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Case Reports
. 2014 Jan;18(1):114-7.
doi: 10.4103/0973-029X.131932.

Plexiform neurofibromatosis involving face and oral cavity

Dorairaj Jayachandran  1 , Selvaraj Sunantha  2 , Hema Gopalaiah  3 , Gajendra Veeraraghavan  4
Affiliations
Case Reports

Plexiform neurofibromatosis involving face and oral cavity

Dorairaj Jayachandran et al. J Oral Maxillofac Pathol. 2014 Jan.

Abstract

Plexiform neurofibromas (PNFs) are one of the most common and debilitating complications of neurofibromatosis type I (NF-I). They account for substantial morbidity, disfigurement, functional impairment and are life threatening. PNFs can also be subjected to transformation into malignant peripheral nerve sheath tumor (MPNST). This complication is refractory to treat due to paucity of effective therapies for malignant soft tissue sarcomas in general and also the delay in diagnosis from a preexisting tumor. We report a case of PNF of face involving oral cavity with literature review.

Keywords: Café- au- lait macules; facial plexiform neurofibromatosis; peripheral nerve sheath tumor; von Recklinghausen's.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
Clinical image showing diffuse swelling involving the tongue and lower lip
Figure 2
Figure 2
The photomicrograph of the sections shows bundles of Schwann cells in myxomatous stroma (H&E stain, ×100)
Figure 3
Figure 3
The photomicrograph showing tumor cells positive for S-100 protein (IHC stain, ×100)
See this image and copyright information in PMC

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