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Gallbladder neuroendocrine tumor, grade 1/2

MedGen UID:
474749
Concept ID:
C3273116
Neoplastic Process
Synonyms: Gallbladder NET; gallbladder NET; Gallbladder Neuroendocrine Tumor; gallbladder neuroendocrine tumor; Gallbladder neuroendocrine tumor; gallbladder neuroendocrine tumor, grade 1/2; gallbladder neuroendocrine tumour; Gallbladder Well Differentiated Endocrine Tumor; gallbladder well differentiated endocrine tumor; Gallbladder Well Differentiated Endocrine Tumor/Carcinoma; gallbladder well differentiated endocrine tumor/carcinoma; gallbladder well differentiated endocrine tumour
 
Monarch Initiative: MONDO:0015073
Orphanet: ORPHA100086

Definition

A rare, very aggressive neuroendocrine neoplasm characterized by the presence of nodular mass(es) arising from the neck, fundus or body of the gallbladder or by diffuse thickening of the gallbladder wall. Patients may be asymptomatic (diagnosed incidentally after surgical resection of the gallbladder) or may present epigastric pain, abdominal mass and/or non-specific symptoms, such as nausea, jaundice, flushing, cough, wheezing, ascites, and anepithymia. Paraneoplastic syndromes, such as Cushing syndrome, hypercalcemia, acanthosis nigricans, bullous pemphigoid, dermatomyositis and the Leser-Trélat sign, may be associated. [from ORDO]

Recent clinical studies

Etiology

Gogna S, Samson D, Gachabayov M, Rojas A, Felsenreich DM, Koo D, Gu K, Quintero L, Miller KR, Azim A, Da Eric Dong X
Langenbecks Arch Surg 2022 Feb;407(1):197-206. Epub 2021 Jul 8 doi: 10.1007/s00423-021-02256-z. PMID: 34236488
Amin M, Gao F, Terrero G, Picus J, Wang-Gillam A, Suresh R, Ma C, Tan B, Baggstrom M, Naughton MJ, Trull L, Belanger S, Fracasso PM, Lockhart AC
Am J Clin Oncol 2021 Sep 1;44(9):443-448. doi: 10.1097/COC.0000000000000852. PMID: 34310349
Chen C, Wang L, Liu X, Zhang G, Zhao Y, Geng Z
Int J Clin Exp Pathol 2015;8(7):8218-26. Epub 2015 Jul 1 PMID: 26339390Free PMC Article
Kimura W, Miyata H, Gotoh M, Hirai I, Kenjo A, Kitagawa Y, Shimada M, Baba H, Tomita N, Nakagoe T, Sugihara K, Mori M
Ann Surg 2014 Apr;259(4):773-80. doi: 10.1097/SLA.0000000000000263. PMID: 24253151

Diagnosis

Gogna S, Samson D, Gachabayov M, Rojas A, Felsenreich DM, Koo D, Gu K, Quintero L, Miller KR, Azim A, Da Eric Dong X
Langenbecks Arch Surg 2022 Feb;407(1):197-206. Epub 2021 Jul 8 doi: 10.1007/s00423-021-02256-z. PMID: 34236488
Amin M, Gao F, Terrero G, Picus J, Wang-Gillam A, Suresh R, Ma C, Tan B, Baggstrom M, Naughton MJ, Trull L, Belanger S, Fracasso PM, Lockhart AC
Am J Clin Oncol 2021 Sep 1;44(9):443-448. doi: 10.1097/COC.0000000000000852. PMID: 34310349

Therapy

Amin M, Gao F, Terrero G, Picus J, Wang-Gillam A, Suresh R, Ma C, Tan B, Baggstrom M, Naughton MJ, Trull L, Belanger S, Fracasso PM, Lockhart AC
Am J Clin Oncol 2021 Sep 1;44(9):443-448. doi: 10.1097/COC.0000000000000852. PMID: 34310349

Prognosis

Gogna S, Samson D, Gachabayov M, Rojas A, Felsenreich DM, Koo D, Gu K, Quintero L, Miller KR, Azim A, Da Eric Dong X
Langenbecks Arch Surg 2022 Feb;407(1):197-206. Epub 2021 Jul 8 doi: 10.1007/s00423-021-02256-z. PMID: 34236488
Chen C, Wang L, Liu X, Zhang G, Zhao Y, Geng Z
Int J Clin Exp Pathol 2015;8(7):8218-26. Epub 2015 Jul 1 PMID: 26339390Free PMC Article
Kimura W, Miyata H, Gotoh M, Hirai I, Kenjo A, Kitagawa Y, Shimada M, Baba H, Tomita N, Nakagoe T, Sugihara K, Mori M
Ann Surg 2014 Apr;259(4):773-80. doi: 10.1097/SLA.0000000000000263. PMID: 24253151

Clinical prediction guides

Gogna S, Samson D, Gachabayov M, Rojas A, Felsenreich DM, Koo D, Gu K, Quintero L, Miller KR, Azim A, Da Eric Dong X
Langenbecks Arch Surg 2022 Feb;407(1):197-206. Epub 2021 Jul 8 doi: 10.1007/s00423-021-02256-z. PMID: 34236488
Amin M, Gao F, Terrero G, Picus J, Wang-Gillam A, Suresh R, Ma C, Tan B, Baggstrom M, Naughton MJ, Trull L, Belanger S, Fracasso PM, Lockhart AC
Am J Clin Oncol 2021 Sep 1;44(9):443-448. doi: 10.1097/COC.0000000000000852. PMID: 34310349

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