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Cholesteatoma of the Epididymis1
CHOLESTEATOMA OF THE EPIDIDYMIS1 PALMER R. KUNDERT, LT. CoMDR. (USNR)
The literature on tumors of the epididymis was first reviewed in 1924 by Hinman and Gibson, who incorporated 3 cases of their own. In 1936 Thompson brought the review to date, including 13 cases discovered at the Mayo Clinic. Of the 61 epididymal tumors reviewed by him, 25, or 41 per cent, were benign and 36, or 59 per cent, malignant. Of the benign tumors 8 were myomas, 8 angiomas, 3 fibromas, 2 adenomas, 2 cystic embryomas, 1 lipoma, and 1 cystic dermoid. Since the publication of Thompson's paper additional case reports and reviews of epididymal tumors have occasionally appeared. A careful search of this literature for reports of cholesteatoma of the epididymis brings to light no previous report of such a tumor. CASE REPORT
H. R. L., ARM 3/c, a 23-year-old white man, was admitted to the U. S. Naval Hospital at Jacksonville, Florida, on December 2, 1942, because of a lump in the right scrotum. This was of 3 years' duration during which the size of the mass had gradually increased slightly, but there was no associated pain, tenderness, or loss of weight. The medical history of the patient revealed that he had had mumps in childhood without involvement of the testicles. He also stated that in childhood he had suffered a stab wound in the right scrotum when he accidentally fell on a stiff, sharp stalk of stubble. There was no history of intrascrotal infection at that or any other time. He denied having had any venereal infection. Examination revealed a normally developed and well-nourished man in no acute distress. Pertinent physical findings were limited to the right scrotum. There was an old linear, well-healed, freely movable scar 2½ cm. long on the midlateral aspect of the right scrotum. In the region of the head of the right epididymis was found a smooth, discrete, firm round mass 1½cm. in diameter which was freely movable but did not transilluminate. The tail of the right epididymis was slightly thickened. Laboratory investigation revealed a normal blood count and urinalysis. Blood Kahn and Friedman tests were negative, and the chest film showed no shadows suggestive of metastases. Although a preliminary diagnosis of benign tumor of the right epididymis was made, surgical intervention was decided upon because tumors in this area are sometimes difficult to differentiate from tumors arising in the rete testis (Gilbert). Accordingly on December 10, 1942, the right epididymis and adjacent portion of the vas deferens were removed, under spinal anesthesia, through a right scrotal incision which included excision of the old scar. The patient made an uneventful recovery and was discharged on January 5, 1943. 1 The opinions or assertions contained herein are the private ones of the writer and are not to be construed as official or reflecting the views of the Navy Department or the Naval service at large.
454
CHOLESTEATOMA QF THE EPIDIDYMIS
45&
A study of the specimen was made by H. Brown, Lt. (MC USN), who re-· ported as follows: The gross specimen consists of an irregular mass of tissue measuring 10 by l cm. and having a large follicular cyst at either end measuring 3 by 2 by 2 and 2.5 by 2 by 1.5 cm. respectively, the larger cyst occurring in the globus major of the epididymis. On section both are thick-walled (5 mm.), containing a soft yellow, semi-solid, putty-like material loosely adherent to a rough granular grey-pink -wall, apparently having nun1erous tiny pink papillary projections on the inner surface. There are in the epididymis, between the two larger cysts, several smaller nodules from 5 to 8 mm. in diameter which have thinner walls and which contain firm yellow granular material. Microscopic examination of the sections shmved multiple cysts imbedded in hyalin fibrous tissue containing a fe1v scattered round cells. The cysts in general were unlined but occasional epithelial remnants remained and occasionally a giant cell was found. Contents included acid or hemic-staining amorphous material with occasional cell remnants and many fatty-acid crystals. Cholesterol crystals seen in direct examination had dissolved in fixing solutions. Diagnosis: Cholesteatoma of right epididymis. Since being discharged from the hospital this patient for various reasons has had 6 examinations, none of -which revealed additional pathological changes associated with the above-described leRion. The most recent checkup waR made on September 4, 1945. DISCUSSION
