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Abbreviations

CEA: Carcinoembryonic antigen, C-cell: Chief cells, U/S: Ultrasound, CT: Computerized tomography, PET: Position emission tomography, FNA: Fine needle aspiration, MEN: Multiple endocrine neoplasia, GI: Gastrointestinal, N/A: Not available/Not applicable, N/L: Normal, MRI: Magnetic resonance imaging, PTH: Parathyroid hormone, ACTH: Adrenocorticotropic hormone, GH: Growth hormone, TSH: Thyroid stimulating hormone, LH: Leutinizing hormone, FSH: Follicle stimulating hormone, NSE: Neuron specific enolase, NET: Neuroendocrine tumors, H&E: Hematoxylin and eosin, CK: Cytokeratin, Hb: Hemoglobin, IBD: Inflammatory bowel disease, VMAT: Vesicular monoamine transporter, ECL: Enterochromaffin like, GNET: Gastrointestinal neuroendocrine tumors, PNET: Pancreatic neuroendocrine tumor, PGP: Protein-gene-product, HCL: Hydrochloric acid, VIP: Vasoactive intestinal peptide, NF: Neurofibromatosis, PUD: Peptic ulcer disease, DVT: Deep vein thrombosis, PE: Pulmonary embolism, TTF: Transcription termination factor, SIADH: Syndrome of inappropriate anti-diuretic hormone secretion.

Neuroendocrine tumor Clinical manifestations Dianosis Gold

standard

Other features
Symptoms Signs Blood & Urine Gross Histology Immunohistochemistry Imaging Others
Medullary thyroid carcinoma[1][2][3]

chemistry and

calcitonin levels

Pheochromocytoma[4][5][6][7][8]
  • N/A
Merkel cell carcinoma[9][10][11]
  • Epidermotropism
  • N/A
Parathyroid adenoma[12][13][14]
  • Enlarged
  • smooth
  • Soft
  • Reddish brown in color
Staining for
  • Associated with MEN
  • Associated with HPRT
Paraganglioma[15][16][17][18]
  • Clear margins
  • Encapsulated
N/A
Pituitary gland tumor[19]s[20][21][22]
  • N/A
  • To learn more about pituitary neuroendocrine tumors, click here.
  • To learn more about pituitary hormones, click here.
Neuroendocrine tumors of the testicles[23][24][25] For metastasis and grading:
Neuroendocrine tumors of the ovaries[26][27][28]
  • Well-defined
  • Smooth
  • Solid with cystic areas

For metastasis and grading:

  • N/A
Thymic neuroendocrine cancer[29][30][31][32]

For metastasis and grading:

Jejuno-Ileal neuroendocrine tumors[33][34][35][36]
  • N/A
Appendix neuroendocrine tumors[37][38][39]
  • N/A
Colon neuroendocrine tumors[40][41][42][43]
  • Flat or depressed lesion
  • Plaque or polypoid
  • Wall thickening
  • Lumen narrowing
Rectum neuroendocrine tumors[44][40][45][46][47]
  • Small and multiple
  • Nodules or polypoid
Gastric neuroendocrine tumors Type I GNET[48][49][50]
  • N/A
Type II GNET[48][49][51]
  • N/A
  • N/A
Type III GNET[52][53][54]
  • Hb
  • Urinary 5-hydroxyindole-acetic acid (rare)
  • Large lesion with poor margins
  • Ulcerated appearance
Type IV GNET[55][56][41]
  • Invasion of adjacent structures
  • Large solid lesion
  • N/A
Structural abnormality → no HCL secretion → achlorhydria

hypergastrinemiahyperplasia

Duodenal neuroendocrine tumors Gastrinoma[57][58][59][60]
  • N/A
Somatostatinomas[61][62][63][64]
  • N/A
  • N/A
  • Associated with NF-1
Gangliocytic paraganglionomas[65][66][67][68]
  • Solid tumor
  • Ulceration
  • Sessile or polypod
  • Covered by the smooth
  • Associated with NF-1
Nonfunctioning NET[69]
neuroendocrine carcinomas[70][71][72][73]
  • Polypoid intraluminal mass (CT scan)
Pancreatic neuroendocrine tumours Gastrinoma[57][58][59][60]
  • N/A
Insulinoma[74][75][76][77]
Glucagonoma[78][79][80][81]
  • N/A
  • N/A
Somatostatinoma[61][62][63]
  • N/A
  • N/A
VIPoma[82][83][84][85]
ACTHoma[86][87][88][89]
  • Firm
  • Well circumscribed
  • Encapsulated
  • N/A
Lung neuroendocrine tumors Typical carcinoid tumours[90][91][92][93]
  • Central
  • Polypoid
  • Usually well defined
  • Smooth
  • No necrosis
  • N/A
  • 5‐year survival rate >90%
  • 12% metastasis at presentation
Atypical carcinoid tumours[90][91][92][93]
  • Peripheral
  • Polypoid
  • Irregular margins
  • Smooth
  • Necrosis
  • N/A
  • 5‐year survival rate 40-75%
  • 50% metastasis at presentation
Large cell lung neuroendocrine carcinomas[94][95][96][97] Associated
Small cell lung neuroendocrine carcinomas[98][99][100][101] Associated

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