U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Neuroendocrine Carcinoma, Excluding Head and Neck

MedGen UID:
1729128
•Concept ID:
C5420179
•
Neoplastic Process

Definition

A high-grade carcinoma with neuroendocrine differentiation. This category includes small cell carcinomas, large cell neuroendocrine carcinomas, and Merkel cell carcinomas and excludes neuroendocrine carcinomas that arise from the head and neck. The head and neck neuroendocrine carcinomas include well-differentiated, moderately-differentiated, and poorly-differentiated (high-grade) neuroendocrine carcinomas. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeuroendocrine Carcinoma, Excluding Head and Neck

Professional guidelines

PubMed

The role of molecular genetics in the clinical management of sporadic medullary thyroid carcinoma: A systematic review.
Fussey JM, Vaidya B, Kim D, Clark J, Ellard S, Smith JA
Clin Endocrinol (Oxf) 2019 Dec;91(6):697-707. Epub 2019 Jul 29 doi: 10.1111/cen.14060. PMID: 31301229
Sorafenib for the Treatment of Progressive Metastatic Medullary Thyroid Cancer: Efficacy and Safety Analysis.
de Castroneves LA, Negrão MV, de Freitas RM, Papadia C, Lima JV Jr, Fukushima JT, Simão EF, Kulcsar MA, Tavares MR, Jorge AA, de Castro G Jr, Hoff PM, Hoff AO
Thyroid 2016 Mar;26(3):414-9. Epub 2016 Feb 9 doi: 10.1089/thy.2015.0334. PMID: 26701095
Feasibility of using American Joint Committee on Cancer Classification criteria for staging eyelid carcinomas.
Shinder R, Ivan D, Seigler D, Dogan S, Esmaeli B
Orbit 2011 Oct;30(5):202-7. doi: 10.3109/01676831003664343. PMID: 21957948

Recent clinical studies

Etiology

Induction Toripalimab and Chemotherapy for Organ Preservation in Locally Advanced Laryngeal and Hypopharyngeal Cancer: A Single-Arm Phase II Clinical Trial.
Ou X, Zhai R, Wei W, Chen J, Ou D, Liao T, Xu T, Zhu Y, Wang Y, Huang S, Shi R, Wu B, Chen T, Li Y, Yang Z, Zhou C, Liu Y, Jiang Z, Zeng M, Liu X, Ji D, Ying H, Zhang Z, Hu C, Lu X, Ji Q, He X, Wang Y
Clin Cancer Res 2024 Jan 17;30(2):344-355. doi: 10.1158/1078-0432.CCR-23-2398. PMID: 37955629
Differences in Merkel Cell Carcinoma Presentation and Outcomes Among Racial and Ethnic Groups.
Mohsin N, Martin MR, Reed DJ, Vilasi SM, Miao L, Hill NT, Brownell I
JAMA Dermatol 2023 May 1;159(5):536-540. doi: 10.1001/jamadermatol.2023.0061. PMID: 36920369Free PMC Article
Combined nivolumab and ipilimumab with or without stereotactic body radiation therapy for advanced Merkel cell carcinoma: a randomised, open label, phase 2 trial.
Kim S, Wuthrick E, Blakaj D, Eroglu Z, Verschraegen C, Thapa R, Mills M, Dibs K, Liveringhouse C, Russell J, Caudell JJ, Tarhini A, Markowitz J, Kendra K, Wu R, Chen DT, Berglund A, Michael L, Aoki M, Wang MH, Hamaidi I, Cheng P, de la Iglesia J, Slebos RJ, Chung CH, Knepper TC, Moran-Segura CM, Nguyen JV, Perez BA, Rose T, Harrison L, Messina JL, Sondak VK, Tsai KY, Khushalani NI, Brohl AS
Lancet 2022 Sep 24;400(10357):1008-1019. Epub 2022 Sep 12 doi: 10.1016/S0140-6736(22)01659-2. PMID: 36108657Free PMC Article
Clinical outcome and comparison between squamous and non-squamous cell carcinoma of the larynx.
Chen L, Deng W, Li C, Lau H, Tao L, Wang S, Zhou L, Zhang M
Acta Otolaryngol 2020 Feb;140(2):195-201. Epub 2019 Dec 18 doi: 10.1080/00016489.2019.1700305. PMID: 31852360
Post-Operative Concurrent Chemoradiation for Patients with Non-Squamous Cell Carcinoma of Head and Neck: A Retrospective Cohort of the Uncommon Cancers.
Thamlikitkul L, Petsuksiri J, Ithimakin S
Asian Pac J Cancer Prev 2019 Jun 1;20(6):1727-1734. doi: 10.31557/APJCP.2019.20.6.1727. PMID: 31244293Free PMC Article

