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Atypical carcinoid tumor

MedGen UID:
226834
•Concept ID:
C1266032
•
Neoplastic Process
Synonyms: Atypical Carcinoid Tumor; atypical carcinoid tumor; Malignant Carcinoid Tumor; malignant carcinoid tumor; malignant carcinoid tumour
SNOMED CT: Atypical carcinoid tumor (128658008)
 
Monarch Initiative: MONDO:0006095

Definition

A carcinoid tumor characterized by a high mitotic rate, often associated with the presence of necrosis and nuclear pleomorphism. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAtypical carcinoid tumor

Professional guidelines

PubMed

Updates and management algorithm for neuroendocrine tumors of the uterine cervix.
Salvo G, Gonzalez Martin A, Gonzales NR, Frumovitz M
Int J Gynecol Cancer 2019 Jul;29(6):986-995. doi: 10.1136/ijgc-2019-000504. PMID: 31263021
About thymic carcinomas: challenges in diagnosis and management.
Mlika M, Boudaya S, Braham E, Chermiti F, Sayi A, Marghli A, El Mezni F
Asian Cardiovasc Thorac Ann 2016 May;24(4):350-4. Epub 2016 Mar 21 doi: 10.1177/0218492316640129. PMID: 27002097
Primary renal carcinoid: treatment and prognosis.
Korkmaz T, Seber S, Yavuzer D, Gumus M, Turhal NS
Crit Rev Oncol Hematol 2013 Sep;87(3):256-64. Epub 2013 Mar 9 doi: 10.1016/j.critrevonc.2013.02.003. PMID: 23478151

Recent clinical studies

Etiology

Pulmonary Neuroendocrine Tumors.
Borczuk AC
Surg Pathol Clin 2020 Mar;13(1):35-55. Epub 2019 Nov 29 doi: 10.1016/j.path.2019.10.002. PMID: 32005434
Neuroendocrine Carcinomas of the Larynx and Head and Neck: Challenges in Classification and Grading.
Perez-Ordoñez B
Head Neck Pathol 2018 Mar;12(1):1-8. Epub 2018 Mar 20 doi: 10.1007/s12105-018-0894-6. PMID: 29557536Free PMC Article
Neuroendrocrine tumors of the uterine cervix: A therapeutic challenge for gynecologic oncologists.
Gadducci A, Carinelli S, Aletti G
Gynecol Oncol 2017 Mar;144(3):637-646. Epub 2017 Jan 3 doi: 10.1016/j.ygyno.2016.12.003. PMID: 28057354
Neuroendocrine neoplasms of the larynx: an overview.
Ferlito A, Silver CE, Bradford CR, Rinaldo A
Head Neck 2009 Dec;31(12):1634-46. doi: 10.1002/hed.21162. PMID: 19536850
Atypical carcinoid tumor of the larynx. A critical review of the literature.
Woodruff JM, Senie RT
ORL J Otorhinolaryngol Relat Spec 1991;53(4):194-209. doi: 10.1159/000276219. PMID: 1891252

Diagnosis

Pulmonary Neuroendocrine Tumors.
Borczuk AC
Surg Pathol Clin 2020 Mar;13(1):35-55. Epub 2019 Nov 29 doi: 10.1016/j.path.2019.10.002. PMID: 32005434
Neuroendocrine Carcinomas of the Larynx and Head and Neck: Challenges in Classification and Grading.
Perez-Ordoñez B
Head Neck Pathol 2018 Mar;12(1):1-8. Epub 2018 Mar 20 doi: 10.1007/s12105-018-0894-6. PMID: 29557536Free PMC Article
Primary renal carcinoid: treatment and prognosis.
Korkmaz T, Seber S, Yavuzer D, Gumus M, Turhal NS
Crit Rev Oncol Hematol 2013 Sep;87(3):256-64. Epub 2013 Mar 9 doi: 10.1016/j.critrevonc.2013.02.003. PMID: 23478151
Neuroendocrine neoplasms of the larynx: an overview.
Ferlito A, Silver CE, Bradford CR, Rinaldo A
Head Neck 2009 Dec;31(12):1634-46. doi: 10.1002/hed.21162. PMID: 19536850
Bronchopulmonary neuroendocrine tumors.
Gustafsson BI, Kidd M, Chan A, Malfertheiner MV, Modlin IM
Cancer 2008 Jul 1;113(1):5-21. doi: 10.1002/cncr.23542. PMID: 18473355

