Primary Spinal Cord Melanoma

Primary central nervous system (CNS) melanoma is a rare condition that accounts for only 1% of all melanomas. A 34-year-old Korean female presented with a two-month history of progressive weakness in both legs. Spinal magnetic resonance image (MRI) revealed a spinal cord tumor at the level of T4, which was hyperintense on T1-weighted imaging and hypointense on T2-weighted imaging. The intradural and extramedullary tumor was completely resected and diagnosed as melanoma. There were no metastatic lesions. At three years after surgery, the patient is still alive, with no evidence of tumor recurrence. We present the details of this case along with a comprehensive review of spinal cord melanoma.     

Intramedullary Solitary Fibrous Tumor of Cervicothoracic Spinal Cord

Solitary fibrous tumor is rare benign mesenchymal neoplasm. The spinal solitary fibrous tumor is extremely rare. The authors experienced a case of intramedullary solitary fibrous tumor of cervicothoracic spinal cord in a 48-year-old man with right lower extremity sensory disturbance. Spinal MRI showed intradural mass lesion in the level of C7-T1, the margin between the spinal cord and tumor was not clear on MRI. A Left unilateral laminectomy and mass removal was performed. Intra operative finding, the tumor boundary was unclear from spinal cord and it had intramedullary and extramedullary portion. After surgery, patient had good recovery and had uneventful prognosis. Follow up spinal MRI showed no recurrence of tumor.     

Primary spinal melanoma with bilateral papilledema

A case of primary leptomeningeal malignant melanoma localized in the cervical region in a 41-year-old woman is presented. The only clinical finding was intracranial hypertension with papilledema. A diagnosis of primary CNS melanoma was made after dermatological and ophthalmological consultations, ruled out a metastatic lesion. Primary leptomeningeal melanoma is an extremely rare spinal tumor. Its clinical presentation with signs of increased intracranial pressure but without cord symptoms is unusual. Clinical features of this case including the radiological and histologic findings are described. Diagnosis as well as management is discussed.     

颅内原发性黑色素瘤的诊断和治疗

目的 探讨颅内原发性黑色素瘤的临床特点,提高对此病的诊断治疗水平.方法 回顾性分析自2000年1月至2009年12月收治的8例颅内原发性黑色素瘤患者,男6例,女2例;年龄24～68岁;病灶均为单发,位于额叶4例,顶叶1例,枕叶1例,镰旁1例,小脑脑桥角1例.8例患者均行开颅手术,6例达全切;2例行次全切除+辅助伽玛刀治疗.结果 病理结果均为原发性黑色素瘤;术后7例患者获随访,随访时间10-82个月.死亡2例,分别于术后14个月及21个月死于肿瘤复发;1例于术后6个月复发,再次行手术全切,术后化疗,术后6个月MRI未见复发征象;1例于术后9个月复发,再次行手术全切,术后化疗,术后6个月MRI示肿瘤再次复发;余3例未见复发征象.结论 颅内原发性黑色素瘤发病率低,误诊率较高,治疗以手术切除为主,不全切除者应辅助伽玛刀治疗,复发者可行辅助化疗,延缓复发时间,改善患者预后.

Abstract：

Objective To investigate the clinical features, imaging findings and treatment for primary intracranial melanoma. Method The clinical data including presentation, imaging, treatment and prognosis of 8 patients with primary intracranial melanoma underwent surgery were reviewed during the past ten years. The patients included six males and two females and the age range was from 24 to 68 years. Four lesions were located in frontal lobe and the other four were located in parietal lobe, Occipital lobe, parafalcine and cerebellopontine angle respectively. Total removal was achieved in 6 patients and subtotal resection in 2 who had adjuvant radiosurgery. Results Histological examination showed that all cases were melanoma. Follow - up study was gained in seven cases and the follow - up duration was from 10 to 82 months. Two patients died from recurrence at postoperative 14th months and 21st months respectively. One case had tumor recurrence 6 months after surgery and another operation was performed to gain total removal. Adjuvant chemotherapy was performed and there was no recurrence found in MRI 6 months after the surgery. One patient had tumor recurrence 9 months after surgery and another operation was performed to gain total removal. Adjuvant chemotherapy was performed and follow - up MRI showed recurrence 6 months after surgery. There was no recurrence in the other 3 cases. Conclusions Primary intracranial melanoma is an extremely rare tumor and difficult to get correct preoperative diagnosis. Surgical removal of tumor plays an important role in its treatment. Part of patients could gain profits from radiotherapy and chemotherapy. The prognosis is very poor.     

