Two congenital cases of pigmented epithelioid melanocytoma with unique clinical and genetic features

We report two unique cases of congenital PEM with PRKCA fusion transcripts: a multifocal PEM with an aggressive incompletely resectable scalp tumor and a solitary palmar PEM with newly reported ITGB5-PRKCA fusion. Through these case reports and a summary of previously reported cases, we outline the spectrum of disease of PEM and highlight the key clinical and histopathologic features associated with PEM with PRKCA fusion transcripts. We also discuss the treatment options and suggest that surgical excision without further adjuvant systemic treatment is reasonable first-line therapy given the favorable prognosis.PMID:38478639 | DOI:10.5070/D329562403

Source: Dermatol Online J - March 13, 2024 Category: Dermatology Authors: Hiba Zaaroura Benoit Cyrenne Gino R Somers Karen Wy Wong Kristen M Davidge Evan J Propst Abhaya V Kulkarni Winnie Lo Anita Villani Irene Lara-Corrales Rebecca Levy Source Type: research