Pigmented Neuroectodermal Tumor of Infancy

Pigmented neuroectodermal tumor of infancy is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of newborn infants.We report a 4-month-old male baby admitted to Mofid Children’s Hospital with an asymptomatic swelling in posterior region of the left ear. His parents noticed the swelling at the age of 2 months, which was grew rapidly in a period of 2 months. There was no history of medication during pregnancy and no relevant medical history was present. On clinical examination, a 3x2 cm, non-ulcerative, non-tender, firm, immobile, non-fluctuant, swelling was detected in the posterior auricular region. Radiographically, the lesion appeared as a radiolucent mass with ill-defined and irregular borders suggestive of osteolytic lesion. Routine laboratory investigations were within normal limits. The pre-operative diagnosis was Langerhans cell histiocytosis. The excisional biopsy consisted of multiple fragments of irregular tan-brown soft tissue totally measuring 3.7 x 2.5 x 1.5 cm. Microscopically, the typical features of a pigmented neuroectodermal tumor of infancy were present, characterized by two populations of cells, the large melanotic (pigmented) type and the small (non-pigmented) type. The small cells had morphologic features of primitive neuroepithelial cells while the larger cells had features of melanocytes.We report this case because it is a rare tumor.

Keywords:

Pigmented neuroectodermal tumor of infancy , bone