title: Organ-sparing surgery of the bilateral testicular large cell calcifying sertoli cell tumor in patient with atypical Peutz-Jeghers syndrome.
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.
authors.affiliation: Department of Urology, Medical University of Gdansk, Kliniczna Str. 1a, 80-402 Gdansk, Poland. akaluzny@uck.gda.pl
authors.forename: A
authors.initials: A
authors.lastname: Kaluzny
authors.forename: M
authors.initials: M
authors.lastname: Matuszewski
authors.forename: S
authors.initials: S
authors.lastname: Wojtylak
authors.forename: K
authors.initials: K
authors.lastname: Krajka
authors.forename: W
authors.initials: W
authors.lastname: Cichy
authors.forename: A
authors.initials: A
authors.lastname: Plawski
authors.forename: A
authors.initials: A
authors.lastname: Gintowt
authors.forename: B S
authors.initials: BS
authors.lastname: Lipska
mesh_terms: D000091162:AMP-Activated Protein Kinase Kinases; D002114:Calcinosis / Q000150:complications / Q000473:pathology / Q000601:surgery*; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D021141:Nucleic Acid Amplification Techniques; D009919:Orchiectomy / Q000379:methods*; D010580:Peutz-Jeghers Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D017346:Protein Serine-Threonine Kinases / Q000235:genetics; D012707:Sertoli Cell Tumor / Q000150:complications / Q000473:pathology / Q000601:surgery*; D013736:Testicular Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D055815:Young Adult
chemical_list: D017346:Protein Serine-Threonine Kinases; C109790:STK11 protein, human; D000091162:AMP-Activated Protein Kinase Kinases