Hachem Ralph N Abi, Bared Anthony, Zeitouni Joseph, Younis Ramzi T
Department of Otolaryngology-Head and Neck Surgery, University of Miami, Miller School of Medicine, Miami, FL, USA.
Int J Pediatr Otorhinolaryngol. 2010 Apr;74(4):426-9. doi: 10.1016/j.ijporl.2009.12.012. Epub 2010 Jan 13.
Plexiform neurofibromas are peripheral nerve sheath tumors associated with neurofibromatosis type 1. The maxillary sinus is an extremely rare location of the plexiform neurofibroma and only two adult cases have been previously reported. We report the first case of plexiform neurofibroma of the maxillary sinus occurring in a child with neurofibromatosis type 1. This unusual location presents a management challenge considering the infiltrative nature and the potential malignant degeneration of this type of tumor. MRI is highly valuable to diagnose and plan the surgical approach of the plexiform neurofibroma of the maxillary sinus. Due to the location of the tumor and the patient's age, conservative surgery is highly recommended. We performed an endoscopic total en-bloc resection of the tumor with no recurrence after nine months of follow-up.
丛状神经纤维瘤是与1型神经纤维瘤病相关的周围神经鞘瘤。上颌窦是丛状神经纤维瘤极其罕见的发病部位，此前仅有两例成人病例报道。我们报告了首例发生于1型神经纤维瘤病患儿的上颌窦丛状神经纤维瘤。考虑到这类肿瘤的浸润性本质和潜在的恶性变，这一不寻常的发病部位带来了治疗挑战。MRI对于诊断上颌窦丛状神经纤维瘤及规划手术入路具有很高的价值。由于肿瘤的位置及患者年龄，强烈推荐采取保守手术。我们实施了内镜下肿瘤全块切除，随访9个月无复发。
