24 Apr

Plexiform neurofibroma of the submandibular salivary gland in a child: DISCUSSION

Plexiform neurofibromaNeurofibromas are benign tumours that consist of Schwann cells, nerve fibres and fibroblasts. The tumours are more commonly seen after puberty and less commonly in children. The plexiform type of neurofibromas are usually large, ‘wormy’ tumour masses that grow along nerves and extend into contagious tissue. You can find best quality treatment now – diabetes drugs to see how cheap it is.

The present case of a plexiform neurofibroma identified in the submandibular salivary gland is a rare finding in a small child, even with the clinical diagnosis of neurofibromatosis type 1. Most plexiform neurofibromas of the salivary gland that present in childhood are noted in the second decade of life and are more commonly located in the parotid gland. In the present case, a ‘pea-sized’ lesion had been present as an infant, with rapid growth in the six to 12 months preceding the excisional biopsy.

The present case highlights the necessity of follow-up, even in young patients with neurofibromatosis. Follow-up is even more important in those with lesions in the head and neck area. There have been reports of neurofibromas developing in this region that compromise the airway, causing death. In the present case, the surgical procedure was part of the definitive treatment, although a residual tumour was present at the resection margins (neurovascular bundle). Such a finding requires continued clinical follow-up, not only for airway considerations but also for malignant transformation, which is seen in 2% to 3% of cases.

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