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Int Urol Nephrol. 2012 Aug;44(4):1045-8. doi: 10.1007/s11255-011-0100-1. Epub 2011 Dec 11.

Organ-sparing surgery of the bilateral testicular large cell calcifying sertoli cell tumor in patient with atypical Peutz-Jeghers syndrome.

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  • 1Department of Urology, Medical University of Gdansk, Kliniczna Str. 1a, 80-402 Gdansk, Poland. akaluzny@uck.gda.pl

Abstract

Large cell calcifying sertoli cell tumor (LCCSCT) is an exceptionally rare neoplasm originating from sperm cord cells. The tumors have relatively low malignant potential and unlikely proceed to metastasis formation. The lesions may occur in an isolated form or in ca. 40% of cases may be associated with genetic abnormalities, by and large Peutz-Jeghers syndrome and Carney complex. At presentation, 20% of LCCSCT cases are bilateral and/or multifocal. Owning to characteristic skin lesions and particular hyperechoic ultrasound image of the tumor, preliminary diagnosis of the syndromic LCCSCT is possible in the preoperative period. Consequently, testicle organ-sparing procedure can be attempted, which is especially justified in bilateral lesions. Here, we report a case of a bilateral LCCSCT in a 20-year-old man with atypical Peutz-Jeghers syndrome due to amplification of the exon 1 of STK11 gene who was successfully treated with bilateral testicle-sparing tumorectomies.

PMID:
22160729
PMCID:
PMC3401493
DOI:
10.1007/s11255-011-0100-1
[PubMed - indexed for MEDLINE]
Free PMC Article
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