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Malignant Mesothelioma of the Tunica Vaginalis Testis: A Case Report and Literature Review Jing-Liang Chen1 and Yung-Hsiang Hsu2 Departments of 1Urology and 2Pathology, Buddhist Tzu Chi General Hospital, Hualien, Taiwan. Malignant mesothelioma of the tunica vaginalis testis is a rare but often fatal malignancy. Here, we report one patient with locally advanced disease who has a history of asbestos exposure. We review the literature concerning current management strategies of the disease. Radical surgery plus adjuvant radiotherapy seems to provide the best results. Key Words: asbestos, malignant mesothelioma, tunica vaginalis (Kaohsiung J Med Sci 2009;25:77-81) Malignant mesothelioma is a very rare [1] but often fatal type of testicular malignancy that originates from mesenchymal tissue [1-4]. It usually presents as an incidental finding at the time of hydrocele surgery [4], particularly in patients with prior exposure to asbestos [5-7]. Here, we describe a Taiwanese patient with malignant mesothelioma of the tunica vaginalis, together with a review of the literature. Case Presentation and pelvic computerized tomography (CT) demon strated a localized heterogeneous right scrotal tumor without evidence of local lymphadenopathy (Figure 1). Elevated serum p-HCG (27.5 mlU/mL) was detected. Surgical exploration through scrotal incision was performed. The stony hard lesion with purulent dis charge had destroyed the right testicle and had in vaded the right external inguinal canal. Local resection was done because of chronic inflammation of thick ening tunica vaginalis, as confirmed by frozen section examination. Pus culture grew enterococcus species. A 67-year-old man, who was previously diagnosed with infected right hydrocele in 2004 and had long standing bilateral hydrocele for 30 years, visited our clinics with presentation of painless enlargement of the right scrotum for 2 months. Grossly, the tumor had a size of 8 x 10 x 15 cm and occupied the whole right scro tum up to the right external inguinal canal. Ultrasonog raphy revealed left hydrocele and heterogeneous right testicular tumor. The subsequent abdominal Received: May 26, 2008 Accepted: Jul 21, 2008 Address correspondence and reprint requests to: Dr Jing-Liang Chen, Department of Urology, Buddhist Tzu Chi General Hospital, 707, Section 3, Chung Yang Road, Hualien 970, Taiwan. E-mail: bright4.chen@msa.hinet.net ELSEVIER Figure 1. Computed tomography of the testis shows one hetero geneous tumor (arrow) involving the scrotal wall and invading the right testicle (asterisk). Kaohsiung J Med Sci February 2009 Vol 25 No 2 2009 Elsevier. All rights reserved. 77 J.L. Chen and Y.H. Hsu Figure 2. Gross inspection ofthe bivalve specimen. Note the tumor (arrows) arisingfrom the tunica vaginalis (asterisk) and invading the testicular parenchyma (double asterisks). Figure 3. Histology of malignant mesothelioma of the tunica vaginalis testis in a biphasic pattern (hematoxylin & eosin, 400x). The epithelial (asterisk) and sarcomatous (arrow) types exist concomitantly. A C Figure 4. Immunohistochemistry of malignant mesothelioma of the tunica vaginalis testis: (A) CK(++); (B) EMA(++); (C) vimentin (++). (LSAB, 400x.) However, pathology demonstrated malignant meso thelioma arising from the tunica vaginalis of the testis (Figure 2). Histopathology was characterized by a biphasic pattern with admixed epithelial and spindle cell proliferation (Figure 3). Immunohistochemical staining showed CK (++) (Figure 4A) and EMA (++) (Figure 4B) positivity in epithelial nests, while the spindle cell was positive for vimentin (++) (Figure 4C) and negative for desmin (-). Because of poor wound healing, wide resection was performed to achieve an adequate margin 1 week postoperatively. His postoperative course was smooth and the wound 78 Kaohsiung J Med Sci February 2009 Vol 25 No 2 Malignant mesothelioma ofthe tunica vaginalis testis healed well. No local recurrence has been noticed at regular clinic follow-ups for 7 months. Retrospectively, the patient had short-term occu pational exposure to asbestos 40 years