ABSTRACT
Los síndromes de neoplasia endocrina múltiple (MEN), incluyen una serie de enfermedades con alteraciones genéticas que se caracterizan por la presencia de tumores que afectan a dos o más glándulas endocrinas. Son síndromes con una herencia autosómica dominante e incluyen tres patrones: MEN 1 (síndrome de Wermer), MEN 2 (que incluye MEN 2A o síndrome de Sipple y MEN 2B o síndrome de Wagenmann-Froboese) y MEN 4. Los adenomas paratiroideos y el carcinoma medular tiroideo, son los tumores más frecuentes del MEN tipo 1 y 2 respectivamente. Esos síndromes son más comunes en pacientes jóvenes, con patología de afectación bilateral, múltiple o multifocal y, sobre todo, en pacientes con antecedentes familiares. Es necesario el trabajo en equipo de endocrinólogos, cirujanos, oncólogos y radiólogos para optimizar el tratamiento de esos pacientes.
Multiple endocrine neoplasia (MEN) encompasses a serial of familial genetically disorders in wich tumors simultaneusly occur in two or more endocrine organs. MEN síndromes are autosomal-dominant disorders categorized into three main patterns: MEN 1 (Wermer syndrome), MEN 2 (includes MEN 2A o Sipple syndrome and MEN 2B o Wagenmann-Froboese syndrome) and MEN 4. Parathyroid adenomas and medullary thyroid carcinoma are the most frecuent tumors in MEN 1 and MEN 2 respectively. These entities will be suspected in younger patients, bilateral, multiple or multifocal disease and, specially, in patients with family background. Cooperation between endocrinologist, surgeons, oncologists and radiologists is pivotal for optimizing patient treatment.
Subject(s)
Humans , Multiple Endocrine Neoplasia/diagnostic imaging , Multiple Endocrine Neoplasia Type 2b/diagnostic imaging , Multiple Endocrine Neoplasia Type 2a/diagnostic imaging , Multiple Endocrine Neoplasia Type 1/diagnostic imaging , Pancreatic Neoplasms/diagnostic imaging , Parathyroid Neoplasms/diagnostic imaging , Pituitary Diseases/complications , Pituitary Diseases/diagnostic imaging , Multiple Endocrine Neoplasia/complications , Thyroid Neoplasms/diagnostic imaging , Adrenal Cortex Neoplasms/diagnostic imaging , Adrenocortical Carcinoma/diagnostic imaging , Hyperparathyroidism, Primary/diagnostic imagingABSTRACT
O câncer adrenocortical (CAC) é um tumor raro com um prognóstico reservado. O CAC pode ser diagnosticado pela investigação de síndromes endócrinas, de sintomas devido ao crescimento tumoral ou de incidentaloma adrenal. A investigação hormonal demonstra na maioria dos CACs uma hipersecreção esteroidal, mas a característica dominante é uma co-secreção de cortisol e de andrógenos. A TC mostra um tumor heterogêneo e grande. Os tumores restritos à glândula adrenal têm uma evolução melhor do que tumores invasivos e metastáticos. A remoção completa do tumor é o tratamento de escolha. Nos pacientes com doença metastática ou progressiva, o tratamento com mitotano deve ser iniciado. Este relato de caso tem o intuito de demonstrar a importância do correto diagnóstico de lesões tumorais, pois têm grande influência no tratamento e prognóstico dos pacientes
Adrenocortical cancer (ACC) is a rare malign tumor with a poor prognosis. ACC can be diagnosed by the investigation of endocrine syndromes, signs and symptoms due to tumor growth or an adrenal incidentaloma. Hormonal investigations demonstrate in most ACC steroid oversecretion, but dominant characteristic being a co-secretion of cortisol and androgens. The CT shows a large heterogeneous tumor. Tumors localized to the adrenal gland have a better outcome than invasive and metastatic tumors. The complete tumor removal is the treatment of choice. In patients with metastatic or progressive disease, the treatment with mitotano must be initiated. This case has intention to demonstrate the importance of the correct diagnosis of tumors, therefore it has great influence in the treatment and prognosis of the patients