原发性胸腰椎管内肿瘤延误诊治原因探讨（附44例报告）

本文报告一组44例原发性椎管内肿瘤初期延误诊治,其中10例为腰椎间盘突出而手术,2例为腰椎管狭窄症手术治疗。原发性椎管内肿瘤与脊柱退变性疾病分属两种不同的疾病范畴。为提高诊断和治疗效果,本文就误诊原因作以下讨论。     

原发性腰椎管内肿瘤早期诊治之管见

目的：探讨原发腰椎管内肿瘤早期诊治的方法。方法：对经手术病检证实的17例原发腰椎管内肿瘤病例进行随访和回顾性分析。结果：17例患均经手术切除肿瘤，且通过病检证实。术后随访3－7年。估：11例,良：6例。结果：注重病史的采集和常规检查，有针对性地进行影像学检查，有助于早期诊治。对于原发性腰椎管内肿瘤，手术切除是积极而 肯定的。     

原发性椎管内肿瘤与腰背痛（附73例临床分析）

73例原发性椎管内肿瘤回顾性研究表明，早期症状不典型，易误诊为其它原因引起的颈、肩、腰、背及坐骨神经痛。MRI检查，非离子碘水溶性造影剂脊髓造影为诊断椎管内肿瘤的重要手段。根据临床症状体征特点，针对性选择影像学检查方法，才能早期诊断治疗椎管内肿瘤。     

Primary Malignant Melanoma of Maxillary Gingiva

Malignant melanoma of maxillary gingiva is a rare clinical entity. Mucosal melanoma is more aggressive than cutaneous form and carries comparatively poorer prognosis. High index of suspicion is required for diagnosing malignant melanoma of oral cavity. Any pigmented lesion in the oral cavity should be taken seriously by the clinician.     

椎管内占位性病变的手术治疗

本文报告６１例椎管内占位性病变的手术治疗方法，其部位有：颈椎行“单开门术”暴露椎管，胸椎用电磨行全椎板切除，腰椎则行保留棘上韧带、棘间韧带和棘突的次全椎板切除，并对椎管内外哑铃型肿瘤的手术方法及其它注意事项进行了讨论。     

椎管内结核（附3例报告）

目的：探讨椎管内结核特点，诊疗方法及预后。方法：总结１９８８年９月～１９９６年１０月共收治椎管内结核３例，进行分析讨论。３例中男２例，女１例。平均年龄３４２岁。病灶发生在腰１，２１例，腰３，４２例，其中２例术前曾被误诊。３例均行后路椎板切除，椎管探查、病灶清除术。结果：术后病理结果均为椎管内结核。２例全愈，１例并发完全性截瘫。结论：椎管内结核的临床症状和体征、ＣＴ及ＭＲＩ均和椎管内其它占位性病变难以鉴别，治疗上宜早期手术减压     

Intraspinal Primitive Neuroectodermal Tumour: Report of Two Cases and Review of the Literature

Two patients with primary intraspinal primitive neuroectodermal tumour are presented. In a 32-year-old man, the tumour evolved intradurally from a sacral nerve root. Despite repeated surgery and radiochemotherapy, the patient suffered multiple intraspinal tumour relapses and intracranial seedings, and died 29 months after the first diagnosis. In a 17-year-old male adolescent, the tumour was located in the lumbar epidural space, extending into the paraspinal muscles. Following resection and radiochemotherapy, the patient is free from disease 23 months after the initial presentation. The clinical, radiological, histopathological and cytogenetic findings of both patients are presented and the relevant literature is reviewed. Particular attention is given to the histogenetic relationship between peripheral primitive neuroectodermal tumour and Ewing's sarcoma.     

