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Primary Malignant Tumor of the Epididymis
PRIMARY MALIGNANT TUMOR OF THE EPIDIDYMIS JOSEPH A. LAZARUS
A careful review of the literature dealing with the subject of primary malignant neoplasms of the epididymis reveals a total of 40 cases exclusive of the case reported herewith. One case, reported by Barringer as teratoma of the epididymis, must be excluded, since the diagnosis is based solely upon clinical features, the tumor mostlikely having originated from the testis and secondarily involved the epididymis. Our deductions in regard to primary malignant tumors of the epididymis are drawn from a series of 40 cases including our own. According to Thompson, 40 per cent of tumors originating in the epididymis are benign and 60 per cent malignant. Of 36 malignant tumors which this observer collected, he found 23 of epithelial origin, 12 sarcomata and 1 teratoma. An independent review of the present series of 40 cases of malignant tumors reveals 20 cases (50 per cent) listed as carcinomata. Of this number, 6 are designated simply as carcinoma, 1 columnar-cell carcinoma, 3 squamous-cell carcinomata, 1 epithelioma, 8 adenocarcinomata and 1 embryonal carcinoma. Sato reported 1 case in which the epididymis presented 2 distinct tumors; one a carcinoma and the other a seminoma. There were 17 specimens (42.5 per cent) reported sarcomata. Of this number, 4 were listed primary sarcomata, 1 cystic chondro-sarcoma, 2 melano-sarcomata, 1 cysto-sarcoma, 3 round-cell sarcomata, 1 chondro-sarcoma, 2 small-celled sarcomata, 1 myosarcoma, 1 spindle-cell sarcoma and 1 fibro-sarcoma. Excluding the case of Barringer, there are 2 cases designated malignant teratomata; 1 by BlandSutton and the other by Coleman, Mackie and Simpson. Although Ferrier and Foord, in their classification, note 3 cases of seminomata; 1 by Hinman and Gibson, 1 by Coleman, Mackie and Simpson, and 1 by Eisenberg, Simons and Wallerstein, the author, referring to the original designations of the tumors as published, finds mention of only 2 instances, namely 1 by Eisenberg, Simons and Wallerstein, and the other by Sato. The pathological diagnosis in the case of Hinman and Gibson was adenocarcinoma, while that of Coleman, Mackie and Simpson was designated malignant teratoma. In order to understand the reason of the various discrepancies in the classification of malignant tumors of the testis and epididymis, one must 751
752
JOSEPH A. LAZARUS
go back to the controversy regarding the origin of such tumors. Chevassu was of the opinion that the round, large cells with clear cytoplasm so often seen in tumors involving the testis and epididymis closely resembled spermatocytes. For this reason he concluded that these cells were derived from epithelial cells of the seminiferous tubules and consequently designated these tumors as seminomata. The term spermatocytoma was also applied to this group of neoplasms by Tanner, Debernardi and by Southam and Linell. Instances of seminomata arising primarily in the epididymis without any trace of the tumor in the testis would seem to indicate that such cells do not necessarily originate in germinal epithelium. Ewing is of the opinion that practically all the usual tumors of the testicle are primarily teratomata in which one type of cells predominates to the exclusion of all other components of the teratoma, thus giving the impression that one is dealing with a tumor composed entirely of one type of cells. To this view most pathologists today subscribe. Yet a review of the literature clearly indicates that in many of the cases the tumors have disclosed a multiplicity of tissue elements arising from all three of the germ layers. In fact, Hinman, Gibson and Kutzmann, after carefully analysing the slides of cases of so-called seminomata, were able to find a variety of cellular elements indicating the teratomatous origin of such tumors, and concluded that the designation "seminoma and spermatocytoma" should not be used. Using this same line of reasoning, the author concludes that several of the reported cases of primary tumors of the epididymis in which the predominating cells morphologically resembled sarcoma cells, and which were called sarcomata, were actually instances of embryonal carcinomata. The youngest case occurred in a boy 15 years of age, and the oldest in a man of 80. There were 7 cases between 21 and 30 years, 7 between 31 and 40, and 10 between 41 and 50. In 5 instances no mention was made of the side involved. In the remaining cases, 16 occurred in the right, and 19 in the left epididymis. In 11 cases no mention was made of the duration of symptoms prior to admission. Of the remaining 29 cases, 23 patients (78 per cent) complained of symptoms over 1 month, and in 7 the duration of symptoms was less than 1 month. In 16 of the 40 cases the presence of pain was not mentioned. Of the remaining 24 cases, 18 complained of pain in the affected testis, while 6 stated that the swelling was entirely painless. The pain was described
PRIMARY MALIGNANT TUMOR O:F EPIDIDYMIS
753
as drawing, dragging, sharp, pricking, or was just mentioned as being severe. While in the majority of the cases the pain was confined to the affected testis, in some it radiated upward along the course of the spermatic cord. The influence of trauma as a causative factor in the etiology of malig~ nant tumors of the epididymis is quite uncertain. A review of the histories available in this series of 40 cases indicates an absence of information concerning trauma in 25 of them. In 10 of the protocols there was a positive history of trauma which antedated the appearance of the tumor, and in 5 there was a definite statement in the history regarding the absence of trauma. The preoperative diagnosis was not mentioned in 18 of the 40 cases. In only S cases was a diagnosis positively made of tumor of the epididymis. In 2 cases the preoperative diagnosis was carcinoma of the epididymis or tuberculosis, in 1 carcinoma of the testicle or epididymis, and in 4 tumor of the testis. A diagnosis of epididymitis was made in 8 instances, and in 4 of these 8 cases, the inflammation was believed to be tuberculous. It is obvious why such a variety of diagnoses have been made from the clinical features presented. In cases presenting a large tumor mass involving one side of the scrotum in which it is impossible to delineate the features of the epididymis from the testis proper, the differential diagnosis between tumor of the testis invading the epididymis, and primary massive tumor of the epididymis compressing the testis cannot be made. Also, in cases where the growth in the epididymis is small, and particularly when accompanied by hydrocele, and possibly by a history of a preceding trauma, one cannot make the differential diagnosis between tumor and chronic epididymitis, or even of tuberculous epididymitis. It is only in cases where the tumor is of moderate size and apparently well circumscribed and where the epididymis can be clearly delineated from the normal testis that a diagnosis of tumor of the epididymis is possible. In the absence of lymph node metastases or of metastatic tumors in the abdomen, one cannot be sure of the true character of the growth. A review of the cases in which post-mortem examinations were obtained reveals the fact that metastases occur along the course '.of the inguinal and abdominal lymphatics and veins, and frequently the metastasizing tumor surrounds organs in the path of its extension, with or without invading them. Such metastases are extremely prone to invade the celiac axis and mediastinum.
