A Rare Tumor of the Spermatic Cord: Rhabdomyosarcoma

THE JO"GRNAL OF UROLOGY

Vol. 84, No. 2, August 1960 Pn·nted in U.S.A.

A RARE TUMOR OF THE SPER::VIATIC CORD: RHABDOMYOSARCOMA WILLIAM W. HOFYMAN

AND

SYD~EY S. BAIRD

From the Department of Urology, Dallas Medical and Surgical Clinic, Dallas 4, Texas

The purpose of this paper is to record the nineteenth reported case of rhabclomyosarcoma of the spermatic cord, and to demonstrate the efficacy, though transitory, of a new therapeutic approach in the treatment of its metastasis. Tumors of the testicular appendages, namely, of the epididymis, testicular tunics, and spermatic cord, are infrequent in occurrence (Thompson). Seventy-five to ninety per cent of the tumors involving these extratesticular structures are found in the spermatic cord (Dixon and Moore). To elate, approximately 300 tumors of all kinds involving the spermatic cord have been reported (Cannon and associates). According to Dixon and Moore of the Armed Forces Institute of Pathology, ten to fifteen per cent of these extratesticular tumors arc malignant; the remainder are benign lesions. The most common benign tumors, in order of frequency, are lipoma, the mixed or so-called aclenomatoid tumor, fibroma, and leiomyoma. The malignant tumors, in numerical incidence, are fibrosarcoma, liposarcoma, rhabclomyosarcoma, and leiomyosarcoma. The first recorded rhabclomyosarcoma of the spermatic cord was reported by Rokitansky in 1849. Hirsch reviewed the literature to 1934 and added one case of his own, bringing the total to that year to 15. In 1944, Shivers reported a rhabdomyosarcoma of the spermatic cord in a 16-year-old hoy. Hanson reported a similar case in a 17-year-old youth in 1952, and Gray and Biorn reported the most recent one in the English literature in a 4-year-old child in 1955. Two rhahdomyosarcomas reported by Cannon, and by Goldstein and Cassilli, were considered by the authors to originate in the crcmaster muscle bundles of the scrotal sac itself, and are not counted in this series. Our case is, therefore, the nineteenth rhabclomyosarcoma of the spermatic cord. A review of the reported cases reveals that there is a predilection to occur in youth. The youngest reported case was a boy of 21 months. Five other cases ranged from 3H to 5 years of age. Eight patients were 13 to 18 years of age, Accepted for publication February 19, 1960.

one was 25, one 49, and one 58. The ages of two cases arc not given. The duration of the chief complaint, which characteristically is a painless, palpable mass in the scrotum, has varied from 6 clays to 10 months. It is important to note, however, that this relatively short duration of a tumor mass in spermatic cord rhahdomyosarcoma gives one an erroneous impression that the lesion is a rapidly-growing one. In reviews of the lesion as a general pathologic entity, it is more common to find cases in which a mass was present for up to 50 years before rapid enlargement caused the patient to seek medical attention (Harper and Feder). There are several documented reports in which the mass was present for anywhere from 13 to 16 years (Herzog). Stout, in such a review, reports an average 30-month duration of tumor prior to surgical intervention. It is now the consensus that cross striations, formerly much sought for, are no longer the sine qua non for making a diagnosis of rhahdomyosarcoma (Enterline and Horn, Lewis, ,J. P. Moore, Prince). Similarly, since recent work by Horn and Enterline in establishing the four general classifications of rhahdomyosarcomas, the diagnostic value of cell palisading in tissue sections is also relegated to a secondary importance. The following microscopic characteristics will suffice to confirm the diagnosis of rhahdomyosarcoma (Prince): 1) sufficient picomorphism of the cellular constituents; 2) large numbers of bizarre uni- or multinucleated giant cells with finely granular acidophilic cytoplasm; and 3) the presence of cross striations or longitudinal myofibrillae. There is no unanimity of opinion regarding the tissue of origin of rhahdomyosarcomas in the spermatic cord. The following seven structures are all mentioned by one or more of the authors reporting previous cases and reviewing the literature: 1) cremaster muscle bundles of the funiculus, 2) the gubernaculum of Hunter, 3) embryonic rests of totipotent cells in the funicular structures, 4) misplaced fragments of the embryonic dorsal myotomcs, 5) wolffian

