0022-5347 /81/1264-0563$02.00/0 Vol. 126, October Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1981 by The Williams & Wilkins Co.
RHABDOMYOSARCOMA OF THE PARATESTICULAR TISSUES ALAN FORTUNE*
BRIAN R. BOLTON
AND
From the Departments of Urology and Surgery, Fremantle Hospital, Fremantle, Western Australia
ABSTRACT
We report 2 cases of rhabdomyosarcoma of the paratesticular tissues. Differentiation from chronic epididymal infection, such as tuberculosis, may be difficult but it must not delay operation. Lymphography is of limited value in delineating lymph node metastases. In both cases secondary spread was suggested but not confirmed histologically. Lymphography, because of a definite mortality rate of 0.1 per cent and morbidity rate of 1 per cent, would appear not to be indicated. Rhabdomyosarcoma of the paratesticular tissues is a rare tumor. Spread of these tumors has been demonstrated to occur via the testicular vessels to the para-aortic nodes. In most of the articles previously reported the use of lymphography is supported but falsely positive and negative rates are high and its use should be re-evaluated. Para-aortic lymph node dissection is not universally practiced because of the associated morbidity but recent evidence suggests that it may be beneficial. Herein we present 2 additional cases of paratesticular rhabdomyosarcoma. CASE REPORTS
Case I. A 17-year-old man presented elsewhere with a soft small swelling posterior to the right testis, for which no treatment was given. Following minor trauma 6 months later in September 1977 the mass had enlarged to 6 X 5 cm. and was attached to the epididymis, separate from the testis and nontender. There was no inguinal lymphadenopathy. Diagnosis was intrascrotal tumor, although tuberculous epididymo-orchitis was considered a major possibility in the differential diagnosis. Investigations included hemoglobin 14.2 per cent, total white count 10,900/mm. 3 with 48 per cent neutrophils and 40 per cent lymphocytes, and erythrocyte sedimentation rate 4 mm. in the first hour (Westergren). Urine microscopy and culture were normal as well as blood urea and serum electrolytes. A chest radiograph showed no abnormality. Mantoux reaction was positive. Exploration was performed through a right inguinal incision. The testis was normal in size and consistency but increased vascularity of its posterior surface was noted. The epididymis was enlarged, pink and myxoid. The testis, epididymis and spermatic cord were removed en bloc. Pathological examination revealed an embryonal rhabdomyosarcoma with myxoid areas. Convalescence was uneventful. Pedal lymphography showed "alteration in the para-aortic nodes suggestive of metastases". Thus, the patient was rehospitalized and a block dissection of the superficial and deep inguinal nodes, iliac nodes and right lateral aortic and pre-aortic lymph nodes was performed. None of the nodes was involved with tumor on histological examination. The lymph nodes with filling defects on lymphography showed granulomatous change only. A 2-year combined chemotherapy regimen was started using vincristine, cydophosphamide and actinomycin D. After 30 months of treatment there has been no clinical evidence of recurrence. Liver function tests, chest x-ray and hematological parameters are normal. Case 20 A 17-year-old man presented in January 1978 with a 7-week history of an enlarging right scrotal swelling without Accepted for publication October 24, 1980. * Requests for reprints: Department of Urology, Fremantle Hospital, Fremantle, Western Australia 6160.
