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Adenocarcinoma of the ethmoid following radiotherapy for bilateral retinoblastoma

Journal Article · · Laryngoscope; (United States)
Adenocarcinoma of the ethmoid sinus is rare, representing only 4 to 8% of malignancies of the paranasal sinuses. An extraordinary case of papillary adenocarcinoma of the ethmoid sinus arising 30 years following high-dose radiotherapy for bilateral retinoblastoma is presented. Histologically, the findings of a papillary pattern of poorly differentiated, mucicarmine-staining cells enclosing gland-like spaces, and the absence of pseudorosettes, melanin, mesenchymal and peripheral neural elements supports an epithelial origin of this tumor. The high incidence of second fatal primary neoplasms in patients with bilateral retinoblastomas receiving radiation suggests an innate susceptibility that may add to the risk of radiotherapy. Careful long-term head and neck surveillance is mandatory if early aggressive management of these extremely lethal tumors is to be successful.
Research Organization:
Univ. of California, San Francisco
OSTI ID:
6120469
Journal Information:
Laryngoscope; (United States), Journal Name: Laryngoscope; (United States) Vol. 80:1; ISSN LARYA
Country of Publication:
United States
Language:
English