Pancreatic Neuroendocrine Tumor





KEY FACTS


Terminology





  • Historically known as islet cell or carcinoid tumors



Imaging





  • Variable: Range from < 1 cm to > 20 cm; typically 1-5 cm



  • Small tumors: Well-defined, round, hypoechoic; can be isoechoic with focal contour deformity



  • Larger tumors are well demarcated, lobulated, and more heterogeneous with cystic change/necrosis and calcification




    • Displaces, rather than invades, adjacent structures



    • Intratumoral calcification suggests malignancy



    • 60-90% have adenopathy and liver metastases at clinical presentation




  • Absence of pancreatic ductal dilation



  • Color Doppler: Intratumoral flow



Top Differential Diagnoses





  • Mucinous cystic pancreatic neoplasm



  • Solid pseudopapillary neoplasm



  • Pancreatic ductal carcinoma



  • Pancreatic metastases or lymphoma



  • Serous cystadenoma of pancreas



Pathology





  • All pETs have malignant potential



  • ↑ serum chromogranin A is 70% sensitive for pancreatic endocrine tumors



Clinical Issues





  • 2-3% of all pancreatic neoplasms; peak: 4th-6th decades; younger in familial cases; most occurring sporadically



  • Familial syndromes: Multiple endocrine neoplasia type I; von Hippel-Lindau, neurofibromatosis type 1, tuberous sclerosis



  • Functioning tumors: Smaller at time of presentation due to hormonal production of insulin, glucagon, and gastrin



  • Nonfunctional tumors (60-80%): Usually asymptomatic but larger size at time of diagnosis; may cause mass effect and abdominal pain from primary or metastases



  • Prognosis: Best for insulinomas (most common functional)




    • 50-80% noninsulinomas recur or metastasize




  • Poor prognostic features: Size > 2-4 cm; cystic change, calcification, necrosis



  • Surgical resection is only curative treatment for pancreatic endocrine tumors



  • Insulinoma (Whipple triad): Recurrent symptomatic hyperglycemia



  • Gastrinoma (Zollinger-Ellison syndrome): Peptic ulcer, esophagitis, and diarrhea



  • Glucagonoma (4D syndrome): D ermatitis, d iabetes, D VT, and d epression



Diagnostic Checklist





  • Differentiate from other solid, cystic, hypervascular tumors



  • Correlate with clinical and biochemical information







Graphic demonstrates a well-circumscribed, round, solid mass in the pancreatic body with regional metastatic lymphadenopathy .








Transverse ultrasound through the left upper quadrant shows a large soft tissue mass arising from the pancreatic tail . This was a nonfunctioning neuroendocrine tumor, proven by biopsy of liver metastases.








Transverse ultrasound through the epigastric region shows a small soft tissue mass in the pancreatic head in a child who presented with abdominal pain and an enlarged liver. Biopsy of a liver metastasis revealed the diagnosis of a nonfunctioning neuroendocrine tumor.








Transverse color Doppler ultrasound of the same patient shows abundant color flow in the pancreatic head nonfunctioning neuroendocrine tumor .








Longitudinal ultrasound through the midline shows a pancreatic head nonfunctioning neuroendocrine tumor with liver metastases .

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Nov 10, 2024 | Posted by in ULTRASONOGRAPHY | Comments Off on Pancreatic Neuroendocrine Tumor

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