Large laminated calcifications are seen scattered throughout the stroma.
Calcifications are seen against a densely collagenous stroma.
The neoplastic cells are usually round, but can be cuboidal or spindled. Cord-like growth is evident.
A closer view of the neoplastic cells reveals stippled chromatin and small nucleoli. Small nests or tubules are trying to form.
Large cell calcifying Sertoli Cell Tumor (LCCSCT) is a rare variant of Sertoli cell tumor with only 50-60 cases have been reported in the literature. 40% of cases occur as part of Carney complex (cardiac mxyomas, skin hyperpigmentation and endocrine over-reactivity including pituitary adenoma and nodular hyperplasia of adrenal cortex) or Peutz-Jeghers syndrome (hamartomatous polyps in GI tract and pigmented macules on lips and oral mucosa)(Fletcher, Zhou).
Grossly, a small (less than 4 cm) firm yellow-tan well-circumscribed nodule is seen. Microscopically, neoplastic cells with abundant eosinophilic cytoplasm are seen in sheets, nests, clusters or cords, separated by fibrous or myxoid stroma. 50% of cases grow within the seminiferous tubules (intratubular growth). In keeping with its name, large laminated calcifications are seen scattered throughout the tumor.
Although the neoplastic cells resemble Leydig tumor cells in many instances, ultrastructural studies demonstrate features of Sertoli cells. Furthermore, the large calcifications are not seen in Leydig cell tumors.
Patients are generally in their 20s and present with a testicular mass, which is bilateral in 40% and unilateral in 60% of cases. Usually unicentric tumors, but when there is multicentricity, it may be associated with Carney complex or Peutz-Jegher syndrome.
Only about 10 reported cases have been malignant, with necrosis and extension beyond the testis. Malignant cases occur in older patients (mean age of 39 years), compared to benign tumors, which occur in an average age of 17 (Zhou).
Fletcher CDM, ed. Diagnostic Histopathology of Tumors. 3rd Ed. Philadelphia, PA: Elsevier; 2007: 836.
Zhou M, Magi-Galluzzi, C. Genitourinary Pathology: Foundations in Diagnostic Pathology. Philadelphia, PA: Elvesier; 2006: 587-9.