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Clinical Problem Solving: Pathology
January 2007

Pathology Quiz Case 2: Diagnosis

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Copyright 2007 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2007

Arch Otolaryngol Head Neck Surg. 2007;133(1):97-98. doi:10.1001/archotol.133.1.97

Neurofibroma is a benign peripheral nerve tumor. It can be localized or diffuse. The latter usually occurs in association with von Recklinghausen disease (neurofibromatosis type 1). By definition, SNF (also known as isolated or localized neurofibroma) is not associated with neurofibromatosis type 1.1,2 Solitary neurofibroma in the parapharyngeal space is rare.3 In this clinically silent location, SNF usually reaches a considerable size before it becomes symptomatic. Symptoms are nonspecific and depend heavily on the exact location and extension of the lesion.4 Interestingly, patients with SNF of the parapharyngeal space usually do not complain of neurologic deficits. Clinically, SNF of the parapharyngeal space typically presents as a slow-growing, painless mass. Grossly, it can have either a gray-white or a yellow cut surface. It can also have a gelatinous appearance, as in the present case. The gelatinous quality can be so prominent that it suggests the diagnosis of myxoma, as was the initial diagnostic impression on gross examination in this case. When neurofibroma is confined within the peripheral nerve from which it arises, it usually produces a fusiform or ovoid mass and remains encapsulated by the perineurium, which is a common occurrence in cases of neurofibromas that arise in the deep tissues, such as the parapharyngeal space. However, when neurofibroma breaks out of the confines of the nerve, it grows into the adjacent tissues, presenting as an ill-defined infiltrative mass, which is common in cases of neurofibromas that arise in the superficial tissues, such as cutaneous neurofibromas.4

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