Somatostatinomas are a tumor of the delta cells of the endocrine pancreas that produces somatostatin. Increased levels of somatostatin inhibit pancreatic hormones and gastrointestinal hormones. Thus, somatostatinomas are associated with mild diabetes mellitus (due to inhibition of insulin release), steatorrhoea and gallstones (due to inhibition of cholecystokinin release), and achlorhydria (due to inhibition of gastrin release). Somatostatinomas are commonly found in the head of pancreas. Only ten percent of somatostatinomas are functional tumours [9], and 60–70% of tumours are malignant. Nearly two-thirds of patients with malignant somatostatinomas will present with metastatic disease. Pathophysiology
In a normal subject, actions of somatostatin include:
In the anterior pituitary gland, the effects of somatostatin are:Inhibit the release of growth hormone, thus opposing the effects of growth hormone-releasing hormone (GHRH)
Inhibit the release of thyroid-stimulating hormone (TSH)Somatostatin suppresses the release of gastrointestinal hormonesGastrin
Cholecystokinin (CCK)
Secretin
Motilin
Vasoactive intestinal peptide (VIP)
Gastric inhibitory polypeptide (GIP)
EnteroglucagonLowers the rate of gastric emptying, and reduces smooth muscle contractions and blood flow within the intestine
Suppresses the release of pancreatic hormonesInhibits the release of insulin
Inhibits the release of glucagonSuppresses the exocrine secretory action of pancreas.This explains how abnormally elevated somatostatin can cause diabetes mellitus, by inhibiting insulin secretion, steatorrhoea by inhibiting cholecystokinin and secretin, gall stones by inhibiting cholecystokinin which normally induce gallbladder myocytes to contract, and hypochlorhydria caused by inhibiting gastrin, which normally stimulate acid secretion.Somatostatinomas are associated with calcium deposits called psammoma bodies.
An endolymphatic sac tumor (ELST) is a very uncommon papillary epithelial neoplasm arising within the endolymphatic sac or endolymphatic duct. This tumor shows a very high association with Von Hippel–Lindau syndrome (VHL). Classification
The ELST has been referred to as adenocarcinoma of endolymphatic sac, Heffner tumor, papillary adenomatous tumor, aggressive papillary adenoma, invasive papillary cystadenoma, and papillary tumor of temporal bone. However, these names are not encouraged as they do not accurately classify the current understanding of the tumor. Signs and symptoms
Patients with ELST may present clinically with progressive or fluctuating, one sided sensorineural hearing loss which may mimick Ménières disease due to the development of tumor associated endolymphatic hydrops. Patients may also experience tinnitus, vertigo, and loss of vestibular function (ataxia). Alternatively, symptom onset may be sudden, due to intralabyrinthine hemorrhage. Patients may also present with other symptoms related to von Hippel–Lindau syndrome in other anatomic sites, which will result in imaging evaluation of the head. Imaging findings
Imaging studies help to identify the tumor and the specific anatomic site of involvement. Magnetic resonance images show a hyperintensity (hypervascularity) of a heterogeneous mass by T1 weighted images. Computed tomography shows a multilocular, lytic destructive temporal bone mass, centered on the vestibular aqueduct (between internal auditory canal and sigmoid sinus). Pathogenesis
The von Hippel–Lindau tumor suppressor gene generally has a germline mutation. This suppressor gene is also called elongin binding protein and G7 protein. The VHL protein is involved in up-regulation of hypoxic response via the [[hypoxia inducible factor [HIF]-1 alpha]].