Plexiform Neurofibroma of the Submandibular Salivary Gland: A Rare Tumour

Abstract

A 15 year old boy presented with swelling in the submandibular region. X ray of the part showed faint radio opaque shadow. A provisional diagnosis of sialadenitis with sialiolithiasis was made. Excised mass was reported histopathologically as plexiform neurofibroma of submandibular salivary gland. Plexiform neurofibroma of the salivary gland is a rare benign tumour, often present in the parotid gland. It is very rare in submandibular salivary gland. It is a slow growing, locally infiltrating tumour.

Peripheral nerve sheath tumors arising in salivary glands: A clinicopathologic study

“…Neurofibromas arising from salivary gland tissue have been most commonly reported within the parotid, with fewer cases also documented within the submandibular gland [14]. All five patients in this clinical series had lesions within the parotid gland.…”

“…The two patients within this series with solitary schwannoma arising from the salivary gland presented with a tender, mobile mass. Most arise in the parotid gland; only rare cases have been described in the submandibular gland (n=16) [13][14][15][16][17][18][19][20][21] and sublingual gland (n=1) [21];…”

“…A single institution retrospective analysis by Cho et al found that neurofibromas and schwannoma comprised 0.2% (n=1) and 1.1% (n=6) respectively of 527 mesenchymal tumors found in salivary glands [1]. Of the 22 neurofibromas [2], 9 [3][4][5][6][7][8][9][10][11] MPSNT, and approximately 150 schwannomas [12][13][14][15][16][17][18][19][20][21][22] in the literature that have been found to arise in the salivary gland, the vast majority have been reported as single case reports or small series [13].…”

Plexiform Neurofibroma Involving the Lacrimal Gland

“…Neurofibromas constitute 0.4% of all salivary neoplasms [ 10 ]. Plexiform neurofibromas are slow-growing, locally infiltrating benign tumors [ 10 ].…”

“…Neurofibromas constitute 0.4% of all salivary neoplasms [ 10 ]. Plexiform neurofibromas are slow-growing, locally infiltrating benign tumors [ 10 ]. They arise from diffuse enlargement of multiple nerve fascicles and its branches, resulting in nerve thickening [ 10 ].…”