754
JOSEPH A. LAZARUS
The treatment of primary carcinoma of the epididymis may be conveniently divided into : (1) radical; (2) simple orchidectomy; (3) irradiation; and (4) a combination of irradiation and surgery. Hinman advocates radical surgical removal of the testicle, cord, retroperitoneal and lumbar lymph nodes. The operative mortality of his first 46 cases of carcinoma of the testis was 11 per cent. In a later report from a series of 79 cases, he estimates his cures as 30 per cent. On the other hand, Cairns reported 74 cases of which number 55 were subjected to simple orchidectomy and 19 to radical operation. There was no operative mortality, and· of the 55 cases subjected to simple orchidectomy, 33 per cent recovered. Of the 19 cases having radical operation, the percentage of recoveries was only 31.2 per cent. In view of the high degree of radio-sensitivity shown by embryonal carcinoma tissue, the modern trend in treatment is a combination of surgery and x-ray or radium treatment. Higgins and Randall and Bothe advise a course of preoperative roentgen therapy, simple orchidectomy followed by a prolonged course of deep x-ray therapy. The technique of divided doses, according to the method of Coutard, has been employed with gratifying results at the General Memorial Hospital in the treatment of malignant tumors of the testis, and according to Adair, Ferguson has obtained freedom from disease in a 3 year period in 52.6 per cent of cases. A study of the types of therapeutic procedures indicated in this series of cases reveals orchidectomy to have been the procedure of choice, since it was carried out in 23 patients. In 6 additional cases orchidectomy was performed followed by deep x-ray treatment. In 2 cases partial epididymectomy was performed, in 3 complete epididymectomy, and in 1 partial epididymectomy followed by x-ray treatment. No mention was made regarding therapy in 5 cases. In this series of 40 cases, no mention was made of end-results in 10. Of the remaining 30 cases, 2 died shortly after operation; 1 presented a recurrence and evidence of metastases within 6 months after operation. Six patients died within 1 year after operation ; 1 died within 2 years and 1 passed the 2 year limit before his decease. In 2 instances it was stated that the patient died, but there was no mention made regarding the length of life following treatment. Seven patients were reported well and free of metastases 6 months after operation ; while 6 additional patients were alive and well up to 1 year following treatment; and 5 were reported well over the 1 year period. In other words, at least 40 per
PRIMARY MALIGNANT TUMOR OF EPIDJDYMIS
755
cent of the patients were reported dead within 2 years, and about 27 per cent were dead within 1 year. Of those cases reported well between a few months and 19 years after operation, there were 18 patients who had undergone simple orchidectomy (77 per In 4 of these 18 cases deep roentgen therapy supplemented the operative procedure, As regards the efiicacy of supplemental deep roentgen treatment in prolonging life, we find by analyzing our figures that 4 patients had such treatment. In one of the 4 no time was given; while in the remaining three, 1 was reported well 6 months; one, 2 years; and the other 6 months after treatment. On the other hand, there were S patients alive over 1 year who had no roentgen treatment. Case 21 was reported well 1 year after simple orchidectomy; case 25, 2½ years after orchidectomy; case 19 years after partial epididymectomy; case 36, 1 year after simple orchidectomy; and case 14, 15 months after epididymectomy. to case 33, reported Thompson, who was alive 19 years after partial epididymectomy, it might be stated that although the diagnosis was that of adenocarcinoma, grade I, the microscopical description of the tumor indicated that it was adenomatous in character, containing glands lined with broad epithelial cells, the tumor bearing a close resemblance to a mixed tumoL There is some doubt in the writer's mind as to the malignant status of this growth. It appears that before this question can be adequately settled, further follow-up work and a greater number of cases are required. If we are to apply the conclusions drawn from a larger series of primary malignant tumors of the testis treated by x-ray and surgery to malignant tumors of the epididymis, we are forced to conclude that irradiation therapy is an extremely important adjuvant to surgery. Case report. ML M. aged 44 years, a clerk, married and the father of 2 children., was first seen by me on July 1936, complaining of a tender swelling in the left testis of 6 months' duration. The family and past history were negative. He had never had a venereal disease, and did not recall ever receiving an injury to the testis. Six months previously he noted a slight swelling in the left testis which gradually increased in size and became tender to the touch. There was no loss of weight nor any other symptoms save a tendency to tire easily on slight exertion. The physical examination, except for the genitalia, was essentially negative. The penis and right testis were normal. Examination of the left testis re· vealed a hard, indurated, slightly tender mass about the size of a crab-apple
756
JOSEPH A. LAZARUS
situated in the body of the epididymis, but apparently not involving the testis itself. The induration extended along the cord as far as the external ring. Inguinal lymph nodes were not involved. Roentgenological examination of the lungs revealed no evidence of metastasis. Hemoglobin: 87 per cent. Preoperative diagnosis: Tumor of epididymis. Operation (July 20, 1936): Under spinal anesthesia the left testis and the spermatic cord as far as the internal inguinal ring were removed. Pathological report (Dr. A. A. Eisenberg, Sydenham Hospital): The specimen measured 10 x 6 cm., and consisted of a testis and of a tumor and epididy-
FIG.
1. Showing position of tumor with reference to epididymis and testicle
mis as one mass (fig. 1). The external surface presented a greyish purple color. The testis itself measured 4 x 2½ x 2½ cm. The tunica communis and albuginea were thickened and edematous; numerous dilated vessels were found in the upper portion of the tunica at the line of junction between the tumor and testis. The testis had a spongy yellow appearance. The tumor, which was well encapsulated, was situated above the testis and measured 5 x 4 x 3 cm., and presented on section a uniform whitish appearance. The rest of the epididymis was firm and on section presented whitish alternating with brownish areas. A portion of spermatic cord attached to the epididymis measured 6 cm. in length. The testicle proper showed no involvement.
FIG.
FIG.
2. Photomicrograph, low power, X 100
3. Photomicrograph, high power, indicating nature of tumor cells and whorl ar-
rangen1en t.
757
"'..,,u"" -
REFERENCE
AGE
SIDE
SIGNS AND SYMPTOMS
--- --1 Lente and Jacobus: Trans. Not Not Not stated N. Y. Path. Soc., 4: 119, stated stated 1881-82
DIAGNOSIS
TREATMENT
PATHOLOGY AND DIAGNOSIS
RESULT
Not stated
Not stated
Growth showed numerous cysts and hyaline cartilage. Testicle normal. Diagnosis: Cystic chrondro-sarcoma of epididymis
Not stated
Not stated
Castration
Not stated
--Not 3 weeks before enlargement of scrotum; Not stated 50 stated pains. Epididymis firm
Hard new growth, large as a marble. Vas infiltrated. Microscopic: small round cells
Castration
Diagnosis: Primary sarcoma of tail of epididymis
to Metastases nerve centers, skin and lungs. Death
--- ---
2 Edwards, F. S.: Trans. Path. Soc. London, 38: 221, 1887 3 Kocher and Langhans: Deut. Chir ., 1, b: 504, 1887
_,.
en 00
50
Left
Painful nodule just above left testicle about size of walnut
4 Kocher and Langhans: Deut. Chir ., 1, b: 504, 1887
Not Not Not stated stated stated
Not stated
Not stated
Melano-sarcoma of globus minor which Not stated invaded testicle
5 Kocher and Langhans: Deut. Chir., 1, b: 504, 1887
Not Not Not stated stated stated
Not stated
Not stated
A thick walled cyst with tubuli and round cells. Testicle uninvolved. Diagnosis: Cysto-sarcoma of epididymis
Not stated
6 Rydygier: Arch. f. Klin. Chir., 42: 769, 1891
42
Right, later left
Swelling the size of a berry in epididymis; pains
Not stated
Castration right. 4 weeks later castration left
Tumor measured 1.5 cm. x 1.0 cm. All right 40 days Diagnosis: Melano-sarcoma left side after operation
7 Bazy: Bull. et mem. Soc. et Chir. de Par., 18: 488, 1892; also 19: 481, 1893
48
Left
Epididymitis 25 years previously. Two months ago, injury. Tumor size of goose egg, firm
Lues?
Castration
Round-cell sarcoma arising from rete testis and invading epididymis but not testicle
Not stated
8 Blumer: Albany Med. Ann., 18: 593, 1897; also 19: 315, 1898
50
Two months before injury to testicle, Not stated followed by painless swelling
Not stated
Castration
Specimen consists of enlarged testis and epididymis. Testis is size of pigeon's egg and is compressed by enlarged epididymis. Only portion of testis which shows disease is in immediate neighborhood of e pididymis. Epididymis normal. Diagnosis: Small celled sarcoma of epididymis
Patient died less than 1 year later
-
---
9 Kolster: Arch. f. Path. Anat., 155: 391, 1899
52
Left
Dyspnoea
Mediastinal tumor; with metastases
None
Sarcoma of epididymis, generalized metastases. Testis normal
Death
10 Russell and Wood: Brit. M. J., 2: 1193, 1899
15
Left
Injuries to testis. Ovoid tumor 4 inches. Later fluctuating area
Tumor of testicle
Orchidectomy
Tumor weighed ¾pound. Testis not involved. Diagnosis: Sarcoma of epididymis
In good health 5
-
months later
Kenntnis der auf traumatischer Grundlage entstandenen Hodensarkome, Muenchen, 1900 ·
11 Grasman: Zur
--12
Wrobel : Beitrage zur Kenntnis der malignen Hodengeschwuelste, Bresslau, 1902
13 Sprandri: Rev . Veneta di Soc. Med., U: 167, 1904
42
Left
Six months before crushing injury to
Tumor of testicle Orchidectomy
testis, swelling which later increased
Sarcoma of epididymis was found with no involvement of testis
No recurrence 1 year later
to size of fist
24
Left
Recent swelling of left testis; pains
Not stated
Orchidectomy
Microscopic : Carcinoma of epididymis Death 12 years later. Cause
not stated
--80
Right
1 year heaviness, and 11 months later pain and great enlargement
Not stated
Castration
Testis not involved . Epididyinis 3 times normal. ~1icroscopic: Diag-
Not stated
nosis; chondro-sarcoma
14 St. Thomas' Hospital Reports: 34: 219, 1905
16
15 Colby : Brit. Med. Jour., 1: 199, 1907
32
Left
Injury testis 4 weeks before ; pain .
Not stated
Orchidectomy
Large swelling in scrotum
---
Left
Left globus major enlarged; later, Not stated globus minor. Testis uninvolved . Swelling grew rapidly
Castration
Testis normal, epididymis firm continuous with mass in abdomen. Microscopic : Round cell sarcoma
Death 3 months
Grossly the tumor was cystic. Micro-
Metastasis in hypochondrium. Died in 11 months
scopic: Columnar cell carcinoma
later
31
Right
Enlargement of testis 8 months before
Not stated
Castration and Cystic tumor between testis and epididymis. Microscopic: Tubules removal 1 node in connective tissue, also hyaline in front of vena cava at 3rd cartilage and muscle. Diagnosis: lumbar Malignant teratoma
17 Rowlands and Nicholson: Lancet, I: 304, 1909
46
Right
Injury to testis four months before. Painful. The mass seemed to involve only epididymis
Not stated
Castration
Microscopic : Squamous cell carcinoma of epididymis, tunica vaginalis; metastases in spermatic cord
Seven weeks later developed ascites and died
18 Sakaguchi : Frankfurt. Ztschr. f. Path., IS: 62 , 1914
18
Left
Swelling of testis 14 weeks before
Not stated
Part of epididy- Microscopic diagnosis: Carcinoma of mis removed. epididymis. Metastases to lungs days and lymph nodes Three later castration
Died 9 months later
19 Miyata : Arch. f. Klin . Chir., 101 : 434, 1918
32
Left
Swelling in testis 3 weeks before . Epididymis was thick, hard and tender
Epididymitis? Tumor?