According to Neer, the term cholesteatoma was introduced into the literature in 1838 "to signify a tumor of epithelial cells impregnated ,vith cholestrin crystals." In 1904 McFarland described cholesteatoma as a "peculiar and rare tumor characterized by the formation of pearly bodies consisting of scale-like epithelial cellR with occasional admixture of cholestrin." According to Karsner, cholesterol is usually present in large amounts but may be absent. Kopetzky considers that cholesteatoma comprise "all globular growths which are surrounded by a thin shell of epidermis and connective tissue and which are composed of accumulated horny desquamated epidermis." Considerable confusion has developed in applying the term cholesteatoma to such lesions, since they appear to vary in origin and have been classified on that basis even though they are pathologically difficult to differentiate. Oertel does not consider them as an independent growth but as "secondary changes which overtake certain epithelial and possibly endothelial tumors and which may also be noted in excessive inflammatory hyperplasias." Most writers hmrnver, are of the opinion that they occur either as primary independent growths or secondary to inflammation, suppuration, or trauma. Consequently, cholesteatomas have been designated as primary, congenital, true, secondary, pseudo, false, inflammatory or traumatic. It is generally agreed that those cholesteatomas appearing in the nervous system are congenital primary ne,v growths. They sometimes show close rela,
456
PALMER R. KUNDERT
tion to teratoid growths, occasionally containing hair and sebaceous glands. Beattie and Dickson believe they arise from displaced epidermal epithelial cells. It is the opinion of Humiston and Piette and of Klemptner and Palmisano that the 2 cases of cholesteatoma of the cecum which they reported independently were also primary congenital tumors. The origin of cholesteatoma appearing elsewhere remains disputed, however, especially those occurring in the middle ear. Hektoen and Reisman state that "there is hardly any affection of the ear concerning ,vhich so diverse explanations have been offered as cholesteatoma." According to Klemptner and Palmisano, otologists are dominated by the opinions of Bezold, Politzer, Tweltsch, and Haberman, who hold that most cholesteatomas are secondary to chronic suppuration and that only in isolated instances do they occur as primary growths. Constans recognizes cholesteatoma of the orbit to be either true or false. His criteria for true cholesteatoma of the orbit are that it possesses no inflammatory wall, is located subperiosteally in the orbit, and contains or is composed of cholesterol crystals. Although such writers as Hektoen and Reisman and McFarland believe that epidermization, hornification, and keratosis of the lining occurring in cholesteatomas of the urinary passages is secondary to chronic inflammation, Ewing claims that hornification in certain cases must be regarded as a tendency inherent in the growth. This contention is supported by Klug. According to Humiston and Piette, cholesteatomas have also been found in the gall bladder, mammary gland, and uterus and are attributed to chronic inflammation with squamous epithelial metaplastic transformations of normal epithelial covering or partial destruction of a squamous epithelial duct. Klemptner and Palmisano, however, state that such squamous cells although similar in shape to epidermic cells, cannot assume their function of desquamating. Cholesteatoma of the testis was described by McFarland in 1904 as occurring through metamorphosis of tumor tissue with attendant formation of cysts with smooth walls, which are lined ·with columnar and cuboidal epithelium and contain either a clear fluid or a semi-caseous mass composed of fatty degenerated epithelial fat drops and cholestrin plates. No specific case reports or other reference to cholesteatoma of the testis were discovered in the literature. Traumatic cholesteatoma, as pointed out by Klemptner and Palmisano, have also been produced experimentally and discovered clinically. Kaufman has produced them by transplantation of the cock's comb and Blumberg has found several in the palm, the result of stab wounds. CONCLUSION
From the foregoing discussion it is evident that the origin of cholesteatoma is much disputed and is probably due to multiple causes. The attempt to explain the origin of the tumor reported above and to correlate its development and classification with those of cholesteatoma in general is based primarily on the history of trauma. It is conceivable, though difficult to prove, that this tumor is a true cholesteatoma explainable on the basis of embryonic inclusion of epidermal cells. Much more plausible is the hypothesis that it developed secondar-
CHOLESTEATOMA OF THE EPIDIDYMIS
457
ily to a stab ·wound which may have introduced or transplanted the required epithelial elements. No evidence was found to support infection or suppuration as etiologic factors.
