Abdallah Adam M, Odat Ramez M, Hanifa Hamdah, Shakhatreh Zaid, Sharqiah Qosay M, Daoud Suleiman
Department of Neurosurgery, King Abdullah University Hospital, Irbid, Jordan.
Faculty of medicine, Jordan University of Science and Technology, Irbid, Jordan.
Ann Med Surg (Lond). 2024 Jul 1;86(10):6129-6135. doi: 10.1097/MS9.0000000000002333. eCollection 2024 Oct.
Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft tissue malignant tumor. MPNST in the spinal canal is rarely seen except in cases of neurofibromatosis type 1. However, a long-segment extradural spinal malignant spindle cell neoplasm has not been reported in the current literature.
We present the first reported case of spinal malignant spindle cell neoplasm extended along the spine. The detected lesion is responsible for compressing various segments of the spinal cord, causing thinning of the cord and secondary stenosis of the spinal canal, leading to a condition known as multisegment compression myelopathy.
MPNSTs are typically detected late due to nonspecific symptoms, with a higher incidence in extremities and a notable occurrence in unusual locations. Diagnosis relies on MRI and histopathology, with S_100 positivity as a neural marker. MPNSTs can arise from neurofibromas or Schwann cells, with a significant portion resulting from TP53 mutations or secondary to radiation exposure.
This case stands out due to its unique presentation, characterized by a predominantly spindle cell morphology with certain epithelioid features. It is imperative to recognize this condition for an accurate diagnosis, emphasizing the spindle cell-type MPNST and highlighting its exceptionally poor prognosis.
恶性外周神经鞘瘤（MPNST）是一种罕见且侵袭性强的软组织恶性肿瘤。除1型神经纤维瘤病患者外，椎管内的MPNST很少见。然而，目前文献中尚未报道过长节段硬膜外脊髓恶性梭形细胞瘤。
我们报告首例沿脊柱蔓延的脊髓恶性梭形细胞瘤病例。检测到的病变压迫脊髓各节段，导致脊髓变薄及椎管继发性狭窄，引发多节段压迫性脊髓病。
MPNST通常因症状不具特异性而发现较晚，在四肢发病率较高，在不寻常部位也有明显发生。诊断依靠MRI和组织病理学检查，S_100阳性作为神经标志物。MPNST可起源于神经纤维瘤或施万细胞，很大一部分由TP53突变或放疗引起。
该病例因其独特表现脱颖而出，主要特征为以梭形细胞形态为主且伴有某些上皮样特征。准确诊断必须认识到这种情况，强调梭形细胞型MPNST并突出其预后极差。
