Hemangioblastoma of the Spinal Cord Associated with Holocord Syringomyelia and Syringobulbia

脊髓血管母細胞瘤在脊髓腫瘤中本已少見，而本文報導一例胸髓之脊髓血管母細胞瘤合併全脊髓空洞之上下延伸，更屬罕見。患者是一位36歲女性，自3年前即逐漸發生右下腳無力並合併麻感，入院前2個月；右下腳無力更形明顯，而致行走困難，本院門診時，理學檢查顯示右大腿及下腳無力約4級肌力，自第11胸椎皮節以下疼痛感及輕觸感均明顯下降，且振動及體位感覺在右下腳顯示缺損。磁共振造影，經對比劑注射顯影，顯示第11胸髓有約2×1公分大之脊髓內腫瘤。腫瘤與正常組織邊緣清楚且大部份位於脊髓神經之背部並造成脊髓神經之壓迫。況著腫瘤向上有一連續之髓空洞症，由胸髓沿伸至頸髓，並延至腦幹處，形成長形之全脊髓空洞症，而本身向下更在脊髓圓錐部至第一、二腰椎間形成囊腫。手術採顯微手術沿著腫瘤邊緣，將腫瘤完全取出，但對於頸、胸髓之脊髓空洞症及圓錐部之囊腫則並未刻意引流或行導管手術，術後六週，經行磁共振造影檢查，顯示胸椎第11節脊髓之腫瘤已切除乾淨，且頸部及胸髓之脊髓空洞症也明顯萎縮，患者之神經功能在術後，並未變壞，目前僅呈右腳輕度強直性無力，且排尿、排便功能正常，病理顯示為血管母細胞瘤。

關鍵字

血管母細胞瘤；脊髓腫瘤；脊髓空洞症

並列摘要

Hemangioblastoma occurring in the spinal cord is an uncommon but well-recognized entity. The present report shows a hemangioblastoma in the low thoracic cord uniquely associated with extensive rostral and caudal cysts to form a holocord syringomyelia and syringobulbia. This 36-year-old woman presented with progressive weakness and numbness over her right leg for about 3 years. She had difficulty walking for the previous two months. A neurological examination revealed spastic paresis of her right leg about grade 4 in power. Light touch, vibration and pin-prick pain sensations below thoracic dermatome level T11 were markedly reduced especially in the right lower limb. Gadolinium-enhanced magnetic resonance image (MRI) study demonstrated a 2 cm × 1 cm well-circumscribed mass at T11 in the spinal cord. Syringomyelia and cystic formation were noted rostral and caudal to this mass lesion. The upper syrinx extended from T11 up to the high cervical cord and to the lower medulla causing enlargement of the spinal cord. However, the lower one formed a cyst in the conus medullaris and the region of the cauda equina. During surgery to remove the masses, the big drainage vein was kept patent until the tumor was freed from the spinal cord. Internal debulking of the solid tumor was avoided and the tumor was taken out by a so-called ”en bloc” pattern. Rostral and caudal cystic fluids drained spontaneously after tumor removal without shunting the syrinx to the subarachnoidal space. Post-operative MRI revealed total excision of the mass with a remarkable shrinkage of holocord cysts. Pathology showed a hemangioblastoma. Post-operative neurological function including urination and defecation did not change compared with that before surgery.

並列關鍵字

hemangioblastoma ； spinal cord tumor ； syringomyelia

國際替代計量

Hemangioblastoma of the Spinal Cord Associated with Holocord Syringomyelia and Syringobulbia

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