原发性脊髓恶性黑色素瘤1例报告及文献复习

目的 探讨原发性脊髓恶性黑色素瘤的临床特点。方法 报告1例原发性脊髓恶性黑色素瘤的详细临床及病理资料，并结合献，对该罕见疾病的临床诊断、影像学、组织病理学及治疗等做一复习。结果 MRI显示肿瘤位于于髓外、硬脊膜下，以胸段为甚，波及全脊髓。T1加权稍高信号，T2加权呈高信号，增强后有明显强化。随着病程发展，肿瘤扩散至颅内。手术证实肿瘤位于软脊膜下并侵犯脊髓。组织病理、免疫组织化学电镜均表现为典型的恶性黑色素瘤特征。结论 该病例诊断为原发性脊髓恶性黑色素瘤，因肿瘤广泛扩散，术后化疗及免疫治疗效果不佳，发病约1年后死亡。     

Primary spinal cord melanoma - a case report

Authors present a case of a 57-year-old woman with primary spinal cord malignant melanoma. Intramedullary localization of primary melanoma is extremely rare. The patient presented neurological deficits such as lower limbs paresis and sensory loss. MRI examination showed intramedullar tumor located on the Th10 vertebra level. Surgical treatment with total removal of tumor was performed. Histopathological study confirmed melanoma. Subsequent chemotherapy was given. Tumor was successfully treated by neurosurgery; radio- and chemotherapy with disease free follow up of 9 months. Surgical treatment of melanoma in this location is extremely important as it leads to regression of neurological symptoms and improvement of the quality of life.     

显微手术治疗高颈髓肿瘤(53例报告)

本文报告53例高颈髓肿瘤,计髓外肿瘤31例,髓内肿瘤22例。总的肿瘤全切除率达88.7％,临床改善率为81.1％。无手术死亡病例。作者认为,临床诊断高颈髓肿瘤时,需与运动神经元疾病,多发性硬化,脑干脑炎和颈椎病等相鉴别;MR对确诊高颈髓肿瘤最有帮助。本文还分析了影响肿瘤切除程度的相关因素和描述了手术方法与注意事项,指出显微手术能提高肿瘤全切除率和术后疗效。     

Cordectomy as final treatment option for diffuse intramedullary malignant glioma using 5-ALA fluorescence-guided resection

Background

We present a case of an anaplastic astrocytoma (WHO-grade III, AA III) in a 27-year-old woman treated by spinal cordectomy. The patient was pretreated by surgery, radiation therapy and temozolomide chemotherapy and repeat surgery at recurrence. Later on, she developed paraplegia and a diffuse severe pain syndrome. MRI demonstrated intramedullar invasion from T12 to T9. To assess tumor invasion intraoperatively, we used tumor fluorescence derived from 5-aminolevulinic acid (5-ALA).

Patients course

The spinal cord was amputated caudally to the root entry zones of the T10 sensory roots. Additional cordectomy was performed because of tumor infiltration at the cut end to T9 as identified by intraoperative tumor fluorescence, and as verified histologically. The final transected level was between T8 and T9, and the cut end did not reveal any tumor invasion intraoperatively by tumor fluorescence and postoperatively by MRI and with regard to the pathological result. After surgery, the patient was unchanged concerning spasticity, motor and sensory function, and showed complete relief of pain. She refused additional adjuvant therapy. The patient is free of recurrence 15 months after surgery.

Conclusion

Our observation suggests 5-ALA fluorescence-guided resections to be useful in the context of malignant spinal cord gliomas. Furthermore, our particular case indicates that palliative spinal cordectomy with a wide margin and intraoperative resection using fluorescence guidance may be a final option for patients with recurrent spinal malignant glioma presenting with complete deficit below the lesion.     

Mature neuroblastic tumors with spinal cord compression: report of five pediatric cases

Background Neuroblastic tumors cause spinal cord compression when they arise primarily in the spinal canal or invade it through the radicular foramen. Whereas neuroblastomas (NB) are relatively common and are generally treated with chemotherapy, mature neuroblastic tumors (MNT), which include intermixed ganglioneuroblastomas (iGNB) and ganglioneuromas (GN), are less common and the role of surgery is more prominent. Because MNT are rare and have been separated only recently from NB, their clinical and radiological features as well as the role of surgery are poorly defined.Materials and methods In order to increase our knowledge on MNT, we reviewed our database for cases operated for spinal cord compression in our department since the introduction of magnetic resonance imaging (MRI). We treated four cases of NB and one case of iGNB presenting primarily with spinal cord compression.Report of cases MNT represented 10% of spinal tumors and 1.6% of all tumors of the nervous system in our pediatric neurosurgical practice. The neurological and oncological outcomes were generally favorable after surgical resection, followed by orthotic treatment. In one case with neurofibromatosis type 1, the tumor was inoperable and the child died of tumor progression several years later.Conclusion GN, and some iGNB, are chemo-insensitive and can only be cured by surgical removal. Surgery is an emergency in case of rapidly progressing paraplegia and can be challenging because the tumor is often hard and hemorrhagic. In case of subtotal removal, tumor remnants can stay stable without oncological treatment.     

