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Pigmented Epithelioid Melanocytoma

MedGen UID:
1693568
•Concept ID:
C5230531
•
Neoplastic Process
Synonyms: Epithelioid Blue Nevus; Pigmented epithelioid melanocytoma
SNOMED CT: Pigmented epithelioid melanocytoma (789445008)

Definition

A cutaneous melanocytic neoplasm composed of heavily pigmented epithelioid and dendritic cells, with metastatic potential limited to regional lymph nodes. It can occur as a sporadic lesion or in patients with Carney complex. (WHO 2018) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPigmented Epithelioid Melanocytoma

Professional guidelines

PubMed

ESP, EORTC, and EURACAN Expert Opinion: practical recommendations for the pathological diagnosis and clinical management of intermediate melanocytic tumors and rare related melanoma variants.
de la Fouchardiere A, Blokx W, van Kempen LC, Luzar B, Piperno-Neumann S, Puig S, Alos L, Calonje E, Massi D; ESP Dermatopathology Working Group; EORTC Melanoma Group; EURACAN
Virchows Arch 2021 Jul;479(1):3-11. Epub 2021 Jan 12 doi: 10.1007/s00428-020-03005-1. PMID: 33432480
Differential diagnosis of heavily pigmented melanocytic lesions: challenges and diagnostic approach.
Aung PP, Mutyambizi KK, Danialan R, Ivan D, Prieto VG
J Clin Pathol 2015 Dec;68(12):963-70. doi: 10.1136/jclinpath-2015-202887. PMID: 26602414

Recent clinical studies

Etiology

Attempting to Solve the Pigmented Epithelioid Melanocytoma (PEM) Conundrum: PRKAR1A Inactivation Can Occur in Different Genetic Backgrounds (Common, Blue, and Spitz Subgroups) With Variation in Their Clinicopathologic Characteristics.
de la Fouchardiere A, Tirode F, Castillo C, Buisson A, Boivin F, Macagno N, Pissaloux D
Am J Surg Pathol 2022 Aug 1;46(8):1106-1115. Epub 2022 Mar 22 doi: 10.1097/PAS.0000000000001888. PMID: 35319526
Pigmented Epithelioid Melanocytoma: Morphology and Molecular Drivers.
Benton S, Zhao J, Asadbeigi S, Kim D, Zhang B, Gerami P
Surg Pathol Clin 2021 Jun;14(2):285-292. Epub 2021 Apr 28 doi: 10.1016/j.path.2021.01.004. PMID: 34023106
ESP, EORTC, and EURACAN Expert Opinion: practical recommendations for the pathological diagnosis and clinical management of intermediate melanocytic tumors and rare related melanoma variants.
de la Fouchardiere A, Blokx W, van Kempen LC, Luzar B, Piperno-Neumann S, Puig S, Alos L, Calonje E, Massi D; ESP Dermatopathology Working Group; EORTC Melanoma Group; EURACAN
Virchows Arch 2021 Jul;479(1):3-11. Epub 2021 Jan 12 doi: 10.1007/s00428-020-03005-1. PMID: 33432480
Distinct Genomic Patterns in Pigmented Epithelioid Melanocytoma: A Molecular and Histologic Analysis of 16 Cases.
Isales MC, Mohan LS, Quan VL, Garfield EM, Zhang B, Shi K, Arva N, Beaubier N, Yazdan P, White K, Taxter TJ, Gerami P
Am J Surg Pathol 2019 Apr;43(4):480-488. doi: 10.1097/PAS.0000000000001195. PMID: 30475255
A systematic review and meta-analysis of animal-type melanoma.
Vyas R, Keller JJ, Honda K, Cooper KD, Gerstenblith MR
J Am Acad Dermatol 2015 Dec;73(6):1031-9. Epub 2015 Sep 26 doi: 10.1016/j.jaad.2015.08.016. PMID: 26412164

