Troublesome and aggressive multiple central giant cell lesions of jaws in 8-year child with neurofibromatosis-1: case report and therapy options

An 8-year-old boy presented with large,hard and stiff pelvi-lingual mass. Intra-oral examination and radiological investigations (panoramic and CT) revealed multiple multilocular radiolucent, mandibular and maxillary, lesions causing significant painless expansions of maxilla and mandible. Mother’s questioning and former pathology report (October 2006) revealed that patient underwent, 3 years ago,enucleation of multiple central giant lesions-CGCLs. Mandibular insicional biopsy confirmed the recurrence of CGCLs, especially in mandible where multiple multilocular radiolucencies crossed midline and caused significant osseous destruction. Normal serum calcium/phosphate/alkaline phosphatase/PTH levels ruled out hyperparathyroidism and body/skin examination revealed NF1 (according to 1987 NIH Consensus Development Conference). Intralesional injection of 10 mg/ml triamcinolone acetonide,at a volume of 1 mL per cm length (Marx and Stern, 2003) over a 3-month period failed to induce any radiographical involution of lesions. Salmon Calcitonin nasal spray therapy was then initiated,twice daily (200 U/spray) for one year but due to unsatisfactory clinical outcome,it was discontinued after 9 months of treatment. At that stage, and under general anesthesia, wide soft tissue reflection and surgical curettage and debulking were performed and CGCLs diagnosis was histologically reconfirmed, but 15 months after surgery, mandibular lesions recurred again. This clinical case will be presented, with emphasis on differential diagnostic means and contemporary treatment options.

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Jan 20, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Troublesome and aggressive multiple central giant cell lesions of jaws in 8-year child with neurofibromatosis-1: case report and therapy options
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