Fazal Zoha Zahid, Kazmi Syeda Maryam, Bajwa Mohammad Hamza, Khan Ahsan Ali
Department of Neurosurgery, The Aga Khan University Hospital, Karachi, Pakistan.
Surg Neurol Int. 2022 Aug 26;13:375. doi: 10.25259/SNI_183_2022. eCollection 2022.
Melanocytic schwannomas (MSs) are rare, malignant peripheral nerve sheath tumors with only 200 cases reported to date. These pose imaging and pathological challenges for definitive diagnosis.
A 25-year-old lady presented at our center with a prolonged history of gait disturbance, left ear tinnitus, headaches, and drowsiness. MRI findings showed a midline cystic lesion in the posterior cranial fossa extending caudally to the D1 vertebral body, with marked central hypointensity, and peripheral hyperintensity on T1-weighted images. A suboccipital craniotomy and debulking of the lesion were performed, showing a hyperpigmented, infiltrative tumor adherent to the surrounding structures. This was confirmed as a melanocytic schwannoma on histopathological analysis.
Posterior fossa MSs involving cervicomedullary region and extending distally to cervicothoracic spinal cord are rare and complex cases, particularly with regard to difficulty diagnosing preoperatively and surgical resection.
黑素细胞性神经鞘瘤（MSs）是罕见的恶性周围神经鞘瘤，迄今为止仅报道了200例。这些肿瘤在明确诊断方面对影像学和病理学提出了挑战。
一名25岁女性因长期步态障碍、左耳耳鸣、头痛和嗜睡就诊于我们中心。MRI检查结果显示，后颅窝有一个中线囊性病变，尾端延伸至第一椎体，在T1加权图像上中央明显低信号，周边高信号。行枕下开颅手术并对病变进行减压，术中见一色素沉着、浸润性肿瘤，与周围结构粘连。组织病理学分析证实为黑素细胞性神经鞘瘤。
累及颈髓区域并向远侧延伸至颈胸段脊髓的后颅窝MSs是罕见且复杂的病例，尤其是在术前诊断和手术切除方面存在困难。
