Cases reported "Spinal Cord Neoplasms"

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1/45. Angiotropic large B-cell lymphoma with clinical features resembling subacute combined degeneration of the cord.

    Angiotropic large cell lymphoma is a rare neoplastic disorder associated with a high mortality. The hallmark of the disease is lymphoid proliferation confined to the intravascular compartment without local tissue or vessel wall infiltration [1]. This feature is so striking that the disease was originally thought to arise from endothelial tissue and early cases were described as malignant angioendotheliomatosis. However, application of immunohistochemical methods for detection of lymphoid markers such as the CD45 and CD20 cell surface markers has confirmed its lymphoid origin, usually of B-cell lineage [2]. Clinical manifestations of the disease are protean and are due to multifocal medium and small vessel occlusion by tumour cells [3]. Characteristic sites of involvement are skin and central nervous system and although an ante-mortem diagnosis can be made from a biopsy specimen, it is often unsuspected [4]. We present a case of angiotropic large B-cell lymphoma in a 74-year-old man who presented with urinary symptoms and had a neurological picture resembling subacute combined degeneration of the cord.
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2/45. Capillary hemangioma of the spinal cord. Report of four cases.

    The authors describe the clinicopathological features of four cases of capillary hemangioma of the spinal cord. All occurred in adult patients. The presenting symptoms were similar to those of more common intramedullary tumors. Radiologically, they resemble other vascular spinal cord tumors. All patients underwent surgery, and the outcomes varied. Histologically, the lesions resembled capillary hemangioma of skin or of soft tissue that is composed of lobules of small capillaries with associated feeding vessels, all enveloped by a delicate fibrous capsule. Capillary hemangiomas of the central and peripheral nervous system are extremely rare. Although examples of these lesions have been described as occurring in the dura mater and in peripheral nerve, including spinal nerve roots, none has occurred within the spinal cord. knowledge of their existence may help practitioners to avoid misdiagnosis of tumor and resultant overtreatment of these benign lesions.
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3/45. Isolated hemangioblastoma of the filum terminale. Case report.

    The filum terminale is an exceptional location for isolated hemangioblastoma. Only five cases are reported in literature, prior to the magnetic resonance imaging era. A 57-year-old man was referred to our Department with a prolonged history of progressive back pain, particularly severe when recumbent, and recurrent unilateral sciatalgia. Computed tomography demonstrated a non-homogeneous mass at L4 level. magnetic resonance imaging displayed earliness and homogeneous enhancement of the mass with tortuous vessels above the rostral pole of the tumour, suggesting the diagnosis of a vascular tumour. The tumour was totally removed. Histological examination confirmed the hemangioblastoma diagnosis. The case indicates that hemangioblastoma, although uncommon, must be taken into consideration in cauda equina tumour diagnosis, and also emphasises the specificity of magnetic resonance features.
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4/45. Benign isolated fibrohistiocytic tumor arising from the central nervous system. Considerations about two cases.

    Benign fibrous histiocytomas (BFHs) are tumors with fibroblastic and histiocytic components without histological anaplasia. Intracerebral lesions are exceptional and to our knowledge a spinal location was not yet described. We describe 2 cases of BFHs of the neural axis: the first, a 22-month-old boy with Down's syndrome, presented with a paraparesis and the magnetic resonance (MR) of the spine disclosed an intradural extramedullary, thoracic mass, totally resected; the second, a 13-year-old boy with left partial motor seizures, in whom the MR of the brain showed an intracerebral, right frontal tumor, also surgically removed. Both patients are free of recurrence, 6 years and 15 months after surgery, respectively. Histological examination and immunoreactivity for vimentin and histiocytic markers favored the diagnosis of BFH. It is likely that these tumors may originate from spinal dura mater mesenchymal stem cells and from the intracerebral perivascular pial sheath or the brain vessel walls themselves, respectively. Other benign, isolated, intracranial fibrohistiocytic neoplasms, namely the juvenile xanthogranuloma, can harbor a clinical, morphological and immunohistochemical profile overlapping the one of the BFH. Intracranial germ cell tumors may be associated with Down's syndrome, although harboring an unusual, non-pineal and non-chiasmatic location. One can speculate that a similar, still unknown genetic mechanism responsible for this association, could also induce the growth of other type of tumors in patients with this syndrome. BFHs should be added to the differential diagnosis of intracerebral or spinal dural attached tumors. Furthermore, we propose to name these intracranial tumors "benign isolated fibrohistiocytic tumors of the CNS".
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5/45. Intramedullary ancient schwannoma of the cervical spinal cord: case report and review of literature.

    We report a rare case of intramedullary ancient schwannoma of cervical spinal cord in a 68 year old patient. About 49 cases of intramedullary schwannomas and neurofibromas have been reported in the literature but to our knowledge there is no report of the 'ancient' variety of intramedullary schwannoma. The cell of origin of these tumours is the schwann cell, which normally does not exist in the parenchyma of the central nervous system. Many theories have been advanced to explain this paradox. According to one theory, these tumours arise from the perivascular nerve plexus of the pial vessels. This plexus was found mostly to exist along the branches of the anterior spinal artery. In our case, the tumour was supplied by two branches of the anterior spinal artery, which may add further support to the above theory.
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6/45. Atraumatic bloodless removal of intramedullary hemangioblastomas of the spinal cord.

