Pigmented Neuroepithelial Tumor

Introduction: Pigmented neuroepithelial tumor is a rare tumor. About 200 cases has been reported till now. It is characteristically occur in maxilla & has a typical bluish color due to the presence of melanin. It is benign tumor with 2% chances of malignancy. It is locally aggressive. Krompecker described it first in 1918 Many names given as the cell of origin was not clear ( Pigmented ameloblastoma, Retinal anlage tumor, Melanotic adamantinoma, Retinal choristoma)

Presentation: Majority present in1st year of life. Median age of occurrence is 4.3 months. There is no sexual predilection.

Swelling in the region of oral cavity

Often feeding & sucking impaired

Sites: More than 90%- head & neck region. Common site- anterior part of maxilla. Other sites are skull, mandible, & brain. The lesion is usually solitary & not multiple. Mucosa over the lesion is usually normal.

Investigations: There are no characteristic radiological findings so pathology remains the cornerstone of diagnosis.

Plain radiograph & CT/MRI: Well-circumscribed, low-density lesion without calcification typically arising from maxilla. As the tumor grows, bone is destroyed suggesting a malignant process.

Treatment: Treatment of choice is complete surgical excision. Developing teeth & surrounding tissues may need to be sacrificed to attain an adequate surgical margin.

Recurrence rate is 10-60%.Radiotherapy & Chemotherapy are used for inoperable recurrence or margin positive resection. Close follow up is necessary to detect recurrence

Permanent reconstruction can be done after growth is completed