Pure Testicular Rhabdomyosarcoma
M.J. Arya MD
Department of Pathology, Yazd University of Medical Sciences, Yazd, Iran
Pure testicular rhabdomyosarcoma is a very rare tumor and few cases have been reported in literature. The tumor usually presents as painless testicular enlargement with early dissemination via the blood stream and lymphatics. The origin of this tumor is presumed to be from overgrowth of a sarcomatous areas of teratoma. Here, a case of testicular rhabdomyosarcoma is presented and its clinical and pathological findings discussed.
Key Words · Rhabdomyosarcoma · testicle · cancer
Case Report
A 16-year-old male presented with a slow growing painless testicular mass since one year prior to admission.
Physical examination revealed a firm, non-tender mass in the right scrotum. A high orchiectomy was carried-out and the specimen was sent for pathologic examination.
Gross examination of the testis showed that it was enlarged and replaced by a whitish firm tumor, measuring 8´ 6´ 4 cm (Fig. 1). The tumor was completely contained within the tunica albuginea.
Microscopic examination revealed a highly cellular tumor composed of pleomorphic cells including round, strap like and racquet-shaped cells including several multinucleated forms arranged in sheets and an alveolar pattern. The cytoplasm of the tumor cells were intensely acidophilic with some cross-striations. The histological picture was that of pleomorphic rhabdomyosarcoma (Fig. 2).
In spite of chemotherapy and radiotherapy, the patient developed metastases to pelvic and periaortic lymph nodes and 17 months after diagnosis returned with fever and productive cough associated with severe cachexia, marked leukopenia and macronodular infiltrates in the lungs and died.
Discussion
Testicular tumors have been reported as occurring in between 0.58 and 2.09% of all male tumors. It is reported by Prince that 1 to 2% of testicular tumors are sarcomatous and only a small percentage of these are rhabdomyosarcomas.1 Therefore, we are dealing with a very rare tumor.
The age incidence varies from 3 months (Alexander 1968) to 67 years (Prince 1942).
These tumors are usually present as painless swelling of the testis. It appears that they are teratomatous in nature and arise from totipotential primitive germ cells.
Rhabdomyosarcoma is regarded as a highly malignant tumor with frequent recurrence and dissemination via the blood stream and lymphatics. Except in children where prognosis is poor long term survival has been reported.2 On microscopic examination, there are pleomorphic rhabdomyoblasts with some cross striations in the acidophilic cytoplasm.
Since these tumors metastasize via lymphatics as well as the blood stream, lymphangiography and dissection of retroperitoneal nodes may be of benefit. Little evidence is found in the literature to suggest that any treatment, other than surgical removal, is of value in the treatment of rhabdomyosarcoma, radiation and chemotherapy appear to have little effect.3
Acknowledgements
In preparing the present report, I wish to thank professor P.V.N. Kumar for his useful suggestions and reviewing this case.
References
1 Lawrence Ravich, Philip H, Lernman, et al. Pure testicular rhabdomyosarcoma. J Urology 1965;94:596-9.
2 Alexander F. Pure testicular rhabdomyosarcoma. Br J Cancer 1968;22:498-501.
3 Kumar PVN, Khezri AA: Pure testicular rhabdomyosarcoma. Br J Urology 1987;59:282.