Bilateral testicular large-cell calcifying sertoli cell tumor and recurrent cardiac myxoma in a patient with Carney's complex.
Large-cell calcifying Sertoli cell tumors are exceedingly rare testicular tumors which can be distinguished from pure Sertoli cell tumors by distinctive microscopic features and multifocal and bilateral calcifications [1]. We describe the characteristic sonographic appearance of the tumor with pathologic correlation. This tumor also constitutes one of the conditions of Carney's complex [2], which includes cardiac and skin myxomas, myxoid mammary fibroadenomas, spotty skin pigmentation, primary pigmented nodular adrenocortical disease, pituitary adenoma and unusual testicular tumors, especially large-cell calcifying Sertoli cell tumor. We report the case of a 10-year-old boy with four of the above conditions. Recognition of this complex is important as cardiac myxomas can lead to lethal complications. Because of a tendency for familial occurrence, family members should be screened carefully [3].
Noszian
I M
IM
Department of Radiology II, Hospital of the Sisters of Mercy, Grieskirchnerstrasse 42, A-4600 Wels, Austria.
Balon
R
R
Eitelberger
F G
FG
Schmid
N
N
D002114:Calcinosis / Q000473:pathology; D002648:Child; D006338:Heart Neoplasms / Q000473:pathology*; D006801:Humans; D008297:Male; D009232:Myxoma / Q000473:pathology*; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D012707:Sertoli Cell Tumor / Q000000981:diagnostic imaging / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology; D013577:Syndrome; D013736:Testicular Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology*; D013737:Testis / Q000473:pathology; D014261:Tricuspid Valve / Q000473:pathology; D014463:Ultrasonography