General Information of Disease (ID: DISYZ23O)

Disease Name Pancreatic endocrine carcinoma
Synonyms
neuroendocrine carcinoma of pancreas; poorly-differentiated pancreatic neuroendocrine neoplasm; high-grade pancreatic neuroendocrine carcinoma; islet cell cancer; poorly-differentiated pancreatic NEN; carcinoma of endocrine pancreas; pancreatic NEC G3; pancreatic endocrine cancer; malignant pancreatic endocrine tumor; poorly differentiated pancreatic endocrine carcinoma; malignant pancreatic endocrine tumour; poorly-differentiated neuroendocrine neoplasm of pancreas; pancreatic NEC; Pancreatic Neuroendocrine cancer; malignant neoplasm of islets of Langerhans; poorly-differentiated NEN of pancreas; malignant islet cell tumor; pancreatic neuroendocrine carcinoma; islet cell carcinoma (morphologic abnormality); high grade pancreatic neuroendocrine carcinoma; malignant islet cell tumour; pancreatic endocrine carcinoma; islet cell carcinoma; endocrine pancreas carcinoma; carcinoma, islet cell, malignant
Definition
An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the pancreas. The mitotic count is more than 20 per 10 HPF. According to the size of the malignant cells, the prominence of the nucleoli, and the amount of cytoplasm, it is classified either as small or large cell neuroendocrine carcinoma.
Disease Hierarchy
DISH4FJX: Malignant pancreatic neoplasm
DISFC6MM: Digestive system carcinoma
DISVGJET: Neuroendocrine cancer
DISH9F1N: Carcinoma
DIS9IAQV: Pancreatic neuroendocrine neoplasm
DISYZ23O: Pancreatic endocrine carcinoma
Disease Identifiers
MONDO ID
MONDO_0005893
MESH ID
D018273
UMLS CUI
C1328479
MedGen ID
233070
Orphanet ID
506098
SNOMED CT ID
187794005

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 3 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
ELK3 TT5OJMV Strong Biomarker [1]
EPHB1 TT8MDAC Strong Biomarker [1]
SLC6A2 TTAWNKZ Strong Biomarker [1]
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This Disease Is Related to 2 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
MEN1 OTN6U6V0 moderate Genetic Variation [2]
TFE3 OTM99ZWH Strong Biomarker [3]
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References

1 Neoplasms of the Neuroendocrine Pancreas: An Update in the Classification, Definition, and Molecular Genetic Advances.Adv Anat Pathol. 2019 Jan;26(1):13-30. doi: 10.1097/PAP.0000000000000201.
2 Pancreatic neuroendocrine carcinomas reveal a closer relationship to ductal adenocarcinomas than to neuroendocrine tumors G3.Hum Pathol. 2018 Jul;77:70-79. doi: 10.1016/j.humpath.2018.03.018. Epub 2018 Mar 26.
3 TFE3 is a diagnostic marker for solid pseudopapillary neoplasms of the pancreas.Hum Pathol. 2018 Nov;81:166-175. doi: 10.1016/j.humpath.2018.07.005. Epub 2018 Jul 18.