Abstract and Introduction
Abstract
A case of atypical ("pagetoid") compound Spitz nevus on the face of a 2-year-old girl is reported with a review of the literature. The nevus was composed of broad but laterally demarcated compound proliferation of enlarged fusiform and epithelioid melanocytes, with florid pagetoid scatter above the junction. Immunohistochemical analyses revealed the Ki-67 proliferation index to be relatively low. Given the histomorphological overlap with melanoma, an array-based comparative genomic hybridization approach revealed a subthreshold gain in chromosome 1q and gain in distal chromosome 17q, with no other associated chromosomal gains or losses. These molecular aberrations suggested a partially transformed tumor, without adequate evidence for a molecular diagnosis of melanoma. Because of the diagnostic dilemma posed by these lesions, recent research has focused on molecular alterations that may help differentiate Spitz tumors from malignant melanomas.
Introduction
Spitz nevus is an uncommon melanocytic lesion composed of epithelioid and spindled cells, with a remarkable histological resemblance to malignant melanoma. First described in 1948, the diagnosis and management of Spitz tumors are still heavily debated today. Spitz nevi display a morphological and biological spectrum ranging from benign to malignant, with 3 main classifications: Spitz tumor, atypical Spitz tumor, and malignant melanoma.[1] Atypical Spitz tumors, defined as a Spitz tumor with 1 or more atypical features, are a heterogeneous group with indeterminate biological potential. A wide spectrum of histopathological morphologies, classified as atypical Spitz nevi, has been reported and includes desmoplastic, angiomatoid, verrucous, plexiform, pagetoid, halo, myxoid, granulomatous, and tubular presentations.[2] Even among expert dermatopathologists, there exists marked interobserver variability in diagnoses of atypical Spitz tumors and malignant melanoma. Because of the diagnostic dilemma posed by these lesions, recent research has focused on molecular alterations that may help differentiate Spitz tumors from malignant melanomas.
We present a case of 2-year-old girl with an atypical Spitz tumor, with peculiar clinical, histopathological, and molecular based findings.
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