376

R.HABDOMYOS.UWOJVL\ OF SPF~HMNI'IC CORD

duct rests, 6) metaplasia from the muscular coats of the n1s clefcrens and/or epiclidymis and 7) unilateral differentiation in teratomas. There is no generally acknowledged etiologic or inciting agent mentioned in either the clinical or experimental material available. The results of treatment are very discouraging. Stout in his comprd1ensive rrvie,v of the entirr pathologic f'.ntit)- states that therr are only a ,-Ny few recorded cures following radical excision of the primary lesion, and these hiwe been reported in lesiorrn OC'cnrring in the extremities. However, even these casf'.S must be considC'red in the light of known C'ascs of survival witb rlrnbdomyosarcoma for ten to fifteen and even fifty yearn. Thrre an, no reported .5 year cures of rhabdomyosarcoma. involving the spermatic ("ord. ,\ majority of the reportf'd rnsC's have all died of widespread metastases within twelve months' time, most of these within t>ight months of initial treatment. The spread of tlw tumor is as frequent by the blooclstn,am as by lymphatirs, and, therefore, radical dissection of the rctroprritoneal lymph glands in rhabdomyosarcomas of the spermati<' cord is not indicated (Chinn and associates, Enterline and Horn, Gray and Biorn, Riddell and Kudish, Stout). [t is known that rhabdomyosarcomas are resistant to x-ra)(Beard r1ncl Hcwit, Cannon and associates, Gray and Biorn, J. P. Moore). Horn ancl Enterline trrated a rhabdomyosarcoma of the testidc with orchiectomy, deep x-ray, and nitrogen mustard. The lesion rapidly progressed to death ,vith widespread metastases found at autopsy. and Bioru treated a rhabdornyosarciorna by high excision, x-ray, and aminopterin, until a severe folic- acid blocking reaction occurred. The patient improved for 4 months, and then rapidly grew ,vorse. A second course of aminopterin produced a shorter re1mss10n, hut the patient ultimately died of ,1-iclespread metastases 20 months after treatnwnt was begun. These 2 cases represent the only chemically treated rhabclomyosarcomas of the spermatic cord notf'd in the recent literature. The authors report the third case treated with anti-tumor drugs. CASE REPORT

c\. l 4-year old hoy was seen in thf' office on D('cember 2, 1958, with the complaint of an Pnlarging left testicular mass first noted 2 weeks previously. The enlargement was painless and

firm. The patient could n'mcmlwr no history of antecedent trauma. Two montlrn earliC'r, the patient had taken a school physical and at that time no scrotal enlargement noted. No significant past or recent medic:1 I history was elicited. An appendectomy had been performed in 19.57. Inventory of system,; was negative. Physical examination rc'vcaled :i weJl developed though obese 14-year-old weighing 150 pounds. Blood pressure 1rns 1:30/70; pulse 7G. The head and bony emincm·<'f' wen' some1vhat more than usually prominc'nt, tlw impression of acromegalic development. His hands and feet wen' rather large and t<'ndcd tn corroborate this clinical impression. Examination of the head, ears, nosP, and throat was The chest was ckar to perc1rnsion and auscti!tn tion. Examination of the heart was There was a healed l\IcBurnr'.:--'s irn,ision in tlw right lower quadrant. No other almonnalitie,; were noted about the abdomen, and no ,,isccrn could be palpated. Examination of tlw extl'nrnl genitalia revealed a normal right tcstielc. TliP left scrotal contents werf' enlarged to 2 inches, werC' firm in consistency, and did nol tram,illuminatc. Rectal examination ,rns nq,a tive, the prostate 1n1s smooth am! small. Ex-amination of the inguinal rC'gions rc,vf'a led palpable lymph node in the left inguinal cn:ase. measuring approximately 1 1 ~ cm. in its gn'atest dimension. Immediate admission to the' was recommended. On hospital admission, the patient',-, n·d blood count and llC'moglobin were normal. The 11hitc blood connt was 8,700 with 20 eosinophils, 2 band cells, 43 segmented polymorphonucleur leukocytes, 33 lymphocytes, and 2 The urinalysis was negative. The VDRL 11m, negativP. An x-ray of tlw cihcst rt'\'ealed ti, densities, one in each hilar region. ThPse \H'n' interpreted by the radiology department rqJresenting a combination of vascular slmdow~ and an area of old inflammatory scarring, rcspPctivcly. lkcause of the possibility of metastasc-',,, a stratograph of the chest was clone to the shadow:,; noted above. It was nltimakl.1 decirfocl that the stratograph rnvealed a chest. An intra venous pyelogram was negative. On December 6, under general a high orchiectomy was p<'rformed, and tlH· palpable lymph node in the left inguinal. region

