Familial Gastric Type 1 Neuroendocrine Tumor

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

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Drugs

Bevacizumab ( Equidacent, AVASTIN, Aybintio, MVASI ), Catumaxomab, Chimeric monoclonal antibody against claudin-18 splice variant 2

Bevacizumab ( Equidacent, AVASTIN, Aybintio, MVASI ), Catumaxomab, Chimeric monoclonal antibody against claudin-18 splice variant 2, Everolimus ( AFINITOR, VOTUBIA ), G17(9) gastrin-diphtheria toxoid conjugate, Human anti-guanylyl cyclase C monoclonal antibody linked to monomethyl auristatin E, Ipatasertib, Plevitrexed, Ramucirumab ( GILOTRIF, CYRAMZA ), Rilotumumab, Tegafur, Gimeracil, Oteracil ( TEYSUNO ), Tesetaxel

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A rare neoplastic disease characterized by occurrence of atypical and aggressive gastric type 1 neuroendocrine tumors (NET) in early adulthood. The tumors often show nodal infiltration requiring total gastrectomy. Synchronous gastric adenocarcinoma has also been reported. Patients present high serum gastrin concentrations and iron-deficiency anemia (rather than megaloblastic anemia, which is a typical feature in patients with sporadic gastric type 1 NET, where the tumor usually arises on the background of autoimmune atrophic gastritis).