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Extracutaneous Merkel Cell Carcinoma

MedGen UID:
1766390
•Concept ID:
C5420281
•
Neoplastic Process
Synonyms: Merkel Cell Carcinoma of Lymph Node; Merkel Cell Carcinoma of Unknown Primary; Nodal Merkel Cell Carcinoma; Unknown Primary Merkel Cell Carcinoma

Definition

An extremely rare Merkel cell carcinoma affecting the lymph nodes. It may represent metastasis from a regressed dermal primary. (WHO 2017) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtracutaneous Merkel Cell Carcinoma

Professional guidelines

PubMed

Advances in the Treatment of Advanced Extracutaneous Melanomas and Nonmelanoma Skin Cancers.
Komatsubara KM, Jeter J, Carvajal RD, Margolin K, Schadendorf D, Hauschild A
Am Soc Clin Oncol Educ Book 2017;37:641-650. doi: 10.1200/EDBK_175265. PMID: 28561682

Recent clinical studies

Etiology

Cutaneous Neuroendocrine Mucinous Carcinomas Are Low-grade But May Be Associated With Other Cancers.
Adkins KE, Busam K, Pulitzer M
Am J Surg Pathol 2023 Oct 1;47(10):1186-1191. Epub 2023 Aug 3 doi: 10.1097/PAS.0000000000002107. PMID: 37530225Free PMC Article
Merkel cell carcinoma of the anorectum: a case report and review of the literature.
van Wyk AC, Moolla Z, Motala AI, Roberts R, Chotey NA, Lakhi HI, Becker JC
Clin J Gastroenterol 2022 Aug;15(4):740-745. Epub 2022 Jun 28 doi: 10.1007/s12328-022-01648-y. PMID: 35764912
INSM1 Is More Sensitive and Interpretable than Conventional Immunohistochemical Stains Used to Diagnose Merkel Cell Carcinoma.
Lilo MT, Chen Y, LeBlanc RE
Am J Surg Pathol 2018 Nov;42(11):1541-1548. doi: 10.1097/PAS.0000000000001136. PMID: 30080705
Clinical and dermoscopic features of combined cutaneous squamous cell carcinoma (SCC)/neuroendocrine [Merkel cell] carcinoma (MCC).
Suárez AL, Louis P, Kitts J, Busam K, Myskowski PL, Wong RJ, Chen CS, Spencer P, Lacouture M, Pulitzer MP
J Am Acad Dermatol 2015 Dec;73(6):968-75. Epub 2015 Oct 2 doi: 10.1016/j.jaad.2015.08.041. PMID: 26433246Free PMC Article
Skin cancer in organ transplant recipients.
Kempf W, Mertz KD, Hofbauer GF, Tinguely M
Pathobiology 2013;80(6):302-9. Epub 2013 Aug 30 doi: 10.1159/000350757. PMID: 24013135

Diagnosis

Merkel cell carcinoma of the anorectum: a case report and review of the literature.
van Wyk AC, Moolla Z, Motala AI, Roberts R, Chotey NA, Lakhi HI, Becker JC
Clin J Gastroenterol 2022 Aug;15(4):740-745. Epub 2022 Jun 28 doi: 10.1007/s12328-022-01648-y. PMID: 35764912
Investigation of the RB1-SOX2 axis constitutes a tool for viral status determination and diagnosis in Merkel cell carcinoma.
Thanguturi S, Tallet A, Miquelestorena-Standley E, Coco C, Le Corre Y, Hainaut-Wierzbicka E, Blom A, Saiag P, Beneton N, Bens G, Zaragoza J, Nardin C, Aubin F, Dinulescu M, Machet MC, Houben R, Schrama D, Collin C, Fromont G, Jullie ML, Macagno N, Gaboriaud P, Berthon P, Touzé A, Guyétant S, Samimi M, Kervarrec T
Virchows Arch 2022 Jun;480(6):1239-1254. Epub 2022 Apr 12 doi: 10.1007/s00428-022-03315-6. PMID: 35412101
MOC-31 Antibody Immunolabeling During Mohs Surgery.
Bae E, Saap L, Breen C, Iwamoto S
Dermatol Surg 2022 Jun 1;48(6):595-600. Epub 2022 Apr 5 doi: 10.1097/DSS.0000000000003446. PMID: 35384902
Advances in the Treatment of Advanced Extracutaneous Melanomas and Nonmelanoma Skin Cancers.
Komatsubara KM, Jeter J, Carvajal RD, Margolin K, Schadendorf D, Hauschild A
Am Soc Clin Oncol Educ Book 2017;37:641-650. doi: 10.1200/EDBK_175265. PMID: 28561682
Skin cancer in organ transplant recipients.
Kempf W, Mertz KD, Hofbauer GF, Tinguely M
Pathobiology 2013;80(6):302-9. Epub 2013 Aug 30 doi: 10.1159/000350757. PMID: 24013135

