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Cutaneous neuroendocrine carcinoma

MedGen UID:
2843
Concept ID:
C0007129
Neoplastic Process
Synonyms: Merkel cell cancer; Merkel cell carcinoma; Merkel cell skin cancer; Merkle tumors
SNOMED CT: Primary cutaneous neuroendocrine carcinoma (5052009); Merkel cell carcinoma (253001006); Merkel cell tumor (253001006); Apudoma of skin (254729005); Merkel cell carcinoma (5052009); Merkel cell tumor (5052009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0030447
Monarch Initiative: MONDO:0019210
Orphanet: ORPHA79140

Definition

A malignant cutaneous tumor of the elderly that is characterized by an aggressive course with regional nodal involvement, distant metastases and a high rate of recurrence. Most patients present with rapidly growing, painless, firm, non-tender, dome-shaped red, occasionally ulcerated skin nodules, which have a red or bluish color, measuring up to several centimeters, on predominantly sun-exposed areas of the body. The overlying skin is smooth and shiny, sometimes exhibiting ulcerative, acneiform or telangiectatic features. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCutaneous neuroendocrine carcinoma

Professional guidelines

PubMed

Singh N, McClure EM, Akaike T, Park SY, Huynh ET, Goff PH, Nghiem P
Curr Treat Options Oncol 2023 Sep;24(9):1231-1258. Epub 2023 Jul 5 doi: 10.1007/s11864-023-01118-8. PMID: 37403007Free PMC Article
Spada F, Bossi P, Caracò C, Sileni VC, Dei Tos AP, Fazio N, Grignani G, Maio M, Quaglino P, Queirolo P, Ascierto PA; DELPHI Panel Members
J Immunother Cancer 2022 Jun;10(6) doi: 10.1136/jitc-2022-004742. PMID: 35701070Free PMC Article
Weber MM, Fottner C
Oncol Res Treat 2018;41(5):306-312. Epub 2018 Apr 26 doi: 10.1159/000488996. PMID: 29742518

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Merkel Cell Carcinoma, 2023

Recent clinical studies

Etiology

Nayak AL, Pickett AT, Delisle M, Dingley B, Mallick R, Hamilton T, Stuart H, Talbot M, McKinnon G, Jost E, Thiboutot E, Francescutti V, Samman S, Easson A, Schellenberg A, Merchant S, La J, Vanderbeck K, Wright F, Berger-Richardson D, Hebbard P, Hershorn O, Younan R, Patocskai E, Rodriguez-Qizilbash S, Meguerditichian A, Tchuente V, Kazandjian S, Mathieson A, Hossain F, Hetu J, Corsten M, Tohmé A, Nessim C, Johnson-Obaseki S
JAMA Netw Open 2023 Nov 1;6(11):e2344127. doi: 10.1001/jamanetworkopen.2023.44127. PMID: 37983027Free PMC Article
Singh N, McClure EM, Akaike T, Park SY, Huynh ET, Goff PH, Nghiem P
Curr Treat Options Oncol 2023 Sep;24(9):1231-1258. Epub 2023 Jul 5 doi: 10.1007/s11864-023-01118-8. PMID: 37403007Free PMC Article
Tetzlaff MT, Nagarajan P
Head Neck Pathol 2018 Mar;12(1):31-43. Epub 2018 Mar 20 doi: 10.1007/s12105-018-0898-2. PMID: 29556962Free PMC Article
Campillo R, Gil-Carcedo E, Alonso D, Vallejo LA, Oñate JM, Gil-Carcedo LM
Acta Otorrinolaringol Esp 2013 Nov-Dec;64(6):396-402. Epub 2013 Oct 4 doi: 10.1016/j.otorri.2013.06.003. PMID: 24094946
Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707

Diagnosis

Singh N, McClure EM, Akaike T, Park SY, Huynh ET, Goff PH, Nghiem P
Curr Treat Options Oncol 2023 Sep;24(9):1231-1258. Epub 2023 Jul 5 doi: 10.1007/s11864-023-01118-8. PMID: 37403007Free PMC Article
Walsh NM, Cerroni L
J Cutan Pathol 2021 Mar;48(3):411-421. Epub 2020 Dec 3 doi: 10.1111/cup.13910. PMID: 33128463
Tetzlaff MT, Nagarajan P
Head Neck Pathol 2018 Mar;12(1):31-43. Epub 2018 Mar 20 doi: 10.1007/s12105-018-0898-2. PMID: 29556962Free PMC Article
Pulitzer MP, Amin BD, Busam KJ
Adv Anat Pathol 2009 May;16(3):135-44. doi: 10.1097/PAP.0b013e3181a12f5a. PMID: 19395876
Veness MJ
Australas J Dermatol 2006 Aug;47(3):160-5. doi: 10.1111/j.1440-0960.2006.00263.x. PMID: 16866994

