Postradiotherapy intratesticular leiomyosarcoma

Postradiotherapy intratesticular leiomyosarcoma

CASE REPORT POSTRADIOTHERAPY INTRATESTICULAR LEIOMYOSARCOMA BENJAMIN K. CANALES, STEPHEN J. LUKASEWYCZ, JUAN C. MANIVEL, AND JON L. PRYOR ABSTRACT...

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CASE REPORT

POSTRADIOTHERAPY INTRATESTICULAR LEIOMYOSARCOMA BENJAMIN K. CANALES, STEPHEN J. LUKASEWYCZ, JUAN C. MANIVEL,

AND

JON L. PRYOR

ABSTRACT We report what we believe to be the first case of high-grade, radiation-induced, intratesticular leiomyosarcoma in a 30-year-old man who had had testicular relapse of acute lymphoblastic leukemia at age 12 years that was treated with standard testicular field radiation (2400 cGy) and chemotherapy. Radiation-induced tumors of this type are rare, have a median latency of 10 years, and are usually dose dependent (around 5000 cGy). Testicular leiomyosarcoma, especially high grade, remains to be fully characterized. After radical orchiectomy, patients should be followed up with serial germ cell tumor markers and imaging to monitor for metastatic spread. The use of retroperitoneal lymph node dissection and chemotherapy remains controversial but is probably not indicated. UROLOGY 66: 657.e19–657e.20, 2005. © 2005 Elsevier Inc.

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eiomyosarcomas are malignant soft-tissue tumors arising from undifferentiated smooth muscle cells of mesenchymal origin. Only 7 cases of intratesticular leiomyosarcoma have been reported.1– 6 We report the first case of radiation-induced intratesticular leiomyosarcoma and briefly review the published data. CASE REPORT A 30-year-old man presented to our clinic with a 5-month history of right testicular pain and swelling. His past medical history was significant for acute lymphoblastic leukemia at the age of 4 years that had been successfully treated with intrathecal methotrexate and brain radiotherapy followed by bone marrow and testicular relapse at 12 years of age. This relapse was successfully treated with systemic chemotherapy (vincristine, prednisone, daunomycin, and L-asparaginase) in addition to standard testicular field radiotherapy of 2400 cGy. Consequently, the patient developed hypogonadotrophic hypogonadism. Eighteen years later (at age 30), physical examination revealed a 26-cm3 firm right testis that was heterogeneous on ultrasonogFrom the Department of Urology, University of Minnesota, Minneapolis, Minnesota Address for correspondence: Jon Pryor, M.D., Department of Urologic Surgery, Box 394, Fairview-University Medical Center, 420 Delaware Street Southeast, Minneapolis, MN 55455-0392. E-mail: [email protected] Submitted: December 31, 2004, accepted (with revisions): March 7, 2005 © 2005 ELSEVIER INC. ALL RIGHTS RESERVED

raphy. The left testis was atrophic at 5 cm3. Alphafetoprotein and beta-human chorionic gonadotropin levels were normal. The patient underwent right radical orchiectomy and left scrotal orchiectomy, because this testis was nonfunctional. Within the right testis, a 4-cm, tan, fleshy tumor was found that was completely confined within the tunica albuginea with no invasion of paratesticular structures (Fig. 1). The remaining specimens were unremarkable. Light microscopy revealed a tumor with an interweaving pattern of elongated cells arranged in fascicles (Fig. 2). The tumor was positive for muscle-specific actin, smooth muscle actin, and desmin and negative for myogenin and inhibin. The final pathologic finding was reported as high-grade intratesticular leiomyosarcoma. Computed tomography of the abdomen, chest, and pelvis was negative for metastatic disease at surgery and at 6 months postoperatively. COMMENT In 1948, Cahen et al.7 established the first guidelines for the diagnosis of a postradiation sarcoma. Since this description, the criteria now include the following: diagnosis of a histologically confirmed sarcoma, development of this sarcoma within or adjacent to the path of the radiation beam, and a latency period of at least 3 to 5 years.8 In general, radiation-induced tumors are high grade, are extremely rare in patients surviving more than 5 years after radiotherapy (incidence ⬃0.2%), and 0090-4295/05/$30.00 doi:10.1016/j.urology.2005.03.027 657.e19

rarely show lymphatic spread (⬃5% of all cases), routine retroperitoneal lymph node dissection is likely not warranted unless the patient exhibits suspicious, enlarged, or biopsy-proven retroperitoneal lymph nodes.11 Mixed germ cell sarcomatous tumors have been associated with a poor prognosis12; therefore, it is recommended that patients with testicular sarcoma undergo serial evaluations of alpha-fetoprotein and beta-human chorionic gonadotropin to uncover any germ cell elements.11 CONCLUSIONS FIGURE 1. Gross specimen of testicular leiomyosarcoma.