    OBJECT: The goal of this study was to summarize the author's personal experience in the surgical treatment of 19 patients with intramedullary spinal cord hemangioblastomas. methods: All cases were from the author's private practice and were treated between 1967 and 1990. In all cases the intramedullary hemangioblastomas were totally removed by using a unique microsurgical technique of bipolar coagulation, which is fully described in this paper. A bipolar forceps was used to shrink each tumor and detach it from its feeding and draining vessels. Tumor resection was successfully accomplished in all patients. Blood loss was minimal, averaging less than 100 ml, and what little bleeding occurred did so during laminotomy or laminectomy. No bleeding occurred during tumor removal, and no transfusions were given. All patients were symptomatic preoperatively, and all recovered or improved following surgery. CONCLUSIONS: The technique described in this paper makes tumor removal safe, effective, and relatively easy.
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7/45. Primary rhabdomyosarcoma of the central nervous system.

    A case of primary rhabdomyosarcoma in the brain stem is described in an 8 year old girl. The clinical data showed a right side hemiplegia then a total paralysis of the left sixth cranial nerve and a paraplegia which became lethal in 3 months. The necrospy revealed a tumoral nodule in the left medulla oblongata and pons with diffuse subarachnoidal extension from the cranial nerves to the cauda equine roots. Histologically the tumor appeared to be polymorph with numerous rhabdomyoblasts which had a clear cross striation and which were sometimes less differentiated without any neuronal or glial elements. Perivascular tumoral cells and blood vessels were closely linked, the Virchow-Robin spaces were clearly involved. The electron microscopic study confirmed that the less differentiated cells were of a rhabdomyoblastic nature. A review of the litterature indicates that these malignant neoplasias are highly exceptional, and can be classified within the group of primary tumors of the neuraxis with muscular elements. The histogenetic origin of these tumors appears to be the ectomesenchyme of neural crests.
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8/45. Comparison of anterior and posterior surgical approaches in the treatment of ventral spinal hemangioblastomas in patients with von hippel-lindau disease.

    OBJECT: Von Hippel-Lindau (VHL) disease is an autosomal-dominant neoplastic syndrome with manifestations in multiple organs, which is evoked by the deletion or mutation of a tumor suppressor gene on chromosome 3p25. Spinal hemangioblastomas (40% of VHL disease-associated lesions of the central nervous system) arise predominantly in the posterior aspect of the spinal cord and are often associated with an intraspinal cyst. Rarely, the tumor develops in the anterior aspect of the spinal cord. Ventral spinal hemangioblastomas are a surgical challenge because of difficult access and because vessels feeding the tumor originate from the anterior spinal artery. The goal of this study was to clarify whether an anterior or posterior surgical approach is better for management of hemangioblastomas of the ventral spinal cord. METHOD:. The authors performed a retrospective analysis of clinical outcomes and findings on magnetic resonance (MR) imaging studies in eight patients (two women and six men with a mean age of 34 /- 15 years) who underwent resection of ventral spinal hemangioblastomas (nine tumors: five cervical and four thoracic). Two surgical approaches were used to resect these tumors. A posterior approach was selected to treat five patients (laminectomy and posterior myelotomy in four patients and the posterolateral approach in one patient); an anterior approach (corpectomy and arthrodesis) was selected to treat the remaining three patients. Immediately after surgery, the ability to ambulate remained unchanged in patients in whom an anterior approach had been performed, but deteriorated significantly in patients in whom a posterior approach had been used, because of motor weakness (four of five patients) and/or proprioceptive sensory loss (three of five patients). This difference in ambulation, despite significant improvements over time among patients in the posterior access group, remained significant 6 months after surgery. In all cases, MR images revealed complete resection of the tumor and in five patients significant or complete resolution of the intramedullary cyst was demonstrated (present in six of eight patients). CONCLUSIONS: The outcomes of these eight patients with hemangioblastomas of the ventral spinal cord indicate that both immediate and long-term results are better when an anterior approach is selected for resection.
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9/45. Capillary haemangiomas of the spinal cord: report of two cases.

    The clinicopathological features of two cases of capillary haemangioma of the spinal cord are described. The presenting symptoms were similar to those of common intradural, intramedullary tumours or tumour-like mass lesions. Radiological features of these two lesions resembled other vascular lesions of the spinal cord. The patients underwent surgery, and recoveries were good. Histologically, the lesions resembled capillary haemangioma of superficial tissues that are composed of lobules of small capillaries with feeding vessels. A fibrous capsule enveloped the lesions. Capillary haemangiomas of the central nervous system are rare. awareness of their existence may help the surgeon to avoid misdiagnosis and overtreatment of these benign tumour-like lesions.
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10/45. Isolated paramedullary hemangioblastoma originating from the first cervical nerve root: case report.

    STUDY DESIGN: A retrospective case of an isolated paramedullary hemangioblastoma originating from the first cervical root is reported. OBJECTIVE: To describe an uncommon type of spinal hemangioblastoma and its operative treatment. SUMMARY OF BACKGROUND DATA: Spinal hemangioblastoma, rare finding accounting for approximately 1.5% to 2.5% of all spinal cord tumors, may have an intramedullary, extramedullary, or extradural location. Cervical hemangioblastomas occur in approximately 45% of the cases and are intramedullary in about 83% of the cases. methods: A 59-year-old man presented with acute subarachnoid hemorrhage in the basal cisterns. Four-vessel angiography showed a highly vascular small tumor at the dorsolateral side of the cervicomedullary junction fed by a branch of the vertebral artery. The lesion was surgically removed. RESULTS: Total removal of the lesion was achieved after identification of both the arterial feeder and the draining vein with the aid of microvascular Doppler sonography. There were no complications, and the patient did well after surgery. CONCLUSIONS: Although hemangioblastomas occurring in the cervicomedullary area usually may cause progressive neural compression, occasionally they also can present clinically as acute subarachnoid hemorrhage. This situation requires urgent and adequate treatment as in the reported case.
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