| Autor: |
Zaaroura H; Department of Paediatrics, University of Toronto, Toronto, Canada Division of Dermatology, Hospital for Sick Children, Toronto, Canada. Zaarourahiba@gmail.com., Cyrenne B, Somers GR, Wong KW, Davidge KM, Propst EJ, Kulkarni AV, Lo W, Villani A, Lara-Corrales I, Levy R |
| Jazyk: |
angličtina |
| Zdroj: |
Dermatology online journal [Dermatol Online J] 2023 Oct 15; Vol. 29 (5). Date of Electronic Publication: 2023 Oct 15. |
| DOI: |
10.5070/D329562403 |
| Abstrakt: |
Pigmented epithelioid melanocytomas (PEM) are intermediate-grade melanocytic lesions with frequent lymph node involvement and rare metastases that tend to follow an indolent course with a favorable outcome. 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. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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