Jayachandran Dorairaj, Sunantha Selvaraj, Gopalaiah Hema, Veeraraghavan Gajendra
Department of Periodontics, Vinayaka Missions Sankarachariyar Dental College, Salem, Tamil Nadu, India.
Depatment of Prosthodontia, Vinayaka Missions Sankarachariyar Dental College, Salem, Tamil Nadu, India.
J Oral Maxillofac Pathol. 2014 Jan;18(1):114-7. doi: 10.4103/0973-029X.131932.
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.
丛状神经纤维瘤（PNFs）是1型神经纤维瘤病（NF-I）最常见且使人衰弱的并发症之一。它们导致了大量的发病、毁容、功能障碍，甚至危及生命。PNFs还可能转变为恶性外周神经鞘瘤（MPNST）。由于一般恶性软组织肉瘤缺乏有效的治疗方法，以及对先前存在的肿瘤诊断延迟，这种并发症难以治疗。我们报告一例累及口腔的面部PNF病例并进行文献复习。