Primary Malignant Melanoma of the Oesophagus

Primary malignant melanoma arising in the oesophagus is a rare condition with a dismal prognosis. The diagnosis is often made following surgical resection even though the endoscopic features may be pathognomonic. The classical treatment is oesophagectomy even though the advanced disease stage at the time of presentation and aggressive biological behaviour of the tumour usually results in a fatal outcome. We report the case of a male patient initially diagnosed with squamous oesophageal carcinoma and treated with conventional neo-adjuvant chemo-radiotherapy. Poor clinical and radiological response resulted in a review of the original histology confirming a diagnosis of primary malignant melanoma of the oesophagus. The subsequent alteration in management conferred the patient an improved quality of life. A short review of the literature on primary malignant melanoma of the oesophagus supplements this case report.     

椎管内黑色素细胞瘤1例

患者男,40岁,主因“行走凼难伴感觉障碍半年余”入院。患者10余年前无明显诱因出现肢体感觉运动障碍直至颈部以下瘫痪．于当地医院行脊髓肿瘤切除术,术后逐渐恢复正常,但无病理结果。半年前无明显诱因再次逐渐出现行走困难伴感觉障碍,呈进行性加蘑。MRI：延颈交界处脊髓右前方,C2～4椎体平面,C5、6椎体平面脊髓右后方,L3～5椎体平面椎管内可见多发短T1短T2异常信号,T1WI压脂后呈稍高信号,     

Primary Malignant Melanoma of the Small Intestine: a Case Report

The small intestine is the most common site of gastro-intestinal metastasis from cutaneous malignant melanoma. A primary origin at this site has been reported in rare cases. We report a case of a 71-year-old man with a primary malignant melanoma in the jejunum. The patient presented with weakness, weight loss, non-specific abdominal pain and episodes of fainting. After clinical examination, laboratory evaluation and radiological work-up, which included CT of the abdomen, the patient was diagnosed with a tumour mass in the jejunum. This diagnosis was confirmed at laparotomy. The patient underwent enterectomy with wide excision of the tumour. A primary malignant melanoma of the small intestine is an extremely rare neoplasm.

A definite diagnosis can only be made after a thorough investigation has been made to exclude the co-existence of a primary lesion elsewhere. Curative resection of the tumour remains the treatment of choice.     

Primary leptomeningeal melanoma with epipharyngeal invasion

Summary A case of primary leptomeningeal malignant melanoma in a 45-year-old woman is reported. The tumour was located at the basal aspect of the brain with a larger aggregation in the left parasellar and cerebello-pontine angle regions. The tumour showed not only spinal meningeal dissemination but also infiltration into the petrous bone and along the trigeminal nerve till it reached the submucosal tissue of epipharynx. There are a few reported cases of primary leptomeningeal melanoma which invaded areas outside the central nervous system.     

Intraspinal dermoid and epidermoid cysts: Long-term outcome and risk factors

Objective: The literature on long-term outcome after resection of intraspinal dermoid and epidermoid tumors is limited. The purpose of this study was to review the progression-free survival (PFS), overall survival (OS), and long-term outcome in a consecutive series of 57 patients with intraspinal dermoid and epidermoid tumors.

Design: Retrospective study.

Methods: A total of 57 patients who underwent surgery at our institution between 2002 and 2010 were reviewed. Patients outcome were determined using the Japanese Orthopaedic Association score (JOA) and the McCormick score.

Results: The follow-up data were 100% complete and the median follow-up time was 9.2 years. Gross total resection was performed in 21 patients (36.84%) and subtotal resection in 36 patients (63.16%). The PFS and OS at 8 years were 78.95% and 100% respectively. A good outcome was observed in 56.14% of patients based on the JOA and McCormick score. The univariate analysis showed that a tumor size of more than 4?cm, subtotal resection and sphincter disturbances were the influencing factors of poor outcome.

Conclusion: The gold standard treatment for intraspinal tumors is gross total resection, but the operation needs to protect the remaining nerve function as much as possible and follow-up should be focused on patients with a high risk of poor outcome.     