Diagnosis

Somatostatin Receptor-PET/CT/MRI of Head and Neck Neuroendocrine Tumors.
Rini JN, Keir G, Caravella C, Goenka A, Franceschi AM
AJNR Am J Neuroradiol 2023 Aug;44(8):959-966. Epub 2023 Jul 13 doi: 10.3174/ajnr.A7934. PMID: 37442593Free PMC Article
Differences in Merkel Cell Carcinoma Presentation and Outcomes Among Racial and Ethnic Groups.
Mohsin N, Martin MR, Reed DJ, Vilasi SM, Miao L, Hill NT, Brownell I
JAMA Dermatol 2023 May 1;159(5):536-540. doi: 10.1001/jamadermatol.2023.0061. PMID: 36920369Free PMC Article
Clinical outcome and comparison between squamous and non-squamous cell carcinoma of the larynx.
Chen L, Deng W, Li C, Lau H, Tao L, Wang S, Zhou L, Zhang M
Acta Otolaryngol 2020 Feb;140(2):195-201. Epub 2019 Dec 18 doi: 10.1080/00016489.2019.1700305. PMID: 31852360
Different outcomes in sporadic versus familial medullary thyroid cancer.
Saltiki K, Simeakis G, Anagnostou E, Zapanti E, Anastasiou E, Alevizaki M
Head Neck 2019 Jan;41(1):154-161. Epub 2018 Dec 11 doi: 10.1002/hed.25463. PMID: 30548085
New tumor entities in the 4th edition of the World Health Organization classification of head and neck tumors: Nasal cavity, paranasal sinuses and skull base.
Thompson LDR, Franchi A
Virchows Arch 2018 Mar;472(3):315-330. Epub 2017 Apr 25 doi: 10.1007/s00428-017-2116-0. PMID: 28444451

Therapy

Induction Toripalimab and Chemotherapy for Organ Preservation in Locally Advanced Laryngeal and Hypopharyngeal Cancer: A Single-Arm Phase II Clinical Trial.
Ou X, Zhai R, Wei W, Chen J, Ou D, Liao T, Xu T, Zhu Y, Wang Y, Huang S, Shi R, Wu B, Chen T, Li Y, Yang Z, Zhou C, Liu Y, Jiang Z, Zeng M, Liu X, Ji D, Ying H, Zhang Z, Hu C, Lu X, Ji Q, He X, Wang Y
Clin Cancer Res 2024 Jan 17;30(2):344-355. doi: 10.1158/1078-0432.CCR-23-2398. PMID: 37955629
Combined nivolumab and ipilimumab with or without stereotactic body radiation therapy for advanced Merkel cell carcinoma: a randomised, open label, phase 2 trial.
Kim S, Wuthrick E, Blakaj D, Eroglu Z, Verschraegen C, Thapa R, Mills M, Dibs K, Liveringhouse C, Russell J, Caudell JJ, Tarhini A, Markowitz J, Kendra K, Wu R, Chen DT, Berglund A, Michael L, Aoki M, Wang MH, Hamaidi I, Cheng P, de la Iglesia J, Slebos RJ, Chung CH, Knepper TC, Moran-Segura CM, Nguyen JV, Perez BA, Rose T, Harrison L, Messina JL, Sondak VK, Tsai KY, Khushalani NI, Brohl AS
Lancet 2022 Sep 24;400(10357):1008-1019. Epub 2022 Sep 12 doi: 10.1016/S0140-6736(22)01659-2. PMID: 36108657Free PMC Article
Utility of KI-67 as a prognostic biomarker in pulmonary neuroendocrine neoplasms: a systematic review and meta-analysis.
Naheed S, Holden C, Tanno L, Pattini L, Pearce NW, Green B, Jaynes E, Cave J, Ottensmeier CH, Pelosi G
BMJ Open 2022 Mar 3;12(3):e041961. doi: 10.1136/bmjopen-2020-041961. PMID: 35241462Free PMC Article
Post-Operative Concurrent Chemoradiation for Patients with Non-Squamous Cell Carcinoma of Head and Neck: A Retrospective Cohort of the Uncommon Cancers.
Thamlikitkul L, Petsuksiri J, Ithimakin S
Asian Pac J Cancer Prev 2019 Jun 1;20(6):1727-1734. doi: 10.31557/APJCP.2019.20.6.1727. PMID: 31244293Free PMC Article
Sorafenib for the Treatment of Progressive Metastatic Medullary Thyroid Cancer: Efficacy and Safety Analysis.
de Castroneves LA, Negrão MV, de Freitas RM, Papadia C, Lima JV Jr, Fukushima JT, Simão EF, Kulcsar MA, Tavares MR, Jorge AA, de Castro G Jr, Hoff PM, Hoff AO
Thyroid 2016 Mar;26(3):414-9. Epub 2016 Feb 9 doi: 10.1089/thy.2015.0334. PMID: 26701095

