Second malignant neoplasms of the head and neck in survivors of retinoblastoma.

Journal Article (Journal Article)

Retinoblastoma is a malignant tumor of the embryonic retina. Although it is rare, it is the most common primary eye tumor of childhood. Life expectancy following treatment is now excellent, but survivors who have heritable retinoblastoma face an increased risk of a second malignant head or neck neoplasm. A second neoplasm, which often occurs in the irradiated field of the original tumor, has become the most significant threat to the survival of these patients. We report the case of a young girl who was cured of her retinoblastoma only to later develop a second nonocular tumor that metastasized to the superficial parotid gland. She underwent a superficial parotidectomy and neck dissection, but the malignancy eventually recurred and required further surgery and radiation therapy. In this article, we discuss the etiology, incidence, sites of occurrence, and management options for a second malignant neoplasm in retinoblastoma survivors. The head and neck surgeon must be vigilant in the diagnosis and management of second neoplasms in this patient population because they often occur in irradiated fields; surgical management is important to patient survival.

Full Text

Duke Authors

Cited Authors

  • Wenzel, CT; Halperin, EC; Fisher, SR

Published Date

  • February 2001

Published In

Volume / Issue

  • 80 / 2

Start / End Page

  • 106 - 112

PubMed ID

  • 11233341

International Standard Serial Number (ISSN)

  • 0145-5613


  • eng

Conference Location

  • United States