Defining the Role of Surgery for Primary Gastrointestinal Tract Melanoma

Objective The objective of the study was to determine the outcomes for primary gastrointestinal melanomas (PGIM). Material and methods The Surveillance, Epidemiology, and End Results database (1973–2004) was queried. Results Overall, 659 cases of PGIM were identified. The annual incidence of PGIM was approximately 0.47 cases per million in 2000. Overall median survival time was 17 months. Tumors were identified in the oral–nasopharynx (32.8%), anal canal (31.4%), rectum (22.2%), esophagus (5.9%), stomach (2.7%), small bowel (2.3%), gallbladder (1.4%), and large bowel (0.9%). Univariate analysis demonstrated age, tumor location, stage, surgery, and lymph node status were significant predictors of improved survival. MST has not been reached for tumors located in the large bowel, while tumors located in the stomach demonstrated the shortest median survival (5 months). Improvement in MST was observed for those patients undergoing surgical resection. The presence of lymph node involvement conferred a poorer prognosis. Multivariate analysis of the cohort identified that location, advanced tumor stage, failure to undertake surgical resection, positive lymph node status, and age were all independent predictors of poorer outcome. Conclusion PGIM occurs most often in the oral–nasopharynx and anal canal. Surgical extirpation is the only identifiable treatment modality that significantly improves survival.     

肛管直肠恶性黑色素瘤

目的　总结肛管直肠恶性黑色素瘤的诊断与治疗经验。　方法　回顾性分析 10例肛管直肠恶性黑色素瘤的临床病例资料。　结果　出现症状至确诊时间平均 8( 2～ 12 )个月 ,首诊确诊 4例 ,误诊 6例。手术 9例 ,8例肿瘤直径 >3cm。其中 8例行腹会阴联合根治术 ,1例以直肠息肉、肛乳头肥大行局部切除 ,1例放弃手术仅行化疗 ,3月后死于肝、肺腹股沟等广泛转移。术后 8例行化疗 ,2例辅助放疗 ,化放疗联合应用 1例。术后 2年内死亡 5例 ,余 4例存活 ,存活最长者已超过 6年。　结论　肛管直肠恶性黑色素瘤恶性程度高 ,早期行腹会阴联合根治术是最优选择。长期生存有赖于早期治疗以及治疗方法的改进     

椎管内肿瘤400例临床分析

作者自1976～1996年共收治椎管内肿瘤400例。本文对400例椎管内肿瘤主要临床表现、特异性检查临床符合率、手术效果及病理结果进行分析，阐明椎管内肿瘤的诊断、定位、定性方法，进一步指出椎管内肿瘤多为良性肿瘤，皆应手术治疗，以期达到不同程度的缓解。术后完全治愈及功能大部分恢复者合计达64．70％。     

椎管内肿瘤的诊断治疗

目的：总结椎管内肿瘤的诊断治疗经验。方法：回顾性分析1992～2003年123例椎管内肿瘤的治疗结果。结果：脊膜瘤24例（19．5％）,神经鞘瘤／神经纤维瘤32例（26．0％）,室管膜瘤8例（6．5％）,星形细胞瘤13例（10．6％）,脂肪瘤10例（8．1％）,转移瘤5例（4．1％）,血管瘤5例（4．1％）,蛛网膜囊肿9例（7．3％）,其它17例（13．8％）。其中髓外硬膜内57．7％,硬膜外21．1％,髓内22．8％。颈段30．1％,胸段37．4％,腰段19．5％,骶段13．0％。髓外肿瘤70．1％,髓内肿瘤60．7％,全切除率69．1％。运动改善率75．2％。感觉障碍改善73．8％,括约肌功能改善77．8％。结论：椎管内肿瘤以良性居多,手术效果好,MRI检查和显微外科技术的应用是提高治疗效果的关键。如果术中破坏了脊柱的稳定性,重建稳定性非常必要。     

Malignant Melanoma of the Kidney Presenting as a Primary Tumor

We report a case of malignant melanoma of the kidney presenting as a primary tumor. This tumor was found incidentally in a 74-year-old woman. The patient underwent a right radical nephrectomy, and has been living tumor free for 2 years and 3 months. This is the first reported case of primary renal malignant melanoma. We discuss the probability that this tumor is renal in origin and directly linked to the origin of malignant melanoma.