Intramedullary neuroma of the cervicomedullary junction. Case report

Neuromas typically arise in the peripheral nervous system in response to traumatic injury at the site of partial or complete nerve transection as new axons from the proximal nerve stump sprout to reinnervate the distal segment. In rare cases neuromas have also been described as intramedullary spinal cord lesions. These lesions have been identified as incidental autopsy findings in association with prior trauma and cervical spondylosis, multiple sclerosis, spinal tumors, and syringomyelia. The authors report the case of a 50-year-old man who had been involved in a motor vehicle accident, during which his car was struck from behind as it was stationary at an intersection, more than 5 years before presentation. A workup for syncopal and presyncopal episodes involved magnetic resonance imaging that revealed a 1.1-cm lesion at the cervicomedullary junction (CMJ). The imaging features of the lesion raised the question of an ependymoma or subependymoma. The lesion was excised, and examination of the tissue demonstrated a neuroma with haphazardly arranged interlacing bundles of axons ensheathed by Schwann cells with interfascicular regions of reactive glial cells and Rosenthal fibers, consistent with those present after traumatic injury. This case may represent the first true traumatic intramedullary neuroma of the CMJ diagnosed in a living patient and treated surgically.     

Intramedullary fibrosarcoma of the cervicomedullary junction: a case report

A 17-year-old girl presented with signs of increased intracranial pressure. On computed tomography, an enhancing intraaxial lesion in the region of the foramen magnum was demonstrated. Surgical excision was performed. The pathological diagnosis was low grade fibrosarcoma. Her subsequent course was complicated by cerebrospinal fluid seeding, a posterior fossa recurrence, and repeated subarachnoid hemorrhage with cerebral vasospasm. A combination of radiotherapy and intraventricular chemotherapy has left the patient symptom-free 2 years after operation. The intramedullary appearance of fibrosarcoma and the unusual subsequent clinical course are discussed.     

Intramedullary melanotic schwannoma of the cervical spinal cord: report of a case

We present a case report of a patient with an intramedullary tumor of the midcervical cord. At surgery, the lesion was found to be highly pigmented, and pathological analysis revealed a melanotic schwannoma. Intramedullary schwannomas and melanotic schwannomas are exceedingly rare. This is the second reported case of an intramedullary melanotic schwannoma of the central nervous system.     

Intramedullary melanotic schwannoma. Report of a case and review of the literature

A case of an intramedullary melanotic schwannoma located in the spinal cord at the T2-T3 level is described. The lesion occurred in a 44-year-old woman with a 10-year history of weakness and sensory numbness in both legs and feet. At operation the lesion appeared as a well-demarcated grey-brown intramedullary mass. Histologically, it was composed of interlacing bundles of spindle cells showing their cytoplasm filled with melanin. Among spinal cord neoplasms, melanotic schwannomas are rare tumours, which have apparently been reported only in three previous instances. The clinical, diagnostic and pathological features, as well as the possible aetiology of these rare tumours are discussed.     

Intra-axial tumors of the cervicomedullary junction

The authors present their experience with the operative management of 20 intra-axial tumors of the cervicomedullary junction. There were two distinct modes of clinical presentation: lower cranial nerve dysfunction and spinal cord dysfunction. Both groups of patients had indolent courses: in 75% of the patients the symptoms had been present for 6 months to 2 years. Radical excision was carried out in all patients. There was no surgical mortality. Postoperative neurological recovery was directly related to the preoperative status; one patient had a significant new deficit. The authors conclude that intrinsic gliomas of the cervicomedullary junction are amenable to radical excision and that an aggressive surgical approach offers the potential for both neurological recovery and long-term survival. The neuroradiological evaluation and operative technique are discussed.     

Intramedullary thoracic schwannoma.

A case of intramedullary thoracic schwannoma in a 40-year-old woman without signs of von Recklinghausen's disease is reported and the hypotheses as to its origin are discussed. The literature covering 11 other cases is reviewed.     

Tussive presentation in cervicomedullary junction malformations

Two adult patients with craniocervical junction malformations presented with tussive symptom onset. These patients had acute onset of permanent neurologic symptoms. One patient had basilar invagination with tonsilar herniation and the second, a Type 1 Arnold-Chiari malformation with cord widening. Possible mechanisms include: 1) external compression of cervicomedullary structures by posterior fossa tissue that had descended through the foramen magnum secondary to increased intracranial pressure, and 2) parenchymal shear stress secondary to this caudal repositioning of these structures.     

