Tumores endócrinos del páncreas / Endocrine tumors of the pancreas

ABSTRACT

Multiple endocrine neoplasia (MEN) is a rare familial disorder that affects multiple endocrine organs. it is inherited in an autosomal dominant pattern with variable penetrance. There are two distinct types of MEN. Dominantly inherited neoplasia are believed to occur from the recessive loss of tumor suppressor gene function. The first mutational event affects the germ cell, is hereditary, and predisposes to neoplasia. Because tumors occur in multiple organs in MEN, the second mutational evento probably occurs in common precursor cells, such as the amine precursor uptake and decarboxylation cells (APUD). The gene locus for tupe 1 MEN has been mapped to cromosome 11q. Type 1 organs affected: parathyroids, pancreas and pituitary. The gene locus for type 2 MEN is thought to be located for chromosome 10, (other organs). Fifty to 60 % of patients with type 1 MEN have pancreaticislet cell tumors. Insulinomas are tumors that originate in the beta cells of the islets of Langerhans, which compose the APUD system, the meaning of which is: A=Amino, P=Precursor, U=Uptake, D=Decarboxylation. The cells fo the APUD system have common cytochemical characteristics with the ability to secret polypeptides and amines. Symptoms are related to the peptide secreted by the tumor, and some tumors can produce multiple peptides. Gastrinomas comprise about 60 % of type 1 MEN-associated islet cell tumors. Gastric acid hypersecretion results from excess gstrin secretion and causes multiple gastric and duodenal ulcers (Zollinger-Ellison syndrome). The author describe an experience with this pathology with special reference to diagnostic methods, treatment, follow-up, laboratory studies and localization of the tumor.