ago. Discussion Since the first case of malignant mesothelioma at the tunica vaginalis of testis was described by Barbera and Rubino in 1957 [1], approximately 100 cases have been reported. Malignant mesothelioma of pleura, peri cardium, and peritoneum are uncommon and malig nant mesothelioma originating in the tunica vaginalis is extremely rare. In the United States the incidence of malignant mesothelioma is about 11 cases per million people per year [2]. About 68-85% of the mesothelio mas arise in the pleura, 9.1-24.1% in the peritoneum, and only 0.3-5% in the tunica vaginalis testis [2-4,8]. In patients with malignant mesothelioma at the tunica vaginalis of the testis, more than two-thirds of the cases were patients older than 45 years of age with a median age of 60 years [4], although malignant mesothelioma has been reported in a 10-year-old child [9]. Patients usually present with hydrocele (56.3%) and sometimes a testicular tumor (32.8%) [4]. Although the diagnosis of malignant mesothelioma of the tunica vaginalis is sel dom observed preoperatively, it should be considered in any patient presenting with scrotal pathology with a history of exposure to asbestos [5-7]. Direct contact with asbestos (34.2-41%) [4,10] or a familial occupa tional history can significantly increase the risk of dev eloping a malignant mesothelioma by a factor of 10 [11-13]. In addition to asbestos, potassium bromate in drinking water was shown to increase the risk of developing malignant mesothelioma of the tunica vagi nalis testis in an animal model [14] and long-standing hydrocele was also considered to be a risk factor of malignant mesothelioma of the tunica vaginalis, as pro posed by Gurdal and Erol [15]. The localization of the tumor is presented with equal incidence in both testi cles [4], and the presence of bilateral malignant meso thelioma in the tunica vaginalis is very rare, with only three cases presented before the present case [16-18]. According to different histopathologic traits, malig nant mesothelioma has been subclassified into three types: epithelial type, the most frequently seen (60.8 75%) in the peritoneal cavity and tunica vaginalis; the biphasic type, as reported in this case, occurring in the serosa membrane (25-37.3%); and the mesenchymal or sarcomatous type, which is found in the pleural cavity (1.9%) [3,9]. Malignant mesothelioma has an expansive and infiltrative growth pattern and nearly 40% of the patients presented initially presented with local invasion, and the two most frequently involved sites were subtunical connective tissue (25.8%) and testicular parenchyma (19.4%). Metastasis occurs early via the lymphatic system to inguinal, para-aortic or supraclavicular nodes, and was reported in 14.9-31% of cases [4,19]. In 11 patients with primary metastatic disease in the study by Plas et al, retroperitoneal lymph nodes were most involved in five cases, followed by inguinal nodes in three and iliac nodes in two. In cases showing disease progression, the common sites of metastasis were lymph nodes (13.8%), lung (9.7%) and liver (4.2%) [4]. In the study by Plas et al, tumor recurrence was more than 60% within 2 years and over 90% within 5 years [4]. Radical primary resection may decrease the rate of tumor recurrence. Univariate analysis to assess the prognostic pa rameters revealed a significantly better correlation in patients of younger age. However, in a multivariate Cox's regression model, there was no statistically sig nificant result [3]. The mean disease-specific survival for patients with or without systemic treatment was 26 and 36 months, respectively [20]. Although some cases were responsive to radiotherapy or chemo therapy [21-24], adjuvant treatments such as chemo therapy or immunotherapy have limited effects in advanced disease [4]. Radiotherapy appeared to be more effective than chemotherapy and as good as the combination of chemotherapy and radiotherapy in the patients with metastatic disease [4]. The DNA hypomethylating agent 5-aza-2'-deoxycytidine may be effective based on the induction and upregulation of the expression of cancer/testis antigen [25]. Malignant mesothelioma of tunica vaginalis is a rare but often fatal malignancy. It should be consid ered a differential diagnosis of inguinoscrotal mass, particularly in patients with exposure to asbestos. Despite aggressive surgical procedures or systematic adjuvant therapies, the prognosis remains poor. References 1. Barbera V, Rubino M. Papillary mesothelioma of the tunica vaginalis. Cancer 1957;10:183-9. Kaohsiung J Med Sci February 2009 Vol 25 No 2 79 J.L. Chen and Y.H. Hsu 2. Antman K, Hassan R, Eisner M, et al. Update on malig nant mesothelioma. Oncology (Williston Park) 2005;19: 1301-9; discussion 9-10, 13-6. 