20) Endocrine Neoplasms of Pancreas скачать в хорошем качестве

20) Endocrine Neoplasms of Pancreas

Endocrine Neoplasms - Insulinoma –Insulin producing adenoma of the β cells is the most common islet cell tumour. Due to excessive secretion of insulin from β cell lesions there will be- i)attack of hypoglycaemia, ii)confusion stupor and loss of consciousness. iii)prompty relived by feeding or parenteral administration of glucose. -this is classical whipple’s triad & very much characteristic of insulinoma. Pathology- 70 % solitary adenomas, 10 % multiple adenoma, 10% metastasizing tumours, 10% a mixed group of diffuse hyperplasia of islets. -these are usually encapsulated, firm, yellow, brown nodules. Microscopically- -Composed of cords and nests of well differentiated β cells. Clinical features – -Blood sugar falls rapidly –to release of epinephrine caused by hypoglycasemia. -sweating, weakness, tachycardia, hunger & trembling. -produces cerebral symptoms –headache, mental confusion, visual disturbance, convulsions & coma. -Bizarre neurologic or psychiatric disorders are not uncommon in this condition. Rx- -Only permanent cure of this condition is excision of tumour. -Dia zoxide may be given to inhibit insulin release from β cells. -this drug inhibits insulin release directly, causes release of glucose from the liver & interferes with peripheral untilization of sugar. -Steptozotocin, a broad spectrum antibiotic is a powerful agent for control of the symptoms of insulinoma –destroys islets but must be cautious about its toxic side effects. -Simple excision is sufficient as these tumours are benign. Zollinger –Ellison Syndrome (Gastrinoma) In 1955 Dr. Zollinger and EllisoN from Ohio state uni. first defined this disease. -Delta cells (D-cells) of islets of langerhan secrete somatostatin & gastrin . -The latter hormone is also found in G cells of the gastric mocosa. -Somatostatinomas are rare tumours. -Gastrinomas are much more common . -In this condition –peptic ulcers develop for high acid secretion of the stomach due to stimulation by gastrin which is being secreted by this tumour known as ‘Gastrinoma’. Pathology- -60 % are malignant & 2/3 have already metastasised when detected. -40 % are benign -30% are adenomas & -10 % hyperplasia of islets. -This usually consists of oaenomas of other endocrine glands such as pituitary, pancreatic islets (β cells ), parathyroid and adrenal contex . -This is not familial disease. Clinical features – -3rd &4th decade of life, -show an ulcerogenic diathesis, -75% of ulcers occurs in the usual sites within the stomach or Ist & IInd parts of the duodenum , -About 2-3 lit of fluid is found in 12 hour nocturnal secretion. -A ratio of basal acid output to maximal output (Histamine –stimulate is more than 60% & this is almost diagnostic of Zollinger Ellison syndrome . -Gastric hyper secretion is associated with distressing watery diarrhoea in about 1/3 of cases. -About 2 to 8 litres of liquid stool daily has been reported & may precede the onset of peptic ulceration. Treatment- -High dose of H2 antagonist /Omeprazole to control the ulcer. Surgical management – -Gastrinomas in the duodenal wall or in the pancreas should be shelled out. -solitary lesions in the tail can be treated by tail resection. -only in case of multiple lesions in the head of pancreas, a Whipple resection (pancreaticoduodenectomy )is necessary, -If no lesion or metastatic is detected then truncal vagotomy & pyloroplasty should be performed. WDHA Syndrome -Watery Diarrhea Hypocalcaemia & gastric Anacidity or hypochlorydria (First letters of each symptoms ) -Diarrrhoea is usually profuse 4 to 5 litr. /day = ‘Pancreatic cholera’ -Cause – secretion of vasoactive intestinal peptide, pancreatic polypeptide, gastrointestinal inhibitor peptide & secretin. -First 3 harmones are secreted by pp cells of the islets & will cause such syndrome. 40% benign tumours 40% malignant tumours with metastasis & -20% hyperplasia of these cells of the islets. Rx- is resection of these tumours.