A clinicopathologic and immunohistochemical analysis of melanotic neuroectodermal tumor of infancy

Melanotic neuroectodermal tumour of infancy

“…Although melanin is produced by the tumour, pigmentation may not be clinically evident (13,14). Local invasion may be accompanied by bony destruction, tooth displacement and feeding difficulties (6,15,16).…”

“…Histology has been proven to be an unreliable guide to clinical outcome (15). Flow cytometry has been attempted, again with little success (25,42).…”

“…(14,28). The nests of smaller cells in MNTI are often positive for neurogenic markers such as synaptophysin, neuron-specific enolase and glial fibrillar acidic protein (15,25,27). The variability seen in staining results likely represents the multiphenotypical character of this tumour (1).…”

“…Surgical excision remains the mainstay of treatment for patients with MNTI, as the only modality with proven efficacy (2,15). Given the benign nature of most tumours, there has been some controversy over the optimal surgical management and necessity of adequate margins.…”

“…Large, epithelioid, melanin-containing cells are arranged in alveolar or tubular formations around clusters of small neuroblastic cells (15,25,26). A differential diagnosis would include other small round cell tumours occurring in infancy, such as rhabdomyosarcoma, neuroblastoma, melanoma and lymphoma.…”