Prepubertal gynecomastia due to testis tumors is a very rare condition. Nearly 5% of the patients with testicular mass present with gynecomastia. Sertoli cell tumors are sporadic in 60% of the reported cases, while the remaining is a component of multiple neoplasia syndromes such as Peutz-Jeghers syndrome and Carney complex. We present a 4-year-old boy with gynecomastia due to Sertoli cell tumo...

Myxomas are mostly seen in the 2(th) and 3(rd) decades. They rarely occur in childhood and maxillofacial region is rarely involved. The recurrence incidence is high. We report this unusual case occurring in a 9-year-old girl in the maxillofacial region and recurrence four months after initial treatment.

In 1981, study of the adrenal pathology in four cases of Cushing syndrome led to characterization of a unique disorder termed primary pigmented nodular adrenal disease (PPNAD). Review of the literature showed that the condition occurred in two families. In one, it had affected two siblings; a third sibling who did not have Cushing syndrome died of cardiac myxoma. To test the hypothesis that the...

We present a case of bilateral foot multiple cutaneous myxomas, associated with Favre-Racouchot syndrome (FRS) which is a dermatologic condition of multiple large comedones and nodules of the periorbital areas. As the mucocutaneous and cardiac manifestations were absent, Carney complex was excluded. To our knowledge, there has been no report of the association of cutaneous myxoma and FRS. Addit...

We report a case of a female patient who was operated at the third relapse of an atrial myxoma caused by Carney complex. The difficult operation was performed without any complications despite extensive adhesions caused by the previous operations. The further inpatient course went without complications and the patient was discharged to the consecutive treatment on the 9th postoperative day. The...

OBJECTIVE Primary pigmented nodular adrenocortical disease (PPNAD) is the main endocrine manifestation of Carney complex, a multiple neoplasia syndrome caused by PRKAR1A gene mutations. The presence of PRKAR1A loss of heterozygosity (LOH) in adrenocortical tumorigenesis remains controversial. The aim of the present study is to investigate the presence of PRKAR1A LOH in adrenocortical cells in a...

Myxoma cordis is a rare disorder. Up to 1951, 128 cases were known in the literature (Mahaim, Prichart) . It has long been a subject of controversy whether these formations were to be regarded as organized thrombi or as tumours, but now they are considered as tumours. The myxomas are the most frequently occurring primary tumours of the heart, viz., about 50 per cent. In 50 per cent of cases the...