The Transglabellar/Subcranial Approach for Surgical Excision of Periocular Second Tumors in Retinoblastoma

Department of Ophthalmology, W. K. Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan 48105, USA.
Orbit (Amsterdam, Netherlands) 02/2008; 27(4):285-91. DOI: 10.1080/01676830802222951
Source: PubMed


To evaluate the use of transglabellar/subcranial approach for surgical resection of periocular second non-ocular tumors in retinoblastoma patients.
Seven retinoblastoma patients with periocular second tumor involving anterior skull base underwent surgical resection by transglabellar/subcranial approach in a single center. The medical records of these patients were retrospectively evaluated.
The most common presenting symptom in retinoblastoma patients with periocular second tumor was difficulty in maintaining the prosthesis in three patients (43%), followed by epistaxis in one (14%), palpable orbital mass in one (14%), persistent periocular swelling in one (14%), and visual loss in one (14%) patient. Periocular second tumors were leiomyosarcoma in three (43%) patients, osteosarcoma in three (43%), and sphenoid wing meningioma in one (14%) patient. Surgical resection by the transglabellar/subcranial approach was the only treatment in one (14%) patient with sphenoid wing meningioma and was combined with chemotherapy in three (43%) patients, and with both external beam radiotherapy and chemotherapy in three (43%) patients. Surgical margins were negative in three (43%) patients and microscopically positive in four (57%) patients. Complications were minor, including cerebral spinal fluid (CSF) leak in one (14%) patient and CSF leak and subgaleal hematoma in one (14%) patient. After 31 months mean follow-up, three (43%) patients were alive and four patients (57%) were dead.
Retinoblastoma patients with periocular second tumors have a poor prognosis. The transglabellar/subcranial approach can be used for surgical resection of periocular second tumor involving skull base with low morbidity.

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    ABSTRACT: Hereditary patients with a history of treated retinoblastoma (RB) have a greatly increased risk of a broad spectrum of secondary malignancies appearing many years later, with a high incidence in the head and neck region. Leiomyosarcomas (LMS) account for up to 58% of these tumors. LMS in the sinonasal region generally are uncommon and are associated with a locally aggressive course and have a poor prognosis. RB may occur in two forms. The hereditary form is generally bilateral but can present unilaterally with a positive family history and typically exhibits a germline mutation in the RB1 gene on chromosome 13. The non-hereditary form is usually unilateral but can show the same germline mutation in up to 10% of cases. Patients with hereditary RB have been shown to have a significantly higher cumulative risk of developing secondary malignancies than those with the non-hereditary form (28 vs. 1.44% respectively). Most reported cases of sinonasal LMS are in patients with a history of the bilateral hereditary form of treated RB. We report a case of LMS of the nasal sinus area in a 35-year-old African American male with a history of non-hereditary unilateral RB and radiation therapy. To the best of our knowledge, this is the first reported case of sinonasal LMS arising in a patient with a history of non-hereditary unilateral RB. The clinical history, radiology, and pathology are presented along with a brief discussion of the literature.
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