Pheochromocytoma and Paraganglioma
Syndrome Gene HNPGL sPGL PCC Multiple PGL Bilateral PCC Malignancy risk Parentof origin preference MEN2 RET Extremely rare − ++ − ++ ± − VHL VHL Rare + ++ +…
Syndrome Gene HNPGL sPGL PCC Multiple PGL Bilateral PCC Malignancy risk Parentof origin preference MEN2 RET Extremely rare − ++ − ++ ± − VHL VHL Rare + ++ +…
1 Neuroendocrine tumour, NET G1 (carcinoid) 2 Neuroendocrine tumour, NET G2 3 Neuroendocrine carcinoma, NEC (small- or large-cell type) 4 Mixed adenoneuroendocrine carcinoma, MANEC 5 Hyperplastic and preneoplastic lesions Adapted…
Biomarkers Specificity CgA, CgB, pancreastatin H PP, NSE, neurokinin, neurotensin I HCG-α, HCG-β L The chromogranin family and pancreatic polypeptide, neuron-specific enolase (NSE) and alpha HCG constitute general markers for…
Tumors Frequency % 10-year survival (%) Papillary carcinoma 80 93 Classic papillary Follicular variant Macrofollicular variant Oncocytic variant Clear cell variant Diffuse sclerosing variant Solid variant Cribriform carcinoma Papillary carcinoma…
Inappropriate hypergastrinemia – gastric pH <2 Gastrinoma/Zollinger-Ellison syndrome Helicobacter pylori antral predominate Retained gastric antrum Gastric outlet obstruction Short bowel syndrome Appropriate hypergastrinemia – associated with hypochlorhydria Pernicious anemia Chronic…
Fig. 16.1 (a, b) Microscopic appearances of ampullary somatostatinoma. Haematoxylin and eosin staining (left) and strongly positive immunostaining with somatostatin (right) (ANNALS IMAGE) Psammoma bodies (round collections of calcium that…
Fig. 26.1 A 45-year-old man with small bowel neuroendocrine tumor, with left hepatectomy for liver metastasis. (a) Axial CT scan (arterial phase) shows new metastatic liver lesion in the remnant…
Location Within NETs (%) Within GEP-NETs Incidence SEER (/100,000) Incidence (% of primary site) 5-year OS (%) GEP-NETs 67 5.25 <2 75–82 Gastric 9–20 0.3 1 45–64 Small intestine 39–42…
Gene Pancreatic NET (%) n = 48 Intestinal NET (%) n = 68 MEN-1 44 2 DAXX 25 0 ATRX 18 0 TSC2 9 0 PTEN 7 0 PIK3CA 1 2 VHL 0 2…
, Cenk Sokmensuer2 and Ece Esin3 (1) Department of Medical Oncology, Cancer Institute, Hacettepe University, Sihhiye, Ankara, Turkey (2) Department of Pathology, School of Medicine, Hacettepe University, Ankara, Turkey (3) Department of…