Castration
20 Thomson-Walker: Proc. Royal Soc. Med., Sect. Urol., 16: 31, 1922-23
32
Right
12 months ago painless lump. Examination revealed nodule size of marble in globus major
The epididymitis or new growth
Epididymectomy Microscopic: Myosarcoma
21 LaPointe and Cain: Bull.
66
Left
Pain in left testis for 2 years. Tumor "G" epididymitis Castration about size of a cherry pit, which was nodular and cystic, situated in globus minor. Patient had "G" 42 years previously
16 Bland-Sutton: Lancet, 2: 1406, 1909
.... ~
etmem. Soc. de Chir. de Par ., 49 : 391, 1923
Testicle uninvolved, encapsulated tumor in epididymis the size of a pear. Microscopic : Sarcoma
Tumor consisted of groups of cells distributed thru a connective tissue matrix which was unaltered save for slight lymphocytic infiltration . Tumor had appearance of basal-cell epithelioma . Diagnosis : EpitheJioma arising from basal cells of epididymis
Not stated
-
Not stated
Well 15 months later Well 1 year later
"'~ ;
I
AGE
Hinman and Gibson: Arch. --78Surg., 8 : 100, 1924
SIDE
Lett
I J
SIGNS AND SYMPTOMS
I
DIAGNOSIS
At first operation Microscopic: Epididymis showed a Pulmonary infibrous tissue matrix containing testis and vas volvement 7 removed. 3 groups of epithelial cells arranged in months after adenocarcinomatous fashion. Testis months later a first operation mass was reuninv olved, but vas was involved moved from lower angle of wound. Followed by x-ray treatment
58
Right
Increase in size of right testis without pain of 1 month. Swelling of right side of scrotum to 3 times size of left. Enlargement was confined to epididymis. Testis felt atrophied
Tumor of epididymis
251 Llambias and BrachettoBrian: La Semana Medica, 38 : 636, 1931
30
Right
Recent swelling in right testis, and globus major
Tbc epididymitis
261 Coleman, Mackie and Simpson: Surg., Gynec. and Obst., 55: 111, 1932
51
A few days before examination, heaviness in right side of scrotum with some enlargement of testis. No history of trauma. Right epi\iidymis hard and nodular and tender to pressure. Testis did not seem to be involved
Tbc of right epididymis
Right
RESULT
I Not stated
241 Scott: Bull . de Spec. med. Chir ., 4: 244, 1930
I
PATHOLOGY AND DIAGNOSIS
Epididymectomy Tumor was primary in body of epi- Alive 8 months didymis, adherent to skin, median later septum and to base of left corpus cavernosum. Microscopically tumor had a fibrous matrix in which numerous acini lined with epithelial cells were imbedded. Basement membrane was lacking in some places. Diagnosis: Adena-carcinoma
I Right ..
J.
TREATMENT
Nodular mass in left side of scrotum, I Not stated accompanied by a drawing sensation 11 months before admission . Epididymis nodular and of a rubbery consistency, with skin adherent to it
22
231 Scholl and Verbrugge: d'Urol., 27: 24, 1929
g
I I
REFERENCE
A severe kick in right scrotum 14 months previously. Three months later testis became tender and swolJen. Patient lost 12 kilograms in weight. Large nodular epididymis with hydrocele
Orchidectomy and resection of structures of the cord
I Spindle-cell sarcoma of epididymis
I Epididymectomy I Abscess with epithelioma. followed by orchidectomy
Apparently well 6 months later
Diagnosis:J Well 2½ Carcinoma (squamous epithelioma later from tubules)
years
Epididymectomy Tumor was situated in body of the 9 months after epididymis and measured 5 x 3 x 2 operation a cm., diffusely infiltrating. Microlarge nodular scopic: Tumor consisted of large mass in right round and spindle shaped cells with upper quadrant. pale, clear cytoplasm and large He died 10 hyperchromatic nuclei. There were months after groups of deeply staining lymphooperation. cytic cells. Diagnosis: Malignant P.M. teratoma composed of highly differentiated embryonal cells. P .M.: Large mass consisting of retroperitoneal lymph nodes containing same type of cells as tumor of epididymis. Right testis contained no tumor . Tumor extended directly into capsule of right kidney, adrenal and ureter, head of pancreas and abdominal aorta
27 Eisenberg, Simons and Wallerstein: Am. J. Cancer, 16: 875, 1932
28 Sato: Gann, 26: 341, 1932
29 Jaisohn and Jordan : J. A. M. A., 100: 1021, 1933
Right
48
---
Painful swelling of 1 year's duration. No trauma. A hard lump was felt in upper pole of epididymis. A.Z. test positive
Carcinoma
Removal of testis Testis normal. Epididymis presented Not stated and part of vas. a fleshy, sarcomatous-like appearX-ray treatance. Microscopic: Cells of neoment plasm are large, some round and some polyhedral. Cytoplasm is clear, with large nuclei. Cells form large masses, with no tendency toward grouping. Stroma is scanty. Testis not involved. Dia¥nosis: Carcinoma (anaplastic sernmoma)
of testis or The of epididymis
36
Left
Painless swelling in left testis for 4 years. In past 3 months swelling had rapidly increased in size
Not stated
Orchidectomy
Two separate tumors of epididymis. Upper was an adeno-carcinoma, and the lower a seminoma
Death 3 months later. No P .M.
34
Left
Pain and swelling of left testis. He had once injured left testis. In lower third of epididymis there was
Epididymitis
Epididymectomy; Squamous-cell carcinoma of epididymis with chronic inflammation orchidectomy
Reported well. 2 months later
a tumor about size of an orange, and
30 Ferrier and Foord: Urol. and Cut. Rev., 38: 646, 1934
73
31 May: J. Urol.,36:391, 1936
49
· ---Right
....,
-
°'
---Left
tender Pain less swelling in scrotum, which was Probably maligsmooth, cystic and transmitted nancy of epilight. 180 cc. of serous fluid aspididymis rated, and later 150 cc. A hard, nodular tumor measuring 1.5 cm. in diameter was felt in globus major, with shoddy nodules in tunica and a large nodule about 2 cm., up spermatic cord. Testis normal
Orchidectomy
Dense tumor in upper pole of epidid- 1 year later pulymis which had perforated tunica monary tn.etas· so that visceral and parietal layers tases occurred, were studded with implants. Tumor and patient was non-encapsulated, firm and died 18 months grayish-white, measuring 3 x 1.5 x 1 after opera· cm. Microscopic: Malignant epition. P.M. thelial neoplasm. Plugs of tumor cells filled many of lymphatics. Diagnosis: Papillary adeno-carcinoma of etdidymis. P.M.: Retroperitonea glands involved with general metastases
6 weeks previously a mass in scrotum, which grew progressively larger. Soreness in left testis. No history of trauma. Soft tumor about size of baseball in region of left testis which transmitted light. After aspirating 5 ounces of amber fluid, a hard, nodular tumor measuring 3 x 2 cm. was felt in glob us major. Also inguinal hernia
Castration plus x-ray treatment
Grayish-white, solid tumor completely 22 months after replacing head of epididymis; rest operation tuof testis normal. :Microscopic: mor felt in left Infiltrating carcinoma of head of abdomeni 2 epididymis. P.M.: tumors in lower Generalized carcinomatosis abdomen. A few months later patient developed a brain lesion with metastases to the skull. Death from generalized car· cinomatosis and sepsis 3 years and 9 months after operation
Tumor of left epididymis, The or malignant; left hydrocele
.
.,
-32~
REFERENCE
Thompson: Surg., Gynec. and Obst ., 62: 712, 1936
AGE
SIDE
--- - -56
Left
DIAGNOSIS
TREATMENT
PATHOLOGY AND DIAGNOSIS
Swelling of the left testis for 6 years, recently grew in size; also small right sided swelling for 15 years. Fluctuating, painless swelling around left testis. Transmitted light. Both testes
Bilateral hydrocele
Bilateral operation for hydrocele, and excision of tumor from the lower pole of the rig~t epidid-
A tumor of bluish-white color, well encapsulated, 7 mm. in diameter, situated in lower pole of right epididymis . Microscopic: Numerous acini lined with well differentiated epithelial cells. Some of cells have large nuclei and larger nucleoli. Diagnosis : Adena-carcinoma, grade I
Patient is well
A firm, well encapsulated tumor, 2.5 cm. in diameter, in globus minor . Microscopic: Tumor was adenomatous, with glands lined with broad epithelial cells . A close resemblance to mixed twnor was noted. Diagnosis : Adeno-carcinoma, grade I
Apparently well after 19 years
Discharged well. No other follow up
ym1s
---·
7 years previously slight enlargement of right testis; during past year, a dull, intermittent pain in testis. Firm swelling, 2.5 cm. in diameter, in globus minor
Tbc epididymitis Partial epididymectomy
30
Left
Nodule in the globus minor 10 r,ears previously. A :firm nodule in ower pole of epididymis, measuring 2 x 2 cm .
