Rhabdomyosarcoma of the Male Urethra

Rhabdomyosarcoma of the Male Urethra

Vol, 9P, .-\pr. Pn'11ted fri C ./3. A, THE ,JOURNAL OF UROLOGY Copyright© 1968 by The Williams & Wilkins Co RHABDrnVIYOSARCOl\IA OF THE J\IALE URET...

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Vol, 9P, .-\pr. Pn'11ted fri C ./3. A,

THE ,JOURNAL OF UROLOGY

Copyright© 1968 by The Williams & Wilkins Co

RHABDrnVIYOSARCOl\IA OF THE J\IALE URETHRA M. R. PAINTER,* E. J. O'SHAUGHNESSY;r P II. LARSON rnn R. K RIEBE Froni the Urologic and Orihopeclic Services, Departments of Surgery and Pa.lho/ogy, 1'ripler Army N!eclical Center, Hawaii

Rhabdomyosarcoma is a rare neoplasm of the infravesical urinary tract, only 46 eases of rhabdomyosareoma of the prostate having been reported. 1 Still more rare is the rhabdomyosarcoma. presenting in the methra.. Batsa.kis reported l embryonal rbalxlomyosarcoma of the urethra. in a female This report deseribes a case of rhabdomyosarcoma presenting in the membranous urethra of the male concomitant with a similar lesion in the thigh. No similar case has been reported in the literature.

open bi.opRy confirmed the diagnosis of rhabdomyosarcoma. A modified Boyd hip disarticulation W:ls [X,rformccl. "\. small blood clot was passed pn tu·cthram, l::l day.~ postoperatively. Marked micro hematnria. was found iu the initial fraction of :3-glas~ test. Excretory urogra.phy and cy~tog raphy indicated a. normal upper truda.nd bladder v;ith a signific:ant re,,iclu urn on !he post-voiding film. Cystourethroscopy revealed fim.briated tumor protruding from the wall of the membranous urethra. The pros1a.tic un:thrn :UH.I

CASE REPORT

A 39-year-old Malayan man presented with a. rnass in the posterior thigh, 1 week in duration. This mass, though painless, enlarged rapidly. The patient had no Rymptoms referable to the urinary tract. He had a cardiova.sc:ular accident when he was 22 years old with a re,,idual of left herniparesrn. Except for the hemipare.ois, phy,sic:al examination revealed only an 8 8 cm., slightly tender, dee11 soft tissue ma.ss over the !JOsterior lateral aspect of the distal right femur, that was well circumscribed and not attached to the skin. Laboratory examinations revealed normal hemogram and urinaly,sis. Chest and bone surveys were normal except for the site of 11coplasm. X-ray of the right thigh n:vea.led a destructive lesion of the distal portion of the fomur. An arteriograrn was sugge.,tin, of 1ieoplasm and an

Frn. 1. Filling clefecl in 1tre1 hr:.1 rndicatill?, rnass.

Accepted for publication May 10, 19(i7. This material has been reviewed by 1.l1e Oflice of The Surgeon General, Department of the Army, and there is no objection to its presentation and/or publication. This review doe,s not imply any indorsement of the opinions advanced or nny recommendation of such products as may be 1rnrned. * Requests for reprints: lJrology Service, Fitzsimons Genera.I Hospital, Denver, Colorado 80240. t Present address: Box 96, National War College, Washington, D. C. 20044. 1 Lemmon, W. T., Jr., Holland, .J.M. a.nd Ketcham, A. S.: R.habdomyosarcoma of the prostate. Surgery, 59: 736, 1966. 2 Batsakis, J. G.: Urogenital rhabdomyosarcoma: Histogencsis and classification. J. Urol., 90: ]80, 196:3.

bladder did not appear involved. A uret.hrogram confirmed the presence of a mass ,n this area. (fig. l). Trnnsurcthral of ihe lesion confirmed the diagnosis of sarcoma. Bi.manual examination at the hllrn of anesthesia. failed to ren:al fixal.icm of bladder m prostate, a.ncl repeat chest. ancl meta,tn.tic bone surveys were 11egative for mc~taotalic disea,r\ Sub'3equently radical prostalectomy. sPmirml vesieulectomy, me1nbra.11ous and bulbar urc:threctomy with hi lateral iliac node di,:sec:1 inn were performed through au approach I month after the original The bladder appearncl uninvolvecl and was rntained to he utilized as a. c:omluit later.

