Orbital melanotic neuroectodermal tumor of infancy successfully treated with chemotherapy and subtotal excision.

Abstract

Melanotic neuroectodermal tumor of infancy is a rare condition that typically presents within the first 6 months of life. It rarely metastasizes but is locally aggressive. Melanotic neuroectodermal tumor of infancy has been reported under several other names including retinal anlage tumor and melanotic progonoma. Most commonly, melanotic neuroectodermal tumor of infancy originates in the maxilla. Tumors in previously reported orbital cases have arisen from adjacent structures or from orbital bone. Recommended treatment of melanotic neuroectodermal tumor of infancy has consisted of complete excision with wide tissue margins, although success has been reported after incomplete resection. For those patients in whom surgical resection is not possible, various chemotherapy approaches have been reported with mixed results ranging from failure to sustained tumor regression. We report a case of orbital melanotic neuroectodermal tumor of infancy adherent to sclera and successfully treated with chemotherapy and subtotal excision.

• Authors: Robert W Hered, Walter Smithwick 4th, Eric Sandler, Jeffrey D Goldstein
• Journal: Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus (J AAPOS) Vol. 11 Issue 5 Pg. 504-5 (Oct 2007) ISSN: 1091-8531 [Print] United States
• PMID: 17532239 (Publication Type: Case Reports, Journal Article)

Chemical References

• Vincristine
• Etoposide
• Ifosfamide

Topics

• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Biopsy
• Diagnosis, Differential
• Etoposide (administration & dosage)
• Follow-Up Studies
• Humans
• Ifosfamide (administration & dosage)
• Infant
• Male
• Neuroectodermal Tumor, Melanotic (diagnosis, drug therapy, surgery)
• Ophthalmologic Surgical Procedures (methods)
• Orbital Neoplasms (diagnosis, drug therapy, surgery)
• Tomography, X-Ray Computed
• Vincristine (administration & dosage)