Case Report Epididymal Angiosarcoma Matthew H. Hayn, Sheldon Bastacky, and Michael E. Franks Sarcomas are rare and account for approximately 1% of all malignancies. The subtype angiosarcoma is derived from vascular and lymphatic tissue and generally has a poor prognosis. Prior radiation therapy is a known risk factor for the development of angiosarcomas. We present what we believe to be the first case of an angiosarcoma arising in the epididymis in an 80-year-old man who presented with right scrotal swelling. UROLOGY 69: 576.e5–576.e7, 2007. © 2007 Elsevier Inc.
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oft tissue tumors, both benign and malignant, of the testes and paratesticular region are uncommon, and their exact incidence is difficult to estimate.1 Sarcomas are also rare, and the subtype angiosarcoma has never been reported arising in the epididymis. Radiation therapy is a known risk factor for the development of angiosarcoma. We report a case of epididymal angiosarcoma in an 80-year-old man who had undergone prior radiation therapy for prostate cancer.
CASE REPORT An 80-year-old man presented with the complaint of a few weeks of right scrotal swelling. His past medical history was significant for prostate cancer that was treated with external beam radiation coupled with hormonal therapy. The radiation treatment was completed in 2001. His most recent prostate-specific antigen level was 1.6 ng/mL, consistent with biochemical recurrence. Physical examination revealed a large right hydrocele and normal left hemiscrotum. Scrotal ultrasound showed a large right hydrocele with debris, as well as a right epididymal head mass that measured 3.0 ⫻ 2.1 ⫻ 2.2 cm (Fig. 1). The mass was hypervascular, and our differential diagnosis included epididymo-orchitis versus neoplasm. The patient was treated with 6 weeks of ciprofloxacin. A repeat ultrasound after the ciprofloxacin showed no change. The patient was taken to the operating room, where he underwent right radical orchiectomy. Final pathology demonstrated a 3-cm, circumscribed, spongy mass that was present in the inferior pole of the epididymis. The mass did not appear to infiltrate the testicular parenchyma grossly or histologically. Histology revealed a vascular neoplasm composed of irregular, anastomosing, capillary-sized blood vessels lined by endothelial cells with focal moderate cytologic atypia (Fig. 2). Mitotic figures were not seen. There was focal hemorrhage and necrosis. Some of the neoplastic vesFrom the Departments of Urology and Pathology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania Address for correspondence: Matthew H. Hayn, M.D., Department of Urology, University of Pittsburgh Medical Center, Kaufmann Medical Building, Suite 700, 3471 Fifth Avenue, Pittsburgh, PA 15213. E-mail:
[email protected] Submitted: August 10, 2006; accepted: January 22, 2007
© 2007 Elsevier Inc. All Rights Reserved
sels were thrombosed. The neoplasm formed a thrombus within a medium-sized blood vessel. The neoplastic cells were immunoreactive with CD31, CD34, and Ulex Europaeus, consistent with vascular endothelial origin (Fig. 3). Cytokeratin immunostains were performed with pankeratin and AE1/AE3, both of which were negative. A Ki-67 (proliferation marker) showed regional variability, with immunoreactivity in 10% to 80% of the neoplastic endothelial cells. The pathology results were consistent with a paratesticular angiosarcoma. On the basis of the final pathology results, a computed tomography (CT) scan of the chest, abdomen, and pelvis was obtained. It revealed no evidence of chest, retroperitoneal, or pelvic involvement or any solid organ metastasis. As of the time of manuscript preparation, no systemic chemotherapy is planned for the patient. At this writing he is 1 year from his original diagnosis and has had serial CT scans of the chest, abdomen, and pelvis every 3 months since his diagnosis, all of which have been negative for tumor recurrence or retroperitoneal nodal disease. Results from his most recent physical examination were also negative for any local recurrence.
