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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
Veness MJ
Australas J Dermatol 2006 Aug;47(3):160-5. doi: 10.1111/j.1440-0960.2006.00263.x. PMID: 16866994

Curated

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

Recent clinical studies

Etiology

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
Harms KL, Chubb H, Zhao L, Fullen DR, Bichakjian CK, Johnson TM, Carskadon S, Palanisamy N, Harms PW
Hum Pathol 2017 Sep;67:78-84. Epub 2017 Jul 21 doi: 10.1016/j.humpath.2017.07.009. PMID: 28739498
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

Mohamed N, Rampisela D, Gowan AC
Dermatol Online J 2023 Jun 15;29(3) doi: 10.5070/D329361428. PMID: 37591268
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
Harms KL, Chubb H, Zhao L, Fullen DR, Bichakjian CK, Johnson TM, Carskadon S, Palanisamy N, Harms PW
Hum Pathol 2017 Sep;67:78-84. Epub 2017 Jul 21 doi: 10.1016/j.humpath.2017.07.009. PMID: 28739498
Eng TY, Boersma MG, Fuller CD, Goytia V, Jones WE 3rd, Joyner M, Nguyen DD
Am J Clin Oncol 2007 Dec;30(6):624-36. doi: 10.1097/COC.0b013e318142c882. PMID: 18091058

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
Pulitzer MP, Amin BD, Busam KJ
Adv Anat Pathol 2009 May;16(3):135-44. doi: 10.1097/PAP.0b013e3181a12f5a. PMID: 19395876
Eng TY, Boersma MG, Fuller CD, Goytia V, Jones WE 3rd, Joyner M, Nguyen DD
Am J Clin Oncol 2007 Dec;30(6):624-36. doi: 10.1097/COC.0b013e318142c882. PMID: 18091058
Azizi L, Marsot-Dupuch K, Bigel P, Mzali S, Meyer B, Tubiana JM
AJNR Am J Neuroradiol 2001 Aug;22(7):1389-93. PMID: 11498434Free PMC Article
Rodrigues LK, Leong SP, Kashani-Sabet M, Wong JH
J Am Acad Dermatol 2001 Aug;45(2):303-8. doi: 10.1067/mjd.2001.114749. PMID: 11464197

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
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
Rodrigues LK, Leong SP, Kashani-Sabet M, Wong JH
J Am Acad Dermatol 2001 Aug;45(2):303-8. doi: 10.1067/mjd.2001.114749. PMID: 11464197

Supplemental Content

Table of contents

    Clinical resources

    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.

    Curated

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

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