Tumor of testis
Partial epididy- Grayish-white, firm, well encapsumectomy, fol ... lated tumor measuring 2 cm. in lowed by X-ray diameter . Microscopic: Same as treatment Case 33. Diagnosis : Adeno-carciuoma, grade I
38
Left
Nodular swelling of left testis 3½ years previously . 1 year before ad.mission, acute pain in testis and left groin. Testis enlarged to 4 times size of normal; tumor which was hard and nodular, seemed to be situated in epididymis
Tumor of epididymis
Orchidectomy
White, solid looking and moderately :firm tumor involved epididymis in its entire extent, and was size of testis. Testis was slightly involved by direct extension. Microscopic : Carcinoma of high-grade of malignancy with little differentiation of cells
Probably dead
53
Right
Swelling of right testis of 2 months' duration. Right testis size of a lemon, and transmitted light. Swelling felt firm
Tumor of testis
Orchidectomy
Y ellowish-white, :firm tumor arising in epididymis and encircling greater part of testis without invading it. Microscopic: Carcinoma of highgrade of malignancy; cells showed no differentiation
Alive and well and free of metastases 1 year after operation
29
Right
Pain in region of right testis. Hard, discrete nodule in epididymis
Chronic epididymitis
Epididymectomy Diagnosis: Adeno -carcinoma, grade I followed by of epididymis. Testis normal right castration
Not stated
54
Right
Injury to right testis 4 years previously. Since then has had a small lump in testis
Not stated
Right castration and deep X-ray treatment
In good heal th 6
27
34 Thompson: Surg., Gynec.
35 Thompson : Surg., Gynec.
and Obst., 62: 712, 1936
36 Thompson : Surg., Gynec.
and Obst., 62 : 712, 1936
37 Thompson: Surg. , Gynec.
and Obst., 62: 712, 1936 38 Thompson: Surg. , Gynec . and Obst., 62: 712 , 1936
still
Right
33 Thompson : Surg., Gynec. and Obst., 62: 712, 1936
and Obst., 62: 712, 1936
Rl
RESULT
SIGNS AND SYMPTOMS
Hard tumor, 1 cm. in diameter, situated in lower pole of epididymis . Microscopic: Adena-carcinoma, grade II of epididymis . Testis normal
months later
39 Williamson and Barry : Urol., 36 : 388, 1936
,o
Lazarus, J. A.: 39: - , 1938
J.
J. Urol.,
26
44
Right
Left
Severe pain in right lower quadrant The epididymitis and right testis of 2 weeks' duration. Examination: Smooth swelling right epididymis
Epididymectomy followed later by orchidectomyandx-ray treatment
Smooth twnor about 2 cm. in diameter involving body of epididymis. Microscopic : Embryonal carcinoma with lymphoid stroma
Apparently well 2 years after
6 months previously soft swelling of Tumor of left left testis which gradually increased epididymis in size. No history of trauma . Hard mass about size of a crab-apple involving left epididymis, induration extending up cord to external ring. Testis apparently not involved. Testis did not transil-
Castration followed by deep x-ray therapy
See case report
Alive but
luminate
e
operation
complaining of pain in left thigh and hip 8 months after operation ; no evidence of metastases
764
JOSEPH A. LAZARUS
Microscopic examination (figs. 2 and 3). A section taken through the tumor showed it to be a very cellular neoplasm composed of large wavy fibrous connective tissue cells scattered throughout the entire section. These whorls were separated from each other by thick trabecula with an occasional area of lymphocytic infiltration. The tumor was quite vascular and in many places there was endothelial proliferation which fills up the lumen of vessels. In several places the tumor had a distinctly fibro-sarcomatous appearance. The testis itself was not involved. Diagnosis: Fibro-sarcoma of epididymis. Following discharge from the hospital the patient was given deep roentgen treatment. On November 25, 1936 he complained of pain in the left hip and thigh which occasionally radiated down the leg. Since his operation he had lost no weight, but developed a slight cough. X-ray of the chest and pelvis were negative. Abdominal examination failed to reveal any abnormal findings suggestive of metastases. He was last seen on March 12, 1937, at which time he complained of slight pain in the left thigh, but presented no other symptoms. Physical examination was essentially negative. X-ray examination of the pelvis was negative. The author gratefully acknowledges his indebtedness to Dr. Paul Reiser for his assistance in the preparation of the bibliography. SUMMARY AND CONCLUSIONS
Primary malignant tumors of the epididymis are extremely rare, there being but 40 cases on record including the one here reported. Although these tumors have been classified under a variety of names, it seems that the majority of them, if subjected to an extremely critical analysis, would fall in the group of malignant teratomata in which one type of cell predominated to the exclusion of all other teratomatous elements. Summarizing the cases according to the designations of the tumors recorded by the authors, we find 20 carcinomata and 17 sarcomata. The youngest case on record is that of a boy 15 years of age, and the oldest of a man aged 80, the greatest incidence occurring in the fifth decade. The left epididymis is slightly more frequently involved than the right. Seventy-eight per cent of patients, in which a history was obtained relative to the duration of symptoms, presented symptoms longer than 1 month prior to seeking medical aid. Although in 16 patients in this series the presence of pain was not mentioned, 75 per cent of the remaining patient s gave a definite history of pain either preceding or accompanying the swelling in the testis. In only 15 patients out of the 40 was information obtained concerning trauma, and in 10 of these 15 cases, a history of trauma definitely antedated the appearance of the tumor. A diagnosis of primary malignant tumor of the epididymis could, in the
PRIMARY MALIGNANT TUMOR OF EPIDIDYMIS
765
majority of the cases, not have been made with certainty prior to operation. In 22 of the 40 cases where a preoperative diagnosis had been made, the diagnosis of tumor of the epididymis was made only 5 times (22 per cent). Simple orchidectomy appears to have been the procedure of choice in the vast majority of the collected cases. Lately, irradiation therapy has been advocated as a valuable adjuvant to surgery. It is impossible as yet to evaluate the advantages of this agent in the end-results, firstly because of the paucity of material, and secondly because so few cases of primary malignant tumors of the epididymis have been subjected to irradiation therapy. Due to the excellent results obtained in the management of malignant tumors of the testis proper with radio-therapeutic agents, it would be reasonable to assume that such treatment might serve as a valuable supplement to surgery. Although radical surgical procedures have been suggested in the treatment of these lesions, analysis of the cases in this series fails to bear out its advantages over the simple operation of orchidectomy. Regarding end-results, this survey indicates a lack of information concerning this feature in 10 cases. Of the remaining 30 cases, 40 per cent were reported dead within 2 years of operation and 27 per cent died within the first year. A case is reported of a primary £.bro-sarcoma of the epididymis treated by orchidectomy and deep roentgen therapy. 1111 Park Ave., New York City. REFERENCES Additional references will be found in the tabulation of •cases from the literature ADAIR: Discussion of paper of Randall and Bothe. Ann. Surg., 105: 390-391, 1937. BARRINGER: Report of a case of teratoma of epididymis. Internat. J. Surg., 33: 116, 1920. CAIRNS: Neoplasms of testicle. Lancet, 1: 845- 850, 1926. CHEvAssu: Tumeurs du testicle. These de doct. Paris, 1906. DEBERNARDI: Beitrage zur Kenntnis der malignen Hodengeschwuelste. Beitr. z. Path. Anat. u. z. Alig. Path., 40: 534---600, 1907. DEVINCENTIIS: Un caso di seminoma a sviluppo endocanalicolare. Pathologica, 25: 885890, 1933. EWING: Neoplastic Diseases, 3rd Ed. Philadelphia and London, W. B. Saunders Co., 1928. HIGGINS: Malignant tumors of testicle. Ann. Surg., 87: 263-291, 1928. HINMAN: Operative treatment of tumors of testicle with a report of 30 cases treated by orchidectomy. J.A.M.A., 63: 2009-2015, 1914. Idem: Radical operation for teratoma testis. Surg., Gynec. and Obst., 37: 429-451 , 1923. HINMAN, GrnsoN, AND KuTZMANN: Malignant tumors of testicle. Ann. Surg., 82: 552-575, 1925. RANDALL AND BOTHE: Value of preoperative irradiation in tumor of testis. Ann. Surg., 105: 3385-390, 1937. SOUTHAM AND LINELL : Pathology of neoplasms of testis. Brit. J. Surg., 11: 223-233, 1923. TANNER: Tumors of testicle; with analysis of 100 original cases. Surg., Gynec. and Obst., 35: 565-572, 1922.