Therapy

Checkpoint Inhibitor Immunotherapy to Treat Temozolomide-Associated Hypermutation in Advanced Atypical Carcinoid Tumor of the Lung.
Sun F, Grenert JP, Tan L, Van Ziffle J, Joseph NM, Mulvey CK, Bergsland E
JCO Precis Oncol 2022 Jun;6:e2200009. doi: 10.1200/PO.22.00009. PMID: 35737914Free PMC Article
A first-in-human phase 1 dose escalation study of spartalizumab (PDR001), an anti-PD-1 antibody, in patients with advanced solid tumors.
Naing A, Gainor JF, Gelderblom H, Forde PM, Butler MO, Lin CC, Sharma S, Ochoa de Olza M, Varga A, Taylor M, Schellens JHM, Wu H, Sun H, Silva AP, Faris J, Mataraza J, Cameron S, Bauer TM
J Immunother Cancer 2020 Mar;8(1) doi: 10.1136/jitc-2020-000530. PMID: 32179633Free PMC Article
Adjuvant Chemotherapy Does Not Confer Superior Survival in Patients With Atypical Carcinoid Tumors.
Anderson KL Jr, Mulvihill MS, Speicher PJ, Yerokun BA, Gulack BC, Nussbaum DP, Harpole DH Jr, D'Amico TA, Berry MF, Hartwig MG
Ann Thorac Surg 2017 Oct;104(4):1221-1230. Epub 2017 Jul 29 doi: 10.1016/j.athoracsur.2017.05.011. PMID: 28760471Free PMC Article
Endobronchial Treatment of Carcinoid Tumors of the Lung.
Dalar L, Ozdemir C, Abul Y, Sokucu SN, Karasulu L, Urer HN, Altin S
Thorac Cardiovasc Surg 2016 Mar;64(2):166-71. Epub 2015 May 18 doi: 10.1055/s-0035-1549274. PMID: 25984778
Primary renal carcinoid: treatment and prognosis.
Korkmaz T, Seber S, Yavuzer D, Gumus M, Turhal NS
Crit Rev Oncol Hematol 2013 Sep;87(3):256-64. Epub 2013 Mar 9 doi: 10.1016/j.critrevonc.2013.02.003. PMID: 23478151

Prognosis

Complex Patterns of Genomic Heterogeneity Identified in 42 Tumor Samples and ctDNA of a Pulmonary Atypical Carcinoid Patient.
Robb TJ, Tsai P, Fitzgerald S, Shields P, Houseman PS, Patel R, Fan V, Curran B, Tse R, Ting J, Kramer N, Woodhouse BJ, Coats E, Le Quesne Stabej P, Reeve J, Parker K, Lawrence B, Blenkiron C, Print CG
Cancer Res Commun 2023 Jan;3(1):31-42. Epub 2023 Jan 10 doi: 10.1158/2767-9764.CRC-22-0101. PMID: 36968225Free PMC Article
Neuroendrocrine tumors of the uterine cervix: A therapeutic challenge for gynecologic oncologists.
Gadducci A, Carinelli S, Aletti G
Gynecol Oncol 2017 Mar;144(3):637-646. Epub 2017 Jan 3 doi: 10.1016/j.ygyno.2016.12.003. PMID: 28057354
Primary renal carcinoid: treatment and prognosis.
Korkmaz T, Seber S, Yavuzer D, Gumus M, Turhal NS
Crit Rev Oncol Hematol 2013 Sep;87(3):256-64. Epub 2013 Mar 9 doi: 10.1016/j.critrevonc.2013.02.003. PMID: 23478151
Neuroendocrine neoplasms of the larynx: an overview.
Ferlito A, Silver CE, Bradford CR, Rinaldo A
Head Neck 2009 Dec;31(12):1634-46. doi: 10.1002/hed.21162. PMID: 19536850
Bronchopulmonary neuroendocrine tumors.
Gustafsson BI, Kidd M, Chan A, Malfertheiner MV, Modlin IM
Cancer 2008 Jul 1;113(1):5-21. doi: 10.1002/cncr.23542. PMID: 18473355

Clinical prediction guides

Complex Patterns of Genomic Heterogeneity Identified in 42 Tumor Samples and ctDNA of a Pulmonary Atypical Carcinoid Patient.
Robb TJ, Tsai P, Fitzgerald S, Shields P, Houseman PS, Patel R, Fan V, Curran B, Tse R, Ting J, Kramer N, Woodhouse BJ, Coats E, Le Quesne Stabej P, Reeve J, Parker K, Lawrence B, Blenkiron C, Print CG
Cancer Res Commun 2023 Jan;3(1):31-42. Epub 2023 Jan 10 doi: 10.1158/2767-9764.CRC-22-0101. PMID: 36968225Free PMC Article
NOL4 is a novel nuclear marker of small cell carcinoma and other neuroendocrine neoplasms.
Lee JH, Shin DH, Lee SY, Park JY, Kim SY, Hwang CS, Lee HJ, Na JY, Kim JY
Histol Histopathol 2022 Nov;37(11):1091-1098. Epub 2022 Oct 25 doi: 10.14670/HH-18-540. PMID: 36282054
Anatomic Pulmonary Resection is Associated With Improved Survival in Typical Carcinoid Lung Tumor Patients.
Del Calvo H, Nguyen DT, Chan EY, Chihara R, Graviss EA, Kim MP
J Surg Res 2022 Jul;275:352-360. Epub 2022 Mar 24 doi: 10.1016/j.jss.2022.02.048. PMID: 35339287
Atypical carcinoid tumor of the lung: a surveillance, epidemiology, and end results database analysis.
Steuer CE, Behera M, Kim S, Chen Z, Saba NF, Pillai RN, Owonikoko TK, Khuri FR, Ramalingam SS
J Thorac Oncol 2015 Mar;10(3):479-85. doi: 10.1097/JTO.0000000000000419. PMID: 25371080
Clinical outcomes of atypical carcinoid tumors of the lung and thymus: 7-year experience of a rare malignancy at single institute.
Han B, Sun JM, Ahn JS, Park K, Ahn MJ
Med Oncol 2013 Mar;30(1):479. Epub 2013 Feb 3 doi: 10.1007/s12032-013-0479-x. PMID: 23377986

Recent systematic reviews

Neuroendrocrine tumors of the uterine cervix: A therapeutic challenge for gynecologic oncologists.
Gadducci A, Carinelli S, Aletti G
Gynecol Oncol 2017 Mar;144(3):637-646. Epub 2017 Jan 3 doi: 10.1016/j.ygyno.2016.12.003. PMID: 28057354

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