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10 October 2012 - Case of the Week #254
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Thanks to Dr. Saroona Haroon, The Aga Khan University Hospital (Pakistan), for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.
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Case of the Week #254
A 45 year old man had a 2 month history of a rapidly enlarging, painless testicular swelling. The clinical impression was a neoplastic lesion, such as seminoma.
What is your diagnosis?
Immunostains were performed:
Left to right: vimentin, desmin, alpha smooth muscle actin, PAS, PLAP
The behavior of leiomyosarcoma is site related. Paratesticular leiomyosarcomas are rare, and usually low-grade (Am J Surg Pathol 2001;25:1143). The mean patient age is 62 years (range 34-86 years), and most cases involve the testicular tunica or spermatic cord. The mean size is 5 cm (range 2-9 cm). Tumors have fascicular growth of a spindle cell component resembling leiomyosarcoma at other sites, with nuclear pleomorphism, mitotic activity and coagulative necrosis. Most tumors are immunoreactive for muscle specific actin, smooth muscle actin and desmin, and a third are CD34+. Focal staining may also be seen for cytokeratin.
The differential diagnosis includes variants of leiomyoma, also rare, which may be cellular or hemorrhagic, or have mitotic activity, bizarre nuclei, epithelioid features or myxoid stroma.
Radical orchiectomy / high inguinal orchiectomy followed by surveillance or radiation or chemotherapy appears to be the treatment of choice. High grade tumors are aggressive, and may metastasize to lymph nodes, liver or lungs, and cause death (Med Trop (Mars) 2002;62:531). Those with low grade disease occasionally have recurrences, but no deaths.
Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
30100 Telegraph Road, Suite 408
Bingham Farms, Michigan (USA) 48025
Email: [email protected]
Alternate email: [email protected]