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H. Demirci, C.C. Nelson, L.J. Marentette; The Transglabellar / Subcranial Approach for Treatment of Periocular Second Malignant Neoplasms After Retinoblastoma . Invest. Ophthalmol. Vis. Sci. 2006;47(13):2826.
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© ARVO (1962-2015); The Authors (2016-present)
To describe a new surgical approach for the treatment of periocular second non–ocular tumors in patients with retinoblastoma.
Seven consecutive retinoblastoma patients with periocular second non–ocular tumors were treated with transglabellar/subcranial approach to the anterior skull base alone or in combination with chemotherapy and radiotherapy. The clinical features, management and prognosis of these patients were evaluated retrospectively.
All seven patients had hereditary, bilateral retinoblastoma and were treated with external beam radiotherapy. Six patients had unilateral enucleation and one patient underwent bilateral enucleation. The mean time interval between the management of retinoblastoma and development of periocular second malignant tumor was 29 years. Difficulty in maintaining the prosthesis was the most common presenting symptom in 3 patients (43%), epistaxis was present in 2 patients (29%), palpable mass in 1 patient (14%), persistent swelling in 1 (14%), visual loss in 1 (14%), nasal congestion in 1(14%) and frontal headache in 1 patient (14%). Histopathologic diagnosis of the second nonocular tumors were leiomyosarcoma in 3 patients (43%), osteosarcoma in 3 patients (43%) and sphenoid wing meningioma in 1 patient (14%). The transglabellar/subcranial approach was the only treatment in 1 patient (14%) and was combined with external beam radiotherapy and chemotherapy in 3 patients (43%) and with chemotherapy in 3 patients (43%). After a 32 months follow–up, three patients (43%) were alive and four patients (57%) were dead due to second tumor.
The patients with second tumors following retinoblastoma have a poor prognosis. The transglabellar/subcranial approach provides a good exposure to the anterior skull base tumors and wider resection of tumor, allowing less adjuvant therapy.
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