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Neuroendocrine Carcinoma of Unknown Primary

MedGen UID:
1720013
•Concept ID:
C5235863
•
Neoplastic Process

Definition

A neuroendocrine carcinoma that has metastasized from an unknown primary site. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeuroendocrine Carcinoma of Unknown Primary

Professional guidelines

PubMed

Radioactive iodine in the management of medullary carcinoma of the thyroid.
Rowell NP
Br J Radiol 2023 Aug;96(1148):20220660. Epub 2023 Jun 29 doi: 10.1259/bjr.20220660. PMID: 37335288Free PMC Article
SEOM clinical guideline on unknown primary cancer (2017).
Losa F, Soler G, Casado A, Estival A, Fernández I, Giménez S, Longo F, Pazo-Cid R, Salgado J, Seguí MÁ
Clin Transl Oncol 2018 Jan;20(1):89-96. Epub 2017 Dec 11 doi: 10.1007/s12094-017-1807-y. PMID: 29230692Free PMC Article
Diagnosis and management of metastatic neoplasms with unknown primary.
Bochtler T, Löffler H, Krämer A
Semin Diagn Pathol 2018 May;35(3):199-206. Epub 2017 Nov 26 doi: 10.1053/j.semdp.2017.11.013. PMID: 29203116

Recent clinical studies

Etiology

Aspirin for Metabolic Dysfunction-Associated Steatotic Liver Disease Without Cirrhosis: A Randomized Clinical Trial.
Simon TG, Wilechansky RM, Stoyanova S, Grossman A, Dichtel LE, Lauer GM, Miller KK, Hoshida Y, Corey KE, Loomba R, Chung RT, Chan AT
JAMA 2024 Mar 19;331(11):920-929. doi: 10.1001/jama.2024.1215. PMID: 38502074Free PMC Article
Second primary cancers and survival among neuroendocrine tumor patients.
Bateni SB, Coburn NG, Law C, Singh S, Myrehaug S, Assal A, Hallet J
Endocr Relat Cancer 2023 Aug 1;30(8) Epub 2023 Jun 26 doi: 10.1530/ERC-22-0337. PMID: 37184947
Expression and prognostic significance of programmed death-ligand 1 (PD-L1) in Merkel cell carcinoma.
Richter I, Dvorak TJECJBTBJ
J BUON 2019 Sep-Oct;24(5):2155-2160. PMID: 31786889
Gamma Knife Radiosurgery for Metastatic Brain Tumors from Cancer of Unknown Primary.
Matsunaga S, Shuto T, Kobayashi N
World Neurosurg 2019 Feb;122:e1465-e1471. Epub 2018 Nov 20 doi: 10.1016/j.wneu.2018.11.086. PMID: 30468925
Multiple endocrine neoplasia type 2: achievements and current challenges.
Machens A, Dralle H
Clinics (Sao Paulo) 2012;67 Suppl 1(Suppl 1):113-8. doi: 10.6061/clinics/2012(sup01)19. PMID: 22584715Free PMC Article

Diagnosis

Overview of the 2022 WHO Classification of Neuroendocrine Neoplasms.
Rindi G, Mete O, Uccella S, Basturk O, La Rosa S, Brosens LAA, Ezzat S, de Herder WW, Klimstra DS, Papotti M, Asa SL
Endocr Pathol 2022 Mar;33(1):115-154. Epub 2022 Mar 16 doi: 10.1007/s12022-022-09708-2. PMID: 35294740
Consensus on molecular imaging and theranostics in neuroendocrine neoplasms.
Ambrosini V, Kunikowska J, Baudin E, Bodei L, Bouvier C, Capdevila J, Cremonesi M, de Herder WW, Dromain C, Falconi M, Fani M, Fanti S, Hicks RJ, Kabasakal L, Kaltsas G, Lewington V, Minozzi S, Cinquini M, Öberg K, Oyen WJG, O'Toole D, Pavel M, Ruszniewski P, Scarpa A, Strosberg J, Sundin A, Taïeb D, Virgolini I, Wild D, Herrmann K, Yao J
Eur J Cancer 2021 Mar;146:56-73. Epub 2021 Feb 12 doi: 10.1016/j.ejca.2021.01.008. PMID: 33588146Free PMC Article
Immunohistochemistry in the diagnosis and classification of neuroendocrine neoplasms: what can brown do for you?
Bellizzi AM
Hum Pathol 2020 Feb;96:8-33. Epub 2019 Dec 17 doi: 10.1016/j.humpath.2019.12.002. PMID: 31857137Free PMC Article
Head and neck carcinoma of unknown primary.
Kennel T, Garrel R, Costes V, Boisselier P, Crampette L, Favier V
Eur Ann Otorhinolaryngol Head Neck Dis 2019 Jun;136(3):185-192. Epub 2019 Apr 17 doi: 10.1016/j.anorl.2019.04.002. PMID: 31005456
PET/CT and PET/MRI in head and neck malignancy.
Szyszko TA, Cook GJR
Clin Radiol 2018 Jan;73(1):60-69. Epub 2017 Oct 10 doi: 10.1016/j.crad.2017.09.001. PMID: 29029767

