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large cell calcifying Sertoli cell tumor

Monday 25 January 2010

Definition: Tumor originating from Sertoli cells are a rare subset of sex cord-stromal tumors comprising not more than 1% of all testicular tumors.

Images

- large cell calcifying Sertoli cell tumour

Large cell calcifying Sertoli cell tumor (LCCSCT) is a distinct subtype of Sertoli cell tumors occurring almost invariably in the testis.

LCCSCT may be multicentric or bilateral and, as such, are frequently seen in association with Carney complex or Peutz-Jeghers syndrome.

Malignant behavior of LCCSCT is exceptionally rare.

Synopsis

- Variant occurring in childhood

  • average age 16 years
  • sporadic and associated with Carney and Peutz–Jeghers syndromes

- 20% malignant
- 40% bilateral
- Nests / cords of polygonal cells with eosinophilic cytoplasm
- Myxoid / hyaline stroma
- Foci of calcification
- Note: calcification may be absent even though called LCCST.

See also

- testicular sex cord-stromal tumors

References

- Large cell calcifying Sertoli cell tumor: a clinicopathologic study of 1 malignant and 3 benign tumors using histomorphology, immunohistochemistry, ultrastructure, comparative genomic hybridization, and polymerase chain reaction analysis of the PRKAR1A gene. Petersson F, Bulimbasic S, Sima R, Michal M, Hora M, Malagon HD, Matoska J, Hes O. Hum Pathol. PMID: 20004940