Long Term Follow-Up in Patients with Pigmented Epithelioid Melanocytoma
RV Mandal, R Murali, KF Lundquist, BD Ragsdale, PJ Heenan, SW McCarthy, MJ Mihm, RA Scolyer, A Zembowicz. Massachusetts General Hospital, Boston, MA; Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Central Coast Pathology, San Luis Obispo, CA; Cutaneous Pathology, Nedlands, Western Australia, Australia; Lahey Clinic, Burlington, MA; DermatopathologyConsultations.com, Belmont, MA
Background: Pigmented epithelioid melanocytoma (PEM) is a recently defined entity encompassing epithelioid blue nevus of Carney complex and some tumors previously considered as a so-called animal-type melanoma. Loss of the Carney complex gene, protein kinase regulatory subunit R1 is observed in the majority of sporadic and Carney complex-associated PEMs. PEMs frequently metastasize to lymph nodes, and yet the initial average 2 year follow-up suggested a more favorable outcome than for conventional melanomas.
Design: In this report, we detail long-term follow up of 28 patients from North America and Australia with PEM.
Results: The patient's median age was 21 years, with 9 males and 16 females. Nine of the patients developed sentinel node metastases. After a median of 67 months of follow-up (range 39 to 216 months), all patients are alive and free of disease.
Conclusions: These findings provide further evidence that PEM is a unique low-grade melanocytic tumor with limited metastatic potential (to lymph nodes), but an indolent long-term clinical course.
Wednesday, March 11, 2009 9:30 AM
Poster Session V # 51, Wednesday Morning