A careful review of the literature dealing with the subject of primary malignant neoplasms of the epididymis reveals a total of 40 cases exclusive of the case reported herewith. One case, reported by Barringer as teratoma of the epididymis, must be excluded, since the diagnosis is based solely upon clinical features, the tumor mostlikely having originated from the testis and secondarily involved the epididymis. Our deductions in regard to primary malignant tumors of the epididymis are drawn from a series of 40 cases including our own. According to Thompson, 40 per cent of tumors originating in the epididymis are benign and 60 per cent malignant. Of 36 malignant tumors which this observer collected, he found 23 of epithelial origin, 12 sarcomata and 1 teratoma. An independent review of the present series of 40 cases of malignant tumors reveals 20 cases (50 per cent) listed as carcinomata. Of this number, 6 are designated simply as carcinoma, 1 columnar-cell carcinoma, 3 squamous-cell carcinomata, 1 epithelioma, 8 adenocarcinomata and 1 embryonal carcinoma. Sato reported 1 case in which the epididymis presented 2 distinct tumors; one a carcinoma and the other a seminoma. There were 17 specimens (42.5 per cent) reported sarcomata. Of this number, 4 were listed primary sarcomata, 1 cystic chondro-sarcoma, 2 melano-sarcomata, 1 cysto-sarcoma, 3 round-cell sarcomata, 1 chondro-sarcoma, 2 small-celled sarcomata, 1 myosarcoma, 1 spindle-cell sarcoma and 1 fibro-sarcoma. Excluding the case of Barringer, there are 2 cases designated malignant teratomata; 1 by BlandSutton and the other by Coleman, Mackie and Simpson. Although Ferrier and Foord, in their classification, note 3 cases of seminomata; 1 by Hinman and Gibson, 1 by Coleman, Mackie and Simpson, and 1 by Eisenberg, Simons and Wallerstein, the author, referring to the original designations of the tumors as published, finds mention of only 2 instances, namely 1 by Eisenberg, Simons and Wallerstein, and the other by Sato. The pathological diagnosis in the case of Hinman and Gibson was adenocarcinoma, while that of Coleman, Mackie and Simpson was designated malignant teratoma. In order to understand the reason of the various discrepancies in the classification of malignant tumors of the testis and epididymis, one must 751
752
JOSEPH A. LAZARUS
go back to the controversy regarding the origin of such tumors. Chevassu was of the opinion that the round, large cells with clear cytoplasm so often seen in tumors involving the testis and epididymis closely resembled spermatocytes. For this reason he concluded that these cells were derived from epithelial cells of the seminiferous tubules and consequently designated these tumors as seminomata. The term spermatocytoma was also applied to this group of neoplasms by Tanner, Debernardi and by Southam and Linell. Instances of seminomata arising primarily in the epididymis without any trace of the tumor in the testis would seem to indicate that such cells do not necessarily originate in germinal epithelium. Ewing is of the opinion that practically all the usual tumors of the testicle are primarily teratomata in which one type of cells predominates to the exclusion of all other components of the teratoma, thus giving the impression that one is dealing with a tumor composed entirely of one type of cells. To this view most pathologists today subscribe. Yet a review of the literature clearly indicates that in many of the cases the tumors have disclosed a multiplicity of tissue elements arising from all three of the germ layers. In fact, Hinman, Gibson and Kutzmann, after carefully analysing the slides of cases of so-called seminomata, were able to find a variety of cellular elements indicating the teratomatous origin of such tumors, and concluded that the designation "seminoma and spermatocytoma" should not be used. Using this same line of reasoning, the author concludes that several of the reported cases of primary tumors of the epididymis in which the predominating cells morphologically resembled sarcoma cells, and which were called sarcomata, were actually instances of embryonal carcinomata. The youngest case occurred in a boy 15 years of age, and the oldest in a man of 80. There were 7 cases between 21 and 30 years, 7 between 31 and 40, and 10 between 41 and 50. In 5 instances no mention was made of the side involved. In the remaining cases, 16 occurred in the right, and 19 in the left epididymis. In 11 cases no mention was made of the duration of symptoms prior to admission. Of the remaining 29 cases, 23 patients (78 per cent) complained of symptoms over 1 month, and in 7 the duration of symptoms was less than 1 month. In 16 of the 40 cases the presence of pain was not mentioned. Of the remaining 24 cases, 18 complained of pain in the affected testis, while 6 stated that the swelling was entirely painless. The pain was described
PRIMARY MALIGNANT TUMOR O:F EPIDIDYMIS
753
as drawing, dragging, sharp, pricking, or was just mentioned as being severe. While in the majority of the cases the pain was confined to the affected testis, in some it radiated upward along the course of the spermatic cord. The influence of trauma as a causative factor in the etiology of malig~ nant tumors of the epididymis is quite uncertain. A review of the histories available in this series of 40 cases indicates an absence of information concerning trauma in 25 of them. In 10 of the protocols there was a positive history of trauma which antedated the appearance of the tumor, and in 5 there was a definite statement in the history regarding the absence of trauma. The preoperative diagnosis was not mentioned in 18 of the 40 cases. In only S cases was a diagnosis positively made of tumor of the epididymis. In 2 cases the preoperative diagnosis was carcinoma of the epididymis or tuberculosis, in 1 carcinoma of the testicle or epididymis, and in 4 tumor of the testis. A diagnosis of epididymitis was made in 8 instances, and in 4 of these 8 cases, the inflammation was believed to be tuberculous. It is obvious why such a variety of diagnoses have been made from the clinical features presented. In cases presenting a large tumor mass involving one side of the scrotum in which it is impossible to delineate the features of the epididymis from the testis proper, the differential diagnosis between tumor of the testis invading the epididymis, and primary massive tumor of the epididymis compressing the testis cannot be made. Also, in cases where the growth in the epididymis is small, and particularly when accompanied by hydrocele, and possibly by a history of a preceding trauma, one cannot make the differential diagnosis between tumor and chronic epididymitis, or even of tuberculous epididymitis. It is only in cases where the tumor is of moderate size and apparently well circumscribed and where the epididymis can be clearly delineated from the normal testis that a diagnosis of tumor of the epididymis is possible. In the absence of lymph node metastases or of metastatic tumors in the abdomen, one cannot be sure of the true character of the growth. A review of the cases in which post-mortem examinations were obtained reveals the fact that metastases occur along the course '.of the inguinal and abdominal lymphatics and veins, and frequently the metastasizing tumor surrounds organs in the path of its extension, with or without invading them. Such metastases are extremely prone to invade the celiac axis and mediastinum.