FIGURE 2. Photomicrograph of tumor.

occur at a median latency of approximately 10 years.9 The development of the disease is dose dependent, usually at a median dose of 5000 cGy.9 This case fulfilled the criteria for radiation-induced sarcoma. All 7 reported cases of intratesticular leiomyosarcoma have been either low grade or unspecified, and all were treated by orchiectomy.1– 6 Disease recurred in only 1 patient, a 70-year-old man who died of pulmonary metastasis approximately 14 months after orchiectomy.6 Retroperitoneal lymph node dissection after orchiectomy was reported in only one instance, with negative lymph nodes.4 No previous case of high-grade intratesticular leiomyosarcoma has been described, although high-grade paratesticular leiomyosarcomas have been associated with aggressive spread and metastasis.10 The role of primary or adjuvant chemotherapy for this tumor has never been reported, although metastatic and unresectable postradiation sarcomas in other parts of the body have been treated with a combination of doxorubicin and ifosfamide.9 Because primary soft-tissue sarcomas

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Intratesticular leiomyosarcoma of this high grade was likely caused by the previous testicular radiotherapy. Testicular leiomyosarcoma, especially the high-grade variants, remains to be fully characterized. Treatment should include radical orchiectomy and careful follow-up to monitor for metastatic spread and germ cell tumor markers. The use of retroperitoneal lymph node dissection and chemotherapy remains controversial but is probably not indicated. REFERENCES 1. Yachia D, and Auslaender L: Primary leiomyosarcoma of the testis. J Urol 141: 955–956, 1989. 2. Pellice C, Sabate M, Combalia A, et al: Leiomyosarcome du testicle. J d’Urol 100: 46 – 48, 1994. 3. Washecka RM, Mariani AJ, Zuna RE, et al: Primary intratesticular sarcoma: immunohistochemical ultrastructural and DNA flow cytometric study of three cases with a review of the literature. Cancer 77: 1524 –1528, 1996. 4. Froehner M, Fischer R, Leike S, et al: Intratesticular leiomyosarcoma in a young man after high dose doping with oral-turinabol: a case report. Cancer 86: 1571–1575, 1999. 5. Ali Y, Kehinde EO, Makar R, et al: Leiomyosarcoma complicating chronic inflammation of the testis. Med Principles Pract 11: 157–160, 2002. 6. Hachi H, Bougtab A, Amhajji R, et al: A propos d’un cas de leiomyosarcome testiculare. Med Trop 62: 531–533, 2002. 7. Cahen WG, Woodard HQ, Higginbotham NL, et al: Sarcoma arising in irradiated bone: a report of eleven cases. Cancer 1: 3–29, 1948. 8. Arlen M, Higginbotham NL, Huvos AG, et al: Radiation-induced sarcoma of bone. Cancer 28: 1087–1099, 1971. 9. Patel SR: Radiation-induced sarcoma. Curr Treat Options Oncol 1: 258 –261, 2000. 10. Fischer C, Goldblum JR, Epstein JI, et al: Leiomyosarcoma of the paratesticular region: a clinicopathologic study. Am J Surg Pathol 25: 1143–1149, 2001. 11. Washecka RM, Mariani AJ, Zuna RE, et al: Primary intratesticular sarcoma: immunohistochemical ultrastructural and DNA flow cytometric study of three cases with a review of the literature. Cancer 77: 1524 –1528, 1996. 12. Matoska J, and Talerman A: Spermatocytic seminoma associated with rhabdomyosarcoma. Am J Clin Pathol 94: 89 – 95, 1989.

UROLOGY 66 (3), 2005