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Intraparotid neurofibromatosis presenting with multiple nodules.

Neurofibroma of the parotid gland is extremely rare. When it does occur, it usually does so in patients with neurofibromatosis type 1 (NF-1). We describe a case of intraparotid neurofibromatosis that was even more unusual because it occurred in the absence of a definitive diagnosis of NF-1 and because the patient had multiple nodules.

The patient was a 41-year-old woman who had a several-year history of multiple nodules on the right side of her neck and face. She noted that these nodules were painful and required management with analgesic medication. She had previously declined to undergo surgery, but she was seeking surgical treatment at this time because the pain had increased and because she wished to improve the cosmetic appearance of the affected areas.

Magnetic resonance imaging (MRI) was performed to further determine the nature of the lesions. The scan identified multiple nodular masses throughout the right parotid gland (figure 1).

Intraoperatively, multiple masses were found to be adherent to the facial nerve epineurium (figure 2, A). These semisolid masses were found both superficially and deep to all branches of the facial nerve, as well as the main trunk. Several were found in the submandibular space adherent to the marginal mandibular nerve. All were removed, with care taken to protect the facial nerve (figure 2, B).

Postoperatively, the patient did very well. Her pain, which she had rated as 10/10 before surgery, had resolved, and she exhibited no signs of facial palsy. Pathologic evaluation of the nodules was performed to rule out the presence of a plexiform neurofibroma. Although this case did not meet the strict criteria for plexiform neurofibroma, the index of suspicion remained high for NF-1, given the presence of multiple nodules and their atypical location. The patient was referred for further testing to rule out NF-1.

[FIGURE 1 OMITTED]

A neurofibroma is a benign nerve sheath tumor of Schwann cell origin. Neurofibromas occur in the peripheral nervous system. When they are not associated with NF-1, they are usually solitary. Often asymptomatic initially, these lesions may cause pain, weakness, and numbness as they grow as a result of compression of the underlying nerve fascicles.

The presence of multiple neurofibromas requires investigation for neurofibromatosis. (1) Imaging (MRI with and without gadolinium) will aid in making the diagnosis and in helping delineate other neurofibromas. Surgical removal is recommended for symptomatic patients and for those with NF-1.

Solitary neurofibromas usually occur in the absence of NF-1. In such cases, close monitoring is required to ensure that other stigmata of NF-1 do not develop in the future. A solitary neurofibroma may herald the onset of others. It is not uncommon for solitary neurofibromas to develop in the head and neck.

[FIGURE 2 OMITTED]

A plexiform neurofibroma is almost exclusively seen in NF-1, and it must be ruled out by pathology. It is critical to identify plexiform features on pathology because there is a 10 to 15% risk of malignant transformation in patients with NF-1 and plexiform neurofibromas. (1) Most transformations occur in the third decade of life. (2) Although surgery is recommended for treatment, its success is somewhat limited by the multifocal nature of the disease, which leads to a high recurrence rate. (3)

References

(1.) Barnes L, ed. Surgical Pathology of the Head and Neck. 2nd ed. Boca Raton, Fla.: CRC Press; 2001.

(2.) Kosaka M, Miyanohara T, Mochizuki Y, Kamiishi H. A rare case of a facial-nerve neurofibroma in the parotid gland. Br J Hast Surg 2002;55(8):689-91.

(3.) Fadda MT, Verdino G, Mustazza MC, et al. Intra-parotid facial nerve multiple plexiform neurofibroma in patient with NF1. Int J Pediatr Otorhinolaryngol 2008;72(5):553-7.

Ryan E Osborne, MD, FACS; Jason S. Hamilton, MD, FACS; Reena Gupta, MD, FACS

From the Osborne Head and Neck Institute, Los Angeles.
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Title Annotation:HEAD AND NECK CLINIC
Author:Osborne, Ryan F.; Hamilton, Jason S.; Gupta, Reena
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Jun 1, 2013
Words:631
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