Case report: Large-cell calcifying Sertoli cell tumor

Case history

21-year-old male with a painless mass in the right testicle and a prior history of cutaneous lesions.

Histologic findings

Figures show a proliferation composed of large, polygonal cells with ample, eosinophilic cytoplasm, round nuclei, evident nucleoli, with a trabecular, cord-like pattern of growth, associated with foci of dystrophic calcification and psammomatous bodies, within a myxoid loose stroma.

Discussion

Large-cell calcifying Sertoli cell tumor is an unusual morphological variant of Sertoli cell tumors composed of large polygonal Sertoli cells associated with calcification. Around one third of all cases appear in patients with the Carney or, less frequently, with the Peutz-Jeghers syndrome.

Clinically, it presents as a small (<2 cm), painless testicular mass, which can be bilateral and multifocal in about 40% of the cases, especially in syndromic settings. In non-sporadic cases extratesticular manifestations are related to the associated syndrome, such as myxomas of the skin, soft-tissue, and heart and myxoid lesions of the breast, facial lentigines, cutaneous blue nevi, secondary Cushing syndrome due to pigmented adrenocortical nodular hyperplasia, pituitary somatotroph adenomas, and psammomatous melanotic schwannomas.

Microscopically, solid, tubular, and cord-like growth patterns can be observed. Tumor cells are large polygonal with abundant eosinophilic, finely granular cytoplasm, vesicular nuclei with prominent nucleoli, within a myxohyaline stroma. Distinct calcifications characterized by large, laminated calcified nodule, psammoma bodies and focal ossification, and neutrophilic infiltration, can be also found.

The presence of large tumor size (>4 cm), overt nuclear atypias, increased mitotic activity (>3 mitosis/10 HPF), necrosis, extratesticular extension, and vascular invasion are associated with clinical aggressive behavior. Malignant tumors appear in patients older than 25 years old and are usually not associated with syndromes.

Tumor cells are positive for vimentin, S-100, EMA, melan-A, desmin, and inhibin, and focally positive for cytokeratins. The main differential diagnoses of large-cell calcifying Sertoli cell tumor include Sertoli cell and Leydig cell tumors.

Most of the cases behave indolently, and surgical excision is the treatment of choice.