local pain but a recent constant throbbing mild suprapubic pain. On examination the patient was well with no palpable lymphadenopathy and a 6 cm. firm mass posterior to and separate from the testis. Investigations included hemoglobin 17.3 per cent and total white count 10,300/mm. 3 with 74 per cent neutrophils, 17 per cent lymphocytes and 5 per cent eosinophils. Liver function tests, chest x-ray, serum urea and electrolytes were normal. A Mantoux test was not done. A pedal lymphogram showed a gap in the common iliac lymph gland chain on the right side but normal lymph vessels in this area made this observation of doubtful significance. A filling defect in l para-aortic node was present, suggesting metastases. Liver and bone scans were normal. Through a right inguinal incision en bloc excision of the testis, epididymis and spermatic cord to the internal inguinal ring was performed. A 6.0 X 5.5 X 3.0 cm. tumor was adherent to the lower pole of the testis posteriorly. Histological diagnosis was a poorly differentiated rhabdomyosarcoma with vessel invasion. A block dissection of the inguinal, iliac and right paraaortic nodes was performed 3 weeks after orchiectomy. Histol-ogy was normal. Identical followup treatment to case 1 was instituted and there has been no evidence of recurrence after 27 months. DISCUSSION
There have been few reported cases of rhabdomyosarcoma of the paratesticular tissues. Aspects of the differential diagnosis and the usefulness of lymphography need clarification. Differential diagnosis. Although testicular tumors generally are classically said to present as painless intrascrotal masses, as exemplified by our 2 cases, it is important to bear in mind that their presentation can range from "the obvious to the bizarre" .1 In particular, one must be careful not to be misled by a history of trauma, as in case l, since this may be a result of the patient trying to ascribe a cause to the lump or, conversely, trauma attracting the patient's attention to a previously unnoticed mass. Likewise, a history of pain should not distract from consideration of tumor because this may be due to bleeding into the tumor or the coexistence of a painful inflammatory condition. Indeed, of the 491 cases reported by Patton and associates pain was a presenting symptom in 23 per cent. 2 One of the most common sources of misdiagnoses is epididymitis.3 The presence of classical signs of acute inflammation together with pyuria and/or bacteriuria makes the diagnosis acute epididymitis fairly straightforward. However, in subacute and chronic forms the aforementioned findings may be absent and a differential diagnosis can be much more difficult. In particular, tuberculous epididymitis must be considered, since tuberculosis is still a relatively common disease in some parts of the world. In the absence of evidence of tuberculosis in other parts of the body, one must then make a decision whether to
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perform exploration or wait the 6 weeks or so required for laboratory confirmation of tuberculosis. Generally, it is agreed that if there is any reasonable degree of suspicion of the presence of intrascrotal tumor, exploration should be undertaken through an inguinal incision. Trans-scrotal biopsy or aspiration should be avoided since seeding in this site is common. 4 In most instances a confident diagnosis can be made on macroscopic appearance but, if not, frozen section biopsy should be used. If any doubt exists radical orchiectomy should be performed. The role of lymphography. Banowsky and Shultz found evidence of lymphatic spread in 29 of 101 cases of sarcoma of the tunics and cord. 5 Thus, if one followed classical surgical dictum, retroperitoneal dissection should be performed in continuity with removal of the primary tumor. However, there is some resistance to this approach because of the high morbidity associated with retroperitoneal dissection, particularly subsequent infertility. Kedia and associates reported infertility in 49 of 52 patients, although sexual function usually remained normal. 6 This is due to interruption of Tl2, Ll sympathetics, leading to failure of propulsive activity in the remaining vas deferens. Colodny and Hopkins suggest that this complication can usually be avoided by performing an "extended ipsilateral" dissection if the nodes are not obviously involved at operation. 7 That retroperitoneal dissection should be done at all in these tumors is supported by Malek and Kelalis in their series of 10 children with rhabdomyosarcoma of paratesticular tissue. 8 They found that delay in recognition and excision of retroperitoneal metastases result in a much worse prognosis. Thus, one is led to consider the usefulness of lymphography in the detection of retroperitoneal node involvement. Wilkinson and MacDonald are enthusiastic about lymphography.9 It should be indicated, however, that 45 per cent of their 260 patients were suffering from seminomas and much of the use of lymphography was related to staging and monitoring response to radiotherapy. Furthermore, since none of their patients underwent lymphadenectomy, they have no histopathological correction of their lymphographic interpretations. Tavel and associates concluded that lymphangiography can be a useful means of evaluating the retroperitoneum. 