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PAINTER AND ASSOCIA TEE'

Frn. 2. A, tumor in lymphatics of membranous urethra. XlOO. B, tumor replacing urothelinm of mernbranons urethra. X350. The postoperative eourse was ]Jlagued by an intermittent temperature ri-;e to 101 degrees daily which had pernistecl from the day of aclmi,sion. Chest x-ray, 15 clays after hospitalization, revealed a bilateral n1icl-lu11g field nodularity. Despite initiation of actinon1ycin D therapy, the patient died 27 days postoperatively. Examination of the surgical specim.en disclosed a prostate which microscopirally contained many small nests of tumor, and the mucosa of the lurnen of the right seminal vesicle was abo replaced with tumor. The mucosa of the membranous urethra was entirely replaced by tumor with occasional small submucosal nests (fig. 2). Histologically the tun10r was one of a pleomorphic pattern of multinucleated giant cells, large cells with vesicular nuclei and eosinophilic cytoplasm, and strap and racquet-shaped cells. Mitotic figures were abundant. Special stains helped to demonstrate indistinct cro.~s striations in tumor cells. The pattern was characteristic of the plemnorphic variety of rhabclomyosarcoma.a The left seminal vesicle and iliac nodes were uninvolved. Postmortem exmnination disclosed tumor in 3 Horn, R. C., Jr. and Enterline, H. T.: Rhabdomyosarcoma: A clinicopathological study and classification of 39 cases. Cancer, 11: 181, i958.

the midline of the anterior pelvis displacing the bladder, with the tumor eroding the wall of the sigmoid colon leading to the in1mecliate cause of death fron, purulent peritonitis. Metastatic foci were de1nonstrahle in the lungs, adrenals and thyroid. DISCUSSION

0 ur patient's poor response to operative and chemotherapeutic attempts at cure or palliation did not differ from cases of rhabdomyosarcoma of the prostate previously reported. The average life expectancy after diagnosis is 7 to 11 months; our patient died 6 months after admission.I. 4 However, this case i., perplexing histologically. The pleomorphic variety of rhabdomyosarcoma usually arises in the extremities of adults in contrast to other varieties which favor a more central origin. Although other parts of the male urogenital system are not uncommon sites for either primary or metastatic rhabdomyosarcoma, involvement of the male urethra per se has not previously been reported. In view of the original presentation as a thigh tumor and the time interval between disarticulation and urethral symptoms, blood-borne metastases to the urethra, prostate and right seminal vesicle must be 4 Stirling, W. C. and Ash, J. E.: Sarcoma of the prostate. J. UroL, 41: 515, 1939.

ltHABDOl\lYOSARCOJ\IA OF l\lALJ<] Ul-tETHHA

consider8d as probable. :However, no other metastatic le.,ion was found at. the time that the urethral lesion was discovered which was only 2 weeks following disarticulation of the right hip. The metastatic: pattern to the ureLhra, while and a primary tumor probable, would be of the urethra cannot be histologically excluded. This case may represent a double primary pleornorphic rhabclomyosarcoma presenting as a !high and a urethral lesion.

Sl';\HIARY

.·\. urethral presentation of a rhabdornyosarcoma in a man with an :WCOlll[lLll1)'· ing similar 11coplasm of the di.st.al ·:s described. A similar case bas not been reported in the literature. Despite and adequate operative patient died 6 m011ths fron1 the time of within the average life expectancy noted rn utlwi cnsl,s of urogenital rhahclomyo.~a.rc(nna.