Bilateral testicular large-cell calcifying Sertoli cell tumor and recurrent cardiac myxoma in a patient with Carney’s complex

Large Cell Calcifying Sertoli Cell Tumor

“…The LCCSCT, one of the rarest sex cord-stromal tumors of the testis, was initially described by Lange et al6 in 1960 and then formally introduced as a distinct entity by Proppe and Scully in 1980 5. To date, 45 clinically benign,1,5,9,12,25,27,29–45 53 clinically ambiguous,1,16,27,36,38,43,46–78 and 17 clinically malignant1–3,5,23–28 cases have been reported in the English language literature, including this series, according to our comprehensive review (Tables 2, 3, Supplementary Table 1, Supplemental Digital Content 1, http://links.lww.com/PAS/B288).…”

“…The LCCSCT, one of the rarest sex cord-stromal tumors of the testis, was initially described by Lange et al 6 in 1960 and then formally introduced as a distinct entity by Proppe and Scully in 1980. 5 To date, 45 clinically benign, 1,5,9,12,25,27,[29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45] FIGURE 1. There are multiple, solid, tan, encapsulated, and abutting nodules on the cut surface of this testis from a patient with the Carney complex.…”

Large-cell calcifying Sertoli cell tumour of the testis: Associated organ anomalies

“…1 Forty-seven cases have been reported in the literature. [3][4][5][6][7] Most of the patients were less than 20 years of age. 2 Twelve (25%) of the 47 reported cases were bilateral.…”

“…2 Twelve (25%) of the 47 reported cases were bilateral. 1,4,8,9 Because of the high incidence of bilateral tumours, the contralateral testicle should be examined carefully and long-term follow up with periodic physical examination and ultrasound are warranted. The LCCSCT may be associated with hormonal abnormalities including gynecomastia, precocious puberty and amenorrhoea and may occur in patients…”