Biju Rakesh Danny, Duignan Maria, Maharaj Monish, Ziad Fouzia, Hussain Zakier
Department of Neurosurgery, Waikato Hospital, Hamilton, New Zealand.
Department of Pathology, Waikato Hospital, Hamilton, New Zealand.
Asian J Neurosurg. 2020 Aug 28;15(3):709-712. doi: 10.4103/ajns.AJNS_142_20. eCollection 2020 Jul-Sep.
Melanotic schwannoma is a rare form of nerve sheath tumor composed of melanin-producing neoplastic Schwann cells. Less than 200 cases have been reported worldwide. The entity has been associated with Carney complex, a rare genetic disorder characterized by multiple benign tumors. A 38-year-old female presented to our unit with sudden-onset lower back pain and radiculopathy triggered by a mechanical injury. Imaging demonstrated a lesion within the left L5/S1 neural exit foramen with remodeling of bony architecture typical of a chronic, benign process. She proceeded for resection and histology revealed a psammomatous melanotic schwannoma. The patient recovered well with improvement in symptomology. Due to the aggressive nature of the disease, she remains under surveillance for local recurrence and distant metastasis. Clinicians should be aware of this malignant entity, despite its possible presentation with radiological features of a chronic, benign process. Unusual characteristics such as hemorrhage should be treated with a high index of suspicion.
黑色素性神经鞘瘤是一种罕见的神经鞘瘤,由产生黑色素的肿瘤性施万细胞组成。全球报道的病例不到200例。该实体与卡尼综合征有关,卡尼综合征是一种罕见的遗传性疾病,其特征为多发性良性肿瘤。一名38岁女性因机械性损伤引发急性下背痛和神经根病前来我院就诊。影像学检查显示左侧L5/S1神经出口孔内有一病变,伴有典型慢性良性过程的骨质结构重塑。她接受了手术切除,组织学检查显示为砂粒体性黑色素性神经鞘瘤。患者恢复良好,症状有所改善。由于该疾病具有侵袭性,她仍在接受局部复发和远处转移的监测。临床医生应意识到这种恶性实体,尽管其可能表现出慢性良性过程的放射学特征。对于出血等不寻常特征应高度怀疑并进行处理。