Vi Poma
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Vi Poma

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  • ตับอ่อน มีขนาดความยาวประมาณ 6 นิ้ว มีรูปร่างคล้ายปลาดุก เป็นต่อมขนาดใหญ่ ตั้งอยู่ทางด้านหลังของกระเพาะอาหาร ใกล้กับลำไส้เล็กส่วนดูโอดินัม ซึ่งเป็นลำไส้เล็กส่วนต้น
  • endocrine tumor ผู้ป่วยมักมีอาการอันเนื่องมาจากความผิดปกติของฮอร์โมนที่เนื้องอกสร้างขึ้น การจำแนก endocrine tumor ของตับอ่อนจึงอาศัยชนิดของฮอร์โมนที่ตรวจพบในเลือดหรือในเนื้อเยื่อของ tumor เป็นสำคัญ

Vi Poma Vi Poma Presentation Transcript

  • VIPoma
  • Neck of Pancreas Anatomy of Pancreas
  •  
    • ตับอ่อน มีหน้าที่สำคัญ 2 ประการ คือ
    • 1. ต่อมมีท่อ ( Exocrine) : มีหน้าที่ สร้างน้ำย่อย ที่เรียกว่า “ น้ำย่อยของตับอ่อน ” ( Pancreatic juice ) ประกอบด้วยเอ็นไซม์ ( enzymes ) หลายชนิด ซึ่งมีหน้าที่ ช่วยในการย่อยอาหาร จำพวกแป้ง โปรตีน ไขมัน ให้มีขนาดเล็กลง จนสามารถซึมผ่านผนังของลำไส้เข้าสู่ร่างกายได้
    • 2. ต่อมไร้ท่อ ( Endocrine gland ) : สร้างฮอร์โมน หลายชนิด ซึ่งจะส่งเข้ากระแสเลือดโดยตรง ฮอร์โมนที่สำคัญ ได้แก่ Insulin , Glucagon , Somatostatin , Pancreatic polypeptide
    หน้าที่ของตับอ่อน
    • Non-Endocrine origin
    • - Non functional pancreatic endocrine neoplasms typically present later in the couse of their disease,when their tumors being to cause symptoms related to a mass effect.
    • - No associated evevation in plasma hormone level
    • Endocrine origin
    • - Most pancreatic endocrine neoplasms discovered clinically are functional
    • - Tumors secrete one or more hormonal product into the blood, which leads to a recognizable clinical syndrome
    • - Pancreatic polypeptide (PP) is a product that appears to be a marker for pancreatic endocrine tumors
    Pancreatic Tumor
  • Exocrine Tumor
    • Benign
    • - Serous cystadenoma
    • - Mucinous cystadenoma
    • - Intraductal papillary-mucinous adenoma
    • - Mature teratoma Borderline tumors ( uncertain malignant potential )
    • - Mucinous cystic tumor with moderate dysplasia
    • - Intraductal papillary-mucinous tumor with moderate dysplasia
    • - Solid-pseudopapillary tumor
    • Malignant
    • - Severe ductal dysplasia-carcinoma in situ
    • - Ductal adenocarcinoma:  * Mucinous noncystic carcinoma  * Signet ring cell carcinoma  * Adenosquamous carcinoma  * Undifferentiated (anaplastic) carcinoma  * Mixed ductal-endocrine carcinoma
    • - Osteoclast-like giant cell tumor
    • - Serous cystadenocarcinoma
    • - Mucinous cystadenocarcinoma:  * Noninvasive  * Invasive
    • - Intraductal papillary-mucinous carcinoma:  * Noninvasive  * Invasive (papillary-mucinous carcinoma)
    • - Acinar cell carcinoma:  * Acinar cell cystadenocarcinoma  * Mixed acinar-endocrine carcinoma
    • - Pancreatoblastoma
    • - Solid-pseudopapillary carcinoma
    • - Miscellaneous carcinomas
  • Clinical presentations of pancreatic endocrine tumor Name ( synonym ) Hormone responsible Clinical symptoms Principle of Medical Treatment Insulinoma (insulin-producing tumor) or Beta cell tumor Insulin Whipple triad - Mental confuse, weakness,fatigue and convulsion , palpitation - Fasting hypoglycemia - Relief of hypoglycemic symptoms after glucose administation May require immediate potassium replasement and glucose administration
  • Clinical presentations of pancreatic endocrine tumor Name ( synonym ) Hormone responsible Clinical symptoms Principle of Medical Treatment Gastrinoma (gastrin-producing tumor; ulcerogenic tumor) or G-cell tumors Gastrin Zollinger-Ellison syndrome * Severe gastrointestinal ulcerative disease * Gastric acid hypersecretion * Steatorrhea (fatty diarrhea) 1 . Stabilize hemodynamic and control bleeding from gastrointestinal ulcers 2 . Establish a nonacidic gastric pH with the use of proton pump inhibitors, eg, omeprazole 3 . Administration of the somatostatin analogue octreotide or chemotherapeutic agent
  • Clinical presentations of pancreatic endocrine tumor Name ( synonym ) Hormone responsible Clinical symptoms Principle of Medical Treatment Glucagonoma (glucagon-producing tumor) or Alpha cell tumors Glucagon
    • Glucagonoma syndrome
    • Abnormal glucose tolerance test
    • Normochromic normocytic anemia
    • Skin rash จากการหลั่งฮอร์โมนกลูคากอนมากเกินไป
    Generally have nutritional depletion and often require blood transfusions, total parenteral nutrition, and preoperative control of hyperglycemia
  • Clinical presentations of pancreatic endocrine tumor Name ( synonym ) Hormone responsible Clinical symptoms Principle of Medical Treatment VIPoma (vasoactive intestinal polypeptide) (VIP-producing tumor; diarrheogenic tumor; Verner-Morrison syndrome; pancreatic cholera) VIP
    • WDHA syndrome
    • Watery diarrhea
    • Hypokalemia
    • Achlorhydria
    Primary initial concern in the treatment of a patient who presents first with VIPoma - associated diarrhea is the replacement of volume losses and the correction of acid - base and electrolyte abnormalities
  • Clinical presentations of pancreatic endocrine tumor Name ( synonym ) Hormone responsible Clinical symptoms Principle of Medical Treatment Somatostatinoma (somatostatin-producing tumor) or Delta cell tumors Somatostatin
    • Somatostatinoma syndrome
    • Diabetes mellitus
    • Steatorrhea
    • Cholelithiasis
    • gastric hyposecretion
    Nutritional repletion and control of hyperglycemia are important aspects of good medical care in patients
    • Diagnosis of pancreatic tumors :
    • need imaging studies e.g. ultrasound ,
    • CT scan , MRI
    Investigation
    • Tumors deep in the substance of the pancreatic gland, and therefore close to the main duct, have ill-defined capsules, and tumors larger than 2 cm in diameter should be treated with regional pancreatectomy .
    • Tumors in the body or tail of the pancreas can be managed with distal pancreatectomy
    • Tumors in the neck of the pancreas can now be managed with middle-segment pancreatectomy by oversewing the proximal pancreatic stump and draining the distal pancreatic duct via a pancreaticogastrostomy or pancreaticojejunostomy
    • Lesions in the head or uncinate process of the pancreas can be resected with pancreaticoduodenectomy
    Surgical Treatment