Concomitant Sertoli and Leydig Cell Tumor of the Testis: A Case Report

8_RIU0495_09-30.qxd 9/29/11 7:55 PM Page 173 CASE REVIEW Concomitant Sertoli and Leydig Cell Tumor of the Testis: A Case Report Mohammed Fadl Tazi, MD,1 Youness Ahallal, MD,1 Abdelhak Khallouk, MD,1 Hinde Elfatemi,2 Mohcine Bendahou,2 Elmehdi Tazi,3 Mohammed Jamal El Fassi, PhD,1 Moulay Hassan Farih, MD1 1 Department of Urology, Hassan II Teaching Hospital, Fes, Morocco; 2Department of Pathology, Hassan II Teaching Hospital, Fes, Morocco; 3Department of Medical Oncology, National Institute of Oncology, Rabat, Morocco A rare intratubular gonadal stromal tumor was present in the testis of a 45-year-old man who was admitted to our hospital with the chief complaint of gradual enlargement of the left testis. Tumoral markers were negative and no extension was observed. The tumor comprised an intratubular mixture of two types of tumor cells with intercellular junctions: the predominant tumor cells were consistent with a Sertoli cell origin and cells comprising the minor population consistent with a Leydig cell origin. The patient is disease free after 6-month follow-up. The case is considered to be a testicular mixed tubular Sertoli-Leydig cell tumor. It highlights a rare type of primary tumor of the testis that features a good prognosis. [Rev Urol. 2011;13(3):173-175 doi:10.3909/riu0495] © 2011 MedReviews®, LLC Keywords: Testis cancer • Leydig cell tumor • Sertoli cell tumor eydig cell tumors of the testis account for 1% to 3% of testicular neoplasms.1,2 They occur at all ages, but are most common between age 20 and 50 years. Sertoli cell tumors account for less than 1% of testicular neoplasms and occur at all ages. To our knowledge, the simultaneous coexistence of both components has never been reported in the English literature. L VOL. 13 NO. 3 2011 REVIEWS IN UROLOGY 173 8_RIU0495_09-30.qxd 9/29/11 7:55 PM Page 174 Concomitant Sertoli and Leydig Cell Tumor of the Testis continued Case Report A 45-year-old Moroccan man presented with a 10-month history of painless left testicular enlargement. A detailed clinical history and thorough examination revealed no abnormality. The patient considered himself in good health except for the enormous mass in the left hemiscrotum. He had no gynecomastia. His bone structure and muscular development were decidedly masculine. Examination of the chest and abdomen was normal except for apparent obesity. The pubic hair presented a typical male distribution. The penis was of normal size. The left testis was stony hard and of an irregular contour. Tumor markers (-fetoprotein, human chorionic gonadotropin, and lactate dehydrogenase) were negative. Ultrasonography revealed a 15 cm  8 cm nonhomogeneous testicular mass with multiple hypoechoic nodules. Metastases were not evident in the staging investigations. He was advised surgery. High inguinal orchiectomy was performed and the specimen submitted for histopathological examination. The tumor was 14 cm  7 cm in size and a cut section revealed a brown tumor replacing the testicular tissue, but not appearing to extend beyond the capsule (Figure 1). Microscopic examination revealed a nodular cell proliferation including both Leydig cell tumor component (showing large polygonal cells with abundant eosinophilic cytoplasm, the nuclei are prominent with atypia) and Sertoli cell tumor component (showing cords and nests of cells in a fibrous and edematous stroma) (Figures 2, 3 and 4). The patient was disease free at 6-month follow-up. Discussion Leydig cell tumors exhibit a bimodal peak of incidence in preadolescent boys and older men (age  50 years). About 3% are bilateral and 15% extend beyond the testis at the time of presentation. This tumor has a capacity for estrogenic hypersecretion and gynecomastia is seen in 30% of cases.3 Around 41.7% of Leydig cell tumors Figure 2. Nodular cell proliferation (hematoxylin and eosin stain; original magnification  5). Figure 3. Leydig cell tumor. Large polygonal cells with abundant eosinophilic cytoplasm; the nuclei are prominent with atypia (hematoxylin and eosin stain; original magnification  10). Figure 1. A cut section of the testis revealing a brown tumor replacing the testicular tissue. Figure 4. Sertoli cell tumor. Cords and nests of cells in a fibrous and edematous stroma (hematoxylin and eosin stain; original magnification  20). 