Diagnostic and therapeutic strategy for confounding radiation myelitis

We report a case of confounding radiation myelitis to demonstrate the usefulness of surgical biopsy in ensuring the correct diagnosis and to avoid unnecessary treatment. The patient was a 40-year-old man with a history of epiglottis carcinoma and sarcoidosis. Six months after radiation therapy and chemotherapy for epiglottis carcinoma, he noticed paresthesia and dysesthesia in the left arm and leg. Two months after that, he complained of severe neck pain and rapidly progressing weakness in all extremities. MRI showed an enhanced intramedullary lesion with extensive edema in the cervical spinal cord. Radiation myelitis, intramedullary spinal tumor, and neurosarcoidosis were considered as differential diagnoses. Spinal cord biopsy with laminectomy was performed and radiation myelitis was diagnosed. After the surgery, the lesion was significantly decreased in size even though corticosteroid therapy was rapidly tapered. We emphasize that a spinal cord biopsy is indicated to obtain a pathological diagnosis and to make a clear treatment strategy for patients with associated diseases causing lesions of the spinal cord.     

Primary Intracranial Leptomeningeal Melanomatosis

Primary intracranial malignant melanoma is a very rare and highly aggressive tumor with poor prognosis. A 66-year-old female patient presented a headache that had been slowly progressing for several months. A large benign pigmented skin lesion was found on her back. A brain MRI showed multiple linear signal changes with branching pattern and strong enhancement in the temporal lobe. The cytological and immunohiostochemical cerebrospinal fluid examination confirmed malignant melanoma. A biopsy confirmed that the pigmented skin lesion on the back and the conjunctiva were benign nevi. We report a case of primary intracranial malignant melanoma and review relevant literatures.     

颅内原发黑色素瘤的MRI、CT分析

目的探讨颅内原发黑色素瘤的影像学表现,以提高对该病的认识和诊断正确率。方法回顾性分析经手术病理证实的4例颅内原发黑色素瘤MRI、CT表现;4例患者均进行MRI检查,其中3例同时行CT检查。结果CT平扫均为高密度,密度部分不均匀,增强扫描肿瘤明显强化。4例MRIT1WI肿瘤均表现为高信号为主,其中2例T2WI为低信号,2例为高低混杂信号,肿瘤内信号部分不均匀,增强后扫描肿瘤可见明显强化。结论颅内原发黑色素瘤MRI具有特征性表现,对于显示肿瘤范围、成分及脑膜的浸润和定性诊断较CT有优势。     

Tumor of the conus medullaris treated with antituberculous therapy

This case report concerns a 21-year-old man developing left leg paresis. Evaluation with magnetic resonance imaging (MRI) showed an intramedullary tumor in the conus region. He was planned for surgery but preoperative investigation indicated he had tuberculosis and the tumor was presumed to be a tuberculoma. Antituberculous therapy was started and the patient improved neurologically. The patient was followed clinically and with consecutive MRI during 2 years and the last MRI showed that the lesion had disappeared completely. Intramedullary tuberculomas are rare but important differential diagnosis in patients with spinal cord mass lesions. The role of medical and surgical treatment of intramedullary tuberculomas is discussed.     

颅内黑色素瘤的诊断和治疗

目的 讨论颅内黑色素瘤的临床特点、影像学特征及治疗方案.方法 回顾性分析11例经证实的颅内黑色素瘤患者的资料,分析其临床表现、影像学特征,并对其临床特点和治疗方案进行讨论.结果 头部CT及MRI多可见瘤周有点状或条索状卫星灶.典型的黑色素瘤MRI表现为T1WI高信号、T2WI低信号,该表现具有特异性.11例手术中,4例全切,6例近全切,1例多发性转移瘤部分切除.术后患者多在3-12个月内死亡,最长不超过2年,平均生存期不到6个月.结论 颅内黑色素瘤发病率低,误诊率高,治疗以手术切除为主,放化疗及免疫疗法仅可使部分患者受益,预后极差.     

Intramedullary spinal cord metastasis as the first manifestation of a renal carcinoma

We present the case of a 69 year-old male without known antecedente who presented a clinical loss of distal force in his lower limbs during the last two months. Magnetic resonance imaging (MRI) showed an intramedullary spinal cord mass at the level of the medullaris cone. The patient was operated on; the histological diagnosis was clear cells carcinoma suggestive of metastasis from an unsuspected renal tumor that was later confirmed with an abdominal CT. When the patient's state is good, surgery can correct the neurological deficit produced by an intramedullary spinal cord lesion. The neurological state of our patient improved after the intervention, and 14 months after surgery, he has no neurological deficit in the lower limbs.     