Therapy

Singh N, McClure EM, Akaike T, Park SY, Huynh ET, Goff PH, Nghiem P
Curr Treat Options Oncol 2023 Sep;24(9):1231-1258. Epub 2023 Jul 5 doi: 10.1007/s11864-023-01118-8. PMID: 37403007Free PMC Article
Dika E, Pellegrini C, Lambertini M, Patrizi A, Ventura A, Baraldi C, Cardelli L, Mussi M, Fargnoli MC
Eur J Dermatol 2021 Dec 1;31(6):691-701. doi: 10.1684/ejd.2021.4170. PMID: 35107067
Walsh NM, Cerroni L
J Cutan Pathol 2021 Mar;48(3):411-421. Epub 2020 Dec 3 doi: 10.1111/cup.13910. PMID: 33128463
Weber MM, Fottner C
Oncol Res Treat 2018;41(5):306-312. Epub 2018 Apr 26 doi: 10.1159/000488996. PMID: 29742518
Veness MJ
Australas J Dermatol 2006 Aug;47(3):160-5. doi: 10.1111/j.1440-0960.2006.00263.x. PMID: 16866994

Prognosis

Walsh NM, Cerroni L
J Cutan Pathol 2021 Mar;48(3):411-421. Epub 2020 Dec 3 doi: 10.1111/cup.13910. PMID: 33128463
Weber MM, Fottner C
Oncol Res Treat 2018;41(5):306-312. Epub 2018 Apr 26 doi: 10.1159/000488996. PMID: 29742518
Tetzlaff MT, Nagarajan P
Head Neck Pathol 2018 Mar;12(1):31-43. Epub 2018 Mar 20 doi: 10.1007/s12105-018-0898-2. PMID: 29556962Free PMC Article
Pulitzer MP, Amin BD, Busam KJ
Adv Anat Pathol 2009 May;16(3):135-44. doi: 10.1097/PAP.0b013e3181a12f5a. PMID: 19395876
Veness MJ
Australas J Dermatol 2006 Aug;47(3):160-5. doi: 10.1111/j.1440-0960.2006.00263.x. PMID: 16866994

Clinical prediction guides

Duarte-Bateman D, Shen A, Bullock T, Sadeghi P, Escandón JM, Dedkova E, Gastman BR
Mol Carcinog 2023 Jan;62(1):101-112. Epub 2022 Nov 11 doi: 10.1002/mc.23483. PMID: 36367533Free PMC Article
Harms KL, Zhao L, Johnson B, Wang X, Carskadon S, Palanisamy N, Rhodes DR, Mannan R, Vo JN, Choi JE, Chan MP, Fullen DR, Patel RM, Siddiqui J, Ma VT, Hrycaj S, McLean SA, Hughes TM, Bichakjian CK, Tomlins SA, Harms PW
Clin Cancer Res 2021 May 1;27(9):2494-2504. Epub 2021 Feb 5 doi: 10.1158/1078-0432.CCR-20-0864. PMID: 33547200Free PMC Article
Stanoszek LM, Chan MP, Palanisamy N, Carskadon S, Siddiqui J, Patel RM, Harms KL, Lowe L, Fullen DR, Harms PW
Histopathology 2019 Feb;74(3):504-513. Epub 2018 Dec 5 doi: 10.1111/his.13758. PMID: 30239030
Weber MM, Fottner C
Oncol Res Treat 2018;41(5):306-312. Epub 2018 Apr 26 doi: 10.1159/000488996. PMID: 29742518
Pulitzer MP, Amin BD, Busam KJ
Adv Anat Pathol 2009 May;16(3):135-44. doi: 10.1097/PAP.0b013e3181a12f5a. PMID: 19395876

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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    • 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.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Merkel Cell Carcinoma, 2023

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