754
JOSEPH A. LAZARUS
The treatment of primary carcinoma of the epididymis may be conveniently divided into : (1) radical; (2) simple orchidectomy; (3) irradiation; and (4) a combination of irradiation and surgery. Hinman advocates radical surgical removal of the testicle, cord, retroperitoneal and lumbar lymph nodes. The operative mortality of his first 46 cases of carcinoma of the testis was 11 per cent. In a later report from a series of 79 cases, he estimates his cures as 30 per cent. On the other hand, Cairns reported 74 cases of which number 55 were subjected to simple orchidectomy and 19 to radical operation. There was no operative mortality, and· of the 55 cases subjected to simple orchidectomy, 33 per cent recovered. Of the 19 cases having radical operation, the percentage of recoveries was only 31.2 per cent. In view of the high degree of radio-sensitivity shown by embryonal carcinoma tissue, the modern trend in treatment is a combination of surgery and x-ray or radium treatment. Higgins and Randall and Bothe advise a course of preoperative roentgen therapy, simple orchidectomy followed by a prolonged course of deep x-ray therapy. The technique of divided doses, according to the method of Coutard, has been employed with gratifying results at the General Memorial Hospital in the treatment of malignant tumors of the testis, and according to Adair, Ferguson has obtained freedom from disease in a 3 year period in 52.6 per cent of cases. A study of the types of therapeutic procedures indicated in this series of cases reveals orchidectomy to have been the procedure of choice, since it was carried out in 23 patients. In 6 additional cases orchidectomy was performed followed by deep x-ray treatment. In 2 cases partial epididymectomy was performed, in 3 complete epididymectomy, and in 1 partial epididymectomy followed by x-ray treatment. No mention was made regarding therapy in 5 cases. In this series of 40 cases, no mention was made of end-results in 10. Of the remaining 30 cases, 2 died shortly after operation; 1 presented a recurrence and evidence of metastases within 6 months after operation. Six patients died within 1 year after operation ; 1 died within 2 years and 1 passed the 2 year limit before his decease. In 2 instances it was stated that the patient died, but there was no mention made regarding the length of life following treatment. Seven patients were reported well and free of metastases 6 months after operation ; while 6 additional patients were alive and well up to 1 year following treatment; and 5 were reported well over the 1 year period. In other words, at least 40 per
PRIMARY MALIGNANT TUMOR OF EPIDJDYMIS
755
cent of the patients were reported dead within 2 years, and about 27 per cent were dead within 1 year. Of those cases reported well between a few months and 19 years after operation, there were 18 patients who had undergone simple orchidectomy (77 per In 4 of these 18 cases deep roentgen therapy supplemented the operative procedure, As regards the efiicacy of supplemental deep roentgen treatment in prolonging life, we find by analyzing our figures that 4 patients had such treatment. In one of the 4 no time was given; while in the remaining three, 1 was reported well 6 months; one, 2 years; and the other 6 months after treatment. On the other hand, there were S patients alive over 1 year who had no roentgen treatment. Case 21 was reported well 1 year after simple orchidectomy; case 25, 2½ years after orchidectomy; case 19 years after partial epididymectomy; case 36, 1 year after simple orchidectomy; and case 14, 15 months after epididymectomy. to case 33, reported Thompson, who was alive 19 years after partial epididymectomy, it might be stated that although the diagnosis was that of adenocarcinoma, grade I, the microscopical description of the tumor indicated that it was adenomatous in character, containing glands lined with broad epithelial cells, the tumor bearing a close resemblance to a mixed tumoL There is some doubt in the writer's mind as to the malignant status of this growth. It appears that before this question can be adequately settled, further follow-up work and a greater number of cases are required. If we are to apply the conclusions drawn from a larger series of primary malignant tumors of the testis treated by x-ray and surgery to malignant tumors of the epididymis, we are forced to conclude that irradiation therapy is an extremely important adjuvant to surgery. Case report. ML M. aged 44 years, a clerk, married and the father of 2 children., was first seen by me on July 1936, complaining of a tender swelling in the left testis of 6 months' duration. The family and past history were negative. He had never had a venereal disease, and did not recall ever receiving an injury to the testis. Six months previously he noted a slight swelling in the left testis which gradually increased in size and became tender to the touch. There was no loss of weight nor any other symptoms save a tendency to tire easily on slight exertion. The physical examination, except for the genitalia, was essentially negative. The penis and right testis were normal. Examination of the left testis re· vealed a hard, indurated, slightly tender mass about the size of a crab-apple
756
JOSEPH A. LAZARUS
situated in the body of the epididymis, but apparently not involving the testis itself. The induration extended along the cord as far as the external ring. Inguinal lymph nodes were not involved. Roentgenological examination of the lungs revealed no evidence of metastasis. Hemoglobin: 87 per cent. Preoperative diagnosis: Tumor of epididymis. Operation (July 20, 1936): Under spinal anesthesia the left testis and the spermatic cord as far as the internal inguinal ring were removed. Pathological report (Dr. A. A. Eisenberg, Sydenham Hospital): The specimen measured 10 x 6 cm., and consisted of a testis and of a tumor and epididy-
FIG.
1. Showing position of tumor with reference to epididymis and testicle
mis as one mass (fig. 1). The external surface presented a greyish purple color. The testis itself measured 4 x 2½ x 2½ cm. The tunica communis and albuginea were thickened and edematous; numerous dilated vessels were found in the upper portion of the tunica at the line of junction between the tumor and testis. The testis had a spongy yellow appearance. The tumor, which was well encapsulated, was situated above the testis and measured 5 x 4 x 3 cm., and presented on section a uniform whitish appearance. The rest of the epididymis was firm and on section presented whitish alternating with brownish areas. A portion of spermatic cord attached to the epididymis measured 6 cm. in length. The testicle proper showed no involvement.
FIG.
FIG.
2. Photomicrograph, low power, X 100
3. Photomicrograph, high power, indicating nature of tumor cells and whorl ar-
rangen1en t.
757
"'..,,u"" -
REFERENCE
AGE
SIDE
SIGNS AND SYMPTOMS
--- --1 Lente and Jacobus: Trans. Not Not Not stated N. Y. Path. Soc., 4: 119, stated stated 1881-82
DIAGNOSIS
TREATMENT
PATHOLOGY AND DIAGNOSIS
RESULT
Not stated
Not stated
Growth showed numerous cysts and hyaline cartilage. Testicle normal. Diagnosis: Cystic chrondro-sarcoma of epididymis
Not stated
Not stated
Castration
Not stated
--Not 3 weeks before enlargement of scrotum; Not stated 50 stated pains. Epididymis firm
Hard new growth, large as a marble. Vas infiltrated. Microscopic: small round cells
Castration
Diagnosis: Primary sarcoma of tail of epididymis
to Metastases nerve centers, skin and lungs. Death
--- ---
2 Edwards, F. S.: Trans. Path. Soc. London, 38: 221, 1887 3 Kocher and Langhans: Deut. Chir ., 1, b: 504, 1887
_,.
en 00
50
Left
Painful nodule just above left testicle about size of walnut
4 Kocher and Langhans: Deut. Chir ., 1, b: 504, 1887
Not Not Not stated stated stated
Not stated
Not stated
Melano-sarcoma of globus minor which Not stated invaded testicle
5 Kocher and Langhans: Deut. Chir., 1, b: 504, 1887
Not Not Not stated stated stated
Not stated
Not stated
A thick walled cyst with tubuli and round cells. Testicle uninvolved. Diagnosis: Cysto-sarcoma of epididymis
Not stated
6 Rydygier: Arch. f. Klin. Chir., 42: 769, 1891
42
Right, later left
Swelling the size of a berry in epididymis; pains
Not stated
Castration right. 4 weeks later castration left
Tumor measured 1.5 cm. x 1.0 cm. All right 40 days Diagnosis: Melano-sarcoma left side after operation
7 Bazy: Bull. et mem. Soc. et Chir. de Par., 18: 488, 1892; also 19: 481, 1893
48
Left
Epididymitis 25 years previously. Two months ago, injury. Tumor size of goose egg, firm
Lues?
Castration
Round-cell sarcoma arising from rete testis and invading epididymis but not testicle
Not stated
8 Blumer: Albany Med. Ann., 18: 593, 1897; also 19: 315, 1898
50
Two months before injury to testicle, Not stated followed by painless swelling
Not stated
Castration
Specimen consists of enlarged testis and epididymis. Testis is size of pigeon's egg and is compressed by enlarged epididymis. Only portion of testis which shows disease is in immediate neighborhood of e pididymis. Epididymis normal. Diagnosis: Small celled sarcoma of epididymis
Patient died less than 1 year later
-
---
9 Kolster: Arch. f. Path. Anat., 155: 391, 1899
52
Left
Dyspnoea
Mediastinal tumor; with metastases
None
Sarcoma of epididymis, generalized metastases. Testis normal
Death
10 Russell and Wood: Brit. M. J., 2: 1193, 1899
15
Left
Injuries to testis. Ovoid tumor 4 inches. Later fluctuating area
Tumor of testicle
Orchidectomy
Tumor weighed ¾pound. Testis not involved. Diagnosis: Sarcoma of epididymis
In good health 5
-
months later
Kenntnis der auf traumatischer Grundlage entstandenen Hodensarkome, Muenchen, 1900 ·
11 Grasman: Zur
--12
Wrobel : Beitrage zur Kenntnis der malignen Hodengeschwuelste, Bresslau, 1902
13 Sprandri: Rev . Veneta di Soc. Med., U: 167, 1904
42
Left
Six months before crushing injury to
Tumor of testicle Orchidectomy
testis, swelling which later increased
Sarcoma of epididymis was found with no involvement of testis
No recurrence 1 year later
to size of fist
24
Left
Recent swelling of left testis; pains
Not stated
Orchidectomy
Microscopic : Carcinoma of epididymis Death 12 years later. Cause
not stated
--80
Right
1 year heaviness, and 11 months later pain and great enlargement
Not stated
Castration
Testis not involved . Epididyinis 3 times normal. ~1icroscopic: Diag-
Not stated
nosis; chondro-sarcoma
14 St. Thomas' Hospital Reports: 34: 219, 1905
16
15 Colby : Brit. Med. Jour., 1: 199, 1907
32
Left
Injury testis 4 weeks before ; pain .
Not stated
Orchidectomy
Large swelling in scrotum
---
Left
Left globus major enlarged; later, Not stated globus minor. Testis uninvolved . Swelling grew rapidly
Castration
Testis normal, epididymis firm continuous with mass in abdomen. Microscopic : Round cell sarcoma
Death 3 months
Grossly the tumor was cystic. Micro-
Metastasis in hypochondrium. Died in 11 months
scopic: Columnar cell carcinoma
later
31
Right
Enlargement of testis 8 months before
Not stated
Castration and Cystic tumor between testis and epididymis. Microscopic: Tubules removal 1 node in connective tissue, also hyaline in front of vena cava at 3rd cartilage and muscle. Diagnosis: lumbar Malignant teratoma
17 Rowlands and Nicholson: Lancet, I: 304, 1909
46
Right
Injury to testis four months before. Painful. The mass seemed to involve only epididymis
Not stated
Castration
Microscopic : Squamous cell carcinoma of epididymis, tunica vaginalis; metastases in spermatic cord
Seven weeks later developed ascites and died
18 Sakaguchi : Frankfurt. Ztschr. f. Path., IS: 62 , 1914
18
Left
Swelling of testis 14 weeks before
Not stated
Part of epididy- Microscopic diagnosis: Carcinoma of mis removed. epididymis. Metastases to lungs days and lymph nodes Three later castration
Died 9 months later
19 Miyata : Arch. f. Klin . Chir., 101 : 434, 1918
32
Left
Swelling in testis 3 weeks before . Epididymis was thick, hard and tender
Epididymitis? Tumor?