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WILLIAM W. HOFFMAN AND SYD_:,,;Ey S. BAIRD

was removed. Postoperative course was uneventful. The pathology rnport by Dr. Thomas R. Cox of Baylor Hospital was: "Sections of the tumor

Frn. 1. Gross specimen. Sections revealed no epididymal involvement. Bulk of tumor completely effaced cord structures.

from the spermatic cord show bands and sheets of tumor in a fibrous stroma. The tumor cells vary from small, poorly-differentiated cells with scanty spindle cytoplasm with hyperchromatic nuclei and a somewhat myxomatous stroma, to areas in which there arc definite strap cells with striations and large basket type cells of a rhabdomyosarcoma. The testicle is not remarkable except for minimal spermatogenesis. In many areas, it is almost infantile in character. The lymph node submitted with the specimen shows no involvement by tumor. ::"Jo actual involvement and invasion of the testis are noted. Diagnosis: Rhabdomyosarcoma of spermatic cord" (figs. 1 and 2). Having established the diagnosis of rhabdomyosarcoma, consultation with the radiotherapy department was requested. Despite the fact that the lesion is not ordinarily radiosensitive, it was recommended that the inguinal and iliac nodes and the abdominal pcriaortic nodes be treated by cobalt irradiation. Over a period of 3 weeks each of these node-bearing areas was, therefore, delivered approximately 3,000 r. Two weeks after the cobalt therapy was completed, the patient was re-admitted to the

Fm. 2. Photomicrograph (120X) of tumor shown in figure 1. Tumor fulfills diagnostic criteria enumerated in text.

RHABDOi\JYOSAHCO!VL\ OF SPEB.MATIC CORD

shows therape11tic regimen and hematologic rehponse hospital lwc:mrne of cmset of severe cough and pain in the right l'hest. A chest x-m)· revealed massive mc:tasta.sis of the so-called wow-flake variet)· in both lungs. \\'c; then obtained consultation ,vith Dr . .J. :u. Hill and Dr. E. Loeb of the Wadley Haematology fnstitute. AJ! of the subsequent medical care \\'as tmclertaken for us by Drs. Hill and Loeb on their service at Ba)·lor Hospital. Becairnc reports indicated quantitative remissions of pulmonary metastases proclucccl by rhabdomyosarc:01na following the use of actinomycin D (G. C :\Ioore ancl the patient was giYen 3,500 micrograms of this agent in a period of 2 weeks (fig. ;3). The drug produeed no visible salutary effect upon the patient's condition in the expcictecl time of 7~14 clays. He became progn,s~in·ly more dyspneic and a chest x-ray rc,·ealed rapid proliferation of the metastatic In vie,Y of this tumor in his chest (fig. 4, apparent failure of actinomycin D, it \Yas dec:idecl to give the patient massive closrs of A.nticipating the severe marrow """'''' produced by this drug, a total of 80 cc of tlw patient's bone marrow was 1Yithclra,n1 and frozrn m Tocantin's buffer solution with 30 per c,ent of glycerol according to the technique of Polge-Smith-Parkes. In the period of 2 \Yecks fo1lowing this marrow aspiration, he received a total of 270 mg. amethoptcrin. He responclecl rather violently to the drug; nausea and vomiting dcvclopecl, and his tern00 '

