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Pigmented epithelioid melanocytoma
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Pigmented epithelioid melanocytoma

Contributors: Steven Nguyen BLA, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Pigmented epithelioid melanocytoma (PEM; also known as atypical epithelioid blue nevus [EBN] and animal-type melanoma) is a rare, heavily pigmented melanocytic neoplasm with borderline malignant potential (categorized by the World Health Organization [WHO] as a "high-grade intermediate melanocytic tumor"). It frequently involves regional lymph nodes. Distant metastases are very rare, and deaths from PEM are not typical but have been reported.

PEM is seen in men and women of every race / ethnicity and age, but it is most commonly found in children, teenagers, and young adults (the median age is 20-27 years). It can occur in any part of the body, including the genital area and on the conjunctiva, but most of the lesions have been described on the extremities, followed by the head and neck.

PEM may arise in association with another melanocytic nevus, such as a compound nevus. This type is known as the combined type of PEM, or epithelioid blue nevus. Combined PEMs are a manifestation of Carney complex.

Codes

ICD10CM:
C44.90 – Unspecified malignant neoplasm of skin, unspecified
C49.9 – Malignant neoplasm of connective and soft tissue, unspecified

SNOMEDCT:
789445008 – Pigmented epithelioid melanocytoma

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Therapy

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References

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Last Reviewed:12/19/2023
Last Updated:12/21/2023
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Pigmented epithelioid melanocytoma
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A medical illustration showing key findings of Pigmented epithelioid melanocytoma
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