Plexiform neurofibroma of the submandibular salivary gland in a child: CASE PRESENTATION Part 1
A three-year-old boy with a history of inherited neurofibromatosis type 1 presented with a 3×4 cm left submandibular triangle cervical mass that had grown rapidly over the preceding six to 12 months. A pea-sized mass in the same region had been present since infancy and had been aspirated when the patient was approximately one to one-and-a-half years of age. His medical history included a tonsillectomy for an unrelated problem. The child was otherwise healthy, and the only manifestations of neurofibromatosis were the presence of multiple cafe au lait spots. Results of a neurological examination were normal.
A computed tomography scan was performed with and without intravenous contrast enhancement of the neck. A mass measuring about 4.5 cm in its maximum dimension was associated with the left submandibular gland. On contrast enhancement, the lesion was seen to have well-defined margins and was predominantly composed of low soft tissue density, within which there were several interconnecting strands of a higher density (Figure 1). In a patient with neurofibromatosis type 1, neurofibroma would be the most likely cause of the mass. However, in the pediatric population, the salivary gland tumours that should be included in the differential diagnosis, in descending order of frequency, are capillary hemangiomas, pleomorphic adenomas, mucoepidermoid carcinoma, lymphangiomatous-type tumours and acinic cell carcinoma.
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Figure 1) Computed tomography axial section with contrast enhancement showing a left submandibular gland mass (arrow) measuring 4.5 cm in maximum dimension and having a predominantly low soft tissue density with several higher density interconnecting strands