The transglabellar/subcranial approach for surgical excision of periocular second tumors in retinoblastoma.
OBJECTIVE
To evaluate the use of transglabellar/subcranial approach for surgical resection of periocular second non-ocular tumors in retinoblastoma patients.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Demirci
Hakan
H
Department of Ophthalmology, W. K. Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan 48105, USA.
Marentette
Lawrence J
LJ
Nelson
Christine C
CC
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003399:Craniotomy / Q000379:methods*; D015353:Eye Enucleation; D005138:Eyebrows; D005260:Female; D005624:Frontal Bone; D006801:Humans; D007890:Leiomyosarcoma / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D008577:Meningeal Neoplasms / Q000601:surgery; D008579:Meningioma / Q000601:surgery; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000401:mortality / Q000601:surgery*; D013508:Ophthalmologic Surgical Procedures; D009918:Orbital Neoplasms / Q000601:surgery; D012516:Osteosarcoma / Q000601:surgery; D010255:Paranasal Sinus Neoplasms / Q000601:surgery; D011878:Radiotherapy; D019572:Retinal Neoplasms / Q000473:pathology* / Q000601:surgery; D012175:Retinoblastoma / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D015996:Survival Rate; D014057:Tomography, X-Ray Computed