2009 ASN Abstracts

Edward Tay, Rishi Gupta
Cleveland Clinic, Cleveland, OH, United States
Introduction: Spinal cord infarct is much less frequent than cerebral infarction, accounting for only 1% of all strokes. Most cases of spinal cord ischemia have been associated with aortic surgery or prolonged arterial hypotension. We describe a case report of spinal cord ischemia complicated by haemorrhage, initially thought to be a case of transverse myelitis.
Patients (or Materials) and Methods: A 42 year old Kuwaiti woman with history of cardiac valve surgery 2 months ago, on anticoagulation, presented with several day history of lower extremity weakness and bowel and bladder incontinence. Exam was significant for a T8 sensory level, flaccid paraplegia, and loss of LE reflexes. Infectious and inflammatory workup was negative. MRI spine and spinal angiogram were done which revealed the diagnosis. We present a case of spinal cord ischemia complicated by haemorrhage found on imaging and looked at the relevant literature.
Results: MRI T1 thoracic and lumbar spine revealed blood products in the lower thoracic and entire lumbar spine. A spinal angiogram revealed the absence of the artery of Adamkiewicz. CT abdomen showed splenic and liver infarcts consistent with thromboembolic disease.
Conclusion: MRI imaging can be helpful in the diagnosis of spinal cord ischemia. Sagittal and axial T2 weighted images are considered the most useful. Spinal angiography can also be of use in diagnosing AVMs of the spine and spinal artery occlusion. Certain clinical features are associated with different areas of the spinal cord. This combined with MR imaging and selective use of spinal angiography can help lead to a definitive diagnosis.     

Intramedullary subependymoma of the cervical spinal cord: a case report with immunohistochemical study

Spinal subependymomas, which have a relatively benign nature, are very rare tumors. It is difficult to distinguish spinal subependymomas from other intramedullary spinal tumors based on neuroradiological findings. A case of cervical intramedullary subependymoma in a 63-year-old female is reported. The diffused enlargement of the spinal cord at C2 level involved the lesion with isointensity on a T1-weighted MRI and relatively high intensity on a T2-weighted MRI. Enhancement in the small part of the tumor was observed on a T1-weighted MRI with gadolinium administration. The tumor occupied the left side of the spinal cord, and was totally removed through a laminoplasty of C2. Immunohistochemistry was useful for pathological diagnosis. The clinical feature of this patient is described with the review of literatures.     

Primary intracerebral malignant melanoma with leptomeningeal spread, extradural extension and lung metastasis

A rare primary intracerebral solitary malignant melanoma with extracranial metastasis is reported. The patient was a 66-year-old man who was revealed to have a tumor in the right temporal lobe by MRI. The tumor was totally resected and the histopathological diagnosis was malignant melanoma. Despite postoperative treatment with chemo-radiotherapy, tumor recurred with multiple subarachnoid dissemination. Complete autopsy showed marked leptomeningeal dissemination of tumor in the cerebro-spinal compartment. In the outside of the central nervous system, a microscopic metastatic nodule was found in the right lung. Metastatic tumor in the spinal cord showed the extradural extension which might have led to hematogenous metastasis to the lung.     

脑膜恶性黑色素瘤病三例临床病理及神经影像学特点

目的 探讨脑膜恶性黑色素瘤病的临床、病理及神经影像学特点.方法 对我院收治的经病理确诊的3例脑膜恶性黑色素瘤病患者进行观察,总结其临床、脑脊液细胞学、神经影像学、脑膜组织病理等方面特点.结果 3例患者均以头痛起病,随后出现脑膜刺激征.其中1例皮肤有巨大黑色素痣,1例额部黑痣破溃经久不愈,另一例无皮肤及内脏黑色素瘤,为原发性.3例患者颅脑MRI强化后显示软脑膜及蛛网膜弥漫性较均匀强化并增厚.脑脊液可见大最的异形细胞.脑膜呈黑褐色或深棕色,光镜下肿瘤细胞呈多形性,核大而圆或呈不规则形,胞质丰富,核分裂象可见,胞质内黑色素颗粒聚集,细胞排列紊乱.免疫组织化学分析S-100蛋白、波形蛋白、黑色素瘤抗体HMB-45等胞质反应阳性.结论 脑膜恶性黑色素瘤病临床主要表现为头痛及脑膜刺激征,脑脊液可见大量的异形肿瘤细胞.脑膜病理见肿瘤细胞胞质内黑色素颗粒聚集、瘤细胞排列紊乱.颅脑强化MRI对该病的诊断具有一定价值.     

Intramedullary epidermoid cyst. Contribution of magnetic resonance imaging

Epidermoid tumors are seldom found as a cause of spinal cord compression; still fewer involve the spinal cord itself. We report a case of a thoracic intramedullary epidermoid tumor documented by MRI.