Therapy

Aspirin for Metabolic Dysfunction-Associated Steatotic Liver Disease Without Cirrhosis: A Randomized Clinical Trial.
Simon TG, Wilechansky RM, Stoyanova S, Grossman A, Dichtel LE, Lauer GM, Miller KK, Hoshida Y, Corey KE, Loomba R, Chung RT, Chan AT
JAMA 2024 Mar 19;331(11):920-929. doi: 10.1001/jama.2024.1215. PMID: 38502074Free PMC Article
Hyaluronan heterogeneity in pancreatic ductal adenocarcinoma: Primary tumors compared to sites of metastasis.
Placencio-Hickok VR, Lauzon M, Moshayedi N, Guan M, Kim S, Nissen N, Lo S, Pandol S, Larson BK, Gong J, Hendifar AE, Osipov A
Pancreatology 2022 Jan;22(1):92-97. Epub 2021 Oct 4 doi: 10.1016/j.pan.2021.09.015. PMID: 34657790Free PMC Article
Consensus on molecular imaging and theranostics in neuroendocrine neoplasms.
Ambrosini V, Kunikowska J, Baudin E, Bodei L, Bouvier C, Capdevila J, Cremonesi M, de Herder WW, Dromain C, Falconi M, Fani M, Fanti S, Hicks RJ, Kabasakal L, Kaltsas G, Lewington V, Minozzi S, Cinquini M, Öberg K, Oyen WJG, O'Toole D, Pavel M, Ruszniewski P, Scarpa A, Strosberg J, Sundin A, Taïeb D, Virgolini I, Wild D, Herrmann K, Yao J
Eur J Cancer 2021 Mar;146:56-73. Epub 2021 Feb 12 doi: 10.1016/j.ejca.2021.01.008. PMID: 33588146Free PMC Article
Vandetanib: in medullary thyroid cancer.
Frampton JE
Drugs 2012 Jul 9;72(10):1423-36. doi: 10.2165/11209300-000000000-00000. PMID: 22715896
Merkel cell carcinoma: critical review with guidelines for multidisciplinary management.
Bichakjian CK, Lowe L, Lao CD, Sandler HM, Bradford CR, Johnson TM, Wong SL
Cancer 2007 Jul 1;110(1):1-12. doi: 10.1002/cncr.22765. PMID: 17520670

Prognosis

Aspirin for Metabolic Dysfunction-Associated Steatotic Liver Disease Without Cirrhosis: A Randomized Clinical Trial.
Simon TG, Wilechansky RM, Stoyanova S, Grossman A, Dichtel LE, Lauer GM, Miller KK, Hoshida Y, Corey KE, Loomba R, Chung RT, Chan AT
JAMA 2024 Mar 19;331(11):920-929. doi: 10.1001/jama.2024.1215. PMID: 38502074Free PMC Article
Immunohistochemistry in the diagnosis and classification of neuroendocrine neoplasms: what can brown do for you?
Bellizzi AM
Hum Pathol 2020 Feb;96:8-33. Epub 2019 Dec 17 doi: 10.1016/j.humpath.2019.12.002. PMID: 31857137Free PMC Article
Expression and prognostic significance of programmed death-ligand 1 (PD-L1) in Merkel cell carcinoma.
Richter I, Dvorak TJECJBTBJ
J BUON 2019 Sep-Oct;24(5):2155-2160. PMID: 31786889
Gamma Knife Radiosurgery for Metastatic Brain Tumors from Cancer of Unknown Primary.
Matsunaga S, Shuto T, Kobayashi N
World Neurosurg 2019 Feb;122:e1465-e1471. Epub 2018 Nov 20 doi: 10.1016/j.wneu.2018.11.086. PMID: 30468925
Merkel cell carcinoma: The first human cancer shown to be associated with a polyomavirus.
Samimi M, Touzé A
Presse Med 2014 Dec;43(12 Pt 2):e405-11. Epub 2014 Nov 6 doi: 10.1016/j.lpm.2014.09.008. PMID: 25455636

