The transglabellar/subcranial approach for surgical excision of periocular second tumors in retinoblastoma

Orbit. 2008;27(4):285-91. doi: 10.1080/01676830802222951.


Purpose: 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.

MeSH terms

  • Adolescent
  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Combined Modality Therapy
  • Craniotomy / methods*
  • Eye Enucleation
  • Eyebrows
  • Female
  • Frontal Bone
  • Humans
  • Leiomyosarcoma / surgery
  • Magnetic Resonance Imaging
  • Male
  • Meningeal Neoplasms / surgery
  • Meningioma / surgery
  • Middle Aged
  • Neoplasms, Second Primary / mortality
  • Neoplasms, Second Primary / surgery*
  • Ophthalmologic Surgical Procedures*
  • Orbital Neoplasms / surgery
  • Osteosarcoma / surgery
  • Paranasal Sinus Neoplasms / surgery
  • Radiotherapy
  • Retinal Neoplasms / pathology*
  • Retinal Neoplasms / surgery
  • Retinoblastoma / pathology*
  • Retinoblastoma / surgery
  • Retrospective Studies
  • Survival Rate
  • Tomography, X-Ray Computed