Carcinoma corticoadrenal, reporte de caso

Abstract

El carcinoma corticoadrenal es una neoplasia rara, altamente agresiva, de distribución bimodal, con predominio en el sexo femenino, de la cual el 20% de los casos se diagnostican de manera incidental. Se presenta el caso de un varón de 43 años con dolor de tipo cólico en el flanco izquierdo, pérdida de peso y fiebre intermitente. La tomografía computarizada mostró un tumor en la glándula suprarrenal izquierda y metástasis hepática. Se realizó adrenalectomía radical en bloque, con reporte anatomopatológico de carcinoma corticoadrenal, Weiss de 7 puntos y Ki67 40%. El carcinoma corticoadrenal es una neoplasia agresiva y de presentación clínica variable. La terapia sistémica es importante incluso en pacientes con enfermedad localizada e independientemente de la cirugía.

Adrenocortical carcinoma with sarcomatoid pattern. Importance of differential diagnoses

“…Most of the etiology of adrenocortical carcinomas are usually sporadic, but they have also been associated with some hereditary disorders such as: Li-Fraumeni Syndrome, Beck with-Wiedemann syndrome, Lynch syndrome, multipleendocrine neoplasia of type 1 and familial adenomatous polvposis. 2 In the case of Li-Fraumeni Syndrome with a TP53 (17p13) mutation associated with breast cancer, sarcomas, leukemia and adrenal carcinomaswhere 16-70% of adrenal carcinomas have a somatic mutation in TP53; In Gardner Syndrome with an APCmutation (5q21) it is associated with colon cancer, desmoid tumor, osteomas, thyroid carcinoma, adrenalcarcinoma (7-13% of patients), and in 33% of adrenal carcinomas they have a activation of Wnt/B-cateninwhere it is usually an area of frequent mutation; and Beckwith-Wiedemann Syndrome with a CDKNIC/ IGF-IImutation associated with Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma and adrenalcarcinoma where 60-90% of adrenal carcinomas have overexpression of IGF-2(0pin Oncology 16(1):p 8-12, Jan 2004). The clinical presentation of Li-Fraumeni Syndrome is 33% related to the hormonal excess of its symptoms in which the following pathologies are involved: 45% Cushing's syndrome, 30% Cushing's syndrome + virilization, 10% virilization, 10% Conn Syndrome and < 5% feminization.…”