neuroendocrine%20tumors
NEUROENDOCRINE TUMORS
Neuroendocrine tumors are rare, small, slow-growing epithelial neoplasms with predominant neuroendocrine differentiation found in most organs of the body.
They arise from cells throughout the diffuse endocrine system.
Carcinoid tumors and pancreatic neuroendocrine tumors are the most common.
Carcinoid tumors arise from the lungs & bronchi, stomach, small intestine, appendix, rectum or thymus.
Majority of the neuroendocrine tumors are sporadic but some tumors are caused by inherited genetic syndromes such as multiple endocrine neoplasia, Von-Hippel Lindau disease, tuberous sclerosis complex and neurofibromatosis.
They have the ability to store and secrete various peptides and neuroamines.

Follow Up

  • NETs are slow growing but they may progress faster if they are poorly differentiated or have a high Ki67 index > 5%
  • Patients should be monitored more closely during the first year after diagnosis to determine the status of the disease
    • Assess clinical manifestations, biomarkers, presence of new sites of disease, & quality of life during treatment
    • Routine evaluations, such as echocardiogram for patients with elevated 5-HIAA, to detect carcinoid heart disease in its early stages can improve prognosis
  • PET scan & somatostatin scintigraphy are not advised for routine surveillance
  • It is recommended to have re-evaluation of the patient 3-12 months after surgical resection or earlier if the patient is symptomatic
    • >1 year following surgical resection, every 6-12 months is recommended schedule for follow-up
    • Abdominal or pelvic multiphasic CT or MRI, gastrin, CgA & 5-HIAA levels may be used for disease monitoring
  • Low-risk patients (eg carcinoid tumor of the appendix) usually require no follow-ups
    • Low-risk patients are those having <2 cm primary tumor (<1 cm for rectal tumors), no nodal involvement & have low Ki67 < 5%
  • Repeat rectal MRI or endoscopic ultrasound 6-12 months after initial treatment is recommended for rectal NETs 1-2 cm in size & for gastric NETs type 1 or 2 for the 1st 3 months then annually if negative for pathologies
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