Two Congenital Cases of Pigmented Epithelioid Melanocytoma Studied by Fluorescent in situ Hybridization for Melanocytic Tumors: Case Reports and Review of These Recent TopicsBattistella M. · Prochazkova-Carlotti M. · Berrebi D. · Bennaceur S. · Edan C. · Riffaud L. · Rütten A. · Fraitag S.
aDermatology and bPathology, Necker-Enfants Malades Hospital, APHP, Paris V – Descartes University, and cPathology and dPlastic Surgery, Robert-Debré Hospital, APHP, Paris, eHistology and Molecular Pathology of Tumours, EA 2406, Victor Segalen Bordeaux 2 University, Bordeaux, and fDepartment of Pediatrics and gNeurosurgery, Rennes University Hospital, Rennes, France; hDermatopathologische Gemeinschaftspraxis, Friedrichshafen, Germany
The term ‘pigmented epithelioid melanocytoma’ (PEM) has recently been proposed as a nosological framework grouping lesions formerly known as animal-type melanomas, sporadic epithelioid blue nevi and Carney complex-associated epithelioid blue nevi. Congenital PEMs have been reported extremely rarely and their prognosis is poorly known. Four-color fluorescent in situ hybridization (FISH) for melanocytic lesions is a recent method developed to assess the malignant potential of ambiguous melanocytic lesions. Here we describe 2 cases of congenital epithelioid and strongly pigmented melanocytic lesions consistent with PEM. No BRAF gene mutation was found in the 2 cases. FISH for melanocytic lesions was also performed. The first case proved entirely negative, whereas the second one showed a positive zone with an extra copy of chromosome 6. The prognosis and management of PEM are discussed, with a review of the available data on the history, demographics, molecular alterations and histopathological aspects of this entity. PEM seems to represent a unique low-grade melanocytic tumor with a limited potential of metastasis to lymph nodes, but a favorable long-term clinical course. The published data about FISH for melanocytic tumors, and especially PEM, are reviewed. Four-color FISH may be a useful tool to assess more accurately the prognosis of these tumors.
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Received: December 4, 2009
Accepted after revision: April 20, 2010
Published online: June 18, 2010
Number of Print Pages : 10
Number of Figures : 5, Number of Tables : 1, Number of References : 69
Vol. 221, No. 2, Year 2010 (Cover Date: August 2010)
Journal Editor: Saurat J.-H. (Geneva)
ISSN: 1018-8665 (Print), eISSN: 1421-9832 (Online)
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