Diagnosis and Treatment of Gastrinomas in Multiple Endocrine Neoplasia Type 1 (MEN-1)
نویسنده
چکیده
Multiple endocrine neoplasia type 1 (MEN-1) is a rare autosomal-dominant disease. It is associated with a broad range of endocrine tumours, most frequently arising in the parathyroid glands, the pituitary and the pancreas. Most neuroendocrine tumours will be diagnosed in the pancreas as non-functioning neuroendocrine tumours or insulinomas. Forty-two percent of the patients will develop a gastrin-secreting neuroendocrine tumour, a gastrinoma. Gastrinomas in MEN-1 tend to be small, multiple and preferentially located in the duodenum. This paper will focus on the specific characteristics of gastrinomas in the setting of MEN-1 compared to sporadic gastrinomas. The developments in understanding the tumorigenesis of these tumours and the consequences for diagnosis and therapy will be discussed.
منابع مشابه
MENJ gene plays a role in all four tumor types The lower rate of 1!q!3 LOH in MEN!-associated and sporadic gastrinomas and sporadic instill
Endocrine tumors (ETs) of pancreas and duodenum occur sporadically and as a part of multiple endocrine neoplasia type 1 (MEN1). The MENJ tumor suppressor gene has been localized to chromosome 11q13 by link age analysis but has not yet isolated. Previous alleic deletion studies in enteropancreatic ETs suggested MENJ gene involvement in tumorigenesis of familial pancreatic ETs (nongastrinomas) an...
متن کاملThe surgical management of hyperparathyroidism and endocrine disease of the pancreas in the multiple endocrine neoplasia type 1 patient.
The surgical management of multiple endocrine neoplasia type 1 (MEN1) parathyroid disease and involvement of the endocrine pancreas remains controversial. Hyperparathyroidism, usually the first clinical manifestation of the syndrome, requires surgical treatment in nearly all patients. We favour a subtotal parathyroidectomy and cervical thymectomy rather than a total parathyroidectomy and autotr...
متن کاملZollinger-Ellison Syndrome In Men1 Patients: Medical Or Surgical Treatment?
Multiple endocrine neoplasia type 1 (MEN1) is characterized by the occurrence of tumors in different endocrine organs mainly parathyroid glands, pancreatic islets and anterior pituitary glands. The pancreatoduodenal neuroendocrine tumors (NET) have a high penetrance with a prevalence of 9%, 53% and 84% at 20, 50 and 80 years of age, respectively [1]. Pancreato-duodenal NETs may secrete hormones...
متن کاملIdentical clonality of sporadic gastrinomas at multiple sites.
Gastrinomas are neuroendocrine neoplasms that occur sporadically and in patients with multiple endocrine neoplasia type 1 (MEN1). In MEN1, multiple gastrinomas have been shown to arise by independent clonal events (Debelenko, et al., Cancer Res., 57: 2238-2243, 1997). The purpose of the present study was to analyze clonality in 20 sporadic gastrinomas from eight patients in whom the tumor was p...
متن کاملA Case of Multiple Endocrine Neoplasia Type 1 with Primary Liver Gastrinoma
Gastrinoma is the most frequent functional pancreaticoduodenal endocrine tumor in patients with multiple endocrine neoplasia type 1 (MEN 1). Primary hepatic gastrinomas in MEN 1 are very rare, with no previous reports published in the literature. We reported the case of a 39 yr old female patient with a history of repeated peptic ulcers and a hypoglycemia episode. Abdominal CT indicated a well-...
متن کامل