Síndrome de Cushing secundario a carcinoma de la glándula suprarrenal izquierda con metástasis hepáticas y trombosis tumoral masiva de la vena cava / Cushing´s syndrome due to a left adrenal cortical carcinoma with metastasis to the liver and a massive vena cava tumor thrombus

Se presenta el caso de una mujer de 47 años con cuadro clínico de siete meses de evolución caracterizado por aumento progresivo de peso, hipertensión arterial y diabetes mellitus de reciente aparición, hirsutismo facial y en tórax, alopecia frontal, alteraciones en la menstruación e hipopotasiemia. Se consideró el diagnóstico de síndrome de Cushing, por lo cual se iniciaron estudios e extensión para establecer su etiología. Durante su hospitalización presentó una evolución tórpida y falleció. En la autopsia clínica se encontró un carcinoma de la glándula suprarrenal izquierda, de 400 g, aproximadamente, con metástasis a hígado y trombosis masiva de la vena cava, lo que finalmente produjo su muerte.

A 47-year-old woman with a seven-month history of increasing weight, hypertension and recently diagnosed diabetes presented features of hirsutism, frontal baldness, amenorrhea and hypokalemia. These characteristics were considered diagnostic of Cushing´s syndrome, and studies were initialized to identify its etiology. During hospitalization, the patient presented a torpid evolution resulting in death. Clinical autopsy revealed a 400 g carcinoma in the left adrenal gland, liver metastasis and a massive vena cava tumor thrombus which was the final cause of death.

Sweet's syndrome preceding carcinoma of the adrenal cortex.

A case of Sweet's syndrome preceding carcinoma of the adrenal cortex is reported. Sweet's syndrome can be a rare premalignant manifestation of carcinoma of adrenal cortex.

Amplification of 9q34 in childhood adrenocortical tumors: a specific feature unrelated to ethnic origin or living conditions

Adrenocortical tumors (ACT) in children under 15 years of age exhibit some clinical and biological features distinct from ACT in adults. Cell proliferation, hypertrophy and cell death in adrenal cortex during the last months of gestation and the immediate postnatal period seem to be critical for the origin of ACT in children. Studies with large numbers of patients with childhood ACT have indicated a median age at diagnosis of about 4 years. In our institution, the median age was 3 years and 5 months, while the median age for first signs and symptoms was 2 years and 5 months (N = 72). Using the comparative genomic hybridization technique, we have reported a high frequency of 9q34 amplification in adenomas and carcinomas. This finding has been confirmed more recently by investigators in England. The lower socioeconomic status, the distinctive ethnic groups and all the regional differences in Southern Brazil in relation to patients in England indicate that these differences are not important to determine 9q34 amplification. Candidate amplified genes mapped to this locus are currently being investigated and Southern blot results obtained so far have discarded amplification of the abl oncogene. Amplification of 9q34 has not been found to be related to tumor size, staging, or malignant histopathological features, nor does it seem to be responsible for the higher incidence of ACT observed in Southern Brazil, but could be related to an ACT from embryonic origin.