Infant with unusual sino-nasal tumor

Introduction:

A 4-year-old boy with progressive exophthalmos for one month was referred to our department. The endonasal inspection showed a mass of soft consistency in the left nasal cavity, which extended to the middle meatus.

Methods:

The contrast-enhanced MRI of the skull revealed a solid, contrast-enhancing mass of the left nasal cavity and ethmoidal cells, infiltrating the orbital cavity up to the optical nerve. Computed tomography showed bone remodeling and notable bone erosions of the left paramedian anterior skull base.

Endonasal endoscopic biopsy was taken, which displayed a spindle-cell, giant cell lesion with focal fibrous bone formation.

Results:

Due to the intraoperative risk of injury to the optic nerve and cavernous sinus, subtotal tumor resection was performed with orbital decompression.

In summary of clinical, radiological and histological findings central giant cell granuloma with no evidence of malignancy was diagnosed.

Unfortunately MRI-follow ups revealed a renewed growth of the residual tumor, requiring revision surgery within 6 months. A near-total resection with only small tumor remnant near the cavernous sinus was performed.

Conclusions:

Central giant cell granulomas usually occur in the jawbone and in rare cases can show malignant transformation. The treatment of choice is surgical excision. In case of tumor recurrence, adjuvant therapy with steroid instillation or monoclonal antibody Denosumab must be considered.

Among others, granulomatous processes, rhabdomyosarcoma, esthesioneuroblastoma or lymphoma must be considered as differential diagnosis.

Publication History

Publication Date:
23 April 2019 (online)