305 Tenney Bldg., Madison 3, wi·s. REFERENCES BEATTIE, J.M., AND DrcKSON, W. E. C.: A Textbook of Pathology. St. Louis: C. V. Mosby Co., 1926, p. 271. CoNSTANS, G. M.: Cholesteatoma of orbit. Arch. Ophth., 30: 236-246, 1943. EWING, JAMES: Neoplastic Diseases-A Treatise on Tumors. Philadelphia: W. B. Saunders Co., 1940, pp. 828, 1024, 1061. GILBERT, J. B.: Studies in malignant tumors of the testis; differential diagnosis of clinically obscure tumors: 4 cases and review of 122 from the literature. J. Urol., 43: 722733, 1940. HEKTOEN, L., AND RrnsMAN, D.: An American Textbook of Pathology. Philadelphia: W. B. Saunders Co. 1902 pp. 655, 993, 999, 1215, 1216. HINMAN, FRANK, AND GrnsoN, THOMAS, E.: Tumors of the epididymis, spermatic cord, and testicular tunic; a review of the literature and report of three cases. Arch. Surg., 8: 100-137, 1924. Hc:J\IISTON, CHARLES E., AND PIETTE, EUGENE C.: True cholestcatoma of the cecum. J. A. M.A., 84: 874-876, 1925. KARSNER, H. T.: Human Pathology. Philadelphia: J.B. Lippincott Co., 1926, p. 944. KLEJ\JPTNER, DIETRICH, AND PALMISANO, DOMINIC, J.: Cholesteatoma of the cecum. Ill. Med. J., 52: 82-84, 1927. KL,'G, W. J.: Cholesteatoma in urinary passages. Beitr. z. ldin. Chir., 127: 123-131, 1922. KoPETZKY, S. J.: Cholesteatoma. Laryngoscope, 43: 118-131, 1933. McFARLAND, JosEPH: A Textbook of Pathology. Philadelphia: W. B. Saunders Co., 1904, p. 243, 685. NEirn, E. D.: Cholesteatoma. S. Clin. N. A. 9: 769-774, 1929. OERTEL, HoRsT: Outlines of Pathology-In its Historical Philosophical and Scientific Foundations. Renouf Publishing Company, 1927, p. 387. THOMPSON, GERSHOJ\I J.: Tumors of the spermatic cord, epididymis and testicular tunic: Review of literature and report of 41 additional cases. Surg., Gynec. & Obst., 62: 712-728, 1936.
The literature on tumors of the epididymis was first reviewed in 1924 by Hinman and Gibson, who incorporated 3 cases of their own. In 1936 Thompson brought the review to date, including 13 cases discovered at the Mayo Clinic. Of the 61 epididymal tumors reviewed by him, 25, or 41 per cent, were benign and 36, or 59 per cent, malignant. Of the benign tumors 8 were myomas, 8 angiomas, 3 fibromas, 2 adenomas, 2 cystic embryomas, 1 lipoma, and 1 cystic dermoid. Since the publication of Thompson's paper additional case reports and reviews of epididymal tumors have occasionally appeared. A careful search of this literature for reports of cholesteatoma of the epididymis brings to light no previous report of such a tumor. CASE REPORT
H. R. L., ARM 3/c, a 23-year-old white man, was admitted to the U. S. Naval Hospital at Jacksonville, Florida, on December 2, 1942, because of a lump in the right scrotum. This was of 3 years' duration during which the size of the mass had gradually increased slightly, but there was no associated pain, tenderness, or loss of weight. The medical history of the patient revealed that he had had mumps in childhood without involvement of the testicles. He also stated that in childhood he had suffered a stab wound in the right scrotum when he accidentally fell on a stiff, sharp stalk of stubble. There was no history of intrascrotal infection at that or any other time. He denied having had any venereal infection. Examination revealed a normally developed and well-nourished man in no acute distress. Pertinent physical findings were limited to the right scrotum. There was an old linear, well-healed, freely movable scar 2½ cm. long on the midlateral aspect of the right scrotum. In the region of the head of the right epididymis was found a smooth, discrete, firm round mass 1½cm. in diameter which was freely movable but did not transilluminate. The tail of the right epididymis was slightly thickened. Laboratory investigation revealed a normal blood count and urinalysis. Blood Kahn and Friedman tests were negative, and the chest film showed no shadows suggestive of metastases. Although a preliminary diagnosis of benign tumor of the right epididymis was made, surgical intervention was decided upon because tumors in this area are sometimes difficult to differentiate from tumors arising in the rete testis (Gilbert). Accordingly on December 10, 1942, the right epididymis and adjacent portion of the vas deferens were removed, under spinal anesthesia, through a right scrotal incision which included excision of the old scar. The patient made an uneventful recovery and was discharged on January 5, 1943. 1 The opinions or assertions contained herein are the private ones of the writer and are not to be construed as official or reflecting the views of the Navy Department or the Naval service at large.