10 However, in their 33 cases of nonseminomatous tumors, which resulted in lymphadenectomy, there was a falsely positive rate of 25 per cent and a falsely negative rate of 17 per cent. On the other hand, of 45 patients with. nonseminomatous testicular tumors who underwent lymphadenectomy Storm and associates found that "lymphangiography is not completely reliable in the diagnosis of metastatic retroperitoneal lymphadenopathy".11 They reported a falsely positive rate of 41 per cent and a falsely negative rate of 39 per cent. The most impressive series reported with histological correlation is that of Kademian and Wirtamen whose 45 cases had a falsely positive rate of 3.4 per cent (1 of 29) and a falsely negative rate of 25 per cent (4 of 16).12 It seems that although the reported falsely positive rates are variable the falsely negative rates are consistently (and probably unacceptably) high, ranging from 1 in 6 to >l in 3. Some of the reasons postulated for the falsely positive rates include 1) microscopic foci being missed on histopathological sampling, 2) inadequacy of dissection (Tavel and associates reported that up to 25 per cent of nodes remain after "adequate" adenectomy. 10), 3) spurious filling defects, such as fatty replacement, fibrosis or chronic inflammatory disease (for example case 1), and 4) node reaction from iodized oil. The falsely negative rates could be owing to the fact that 1)
para-aortic sentinel nodes that are demonstrable on funicular lymphography are not as readily visualized on pedal lymphography13 and 2) micrometastases may be missed on the lymphogram. Finally, when the usefulness of any investigation is considered one must also think of the risks involved. Storm and associates indicated that lymphography has an over-all mortality rate of 0.1 per cent and significant morbidity of approximately 1 per cent. REFERENCES 1. Gordon-Taylor, G. and Wyndham, N. R.: On malignant tumours of the testicle. Brit. J. Surg., 35: 6, 1947. 2. Patton, J. F., Hewitt, C. B. and Mallis, N.: Diagnosis and treatment of tumors of the testis. J.A.M.A., 171: 2194, 1959. 3. Stephen, R. A.: The clinical presentation of testicular tumours. Brit. J. Urol., 34: 448, 1962. 4. Markland, C., Kedia, K. and Fraley, E. E.: Inadequate orchiectomy for patients with testicular tumors. J.A.M.A., 224: 1025, 1973. 5. Banowsky, L. H. and Shultz, G. N.: Sarcoma of the spermatic cord and tunics: review of the literature, case report and discussion of the role of retroperitoneal lymph node dissection. J. Urol., 103: 628, 1970. 6. Kedia, K. R., Markland, C. and Fraley, E. E.: Sexual function after high retroperitoneal lymphadenectomy. Urol. Clin. N. Amer., 4: 523, 1977. 7. Colodny, A. H. and Hopkins, T. B.: Testicular tumors in infants and children. Urol. Clin. N. Amer., 4: 347, 1977. 8. Malek, R. S. and Kelalis, P. P.: Paratesticular rhabdomyosarcoma in childhood. J. Urol., 118: 450, 1977. 9. Wilkinson, D. J. and MacDonald, J. S.: A review of the role of lymphography in the management of testicular tumours. Clin. Rad., 26: 89, 1975. 10. Tavel, F. R., Osius, T. G., Parker, J. W., Goodfriend, R. B., McGonigle, D. J., Jassie, M. P., Simmons, E. L., Tobenkin, M. I. and Schulte, J. W.: Retropertioneal lymph node dissection. J. Urol., 89: 241, 1963. 11. Storm, P. B., Kern, A., Loening, S. A., Brown, R. C. and Culp, D. A.: Evaluation of pedal lymphangiography in staging non-seminomatous testicular carcinoma. J. Urol., 118: 1000, 1977. 12. Kademian, M. and Wirtanen, G.: Accuracy of bipedal lymphangiography in testicular tumors. Urology, 9: 218, 1977. 13. Chiappa, S., Uslenghi, C., Bonadonna, G., Marano, F. and Ravasi, G.: Combined testicular and foot lymphangiography in testicular carcinomas. Surg., Gynec. & Obst., 123: 10, 1966.
EDITORIAL COMMENT These authors review the differential diagnosis of this rare malignant tumor. The 2 patients have experienced a good result after multimodal therapy as it is practiced currently. Surgical excision of the para-aortic and iliac lymph nodes similar to the treatment for nontesticular germinal cell tumors generally is practiced. Resection of the superficial and deep inguinal lymph nodes as reported by these authors is inadvisable and unnecessary unless the scrotum is involved. Dissection behind the great vessels (aorta and vena cava) will eliminate the 25 per cent of nodes said to remain after "adequate adenectomy" as described by Tavel and associates (reference 10 in article). The controversy regarding the adequacy of lymphadenectomy seems to have been resolved by a technique that involves complete dissection behind the great vessels and can be accomplished only by division of the lumbar arteries and veins. The prognosis for rhabdomyosarcoma of the spermatic cord appears to be significantly better than a similar tumor that occurs in relation to the urinary organs of the male subject, and the urinary and genital organs of the female subject. Donald C. Martin Department of Surgery University of California Irvine, California