Adenocarcinoma of the ethmoid sinus is rare, representing only 4-8% 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.
Retinoblastoma, though occurring only once in every 23,000 to 34,000 births, is the most common malignant intraocular
tumor of childhood. Second fatal mesenchymal and epithelial primaries have been described in 8.5% of patients with bilateral
retinoblastomas previously treated with
radiotherapy; however,
papillary adenocarcinoma arising within the paranasal sinuses has not been reported. 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. Agressive treatment including partial maxillectomy, radical pansinusectomy,
radical neck dissection followed by regional
radiotherapy and systemic
chemotherapy failed to prevent the development of fatal hepatic
metastases. 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.