perature rose to 102 for a period clays. His white blood count dropped from to 5,400 and his platrll'ts from 312,040 to HiR hc,moglobin foll from 1 i:i.6 gm to 12.G ;.ttn. Chloromycetin and meticortclone were rn,(cd a,, adjuvant therapy during this acntc plrn:w of marrow depression. Figure ;:3 the details of treatment and hematologic response. At the end of a week, the patic:nt became afobrifo, his dyspnca clisappcarecl, and he felt well to be clisrhargecl. He was sec,n as an at the Wadley Institute 10 after dischargcid from the hospital. At this platelet count was 3,500, liemoglohin 10.:3 gn1.. red blood count 3,i500,000, and white blood cuun,. 708 with 36 eosinophils, 2 band c0lls, 42 mentcd cells, 14 lymphocytes, ancl (-\ mono(>yteo The bone marrow which Jiad been aspirated and stored ,.-as now returned to h·un intravenous infusion without incident. The p:i · tient continued to do well, returned :rn :11, outpatient to the Rescard1 Institute onl 1rnek after his autologous marrow infusion. \[, that time his platelet count was hemoglobin had risen to 12.5 gm., the n-:d blood count was 4,400,000, and white blood count 4,600. ), differential count of his white hloocl cells revealed 8 cosinophils, 2:1 band cdls, 11 segmented and 2 immature lymphocytes. however, was the appearance

380

WILLIAM W. HOFFMAN AND SYDNEY S. BAIRD

FIG. 4. A, chest x-ray prior to institution of AME therapy. B, note clearing of metastases in chest 5 weeks after first does of AME had been administered. Comparable clinical improvement was noted.

done on this day (fig. 4, B) 5 weeks after AME therapy was instituted. Subsequent blood counts on the patient were normal; physically, he felt perfectly well, had gained 8 pounds, and had an excellent appetite. He had no respiratory difficulty and had returned to his ordinary activities as a schoolboy. This remission lasted for a total of 6 weeks. At the end of that time, the patient again became dyspneic and chest x-ray revealed a re-appearance of metastases in his lungs. He was given a second course of 100 mg. amethopterin, this time without a febrile reaction, and without anorexia, nausea, or vomiting. Done marrow depression following this last dose of amethopterin was minimal, the platelet count dropped only to 486,200, the hemoglobin to 11.4 gm., and the white blood count to 4,800. A second symptomatic remission lasted for a period of 4 weeks, but this one without any dramatic clearing of lung metastasis on x-ray. Then the patient once more began to lose weight and to complain of dyspnea on even minimal exertion. He was given 10 mg. nitrogen mustard without any obvious improvement in his degeneratng physical condition or any changes in x-ray studies of the chest. A month after this initial dose of nitrogen mustard, he was given 100 mg. more. Again, there was no effect. He became progressively more dyspneic, had severe melena, and went into pulmonary edema. Final therapy consisted of numerous supportive measures,

including blood transfusions, metacortelone, and broad spectrum antibiotics. He died 9 months after the orchiectomy. Final anatomic diagnosis determined at autopsy by Dr. George J. Race, of the pathology department at Baylor University Medical Center, was: "1) Rhabdomyosarcoma, embryonal type, of left spermatic cord; 2) extensive metastases to lungs, pleura, epicardium, heart, liver, spleen, pancreas, gastrointestinal tract, omentum, lymph nodes, ureters, bladder, prostate, and bone marrow; 3) purpura (thrombocytopenic): subdural confluent ecchymosis, mild subdural hemorrhages, mild petechial hemorrhages of endocardium, pleura, peritoneum, and intestinal mucosa; 4) pulmonary edema and hemorrhages; 5) hyperdistention of intestine due to partial constriction due to fibrous adhesions; 6) ascites; 7) hemosiderosis of spleen." SUMMARY