174 VOL. 13 NO. 3 2011 REVIEWS IN UROLOGY 8_RIU0495_09-30.qxd 9/29/11 7:55 PM Page 175 Concomitant Sertoli and Leydig Cell Tumor of the Testis in adults are diagnosed incidentally on ultrasonography, 29.2% present with a palpable testicular mass, 16.6% with scrotal pain, and 12.5% with gynecomastia.4 Sertoli cell tumors represent only about 1% of testicular neoplasms5,6 and are, almost by definition, sex cord tumors that form defined tubular structures.5,7 To our knowledge, a testicular tumor including both components has not been reported in the literature. Ultrasonographic findings vary, and a hypoechoic nodule with a nonhomogeneous echoic pattern is the most prevalent feature.4 Contrast-enhanced magnetic resonance imaging seems to be superior to ultrasonography.8 As in our case, the neoplastic Sertoli cells have large pleomorphic nuclei and clear cytoplasm with many tubulovesicular cristae and free ribosomes, whereas the neoplastic Leydig cells show relatively small pleomorphic nuclei, dark cytoplasm with rich smooth endoplasmic reticulum, numerous mitochondria, and lipid droplets. Leydig cell tumor is usually benign and only about one-tenth show malignant behavior in the form of metastatic disease, particularly to the lymph node, lung, and liver. Malignant tumors occur exclusively in adults and are unaccompanied by endocrine changes. 2. 3. 4. 5. 6. 7. References 1. Kim I, Young RH, Scully RE. Leydig cell tumors of the testis. A clinicopathological analysis of 40 cases and review of the literature. Am J Surg Pathol. 1985;9:177-192. 8. Cheville JC, Sebo TJ, Lager DJ, et al. Leydig cell tumor of the testis: a clinicopathologic, DNA content and MIB-1 comparison of nonmetastasizing and metastasizing tumors. Am J Surg Pathol. 1998;22:1361-1367. Tichoo SK, Tamboli P, Warner NE, Amin MB. Testicular and paratesticular tumors. In: Weidner N, Cote RJ, Suster S, Weiss LM, eds. Modern Surgical Pathology. Philadelphia: Saunders; 2003:1215-1256. Carmignani L, Salvioni R, Gadda F, et al. Longterm followup and clinical characteristics of testicular Leydig cell tumor: experience with 24 cases. J Urol. 2006;176:2040-2043; discussion 2043. Young RH, Scully RE. Testicular Tumors. Chicago: ASCP Press; 1990. Ulbright TM, Amin MB, Young RH. Tumors of the testis, adnexa, spermatic cord, and scrotum. In: Rosai J, ed. Atlas of Tumor Pathology. Washington, DC: Armed Forces Institute of Pathology; 1999. Young RH, Koelliker DD, Scully RE. Sertoli cell tumors of the testis, not otherwise specified: a clinicopathologic analysis of 60 cases. Am J Surg Pathol. 1998;22:709-721. Fernández GC, Tardáguila F, Rivas C, et al. Case report: MRI in the diagnosis of testicular Leydig cell tumour. Br J Radiol. 2004;77:521-524. Main Points • Leydig cell tumors of the testis account for 1% to 3% of testicular neoplasms; Sertoli cell tumors account for less than 1% of testicular neoplasms. • Ultrasonographic findings vary, and a hypoechoic nodule with a nonhomogeneous echoic pattern is the most prevalent feature. Contrast-enhanced magnetic resonance imaging seems to be superior to ultrasonography. • Neoplastic Sertoli cells have large pleomorphic nuclei and clear cytoplasm with many tubulovesicular cristae and free ribosomes, whereas the neoplastic Leydig cells show relatively small pleomorphic nuclei, dark cytoplasm with rich smooth endoplasmic reticulum, numerous mitochondria, and lipid droplets. • Leydig cell tumor is usually benign and only about one-tenth show malignant behavior in the form of metastatic disease, particularly to the lymph node, lung, and liver. VOL. 13 NO. 3 2011 REVIEWS IN UROLOGY 175