Menéndez Ricardo H, Erice Santiago G, Bas Carlos A, Casas Gabriel, Dillon Horacio S
Departments of 1 Neurosciences.
J Neurosurg Spine. 2015 Mar;22(3):310-3. doi: 10.3171/2014.9.SPINE14360. Epub 2015 Jan 2.
The authors describe a case of spinal cord compression due to an epidural metastasis of malignant chondroid syringoma. Chondroid syringoma is a rare mixed tumor of the skin composed of both epithelial and mesenchymal elements. Although most are benign, malignant forms have been reported. Malignant chondroid syringoma may progress very slowly and the metastatic spread occurs late, appearing years after the original diagnosis. There is only one other report of spinal cord compression secondary to metastasis of malignant chondroid syringoma, which was finally diagnosed by microscopic examination of an autopsy specimen. This 63-year-old woman presented with a 4-week history of progressive paraparesis. Admission MRI of the thoracic spine showed an extradural mass arising from the posterior elements and left pedicle of T-9, which caused posterior compression of the spinal cord. Surgical decompression resulted in resolution of the neurological impairments. The histological results were consistent with metastasis of malignant chondroid syringoma. The patient underwent adjuvant radiotherapy and a favorable outcome was noted at the 2-year follow-up visit. This represents the first reported case of spinal cord compression from a metastasis of a malignant chondroid syringoma histologically confirmed in vivo. The authors' experience in this case suggests that resection followed by radiotherapy might be an acceptable means for achieving short-term, progression-free survival.
作者描述了一例因恶性软骨样汗腺腺瘤硬膜外转移导致脊髓压迫的病例。软骨样汗腺腺瘤是一种罕见的皮肤混合性肿瘤,由上皮和间充质成分组成。虽然大多数是良性的,但也有恶性形式的报道。恶性软骨样汗腺腺瘤可能进展非常缓慢,转移扩散发生较晚,在最初诊断数年之后才出现。仅有另一例关于恶性软骨样汗腺腺瘤转移继发脊髓压迫的报告,最终通过尸检标本的显微镜检查得以确诊。这位63岁女性有4周进行性双下肢轻瘫病史。胸椎入院时的磁共振成像(MRI)显示一个硬膜外肿块,起源于T9的后部结构和左侧椎弓根,导致脊髓后部受压。手术减压使神经功能障碍得到缓解。组织学结果与恶性软骨样汗腺腺瘤转移相符。患者接受了辅助放疗,在2年随访时观察到良好的结果。这是首例在体内经组织学证实的恶性软骨样汗腺腺瘤转移导致脊髓压迫的报告病例。作者在该病例中的经验表明,手术切除后放疗可能是实现短期无进展生存的一种可接受方法。