CASE OF THE WEEK
2021-01 / January 04
Contributors: Anu Abraham, Varsha Manucha
A man in his 50s presented with constipation and urinary frequency. CT scan identified a 20 cm abdominal mass. A biopsy of the mass was performed. He underwent orchiectomy two years back for a 11 cm right testicular mass. Biopsy from the abdominal mass is shown in figures 1 and 2. Figures 3-5 show the tumor in the testis that was retrospectively reviewed.
Quiz
1. What is the correct diagnosis?
a. Seminoma with atypia (formerly anaplastic seminoma)
b. Metastatic Leydig cell tumor
c. Sertoli cell tumor
d. Granulosa cell tumor
e. Embryonal carcinoma
1. b
1. Metastatic Leydig cell tumor
We present a rare case of metastatic Leydig cell tumor. Microscopically the tumor from the abdominal mass was comprised of moderately pleomorphic epithelioid cells with eosinophilic to vacuolated cytoplasm in a nested and trabecular/insular pattern, surrounded by thin vascular channels. The cells had enlarged nuclei with prominent nucleoli. Increased apoptosis was seen, however, no mitotic activity was noted in the limited biopsy specimen. By immunohistochemistry, the tumor was positive for Inhibin and Melan A. Tumor was negative for PLAP, AE1/AE3, C-KIT, OCT-4, SOX-10, HMB-45, and Calretinin. The differentials at this point included adrenocortical tumor and metastatic sex cord-stromal tumor. So, the primary testicular tumor was retrieved and reviewed. The testicular tumor showed large nodules of tumor cells, some of which were necrotic. The nodules showed nested and trabecular pattern of tumor cells with abundant finely vacuolated cytoplasm and large nuclei with prominent nucleoli. The nests were surrounded by delicate fibrovascular septa. The testicular tumor also showed lymphovascular and extratesticular/spermatic cord invasion, increased apoptosis, increased mitotic activity (8/10hpf), significant nuclear atypia, and the presence of necrosis. The primary tumor was positive for inhibin, Melan-A, vimentin, CD99 (weak, membranous), focally positive for keratin, and CD117 (nuclear). The tumor was negative for S100, SOX10, D2-40, PLAP, PAX8, and CD30. Reinke crystals were not seen in both the tumors.
Metastatic Leydig cell tumors are very rare, with few reported cases in the literature. The majority of Leydig cell tumors are benign, but approximately 10 % of cases are malignant and can metastasize. In 1985, Kim I et al analyzed 40 cases of Leydig cell tumors and found that there is no single histologic criterion to define malignant Leydig cell tumors. Malignant tumors are usually larger than benign tumors (>5cm) and show nuclear atypia, increased mitotic figures, infiltrative margin, angiolymphatic invasion, necrosis, DNA aneuploidy, and increased Ki67 proliferative activity. Metastasis is therefore generally regarded as the only reliable criterion of malignancy.
Radical orchiectomy is the treatment of choice for all solid testicular masses. A retroperitoneal lymph node dissection is recommended if the tumor is malignant. Survival from the initial diagnosis of malignant Leydig cell tumor ranges from 2 months to 17 years (median 2 years).
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; 9:177-92.
2. Bertram KA, Bratloff B, Hodges GF, Davidson H. Treatment of malignant Leydig cell tumor. Cancer. 1991; 68:2324-9.
3. Cheville JC, Sebo TJ, Lager DJ, Bostwick DG, Farrow GM. Leydig cell tumor of the testis: a clinicopathologic, DNA content, and MIB-1 comparison of non metastasizing and metastasizing tumors. Am J Surg Pathol, 1998; 22:1361-7.
Anu Abraham, University of Mississippi Medical Center, aabraham@umc.edu
Varsha Manucha, University of Mississippi Medical Center, vmanucha@umc.edu (@VarshaManucha MD)
Testis
Testis; Leydig cell tumor; Metastatic