Therapy

Skin cancer in organ transplant recipients.
Kempf W, Mertz KD, Hofbauer GF, Tinguely M
Pathobiology 2013;80(6):302-9. Epub 2013 Aug 30 doi: 10.1159/000350757. PMID: 24013135

Prognosis

Cutaneous Neuroendocrine Mucinous Carcinomas Are Low-grade But May Be Associated With Other Cancers.
Adkins KE, Busam K, Pulitzer M
Am J Surg Pathol 2023 Oct 1;47(10):1186-1191. Epub 2023 Aug 3 doi: 10.1097/PAS.0000000000002107. PMID: 37530225Free PMC Article
Investigation of the RB1-SOX2 axis constitutes a tool for viral status determination and diagnosis in Merkel cell carcinoma.
Thanguturi S, Tallet A, Miquelestorena-Standley E, Coco C, Le Corre Y, Hainaut-Wierzbicka E, Blom A, Saiag P, Beneton N, Bens G, Zaragoza J, Nardin C, Aubin F, Dinulescu M, Machet MC, Houben R, Schrama D, Collin C, Fromont G, Jullie ML, Macagno N, Gaboriaud P, Berthon P, Touzé A, Guyétant S, Samimi M, Kervarrec T
Virchows Arch 2022 Jun;480(6):1239-1254. Epub 2022 Apr 12 doi: 10.1007/s00428-022-03315-6. PMID: 35412101
INSM1 Is More Sensitive and Interpretable than Conventional Immunohistochemical Stains Used to Diagnose Merkel Cell Carcinoma.
Lilo MT, Chen Y, LeBlanc RE
Am J Surg Pathol 2018 Nov;42(11):1541-1548. doi: 10.1097/PAS.0000000000001136. PMID: 30080705
Skin cancer in organ transplant recipients.
Kempf W, Mertz KD, Hofbauer GF, Tinguely M
Pathobiology 2013;80(6):302-9. Epub 2013 Aug 30 doi: 10.1159/000350757. PMID: 24013135
Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study.
Albores-Saavedra J, Batich K, Chable-Montero F, Sagy N, Schwartz AM, Henson DE
J Cutan Pathol 2010 Jan;37(1):20-7. Epub 2009 Jul 23 doi: 10.1111/j.1600-0560.2009.01370.x. PMID: 19638070

Clinical prediction guides

Investigation of the RB1-SOX2 axis constitutes a tool for viral status determination and diagnosis in Merkel cell carcinoma.
Thanguturi S, Tallet A, Miquelestorena-Standley E, Coco C, Le Corre Y, Hainaut-Wierzbicka E, Blom A, Saiag P, Beneton N, Bens G, Zaragoza J, Nardin C, Aubin F, Dinulescu M, Machet MC, Houben R, Schrama D, Collin C, Fromont G, Jullie ML, Macagno N, Gaboriaud P, Berthon P, Touzé A, Guyétant S, Samimi M, Kervarrec T
Virchows Arch 2022 Jun;480(6):1239-1254. Epub 2022 Apr 12 doi: 10.1007/s00428-022-03315-6. PMID: 35412101
Neurofilament is superior to cytokeratin 20 in supporting cutaneous origin for neuroendocrine carcinoma.
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
INSM1 Is More Sensitive and Interpretable than Conventional Immunohistochemical Stains Used to Diagnose Merkel Cell Carcinoma.
Lilo MT, Chen Y, LeBlanc RE
Am J Surg Pathol 2018 Nov;42(11):1541-1548. doi: 10.1097/PAS.0000000000001136. PMID: 30080705
Primary gastric Merkel cell carcinoma harboring DNA polyomavirus: first description of an unusual high-grade neuroendocrine carcinoma.
Capella C, Marando A, Longhi E, Bernasconi B, Finzi G, Parravicini C, Sessa F, La Rosa S
Hum Pathol 2014 Jun;45(6):1310-4. Epub 2014 Feb 6 doi: 10.1016/j.humpath.2014.01.019. PMID: 24709111
Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study.
Albores-Saavedra J, Batich K, Chable-Montero F, Sagy N, Schwartz AM, Henson DE
J Cutan Pathol 2010 Jan;37(1):20-7. Epub 2009 Jul 23 doi: 10.1111/j.1600-0560.2009.01370.x. PMID: 19638070

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