Original ArticlePigmented Epithelioid Melanocytoma: A Low-grade Melanocytic Tumor With Metastatic Potential Indistinguishable From Animal-type Melanoma and Epithelioid Blue NevusZembowicz, Artur MD, PhD*; Carney, J. Aidan†; Mihm, Martin C.*Author Information From the *Dermatopathology Unit, Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA; and †Department of Laboratory Medicine & Pathology (Emeritus Member), Mayo Clinic, Rochester, MN. Reprints: Artur Zembowicz, MD, PhD, Dermatopathology Unit, Massachusetts General Hospital, Warren 820, 55 Fruit Street, Boston, MA 02114 (e-mail: firstname.lastname@example.org). The American Journal of Surgical Pathology: January 2004 - Volume 28 - Issue 1 - p 31-40 Buy SDC Abstract In the course of a study of borderline melanocytic tumors, we observed a distinctive group of lesions characterized by features very similar to those previously described in the literature as “animal-type melanoma” and epithelioid blue nevus of Carney complex. We have designated these lesions as pigmented epithelioid melanocytoma (PEM). Herein, we present a clinical-pathologic analysis of 41 consecutive PEM from 40 patients and compare them with 11 epithelioid blue nevi from patients with Carney complex. PEM occurred in both sexes of different ethnic backgrounds, including white, Hispanic, black, Asian, and Persian. The median age of occurrence was 27 years (range 0.6–78 years). Tumors had wide distribution with extremities being the most common site. The tumors were formed by deep dermal (mean Breslow's thickness 3.3 mm) proliferation of heavily pigmented epithelioid and/or spindled melanocytes. Five lesions were part of combined nevus. Ulceration was present in 7 cases. Tumor necrosis was present in 1 case. Regional lymph nodes were sampled in 24 cases (59%). In 11 cases, lymph nodes contained metastases (46%). Liver metastases occurred in 1 case. None of the patients died of disease. Clinical follow-up of more than a year (mean 32 months, range up to 67 months) was available in 27 cases (67%). We found no histologic criteria separating metastasizing and nonmetastasizing PEM. Ulceration was the only feature more common in PEM than epithelioid blue nevi of Carney complex. Otherwise, they were histologically indistinguishable. Our data show that PEM is a unique low-grade variant of melanoma with frequent lymph node metastases but indolent clinical course. We suggest that PEM be considered as a provisional histologic entity encompassing both animal-type melanoma and epithelioid blue nevus. © 2004 Lippincott Williams & Wilkins, Inc.