Clinical prediction guides

Dilatation of the main pancreatic duct of unknown origin: causes and risk factors of pre-malignancy or malignancy.
Martin A, Cros J, Vullierme MP, Dokmak S, Sauvanet A, Levy P, Rebours V, Maire F
Surg Endosc 2023 May;37(5):3684-3690. Epub 2023 Jan 13 doi: 10.1007/s00464-022-09854-y. PMID: 36639578
Immunohistochemistry in the diagnosis and classification of neuroendocrine neoplasms: what can brown do for you?
Bellizzi AM
Hum Pathol 2020 Feb;96:8-33. Epub 2019 Dec 17 doi: 10.1016/j.humpath.2019.12.002. PMID: 31857137Free PMC Article
Gamma Knife Radiosurgery for Metastatic Brain Tumors from Cancer of Unknown Primary.
Matsunaga S, Shuto T, Kobayashi N
World Neurosurg 2019 Feb;122:e1465-e1471. Epub 2018 Nov 20 doi: 10.1016/j.wneu.2018.11.086. PMID: 30468925
Immunohistochemistry, carcinomas of unknown primary, and incidence rates.
Stelow EB, Yaziji H
Semin Diagn Pathol 2018 Mar;35(2):143-152. Epub 2017 Nov 23 doi: 10.1053/j.semdp.2017.11.012. PMID: 29224972
Efficacy of radiotherapy for primary tumor in patients with unresectable pancreatic neuroendocrine tumors.
Iwata T, Ueno H, Itami J, Ito Y, Inaba K, Morizane C, Kondo S, Sakamoto Y, Shiba S, Sasaki M, Koga F, Okusaka T
Jpn J Clin Oncol 2017 Sep 1;47(9):826-831. doi: 10.1093/jjco/hyx081. PMID: 28591817

Recent systematic reviews

Detecting undetectable - epidemiology, etiology, and diagnosis of carcinoma of unknown primary - systematic review.
Ostojewska J, Wieczorek I, Pachciński O, Zdziennicki W, Burdan F
Folia Med Cracov 2023 Apr 30;63(1):19-37. doi: 10.24425/fmc.2023.145427. PMID: 37406274
Radioactive iodine in the management of medullary carcinoma of the thyroid.
Rowell NP
Br J Radiol 2023 Aug;96(1148):20220660. Epub 2023 Jun 29 doi: 10.1259/bjr.20220660. PMID: 37335288Free PMC Article
Merkel cell carcinoma: A systematic review of the demographic and clinical characteristics of 847 cases in Japan.
Shinogi T, Nagase K, Inoue T, Sato K, Onita A, Takamori A, Narisawa Y
J Dermatol 2021 Jul;48(7):1027-1034. Epub 2021 Apr 13 doi: 10.1111/1346-8138.15875. PMID: 33847013
Unraveling molecular pathways of poorly differentiated neuroendocrine carcinomas of the gastroenteropancreatic system: A systematic review.
Girardi DM, Silva ACB, Rêgo JFM, Coudry RA, Riechelmann RP
Cancer Treat Rev 2017 May;56:28-35. Epub 2017 Apr 17 doi: 10.1016/j.ctrv.2017.04.002. PMID: 28456055
Neuroendocrine carcinoma of unknown primary: a systematic review of the literature and a comparative study with other neuroendocrine tumors.
Stoyianni A, Pentheroudakis G, Pavlidis N
Cancer Treat Rev 2011 Aug;37(5):358-65. Epub 2011 Apr 9 doi: 10.1016/j.ctrv.2011.03.002. PMID: 21481536

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