Castration
20 Thomson-Walker: Proc. Royal Soc. Med., Sect. Urol., 16: 31, 1922-23
32
Right
12 months ago painless lump. Examination revealed nodule size of marble in globus major
The epididymitis or new growth
Epididymectomy Microscopic: Myosarcoma
21 LaPointe and Cain: Bull.
66
Left
Pain in left testis for 2 years. Tumor "G" epididymitis Castration about size of a cherry pit, which was nodular and cystic, situated in globus minor. Patient had "G" 42 years previously
16 Bland-Sutton: Lancet, 2: 1406, 1909
.... ~
etmem. Soc. de Chir. de Par ., 49 : 391, 1923
Testicle uninvolved, encapsulated tumor in epididymis the size of a pear. Microscopic : Sarcoma
Tumor consisted of groups of cells distributed thru a connective tissue matrix which was unaltered save for slight lymphocytic infiltration . Tumor had appearance of basal-cell epithelioma . Diagnosis : EpitheJioma arising from basal cells of epididymis
Not stated
-
Not stated
Well 15 months later Well 1 year later
"'~ ;
I
AGE
Hinman and Gibson: Arch. --78Surg., 8 : 100, 1924
SIDE
Lett
I J
SIGNS AND SYMPTOMS
I
DIAGNOSIS
At first operation Microscopic: Epididymis showed a Pulmonary infibrous tissue matrix containing testis and vas volvement 7 removed. 3 groups of epithelial cells arranged in months after adenocarcinomatous fashion. Testis months later a first operation mass was reuninv olved, but vas was involved moved from lower angle of wound. Followed by x-ray treatment
58
Right
Increase in size of right testis without pain of 1 month. Swelling of right side of scrotum to 3 times size of left. Enlargement was confined to epididymis. Testis felt atrophied
Tumor of epididymis
251 Llambias and BrachettoBrian: La Semana Medica, 38 : 636, 1931
30
Right
Recent swelling in right testis, and globus major
Tbc epididymitis
261 Coleman, Mackie and Simpson: Surg., Gynec. and Obst., 55: 111, 1932
51
A few days before examination, heaviness in right side of scrotum with some enlargement of testis. No history of trauma. Right epi\iidymis hard and nodular and tender to pressure. Testis did not seem to be involved
Tbc of right epididymis
Right
RESULT
I Not stated
241 Scott: Bull . de Spec. med. Chir ., 4: 244, 1930
I
PATHOLOGY AND DIAGNOSIS
Epididymectomy Tumor was primary in body of epi- Alive 8 months didymis, adherent to skin, median later septum and to base of left corpus cavernosum. Microscopically tumor had a fibrous matrix in which numerous acini lined with epithelial cells were imbedded. Basement membrane was lacking in some places. Diagnosis: Adena-carcinoma
I Right ..
J.
TREATMENT
Nodular mass in left side of scrotum, I Not stated accompanied by a drawing sensation 11 months before admission . Epididymis nodular and of a rubbery consistency, with skin adherent to it
22
231 Scholl and Verbrugge: d'Urol., 27: 24, 1929
g
I I
REFERENCE
A severe kick in right scrotum 14 months previously. Three months later testis became tender and swolJen. Patient lost 12 kilograms in weight. Large nodular epididymis with hydrocele
Orchidectomy and resection of structures of the cord
I Spindle-cell sarcoma of epididymis
I Epididymectomy I Abscess with epithelioma. followed by orchidectomy
Apparently well 6 months later
Diagnosis:J Well 2½ Carcinoma (squamous epithelioma later from tubules)
years
Epididymectomy Tumor was situated in body of the 9 months after epididymis and measured 5 x 3 x 2 operation a cm., diffusely infiltrating. Microlarge nodular scopic: Tumor consisted of large mass in right round and spindle shaped cells with upper quadrant. pale, clear cytoplasm and large He died 10 hyperchromatic nuclei. There were months after groups of deeply staining lymphooperation. cytic cells. Diagnosis: Malignant P.M. teratoma composed of highly differentiated embryonal cells. P .M.: Large mass consisting of retroperitoneal lymph nodes containing same type of cells as tumor of epididymis. Right testis contained no tumor . Tumor extended directly into capsule of right kidney, adrenal and ureter, head of pancreas and abdominal aorta
27 Eisenberg, Simons and Wallerstein: Am. J. Cancer, 16: 875, 1932
28 Sato: Gann, 26: 341, 1932
29 Jaisohn and Jordan : J. A. M. A., 100: 1021, 1933
Right
48
---
Painful swelling of 1 year's duration. No trauma. A hard lump was felt in upper pole of epididymis. A.Z. test positive
Carcinoma
Removal of testis Testis normal. Epididymis presented Not stated and part of vas. a fleshy, sarcomatous-like appearX-ray treatance. Microscopic: Cells of neoment plasm are large, some round and some polyhedral. Cytoplasm is clear, with large nuclei. Cells form large masses, with no tendency toward grouping. Stroma is scanty. Testis not involved. Dia¥nosis: Carcinoma (anaplastic sernmoma)
of testis or The of epididymis
36
Left
Painless swelling in left testis for 4 years. In past 3 months swelling had rapidly increased in size
Not stated
Orchidectomy
Two separate tumors of epididymis. Upper was an adeno-carcinoma, and the lower a seminoma
Death 3 months later. No P .M.
34
Left
Pain and swelling of left testis. He had once injured left testis. In lower third of epididymis there was
Epididymitis
Epididymectomy; Squamous-cell carcinoma of epididymis with chronic inflammation orchidectomy
Reported well. 2 months later
a tumor about size of an orange, and
30 Ferrier and Foord: Urol. and Cut. Rev., 38: 646, 1934
73
31 May: J. Urol.,36:391, 1936
49
· ---Right
....,
-
°'
---Left
tender Pain less swelling in scrotum, which was Probably maligsmooth, cystic and transmitted nancy of epilight. 180 cc. of serous fluid aspididymis rated, and later 150 cc. A hard, nodular tumor measuring 1.5 cm. in diameter was felt in globus major, with shoddy nodules in tunica and a large nodule about 2 cm., up spermatic cord. Testis normal
Orchidectomy
Dense tumor in upper pole of epidid- 1 year later pulymis which had perforated tunica monary tn.etas· so that visceral and parietal layers tases occurred, were studded with implants. Tumor and patient was non-encapsulated, firm and died 18 months grayish-white, measuring 3 x 1.5 x 1 after opera· cm. Microscopic: Malignant epition. P.M. thelial neoplasm. Plugs of tumor cells filled many of lymphatics. Diagnosis: Papillary adeno-carcinoma of etdidymis. P.M.: Retroperitonea glands involved with general metastases
6 weeks previously a mass in scrotum, which grew progressively larger. Soreness in left testis. No history of trauma. Soft tumor about size of baseball in region of left testis which transmitted light. After aspirating 5 ounces of amber fluid, a hard, nodular tumor measuring 3 x 2 cm. was felt in glob us major. Also inguinal hernia
Castration plus x-ray treatment
Grayish-white, solid tumor completely 22 months after replacing head of epididymis; rest operation tuof testis normal. :Microscopic: mor felt in left Infiltrating carcinoma of head of abdomeni 2 epididymis. P.M.: tumors in lower Generalized carcinomatosis abdomen. A few months later patient developed a brain lesion with metastases to the skull. Death from generalized car· cinomatosis and sepsis 3 years and 9 months after operation
Tumor of left epididymis, The or malignant; left hydrocele
.
.,
-32~
REFERENCE
Thompson: Surg., Gynec. and Obst ., 62: 712, 1936
AGE
SIDE
--- - -56
Left
DIAGNOSIS
TREATMENT
PATHOLOGY AND DIAGNOSIS
Swelling of the left testis for 6 years, recently grew in size; also small right sided swelling for 15 years. Fluctuating, painless swelling around left testis. Transmitted light. Both testes
Bilateral hydrocele
Bilateral operation for hydrocele, and excision of tumor from the lower pole of the rig~t epidid-
A tumor of bluish-white color, well encapsulated, 7 mm. in diameter, situated in lower pole of right epididymis . Microscopic: Numerous acini lined with well differentiated epithelial cells. Some of cells have large nuclei and larger nucleoli. Diagnosis : Adena-carcinoma, grade I
Patient is well
A firm, well encapsulated tumor, 2.5 cm. in diameter, in globus minor . Microscopic: Tumor was adenomatous, with glands lined with broad epithelial cells . A close resemblance to mixed twnor was noted. Diagnosis : Adeno-carcinoma, grade I
Apparently well after 19 years
Discharged well. No other follow up
ym1s
---·
7 years previously slight enlargement of right testis; during past year, a dull, intermittent pain in testis. Firm swelling, 2.5 cm. in diameter, in globus minor
Tbc epididymitis Partial epididymectomy
30
Left
Nodule in the globus minor 10 r,ears previously. A :firm nodule in ower pole of epididymis, measuring 2 x 2 cm .