This case represents the nineteenth rhabdomyosarcoma of the spermatic cord reported in the literature. The case is singular in that the patient showed a remarkable radiographic and clinical response to massive therapy with intravenous amethopterin. This remission lasted approximately six weeks. A second course of treatment produced a shorter clinical remission. The rapid hematologic response to autologous bone marrow transplant corroborates the reports

RHABDOlVIYOSARf;OMA OF SPERMATIC CORD

of otlwrs regarding the feasibility of autologous marrow replanting. In the ha.nds of qua.lifiecl personnel, this promises to be a valuable ac\dition to the therapy of malignant disease by removing the limitation to therapeutic agents such as x-ray, nitrngen-mustard, radioactive material, and antimetabolites imposed by the danger of marrow apla.sia. The authors wish to a.C'knowledge the courtesy extended to them Drs. J. :\J. Hill and Eilen Loeb in permitting the use of their clinical data in preparing this report. REFERE?\CES

D. E. A'\'D HE\YIT, L. W.: Rhabdomyosarcoma of testicle: A case report ..J. Urol., 53: :l44, 1945. E. 1VI., Arn.'HEIDE, J. P. AND ALLEN, : 1VIalignau l tumors of the spermatic cord and testicular tunics. South. Med. J.,

BE.,RD,

49: 17, Hl56. CI-llNN,

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CAHILL,

E. E.

A;\"D BISCHOFF,

A. J.:

R.hahdomyosarcoma of the testicle: Case report.. Trans. Weslern 8ec. AUA, 23: 97, 1956. D1x.0N, F. J.

AND MooRI-J, R. A.: Atlas of Tumor Pathology: Tumorn of The Male Sex Organs, Section 8, Fasc. 31, 32. Washington, D. C.: Armed Forces Institute of Pathology, 1952. EN'l'ERLINE, H. T . .'\ND HoRN, R. C., JR.: Alveolar rhabdornyosarcomn.. Arn. J. Clin. Path., 29: 356, J 958. Co1,ns'n;rn, H. H. AND CASILLI, A. R.: Rhabdomvosarcoma of the cremasteric muscle and cc;ncomitant polyorchidism. J. LTrol., 41: 583, 1939. GruY, C. P. AND BIORN, C. 1. Rhabdomyo-

sarcoma of the spennatic cord. J. 402, 1955. HANSI-JN, .J. L., ENOS, W. F., PARK, 0 PosTB1,, A. H.: Rhabdornvosarcoma Oi' spermatic cord. U. S. Armed 1'\,rce.~ Med. 3: 621, 19.52. HARPER, H. Y. AND FEDER, H. M.: Rhnbrlomyo sarcoma. Surgery, 6: 7G, l\l3D HBRTZOG, A. J.: Rhabdomyosarcom,1 of testis: Report of two cases. Arn. J. Ca.rn·er. 28: 131, 1936. HIRSCH, E. F.: H.habdomyosarconm of the ma tic cord. Am. J. Cancer, 20: 3D8, Hl34 HoLLA:-JD, ,J. R.: Methotrexal.e therapy of mf-d,,, static choriocarcinoma. Am. .J. Obst .. Gynec., 75: Hl5, Hl58. HoRN, R. C. AND ENTERLJNJ", H. T: RJ:mbdomy,J·· sarcoma.: A clinicopathological classification of 3 cases. Cancer. 2: , 1,~;wrs, W. H.: Rhabdornyosarcom:1. Cancer search, 3: 86, HJ43. Li, l\I. C., SP,rnc";R, D. B., H1mTz, H.. ANn L1j H. A.: Suppression of trophoblastic tum