COMMENT Sarcomas are rare and account for approximately 1% of all malignancies. The subtype angiosarcoma is derived
Figure 1. Ultrasound demonstrating right epididymal (RT EPI) head mass. 0090-4295/07/$32.00 576.e5 doi:10.1016/j.urology.2007.01.074
from vascular or lymphatic tissue and, similar to other sarcomas, is marked by difficult local control, often leading to regional spread and generally a poor prognosis. There are four typical clinical presentations of angiosarcoma; the most common are primary lesions of the face and scalp. The other settings include lesions at a site of chronic lymphedema, postirradiation, and a primary hepatic mass. The most common site of postirradiation angiosarcoma is the breast.2 Upon review of the literature, ours is the only known report of an angiosarcoma arising in the epididymis. The appearance of the angiosarcoma 4 years after radiation therapy for prostate cancer fulfills Cahan and associates’ criteria of postirradiation angiosarcoma: (1) prior radiation therapy, (2) a latency period of several years since radiation, (3) the development of sarcoma within the irradiated field, and (4) histologic confirmation of the sarcoma.3 Interestingly, the latency period of the angiosarcoma in our case was relatively short given that most angiosarcomas occur 4 to 15 years after radiation.4 The early presentation of this angiosarcoma may be due to epididymal predisposition to develop hydrocele and symptoms of epididymitis with a new mass, which may have expedited its detection. Other reports of lower urinary tract and genital angiosarcoma after irradiation have been rare and include the testis,5 testicular tunica,6 prostate,7 bladder,8 and vagina.9 The risk of developing any type of sarcoma after radiation treatment has been estimated at 0.03% to 0.8%.10 The etiology is thought to be irreversible damage of genetic material with enhanced proto-oncogene expression11 or inactivation of tumor suppressor genes.12 Despite a poor prognosis, the most successful treatment of sarcomas is wide resection of the tumor because lesions tend to be multifocal with regional spread. Systemic therapy has been used for curative intent in an adjuvant setting, as well as palliation in unresectable cases. Reports of response to such agents as docetaxel, doxorubicin, cisplatin, and immunotherapy can be found in the liter-
Figure 2. Histology of the mass revealing an angiosarcoma with focal moderate cytologic atypia. Hematoxylin and eosin stain, original magnification, ⫻60.
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Figure 3. CD31 immunostain highlights the neoplastic endothelial cells lining the vascular channels. Original magnification, ⫻100.
ature.9,13,14 Somewhat paradoxically, radiation has also been used for curative intent and palliation.15 In conclusion, we report the first case of an epididymal angiosarcoma, which arose in the setting of prior radiation therapy. The interval between the end of radiation therapy and diagnosis of angiosarcoma was 4 years, which is a relatively short latency period. The angiosarcoma was associated with a hydrocele, which likely led to early detection and diagnosis. At the time of writing the patient is without any signs of local or distant recurrence. Angiosarcomas are aggressive tumors, associated with a poor prognosis, and the patient remains at high risk of local and distant recurrence. References 1. Folpe AL, and Weiss SW: Paratesticular soft tissue neoplasms. Semin Diag Pathol 17: 307–318, 2000. 2. Yap J, Chuba PJ, Thomas R, et al: Sarcoma as a second malignancy after treatment for breast cancer. Int J Radiat Oncol Biol Phys 52: 1231–1237, 2002. 3. Cahan WG, Woodward HQ, Higinbotham NL, et al: Sarcoma arising in irradiated bone: report of 11 cases. Cancer 1: 3–29, 1948. 4. Arlen M, Higinbotham NL, Huvos AG, et al: Radiation-induced sarcoma of bone. Cancer 28: 1087–1099, 1971. 5. Masera A, Ovcak Z, and Mikuz G: Angiosarcoma of the testis. Virchows Arch 434: 351–353, 1999. 6. Kravtsov VG, Sekamova SM, Kodolova IM, et al: [Angiosarcoma of the testicular tunica]. Arkh Patol 53: 62– 65, 1991. 7. Chandan VS, and Wolsh L: Postirradiation angiosarcoma of the prostate. Arch Pathol Lab Med 127: 876 – 878, 2003. 8. Navon JD, Rahimzadeh M, Wong AK, et al: Angiosarcoma of the bladder after therapeutic irradiation for prostate cancer. J Urol 157: 1359 –1360, 1997. 9. Takeuchi K, Deguchi M, Hamana S, et al: A case of postirradiation vaginal angiosarcoma treated with recombinant interleukin-2 therapy. Int J Gynecol Cancer 15: 1163–1165, 2005. 10. Mark RJ, Poen J, Tran LM, et al: Postirradiation sarcomas. A single-institution study and review of the literature. Cancer 73: 2653–2662, 1994. 11. Hallahan DR, Virudachalam S, Beckett M, et al: Mechanisms of x-ray-mediated protooncogene c-jun expression in radiation-induced human sarcoma cell lines. Int J Radiat Oncol Biol Phys 21: 1677–1681, 1991.
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12. Brachman DG, Hallahan DE, Beckett MA, et al: P53 gene mutations and abnormal retinoblastoma protein in radiation-induced human sarcomas. Cancer Res 51: 6393– 6396, 1991. 13. Mano MS, Fraser G, Kerr J, et al: Radiation-induced angiosarcoma of the breast shows major response to docetaxel after failure of anthracycline-based chemotherapy. Breast 15: 117–118, 2006.
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14. Mathew P, Vakar-Lopez F, and Troncoso P: Protracted remission of metastatic epithelioid angiosarcoma with weekly infusion of doxorubicin, paclitaxel, and cisplatin. Lancet Oncol 7: 92–93, 2006. 15. Ward JR, Feigenberg SJ, Mendenhall NP, et al: Radiation therapy for angiosarcoma. Head Neck 25: 873– 878, 2003.
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