骶管内黑色素性神经鞘瘤1例

1病例资料患者,女,43岁,因"骶部、右侧臀部及右下肢酸痛、麻木不适3年余,加重半个月"于2010年8月13日入院。患者于2008年无明显诱因出现腰骶部、右侧臀部及右下肢酸痛、麻木不适,行走及活     

Intrathoracic psammomatous melanotic schwannoma associated with the Carney complex

The Carney complex is a multiple neoplasia syndrome characterized by myxomas, schwannomas, mucocutaneous spotty pigmentations, and endocrine overactivity with or without endocrine tumors. Herein, we report the rare case of a 49-year-old man with a paravertebral intrathoracic tumor, a history of bilateral adrenalectomy, and resection of an atrial myxoma. A thoracoscopic en-bloc tumor extirpation with minimal safety margins was performed. Histopathologic examination revealed the diagnosis of a malignant psammomatous melanotic schwannoma that is associated with the Carney complex in 50% of these patients. Prognosis of all melanotic schwannomas is usually poor due to local recurrences or metastases. Although treatment guidelines for this rare tumor do not exist, radiotherapy was performed in our patient to prevent possible recurrence or regrowth of this malignant tumor. Twenty-four months after operation the patient showed no signs of tumor recurrence or metastases.     

Spinal melanotic schwannoma: report of a case

A case of spinal dumbbell shaped melanotic schwannoma was reported. A 58-year-old housewife had a 3-months history of progressive gait disturbance. She also complained of mild backache and numbness in both legs. Her family history was not remarkable. When examined on admission, October 10, 1982, mild weakness of both legs with spasticity and sensory impairment below the level of T10 dermatome without sacral sparing were evident. Her deep tendon reflexes were hyperactive on both sides and plantar responses were extensor bilaterally. Sphincteric disturbance was not significant. The function of her cranial nerves was intact. She had neither cutaneous lesions, abdominal mass nor organomegaly. Thoracic plain X-rays revealed erosion of the right side vertebral body and pedicle of the 10th thoracic vertebra. Myelography disclosed a complete block at the same level by an epidural mass. On CT-myelogram, soft tissue density mass compressing the thoracic cord was apparent in the right epidural space of the spinal canal which extended to the paravertebral region through the right intervertebral foramen. Partial destruction of the body and the right side pedicle was easily recognized. Laminectomy from T9 to T11 exposed a large extradural mass which was encapsulated, elastic soft and pigmented in nature. The tumor was dumbbell shaped and extended to the right paravertebral region through the intervertebral foramen. Costotransversectomy was performed to excise the mass entirely. Following the total removal of the tumor, internal fixation was carried out by means of Harrington instrumentation with methylmethacrylate.(ABSTRACT TRUNCATED AT 250 WORDS)     

Dumbbell-shaped nonpsammomatous malignant melanotic schwannoma of the cervical spinal root

Background contextMelanotic schwannoma is a very rare tumor of Schwann cell origin, which can develop in various locations, similar to conventional schwannoma. This tumor has a malignant potential and therefore careful therapy is required.PurposeTo describe a case of melanotic schwannoma with a histopathologically and clinically malignant behavior.Study designCase report.MethodsA 64-year-old man presented with sensory changes in his arm and gait disturbance. Magnetic resonance imaging revealed a dumbbell-shaped tumor at the left C7 spinal root, which was hyperintense on T1-weighted images and generally hypointense on T2-weighted images in comparison with conventional schwannoma; however, the peripheral zone was relatively hyperintense, and the central zone was hypointense like a target sign.ResultsThe tumor was partially resected and diagnosed to be nonpsammomatous malignant melanotic schwannoma. The patient experienced local recurrence and metastases to the bone and lung and finally developed quadriplegia. Radiation therapy failed to palliate the symptoms.ConclusionsSome melanotic schwannomas present with an aggressive behavior, which thus leads to poor prognosis. We should therefore be familiar with its characteristic clinical imaging and pathologic findings to provide a correct diagnosis and appropriate treatment for such patients.     

Spinal malignant melanotic schwannoma. Case report.

The authors report a rare case of spinal cord compression syndrome due to a malignant melanotic schwannoma. Pathogenesis, diagnostic difficulty and therapeutical problems are discussed. The authors conclude that such tumours should be surgically treated due to the possibility of a benign clinical behaviour even in those cases showing malignant histological features.