Prognosis

Utility of KI-67 as a prognostic biomarker in pulmonary neuroendocrine neoplasms: a systematic review and meta-analysis.
Naheed S, Holden C, Tanno L, Pattini L, Pearce NW, Green B, Jaynes E, Cave J, Ottensmeier CH, Pelosi G
BMJ Open 2022 Mar 3;12(3):e041961. doi: 10.1136/bmjopen-2020-041961. PMID: 35241462Free PMC Article
Clinical outcome and comparison between squamous and non-squamous cell carcinoma of the larynx.
Chen L, Deng W, Li C, Lau H, Tao L, Wang S, Zhou L, Zhang M
Acta Otolaryngol 2020 Feb;140(2):195-201. Epub 2019 Dec 18 doi: 10.1080/00016489.2019.1700305. PMID: 31852360
Merkel cell carcinoma of the forehead area: a literature review and case report.
Caldarelli C, Autorino U, Iaquinta C, De Marchi A
Oral Maxillofac Surg 2019 Sep;23(3):365-373. Epub 2019 Jul 24 doi: 10.1007/s10006-019-00793-y. PMID: 31342210
Post-Operative Concurrent Chemoradiation for Patients with Non-Squamous Cell Carcinoma of Head and Neck: A Retrospective Cohort of the Uncommon Cancers.
Thamlikitkul L, Petsuksiri J, Ithimakin S
Asian Pac J Cancer Prev 2019 Jun 1;20(6):1727-1734. doi: 10.31557/APJCP.2019.20.6.1727. PMID: 31244293Free PMC Article
Novel Additions to the AJCC's New Staging Systems for Skin Cancer.
Cañueto J, Román-Curto C
Actas Dermosifiliogr 2017 Nov;108(9):818-826. Epub 2017 Jul 18 doi: 10.1016/j.ad.2017.05.012. PMID: 28732551

Clinical prediction guides

Neuroendocrine and squamous cell phenotypes of NUT carcinoma are potential diagnostic pitfalls that discriminating it from mimickers, such as small cell and squamous cell carcinoma.
Ninomiya H, Sato Y, Inamura K, Dobashi A, Takeuchi K, Mitani H, Mun M, Nishio M, Ishikawa Y
Diagn Pathol 2024 Feb 7;19(1):27. doi: 10.1186/s13000-024-01448-7. PMID: 38326851Free PMC Article
Differences in Merkel Cell Carcinoma Presentation and Outcomes Among Racial and Ethnic Groups.
Mohsin N, Martin MR, Reed DJ, Vilasi SM, Miao L, Hill NT, Brownell I
JAMA Dermatol 2023 May 1;159(5):536-540. doi: 10.1001/jamadermatol.2023.0061. PMID: 36920369Free PMC Article
Combined nivolumab and ipilimumab with or without stereotactic body radiation therapy for advanced Merkel cell carcinoma: a randomised, open label, phase 2 trial.
Kim S, Wuthrick E, Blakaj D, Eroglu Z, Verschraegen C, Thapa R, Mills M, Dibs K, Liveringhouse C, Russell J, Caudell JJ, Tarhini A, Markowitz J, Kendra K, Wu R, Chen DT, Berglund A, Michael L, Aoki M, Wang MH, Hamaidi I, Cheng P, de la Iglesia J, Slebos RJ, Chung CH, Knepper TC, Moran-Segura CM, Nguyen JV, Perez BA, Rose T, Harrison L, Messina JL, Sondak VK, Tsai KY, Khushalani NI, Brohl AS
Lancet 2022 Sep 24;400(10357):1008-1019. Epub 2022 Sep 12 doi: 10.1016/S0140-6736(22)01659-2. PMID: 36108657Free PMC Article
Clinical outcome and comparison between squamous and non-squamous cell carcinoma of the larynx.
Chen L, Deng W, Li C, Lau H, Tao L, Wang S, Zhou L, Zhang M
Acta Otolaryngol 2020 Feb;140(2):195-201. Epub 2019 Dec 18 doi: 10.1080/00016489.2019.1700305. PMID: 31852360
Merkel cell carcinoma of the forehead area: a literature review and case report.
Caldarelli C, Autorino U, Iaquinta C, De Marchi A
Oral Maxillofac Surg 2019 Sep;23(3):365-373. Epub 2019 Jul 24 doi: 10.1007/s10006-019-00793-y. PMID: 31342210

Recent systematic reviews

Utility of KI-67 as a prognostic biomarker in pulmonary neuroendocrine neoplasms: a systematic review and meta-analysis.
Naheed S, Holden C, Tanno L, Pattini L, Pearce NW, Green B, Jaynes E, Cave J, Ottensmeier CH, Pelosi G
BMJ Open 2022 Mar 3;12(3):e041961. doi: 10.1136/bmjopen-2020-041961. PMID: 35241462Free PMC Article
The role of molecular genetics in the clinical management of sporadic medullary thyroid carcinoma: A systematic review.
Fussey JM, Vaidya B, Kim D, Clark J, Ellard S, Smith JA
Clin Endocrinol (Oxf) 2019 Dec;91(6):697-707. Epub 2019 Jul 29 doi: 10.1111/cen.14060. PMID: 31301229

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...