Calcifying Sertoli Cell Tumor Research Articles

Resection of Multiple Recurrent Cardiac Myxomas in an Adult Man With Carney's Complex

Cardiac myxomas have many manifestations, including an association with endocrine overreactivity, unusual testicular tumors, and manifestations in Carney's complex [1McCarthy P.M. Piehler J.M. Schaff H.V. et al.The significance of multiple, recurrent, and “complex” cardiac myxomas.J Thorac Cardiovasc Surg. 1986; 91: 389-396PubMed Google Scholar]. We recently treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years earlier.Echocardiography revealed multiple intracardiac masses in the left atrium (Fig 1A , arrow), right atrium, and right ventricle (Fig 1B, arrow). An ultrasound examination of the scrotum showed multiple bilateral testicular calcifications (Figs 2A, 2B), thought to be calcifying Sertoli cell tumors. A computed tomography scan of the abdomen showed no abnormalities, and the 24-hour urine cortisol level was within the normal reference range. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene.Fig 2View Large Image Figure ViewerDownload (PPT)The patient underwent excision of 4 cardiac myxomas (left atrium, right atrium, and right ventricle; Fig 3) using cardiopulmonary bypass and standard aortic and bicaval cannulation. His postoperative course was unremarkable, and he was dismissed on the fourth postoperative day.Fig 3View Large Image Figure ViewerDownload (PPT) Cardiac myxomas have many manifestations, including an association with endocrine overreactivity, unusual testicular tumors, and manifestations in Carney's complex [1McCarthy P.M. Piehler J.M. Schaff H.V. et al.The significance of multiple, recurrent, and “complex” cardiac myxomas.J Thorac Cardiovasc Surg. 1986; 91: 389-396PubMed Google Scholar]. We recently treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years earlier. Echocardiography revealed multiple intracardiac masses in the left atrium (Fig 1A , arrow), right atrium, and right ventricle (Fig 1B, arrow). An ultrasound examination of the scrotum showed multiple bilateral testicular calcifications (Figs 2A, 2B), thought to be calcifying Sertoli cell tumors. A computed tomography scan of the abdomen showed no abnormalities, and the 24-hour urine cortisol level was within the normal reference range. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene. The patient underwent excision of 4 cardiac myxomas (left atrium, right atrium, and right ventricle; Fig 3) using cardiopulmonary bypass and standard aortic and bicaval cannulation. His postoperative course was unremarkable, and he was dismissed on the fourth postoperative day.

165 TESTICULAR TUMORS WITH PEUTZ-JEGHERS SYNDROME (PJS)

Females with PJS (mucocutaneous pigmentation and intestinal polyposis) have an increased risk of ovarian tumors, with 5% developing a pathologically distinct neoplasm described as a sex cord tumor with annular tubules. We evaluated a 6 yr old male with PJS for bilateral gynecomastia. Testes both measured 3 ml and were normal to palpation. Although two initial estradiol levels were less than 10 pg/ml, a subsequent value was moderately elevated at 24 pg/ml. T4, FSH, LH, T, prolactin and DHAS were all within the normal range for his age. Bone age was slightly advanced at 8.2 yr. Abdominal and pelvic ultrasound, including both adrenals, was normal. Ultrasonography of the testes, however, demonstrated multiple echogenic areas in both testes. Open biopsy revealed multiple minute foci of large-cell, calcifying Sertoli cell tumors. Since the lesions did not appear to be malignant by histology, and were bilateral, the testes were not removed. Subsequently, there has been no progression of the gynecomastia, and serum estradiol has remained <20 pg/ml. Although similar tumors have been reported in two other prepubertal males with PJS, this case is unique in that the tumors were 1) bilateral and multifocal, 2) nonpalpable, and 3) detected by ultrasound. Taken together, these reports strongly suggest that males, as well as females, with PJS are at risk for gonadal tumors and, as such, all patients with PJS should be closely followed for the potential development of gonadal tumors.