Document Type

Article

Publication Date

6-2004

Publisher

Elsevier

Source Publication

Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology

Source ISSN

2212-4403

Original Item ID

DOI: 10.1016/j.tripleo.2003.11.017

Abstract

Post-irradiation sarcoma is a well-defined complication of radiation therapy, yet few reports document such lesions in the head and neck. A 30-year-old man presented for evaluation of an expansile lesion of the left posterior maxilla. His medical history was significant for a childhood ocular malignancy - unilateral retinoblastoma - which was treated with a combination of surgical enucleation of the eye and external beam radiation therapy. Biopsy of his maxillary lesion revealed a spindle cell malignancy that was morphologically and immunohistochemically consistent with a diagnosis of leiomyosarcoma. Further investigation into the case revealed that the patient had three children, every one of whom developed unilateral retinoblastoma in infancy. Compared to the more frequent presentation of bilateral tumors in hereditary cases of retinoblastoma, such cases of heritable unilateral retinoblastoma are exceptional. Importantly, heritable forms of retinoblastoma confer a significant risk for development of second primary cancers, necessitating long-term clinical follow-up in these patients.

Comments

Accepted version. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology, Vol. 97, No. 6 (June 2004): 726-731. DOI. © 2004 Elsevier. Used with permission.

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