Diagnosis

Pigmented Epithelioid Melanocytoma: Morphology and Molecular Drivers.
Benton S, Zhao J, Asadbeigi S, Kim D, Zhang B, Gerami P
Surg Pathol Clin 2021 Jun;14(2):285-292. Epub 2021 Apr 28 doi: 10.1016/j.path.2021.01.004. PMID: 34023106
ESP, EORTC, and EURACAN Expert Opinion: practical recommendations for the pathological diagnosis and clinical management of intermediate melanocytic tumors and rare related melanoma variants.
de la Fouchardiere A, Blokx W, van Kempen LC, Luzar B, Piperno-Neumann S, Puig S, Alos L, Calonje E, Massi D; ESP Dermatopathology Working Group; EORTC Melanoma Group; EURACAN
Virchows Arch 2021 Jul;479(1):3-11. Epub 2021 Jan 12 doi: 10.1007/s00428-020-03005-1. PMID: 33432480
The role of gene fusions in melanocytic neoplasms.
Quan VL, Panah E, Zhang B, Shi K, Mohan LS, Gerami P
J Cutan Pathol 2019 Nov;46(11):878-887. Epub 2019 Jun 20 doi: 10.1111/cup.13521. PMID: 31152596
Unusual Clinical Presentations of Malignant Melanoma: A Review of Clinical and Histologic Features with Special Emphasis on Dermatoscopic Findings.
Cabrera R, Recule F
Am J Clin Dermatol 2018 Nov;19(Suppl 1):15-23. doi: 10.1007/s40257-018-0373-6. PMID: 30374898Free PMC Article
Differential diagnosis of heavily pigmented melanocytic lesions: challenges and diagnostic approach.
Aung PP, Mutyambizi KK, Danialan R, Ivan D, Prieto VG
J Clin Pathol 2015 Dec;68(12):963-70. doi: 10.1136/jclinpath-2015-202887. PMID: 26602414

Therapy

Epithelioid and fusiform blue nevus of chronically sun-damaged skin, an entity distinct from the epithelioid blue nevus of the Carney complex.
Yazdan P, Haghighat Z, Guitart J, Gerami P
Am J Surg Pathol 2013 Jan;37(1):81-8. doi: 10.1097/PAS.0b013e3182608fa5. PMID: 22892599
A case of animal-type melanoma (or pigmented epithelioid melanocytoma?): an open prognosis.
Vezzoni GM, Martini L, Ricci C
Dermatol Surg 2008 Jan;34(1):105-9; discussion 110. Epub 2007 Dec 5 doi: 10.1111/j.1524-4725.2007.34023.x. PMID: 18053035

Prognosis

ESP, EORTC, and EURACAN Expert Opinion: practical recommendations for the pathological diagnosis and clinical management of intermediate melanocytic tumors and rare related melanoma variants.
de la Fouchardiere A, Blokx W, van Kempen LC, Luzar B, Piperno-Neumann S, Puig S, Alos L, Calonje E, Massi D; ESP Dermatopathology Working Group; EORTC Melanoma Group; EURACAN
Virchows Arch 2021 Jul;479(1):3-11. Epub 2021 Jan 12 doi: 10.1007/s00428-020-03005-1. PMID: 33432480
Distinct Genomic Patterns in Pigmented Epithelioid Melanocytoma: A Molecular and Histologic Analysis of 16 Cases.
Isales MC, Mohan LS, Quan VL, Garfield EM, Zhang B, Shi K, Arva N, Beaubier N, Yazdan P, White K, Taxter TJ, Gerami P
Am J Surg Pathol 2019 Apr;43(4):480-488. doi: 10.1097/PAS.0000000000001195. PMID: 30475255
Unusual Clinical Presentations of Malignant Melanoma: A Review of Clinical and Histologic Features with Special Emphasis on Dermatoscopic Findings.
Cabrera R, Recule F
Am J Clin Dermatol 2018 Nov;19(Suppl 1):15-23. doi: 10.1007/s40257-018-0373-6. PMID: 30374898Free PMC Article
Pigmented epithelioid melanocytoma (animal-type melanoma): An institutional experience.
Bax MJ, Brown MD, Rothberg PG, Laughlin TS, Scott GA
J Am Acad Dermatol 2017 Aug;77(2):328-332. Epub 2017 Apr 14 doi: 10.1016/j.jaad.2017.01.029. PMID: 28416343
A systematic review and meta-analysis of animal-type melanoma.
Vyas R, Keller JJ, Honda K, Cooper KD, Gerstenblith MR
J Am Acad Dermatol 2015 Dec;73(6):1031-9. Epub 2015 Sep 26 doi: 10.1016/j.jaad.2015.08.016. PMID: 26412164