454
CHOLESTEATOMA QF THE EPIDIDYMIS
45&
A study of the specimen was made by H. Brown, Lt. (MC USN), who re-· ported as follows: The gross specimen consists of an irregular mass of tissue measuring 10 by l cm. and having a large follicular cyst at either end measuring 3 by 2 by 2 and 2.5 by 2 by 1.5 cm. respectively, the larger cyst occurring in the globus major of the epididymis. On section both are thick-walled (5 mm.), containing a soft yellow, semi-solid, putty-like material loosely adherent to a rough granular grey-pink -wall, apparently having nun1erous tiny pink papillary projections on the inner surface. There are in the epididymis, between the two larger cysts, several smaller nodules from 5 to 8 mm. in diameter which have thinner walls and which contain firm yellow granular material. Microscopic examination of the sections shmved multiple cysts imbedded in hyalin fibrous tissue containing a fe1v scattered round cells. The cysts in general were unlined but occasional epithelial remnants remained and occasionally a giant cell was found. Contents included acid or hemic-staining amorphous material with occasional cell remnants and many fatty-acid crystals. Cholesterol crystals seen in direct examination had dissolved in fixing solutions. Diagnosis: Cholesteatoma of right epididymis. Since being discharged from the hospital this patient for various reasons has had 6 examinations, none of -which revealed additional pathological changes associated with the above-described leRion. The most recent checkup waR made on September 4, 1945. DISCUSSION
According to Neer, the term cholesteatoma was introduced into the literature in 1838 "to signify a tumor of epithelial cells impregnated ,vith cholestrin crystals." In 1904 McFarland described cholesteatoma as a "peculiar and rare tumor characterized by the formation of pearly bodies consisting of scale-like epithelial cellR with occasional admixture of cholestrin." According to Karsner, cholesterol is usually present in large amounts but may be absent. Kopetzky considers that cholesteatoma comprise "all globular growths which are surrounded by a thin shell of epidermis and connective tissue and which are composed of accumulated horny desquamated epidermis." Considerable confusion has developed in applying the term cholesteatoma to such lesions, since they appear to vary in origin and have been classified on that basis even though they are pathologically difficult to differentiate. Oertel does not consider them as an independent growth but as "secondary changes which overtake certain epithelial and possibly endothelial tumors and which may also be noted in excessive inflammatory hyperplasias." Most writers hmrnver, are of the opinion that they occur either as primary independent growths or secondary to inflammation, suppuration, or trauma. Consequently, cholesteatomas have been designated as primary, congenital, true, secondary, pseudo, false, inflammatory or traumatic. It is generally agreed that those cholesteatomas appearing in the nervous system are congenital primary ne,v growths. They sometimes show close rela,
456
PALMER R. KUNDERT
tion to teratoid growths, occasionally containing hair and sebaceous glands. Beattie and Dickson believe they arise from displaced epidermal epithelial cells. It is the opinion of Humiston and Piette and of Klemptner and Palmisano that the 2 cases of cholesteatoma of the cecum which they reported independently were also primary congenital tumors. The origin of cholesteatoma appearing elsewhere remains disputed, however, especially those occurring in the middle ear. Hektoen and Reisman state that "there is hardly any affection of the ear concerning ,vhich so diverse explanations have been offered as cholesteatoma." According to Klemptner and Palmisano, otologists are dominated by the opinions of Bezold, Politzer, Tweltsch, and Haberman, who hold that most cholesteatomas are secondary to chronic suppuration and that only in isolated instances do they occur as primary growths. Constans recognizes cholesteatoma of the orbit to be either true or false. His criteria for true cholesteatoma of the orbit are that it possesses no inflammatory wall, is located