3. Murai Y. Malignant mesothelioma in Japan: analysis of registered autopsy cases. Arch Environ Health 2001;56: 84-8. 4. Plas E, Riedl CR, Pfluger H. Malignant mesothelioma of the tunica vaginalis testis: review of the literature and assessment of prognostic parameters. Cancer 1998;83: 2437-46. 5. Frank AL. Medical and public health approaches to asbestos disease. Mt Sinai JMed 1995;62:401-5. 6. McDonald JC. Health implications of environmental exposure to asbestos. Environ Health Perspect 1985;62: 319-28. 7. Merler E. Mesothelioma incidence decreases parallel to asbestos exposure decrement or interruption: a confir mation of a dose-response relationship, with implica tions in public health. Epidemiol Prev 2007;31:46-52. 8. Serio G, Ceppi M, Fonte A, et al. Malignant mesothe lioma of the testicular tunica vaginalis. Eur Urol 1992; 21:174-6. 9. Antman K, Cohen S, Dimitrov NV, et al. Malignant mesothelioma of the tunica vaginalis testis. J Clin Oncol 1984;2:447-51. 10. Jones MA, Young RH, Scully RE. Malignant mesothe lioma of the tunica vaginalis. A clinicopathologic analysis of 11 cases with review of the literature. Am J Surg Pathol 1995;19:815-25. 11. Vianna NJ, Polan AK. Non-occupational exposure to asbestos and malignant mesothelioma in females. Lancet 1978;1:1061-3. 12. Ascoli V, Cavone D, Merler E, et al. Mesothelioma in blood related subjects: report of 11 clusters among 1954 Italy cases and review of the literature. Am J Ind Med 2007;50:357-69. 13. Huncharek M. Genetic factors in the etiology of malig nant mesothelioma. Eur J Cancer 1995;31A:1741-7. 14. Crosby LM, Morgan KT, Gaskill B, et al. Origin and distribution of potassium bromate-induced testicular and peritoneal mesotheliomas in rats. Toxicol Pathol 2000;28:253-66. 15. Gurdal M, Erol A. Malignant mesothelioma of tunica vaginalis testis associated with long-lasting hydrocele: could hydrocele be an etiological factor? Int Urol Nephrol 2001;32:687-9. 16. McDonald RE, Sago AL, Novicki DE, et al. Paratesticular mesotheliomas. J Urol 1983;130:360-1. 17. Menut P, Herve JM, Barbagelata M, et al. Bilateral malignant mesothelioma of the tunica vaginalis testis. Apropos of a case. Prog Urol 1996;6:587-9. 18. Pelzer A, Akkad T, Herwig R, et al. Synchronous bilat eral malignant mesothelioma of tunica vaginalis testis: early diagnosis. Urology 2004;64:1031. 19. Yamanishi T, Wakisaka M, Ito H, et al. Malignant mesothelioma of the tunica vaginalis testis. Eur Urol 1984;10:207-9. 20. Spiess PE, Tuziak T, Kassouf W, et al. Malignant mesothelioma of the tunica vaginalis. Urology 2005;66: 397-401. 21. Brady LW. Mesothelioma--the role for radiation ther apy. Semin Oncol 1981;8:329-34. 22. Lederman GS, Recht A, Herman T, et al. Long-term survival in peritoneal mesothelioma. The role of radio therapy and combined modality treatment. Cancer 1987;59:1882-6. 23. Lee JD, Perez S, Wang HJ, et al. Intrapleural chemo therapy for patients with incompletely resected malig nant mesothelioma: the UCLA experience. J Surg Oncol 1995;60:262-7. 24. Jaffe J, Roth JA, Carter H. Malignant papillary mesothe lioma of tunica vaginalis testis. Urology 1978;11:647-50. 25. Sigalotti L, Coral S, Altomonte M, et al. Cancer testis antigens expression in mesothelioma: role of DNA methylation and bioimmunotherapeutic implications. Br J Cancer 2002;86:979-82. 80 Kaohsiung J Med Sci February 2009 Vol 25 No 2 rnmn1 2 1 mm 2mm mmm b^ > ^bmbib > MMSU( 2009;25:77-81) : 97 5 B 26 0 m^m : 97 7 B 21 0 :mMMrnrn MamAimwMsrnmmm* 970 707 %mm Kaohsiung J Med Sci February 2009 Vol 25 No 2 81