Clinical prediction guides

Attempting to Solve the Pigmented Epithelioid Melanocytoma (PEM) Conundrum: PRKAR1A Inactivation Can Occur in Different Genetic Backgrounds (Common, Blue, and Spitz Subgroups) With Variation in Their Clinicopathologic Characteristics.
de la Fouchardiere A, Tirode F, Castillo C, Buisson A, Boivin F, Macagno N, Pissaloux D
Am J Surg Pathol 2022 Aug 1;46(8):1106-1115. Epub 2022 Mar 22 doi: 10.1097/PAS.0000000000001888. PMID: 35319526
ESP, EORTC, and EURACAN Expert Opinion: practical recommendations for the pathological diagnosis and clinical management of intermediate melanocytic tumors and rare related melanoma variants.
de la Fouchardiere A, Blokx W, van Kempen LC, Luzar B, Piperno-Neumann S, Puig S, Alos L, Calonje E, Massi D; ESP Dermatopathology Working Group; EORTC Melanoma Group; EURACAN
Virchows Arch 2021 Jul;479(1):3-11. Epub 2021 Jan 12 doi: 10.1007/s00428-020-03005-1. PMID: 33432480
CYSLTR2-mutant Cutaneous Melanocytic Neoplasms Frequently Simulate "Pigmented Epithelioid Melanocytoma," Expanding the Morphologic Spectrum of Blue Tumors: A Clinicopathologic Study of 7 Cases.
Goto K, Pissaloux D, Paindavoine S, Tirode F, de la Fouchardière A
Am J Surg Pathol 2019 Oct;43(10):1368-1376. doi: 10.1097/PAS.0000000000001299. PMID: 31162285
Dermatopathology updates on melanocytic lesions.
Cheung WL, Smoller BR
Dermatol Clin 2012 Oct;30(4):617-22, vi. Epub 2012 Aug 3 doi: 10.1016/j.det.2012.06.014. PMID: 23021049
Pigmented epithelioid melanocytoma: favorable outcome after 5-year follow-up.
Mandal RV, Murali R, Lundquist KF, Ragsdale BD, Heenan P, McCarthy SW, Mihm MC Jr, Scolyer RA, Zembowicz A
Am J Surg Pathol 2009 Dec;33(12):1778-82. doi: 10.1097/PAS.0b013e3181b94f3c. PMID: 19773637

Recent systematic reviews

Clinical and Dermoscopic Features Associated With Difficult-to-Recognize Variants of Cutaneous Melanoma: A Systematic Review.
Pampena R, Lai M, Lombardi M, Mirra M, Raucci M, Lallas A, Apalla Z, Argenziano G, Pellacani G, Longo C
JAMA Dermatol 2020 Apr 1;156(4):430-439. doi: 10.1001/jamadermatol.2019.4912. PMID: 32101255
A systematic review and meta-analysis of animal-type melanoma.
Vyas R, Keller JJ, Honda K, Cooper KD, Gerstenblith MR
J Am Acad Dermatol 2015 Dec;73(6):1031-9. Epub 2015 Sep 26 doi: 10.1016/j.jaad.2015.08.016. PMID: 26412164

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