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Paraganglioma of the cauda equina

机译:马尾副神经节瘤

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Paragangliomas of the cauda equina are rare neuroendocrine tumors. We present a case of nonsecreting paraganglioma of the cauda equina, preoperatively misdiagnosed as neurofiroma. A 42year old lady presented with pain and burning sensation in the lower back. MRI showed an intradural / extra medullary well defined mass at the L4,L5 level. Microscopic examination of the resected tumor revealed paraganglioma. The tumor cells were positive for chromagranin and synaptophysin. Although it is difficult to correctly diagnose paraganglioma preoperatively , especially in the region of cauda equina, it should be included in differential diagnoses. Introduction A paraganglioma of cauda equina region is extremely rare and since most of them are nonsecreting tumours, the pre-operative diagnosis of paraganglioma is very difficult. Histologically these neoplasms may have considerable similarity with ependymoma, the most common neoplasm of the lower spinal cord, and the diagnosis can be easily missed unless special techniques are employed. The clinical and pathological data obtained from this and the other reported examples suggests that paragangliomas of the cauda equina are benign, slowly growing neoplasms. In contrast to ependymomas and to paragangliomas elsewhere, they are well circumscribed, amenable to complete resection and have an excellent prognosis. Case Report A 42year old female presented with pain and burning sensation in the lower back . Pain was moderate and intermittent initially and became continuous in the last six months. Pain was radiating to the gluteal region and increased on coughing and taking deep breath. There was no history of urinary or bowel problem. On clinical examination ,motor and sensory systems were within normal limits. All the reflexes were present. The spine showed no deformity. X ray of the lumbar spine was also normal However MRI showed an intradural / extra medullary well defined mass at the L4,L5 level. Possibilities of neurofibroma and ependymoma were suggested. Laminectomy of L3- S1 with near total excision of the tumor was conducted and 3x3 c.m multilobular pinkish, friable mass arising from cauda equine was excised.Pathological Findings We received three pale tan coloured soft tissue masses measuring 2.5 x 1x0.5c.m.with nodular outer surface. Cut surface of these were pale tan in colour with areas of hemorrhage. On Microscopic examination, The tumor was partially encapsulated and comprised of nests and broad anastamosing trabaculae of round to polygonal cells tumor cells separated by delicate fibrovascular septa ( zellballen pattern).These tumor cells showed moderately abundant eosinophilic cytoplasm , central round to oval nucleus with finely stippled chromatin, occasional nucleolus and intranuclear inclusion. The tumor cells were positive for chromogranin and synaptophysin.
机译:马尾神经节旁神经瘤是罕见的神经内分泌肿瘤。我们提出一例马尾非分泌性神经节旁瘤,术前被误诊为神经纤维瘤。一名42岁的女士在下背部表现出疼痛和灼痛感。 MRI显示在L4,L5水平硬膜内/髓外肿块清晰可见。切除的肿瘤的显微镜检查显示副神经节瘤。肿瘤细胞对嗜铬粒蛋白和突触素呈阳性。尽管术前很难正确诊断神经节旁瘤,特别是在马尾区域,但应将其包括在鉴别诊断中。引言马尾神经节旁神经节瘤极为罕见,而且由于大多数是非分泌性肿瘤,因此术前诊断神经节旁瘤非常困难。从组织学上讲,这些肿瘤可能与室管膜瘤(下脊髓最常见的肿瘤)有相当的相似性,除非采用特殊技术,否则很容易漏诊。从该实例和其他报道的实例中获得的临床和病理数据表明,马尾神经节瘤是良性的,生长缓慢的肿瘤。与室管膜瘤和其他地方的神经节瘤相比,它们具有良好的边界,可以完全切除并且预后良好。病例报告一名42岁的女性腰部疼痛和烧灼感。最初疼痛是中度和间歇性的,在最近六个月中持续疼痛。疼痛扩散到臀区域,并在咳嗽和深呼吸时加剧。没有泌尿或肠病的病史。经临床检查,运动和感觉系统均在正常范围内。所有的反射都出现了。脊柱没有畸形。腰椎的X线检查也正常,但是MRI显示在L4,L5水平硬膜内/髓外肿块清晰可见。提示了神经纤维瘤和室管膜瘤的可能性。进行了L3-S1的椎板切除术,几乎全部切除了肿瘤,并切除了3x3厘米的由马尾引起的多小叶粉红色,易碎的肿块。结节状外表面。它们的切面浅棕褐色,带有出血区域。经显微镜检查,肿瘤被部分包封,由巢状和圆形至多角形的宽吻合气管组成,肿瘤细胞被细密的纤维血管间隔(zellballen模式)所分离,这些肿瘤细胞显示出适度的嗜酸性粒细胞质,中心圆形至卵圆形核,细密斑点染色质,偶有核仁和核内包涵体。肿瘤细胞嗜铬粒蛋白和突触素呈阳性。

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