海绵窦区罕见肿瘤的诊断和显微手术治疗

发布时间：2018-07-28 07:47

【摘要】：目的：海绵窦区肿瘤常见者为脑膜瘤、神经鞘瘤及海绵状血管瘤。而相对罕见的脊索瘤样脑膜瘤、表皮样囊肿及腺样囊性癌的临床报道较少。以我科成功手术治疗的海绵窦区表皮样囊肿、脊索瘤样脑膜瘤及腺样囊性癌为例，结合相关文献总结其临床诊治经验，以提高术前诊断准确率，并选择合适的手术入路提高肿瘤切除率。方法：回顾性分析我科成功手术治疗的海绵窦区脊索瘤样脑膜瘤、表皮样囊肿及腺样囊性癌的临床资料，复习国内外相关文献，比较海绵窦区脑膜瘤、神经鞘瘤、海绵状血管瘤、表皮样囊肿、脊索瘤样脑膜瘤及腺样囊性癌的临床表现、影像学及病理学特征。从常用手术入路的解剖关系、术中处理措施及可能导致的术后并发症的角度，比较不同手术入路的优缺点。结果：海绵窦区肿瘤的临床表现无明显特异性，临床症状主要表现为III～VI颅神经受累。腺样囊性癌多由其它部位侵袭至颅内，可有原发灶相关的症状体征。神经鞘瘤多起源于神经鞘膜细胞，所以常以神经刺激和神经麻痹为首发症状。 MRI上，脑膜瘤大多均匀一致强化，脊索瘤样脑膜瘤常呈不均匀强化，神经鞘瘤可呈均匀或不均匀强化，而腺样囊性癌不均匀强化，呈大小不同的囊腔。在弥散加权成像(DWI)上，表皮样囊肿呈高信号，具有特异性，但不强化。海绵窦区海绵状血管瘤在T2W像上中呈明显高信号，增强扫描显示其强化程度高于脑膜瘤。脑膜瘤与脊索瘤样脑膜瘤病理组织学相似，脊索瘤样脑膜瘤D2-40蛋白表达阳性，而脑膜瘤表达阴性。神经鞘瘤由梭形细胞或小的星状细胞组成，伴有各种退行性变，S-100、Leu-7常呈阳性，局灶性GFAP呈阳性。海绵状血管瘤由血管窦组成，VEGF相关受体表达增高。表皮样囊肿呈结节状或囊状，内容物可见钙化和泥沙样改变。腺样囊性癌的特征性组织学征象是团块状增生的肿瘤细胞，呈筛状及腺样排列。手术切除海绵窦区肿瘤可经翼点或改良翼点入路，，若肿瘤涉及眼眶者可取额眶颧入路，对于后部海绵窦肿瘤可取颞下经颧弓入路。经颞下锁孔入路可切除大部分海绵窦区肿瘤。结论：1、海绵窦区肿瘤的临床症状常不典型，主要表现为海绵窦综合征；2、MRI增强扫描有助于海绵窦区肿瘤的鉴别诊断，确诊有赖于手术后病理结果；3、经颞下锁孔入路可切除大部分海绵窦区肿瘤。
[Abstract]:Objective: meningioma, neurilemmoma and cavernous hemangioma are common tumors in cavernous sinus. But relatively rare chordomatoid meningiomas, epidermoid cysts and adenoid cystic carcinomas are rarely reported. The successful surgical treatment of epidermoid cyst, chordomatoid meningioma and adenoid cystic carcinoma of cavernous sinus region in our department was taken as an example. The clinical experience of diagnosis and treatment was summarized in combination with the relevant literature in order to improve the accuracy of preoperative diagnosis. The appropriate surgical approach was chosen to improve the tumor resection rate. Methods: the clinical data of spinal cord meningioma, epidermoid cyst and adenoid cystic carcinoma of cavernous sinus treated successfully by our department were analyzed retrospectively. Clinical, imaging and pathological features of cavernous hemangioma, epidermoid cyst, chordomatoid meningioma and adenoid cystic carcinoma. The advantages and disadvantages of different operative approaches were compared from the anatomic relationship of common operative approaches, intraoperative management measures and possible postoperative complications. Results: the clinical manifestations of cavernous sinus tumors were not obvious. The main clinical symptoms were III~VI cranial nerve involvement. Adenoid cystic carcinoma usually invades the brain from other sites and may have symptoms and signs associated with primary lesions. Neurilemmoma often originates from nerve sheath cells, so it is often characterized by nerve stimulation and paralysis. On MRI, meningiomas are mostly uniformly enhanced, and chordomatoid meningiomas are often unevenly enhanced. Neurilemmoma showed homogeneous or uneven enhancement, while adenoid cystic carcinoma showed heterogeneous enhancement with different size of cystic cavity. On diffusion-weighted (DWI), epidermoid cysts showed high signal intensity, specificity, but no enhancement. Cavernous hemangioma in cavernous sinus area showed obvious high signal intensity on T 2 W image, and enhanced degree was higher than that of meningioma on contrast enhanced scan. The histopathology of meningioma and chordomatoid meningioma was similar. The expression of D2-40 protein was positive in chordomatoid meningioma, but negative in meningioma. Neurilemmoma consists of spindle cells or small stellate cells with various degenerative changes: S-100 Leu-7 is usually positive and focal GFAP is positive. The expression of VEGF-related receptors in cavernous hemangioma is increased. Epidermoid cysts were nodular or cystic with calcification and silt-like changes in the contents. The characteristic histological features of adenoid cystic carcinoma are lump-like proliferative tumor cells arranged in ethmoid and glandular form. Surgical resection of cavernous sinus tumors can be performed via pterygoid or modified pterygoid approach. If the tumor involves orbit, frontoorbital zygomatic approach can be obtained, and for posterior cavernous sinus tumors, subtemporal approach via zygomatic arch should be adopted. Most cavernous sinus tumors can be removed via subtemporal keyhole approach. Conclusion the clinical symptoms of cavernous sinus tumors are often atypical. The main manifestations are that the enhanced MRI scan of cavernous sinus syndrome is helpful to the differential diagnosis of cavernous sinus tumors, and the diagnosis depends on the pathological results after surgery. 3. Most cavernous sinus tumors can be resected via subtemporal keyhole approach.
【学位授予单位】：苏州大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R739.4

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