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Presented by Dr. Epstein and prepared by J. Stephen Nix
A 44 year old male underwent an orchiectomy for a 5 cm testicular mass
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1. Question
A 44 year old male underwent an orchiectomy for a 5 cm testicular mass
Correct
Answer: D
Histology:
The mass is composed of cords and nests of cells with abundant eosinophilic cytoplasm. Nuclei are enlarged, hyperchromatic, with variable shapes and prominent nucleoli. Mitotic figures are easily identifiable and necrosis is present. Scattered throughout out the tumor are small foci of irregular calcification.
Discussion:
The findings in this case are classic for a large cell calcifying Sertoli cell tumor. Although the tumor is composed of cells that resemble Leydig cells, it is classified as a Sertoli cell tumor since these lesions can express keratin and can have an intratubular component – as Leydig cells reside between seminiferous tubules a Leydig cell tumor would not be expected to be intratubular. The finding of irregular deposits of calcification is unique within the testis for large cell calcifying Sertoli cell tumor. The same criteria for malignancy in these tumors are those for other sex-cord stromal tumors based on size, mitotic figures, necrosis, atypia, and widespread infiltration. Approximately 10% of large cell calcifying Sertoli cell tumors are malignant. Although the majority of large cell calcifying Sertoli cell tumors are sporadic some are associated with the Carney complex which is an autosomal dominant syndrome which has in addition pituitary adenomas, myxomas, pigmented nodular adrenocortical hyperplasia, and skin pigmentation. The best immunohistochemical marker for sex cord stromal tumors in general is SF-1 and if not available inhibin.
Incorrect
Answer: D
Histology:
The mass is composed of cords and nests of cells with abundant eosinophilic cytoplasm. Nuclei are enlarged, hyperchromatic, with variable shapes and prominent nucleoli. Mitotic figures are easily identifiable and necrosis is present. Scattered throughout out the tumor are small foci of irregular calcification.
Discussion:
The findings in this case are classic for a large cell calcifying Sertoli cell tumor. Although the tumor is composed of cells that resemble Leydig cells, it is classified as a Sertoli cell tumor since these lesions can express keratin and can have an intratubular component – as Leydig cells reside between seminiferous tubules a Leydig cell tumor would not be expected to be intratubular. The finding of irregular deposits of calcification is unique within the testis for large cell calcifying Sertoli cell tumor. The same criteria for malignancy in these tumors are those for other sex-cord stromal tumors based on size, mitotic figures, necrosis, atypia, and widespread infiltration. Approximately 10% of large cell calcifying Sertoli cell tumors are malignant. Although the majority of large cell calcifying Sertoli cell tumors are sporadic some are associated with the Carney complex which is an autosomal dominant syndrome which has in addition pituitary adenomas, myxomas, pigmented nodular adrenocortical hyperplasia, and skin pigmentation. The best immunohistochemical marker for sex cord stromal tumors in general is SF-1 and if not available inhibin.