Tumor of testis
Partial epididy- Grayish-white, firm, well encapsumectomy, fol ... lated tumor measuring 2 cm. in lowed by X-ray diameter . Microscopic: Same as treatment Case 33. Diagnosis : Adeno-carciuoma, grade I
38
Left
Nodular swelling of left testis 3½ years previously . 1 year before ad.mission, acute pain in testis and left groin. Testis enlarged to 4 times size of normal; tumor which was hard and nodular, seemed to be situated in epididymis
Tumor of epididymis
Orchidectomy
White, solid looking and moderately :firm tumor involved epididymis in its entire extent, and was size of testis. Testis was slightly involved by direct extension. Microscopic : Carcinoma of high-grade of malignancy with little differentiation of cells
Probably dead
53
Right
Swelling of right testis of 2 months' duration. Right testis size of a lemon, and transmitted light. Swelling felt firm
Tumor of testis
Orchidectomy
Y ellowish-white, :firm tumor arising in epididymis and encircling greater part of testis without invading it. Microscopic: Carcinoma of highgrade of malignancy; cells showed no differentiation
Alive and well and free of metastases 1 year after operation
29
Right
Pain in region of right testis. Hard, discrete nodule in epididymis
Chronic epididymitis
Epididymectomy Diagnosis: Adeno -carcinoma, grade I followed by of epididymis. Testis normal right castration
Not stated
54
Right
Injury to right testis 4 years previously. Since then has had a small lump in testis
Not stated
Right castration and deep X-ray treatment
In good heal th 6
27
34 Thompson: Surg., Gynec.
35 Thompson : Surg., Gynec.
and Obst., 62: 712, 1936
36 Thompson : Surg., Gynec.
and Obst., 62 : 712, 1936
37 Thompson: Surg. , Gynec.
and Obst., 62: 712, 1936 38 Thompson: Surg. , Gynec . and Obst., 62: 712 , 1936
still
Right
33 Thompson : Surg., Gynec. and Obst., 62: 712, 1936
and Obst., 62: 712, 1936
Rl
RESULT
SIGNS AND SYMPTOMS
Hard tumor, 1 cm. in diameter, situated in lower pole of epididymis . Microscopic: Adena-carcinoma, grade II of epididymis . Testis normal
months later
39 Williamson and Barry : Urol., 36 : 388, 1936
,o
Lazarus, J. A.: 39: - , 1938
J.
J. Urol.,
26
44
Right
Left
Severe pain in right lower quadrant The epididymitis and right testis of 2 weeks' duration. Examination: Smooth swelling right epididymis
Epididymectomy followed later by orchidectomyandx-ray treatment
Smooth twnor about 2 cm. in diameter involving body of epididymis. Microscopic : Embryonal carcinoma with lymphoid stroma
Apparently well 2 years after
6 months previously soft swelling of Tumor of left left testis which gradually increased epididymis in size. No history of trauma . Hard mass about size of a crab-apple involving left epididymis, induration extending up cord to external ring. Testis apparently not involved. Testis did not transil-
Castration followed by deep x-ray therapy
See case report
Alive but
luminate
e
operation
complaining of pain in left thigh and hip 8 months after operation ; no evidence of metastases
764
JOSEPH A. LAZARUS
Microscopic examination (figs. 2 and 3). A section taken through the tumor showed it to be a very cellular neoplasm composed of large wavy fibrous connective tissue cells scattered throughout the entire section. These whorls were separated from each other by thick trabecula with an occasional area of lymphocytic infiltration. The tumor was quite vascular and in many places there was endothelial proliferation which fills up the lumen of vessels. In several places the tumor had a distinctly fibro-sarcomatous appearance. The testis itself was not involved. Diagnosis: Fibro-sarcoma of epididymis. Following discharge from the hospital the patient was given deep roentgen treatment. On November 25, 1936 he complained of pain in the left hip and thigh which occasionally radiated down the leg. Since his operation he had lost no weight, but developed a slight cough. X-ray of the chest and pelvis were negative. Abdominal examination failed to reveal any abnormal findings suggestive of metastases. He was last seen on March 12, 1937, at which time he complained of slight pain in the left thigh, but presented no other symptoms. Physical examination was essentially negative. X-ray examination of the pelvis was negative. The author gratefully acknowledges his indebtedness to Dr. Paul Reiser for his assistance in the preparation of the bibliography. SUMMARY AND CONCLUSIONS
Primary malignant tumors of the epididymis are extremely rare, there being but 40 cases on record including the one here reported. Although these tumors have been classified under a variety of names, it seems that the majority of them, if subjected to an extremely critical analysis, would fall in the group of malignant teratomata in which one type of cell predominated to the exclusion of all other teratomatous elements. Summarizing the cases according to the designations of the tumors recorded by the authors, we find 20 carcinomata and 17 sarcomata. The youngest case on record is that of a boy 15 years of age, and the oldest of a man aged 80, the greatest incidence occurring in the fifth decade. The left epididymis is slightly more frequently involved than the right. Seventy-eight per cent of patients, in which a history was obtained relative to the duration of symptoms, presented symptoms longer than 1 month prior to seeking medical aid. Although in 16 patients in this series the presence of pain was not mentioned, 75 per cent of the remaining patient s gave a definite history of pain either preceding or accompanying the swelling in the testis. In only 15 patients out of the 40 was information obtained concerning trauma, and in 10 of these 15 cases, a history of trauma definitely antedated the appearance of the tumor. A diagnosis of primary malignant tumor of the epididymis could, in the
PRIMARY MALIGNANT TUMOR OF EPIDIDYMIS
765
majority of the cases, not have been made with certainty prior to operation. In 22 of the 40 cases where a preoperative diagnosis had been made, the diagnosis of tumor of the epididymis was made only 5 times (22 per cent). Simple orchidectomy appears to have been the procedure of choice in the vast majority of the collected cases. Lately, irradiation therapy has been advocated as a valuable adjuvant to surgery. It is impossible as yet to evaluate the advantages of this agent in the end-results, firstly because of the paucity of material, and secondly because so few cases of primary malignant tumors of the epididymis have been subjected to irradiation therapy. Due to the excellent results obtained in the management of malignant tumors of the testis proper with radio-therapeutic agents, it would be reasonable to assume that such treatment might serve as a valuable supplement to surgery. Although radical surgical procedures have been suggested in the treatment of these lesions, analysis of the cases in this series fails to bear out its advantages over the simple operation of orchidectomy. Regarding end-results, this survey indicates a lack of information concerning this feature in 10 cases. Of the remaining 30 cases, 40 per cent were reported dead within 2 years of operation and 27 per cent died within the first year. A case is reported of a primary £.bro-sarcoma of the epididymis treated by orchidectomy and deep roentgen therapy. 1111 Park Ave., New York City. REFERENCES Additional references will be found in the tabulation of •cases from the literature ADAIR: Discussion of paper of Randall and Bothe. Ann. Surg., 105: 390-391, 1937. BARRINGER: Report of a case of teratoma of epididymis. Internat. J. Surg., 33: 116, 1920. CAIRNS: Neoplasms of testicle. Lancet, 1: 845- 850, 1926. CHEvAssu: Tumeurs du testicle. These de doct. Paris, 1906. DEBERNARDI: Beitrage zur Kenntnis der malignen Hodengeschwuelste. Beitr. z. Path. Anat. u. z. Alig. Path., 40: 534---600, 1907. DEVINCENTIIS: Un caso di seminoma a sviluppo endocanalicolare. Pathologica, 25: 885890, 1933. EWING: Neoplastic Diseases, 3rd Ed. Philadelphia and London, W. B. Saunders Co., 1928. HIGGINS: Malignant tumors of testicle. Ann. Surg., 87: 263-291, 1928. HINMAN: Operative treatment of tumors of testicle with a report of 30 cases treated by orchidectomy. J.A.M.A., 63: 2009-2015, 1914. Idem: Radical operation for teratoma testis. Surg., Gynec. and Obst., 37: 429-451 , 1923. HINMAN, GrnsoN, AND KuTZMANN: Malignant tumors of testicle. Ann. Surg., 82: 552-575, 1925. RANDALL AND BOTHE: Value of preoperative irradiation in tumor of testis. Ann. Surg., 105: 3385-390, 1937. SOUTHAM AND LINELL : Pathology of neoplasms of testis. Brit. J. Surg., 11: 223-233, 1923. TANNER: Tumors of testicle; with analysis of 100 original cases. Surg., Gynec. and Obst., 35: 565-572, 1922.