subperiosteally in the orbit, and contains or is composed of cholesterol crystals. Although such writers as Hektoen and Reisman and McFarland believe that epidermization, hornification, and keratosis of the lining occurring in cholesteatomas of the urinary passages is secondary to chronic inflammation, Ewing claims that hornification in certain cases must be regarded as a tendency inherent in the growth. This contention is supported by Klug. According to Humiston and Piette, cholesteatomas have also been found in the gall bladder, mammary gland, and uterus and are attributed to chronic inflammation with squamous epithelial metaplastic transformations of normal epithelial covering or partial destruction of a squamous epithelial duct. Klemptner and Palmisano, however, state that such squamous cells although similar in shape to epidermic cells, cannot assume their function of desquamating. Cholesteatoma of the testis was described by McFarland in 1904 as occurring through metamorphosis of tumor tissue with attendant formation of cysts with smooth walls, which are lined ·with columnar and cuboidal epithelium and contain either a clear fluid or a semi-caseous mass composed of fatty degenerated epithelial fat drops and cholestrin plates. No specific case reports or other reference to cholesteatoma of the testis were discovered in the literature. Traumatic cholesteatoma, as pointed out by Klemptner and Palmisano, have also been produced experimentally and discovered clinically. Kaufman has produced them by transplantation of the cock's comb and Blumberg has found several in the palm, the result of stab wounds. CONCLUSION
From the foregoing discussion it is evident that the origin of cholesteatoma is much disputed and is probably due to multiple causes. The attempt to explain the origin of the tumor reported above and to correlate its development and classification with those of cholesteatoma in general is based primarily on the history of trauma. It is conceivable, though difficult to prove, that this tumor is a true cholesteatoma explainable on the basis of embryonic inclusion of epidermal cells. Much more plausible is the hypothesis that it developed secondar-
CHOLESTEATOMA OF THE EPIDIDYMIS
457
ily to a stab ·wound which may have introduced or transplanted the required epithelial elements. No evidence was found to support infection or suppuration as etiologic factors.
305 Tenney Bldg., Madison 3, wi·s. REFERENCES BEATTIE, J.M., AND DrcKSON, W. E. C.: A Textbook of Pathology. St. Louis: C. V. Mosby Co., 1926, p. 271. CoNSTANS, G. M.: Cholesteatoma of orbit. Arch. Ophth., 30: 236-246, 1943. EWING, JAMES: Neoplastic Diseases-A Treatise on Tumors. Philadelphia: W. B. Saunders Co., 1940, pp. 828, 1024, 1061. GILBERT, J. B.: Studies in malignant tumors of the testis; differential diagnosis of clinically obscure tumors: 4 cases and review of 122 from the literature. J. Urol., 43: 722733, 1940. HEKTOEN, L., AND RrnsMAN, D.: An American Textbook of Pathology. Philadelphia: W. B. Saunders Co. 1902 pp. 655, 993, 999, 1215, 1216. HINMAN, FRANK, AND GrnsoN, THOMAS, E.: Tumors of the epididymis, spermatic cord, and testicular tunic; a review of the literature and report of three cases. Arch. Surg., 8: 100-137, 1924. Hc:J\IISTON, CHARLES E., AND PIETTE, EUGENE C.: True cholestcatoma of the cecum. J. A. M.A., 84: 874-876, 1925. KARSNER, H. T.: Human Pathology. Philadelphia: J.B. Lippincott Co., 1926, p. 944. KLEJ\JPTNER, DIETRICH, AND PALMISANO, DOMINIC, J.: Cholesteatoma of the cecum. Ill. Med. J., 52: 82-84, 1927. KL,'G, W. J.: Cholesteatoma in urinary passages. Beitr. z. ldin. Chir., 127: 123-131, 1922. KoPETZKY, S. J.: Cholesteatoma. Laryngoscope, 43: 118-131, 1933. McFARLAND, JosEPH: A Textbook of Pathology. Philadelphia: W. B. Saunders Co., 1904, p. 243, 685. NEirn, E. D.: Cholesteatoma. S. Clin. N. A. 9: 769-774, 1929. OERTEL, HoRsT: Outlines of Pathology-In its Historical Philosophical and Scientific Foundations. Renouf Publishing Company, 1927, p. 387. THOMPSON, GERSHOJ\I J.: Tumors of the spermatic cord, epididymis and testicular tunic: Review of literature and report of 41 additional cases. Surg., Gynec. & Obst., 62: 712-728, 1936.