Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Metastatic hemangiopericytoma to the spine is rare, and operative management of these highly vascularized tumors is challenging. Review of the literature identified only seven previously reported cases. Metastases of hemangiopericytomas to the spine are diagnosed often only after a long delay following resection of the primary tumors. To emphasize the clinical and histopathological features of metastatic hemangiopericytoma to the spine and to review treatment options, a case of a delayed metastatic hemangiopericytoma to the cervicothoracic spine is reported: a 48-year-old woman presented with a large metastatic hemangiopericytoma to the cervicothoracic spine causing left-sided cervicobrachialgias and gait disturbance. Magnetic resonance imaging studies revealed a large left-sided dumbbell-shaped intraspinal and extraspinal tumor from C6 to T2. The patient underwent two-staged total removal of the cervicothoracic mass with posterior stabilization and subsequent radiotherapy. Histopathological findings revealed a malignant hemangiopericytoma which had identical features to the primary cranial meningeal tumor removed 8 years earlier. The radicular symptoms and the gait ataxia disappeared, postoperatively. One year later, however, new metastases were present. Patients with hemangiopericytoma should be controlled regularly for local recurrence and systemic tumor spread. The best available treatment for delayed metastasis to the spinal column is complete tumor removal followed by postoperative radiotherapy.
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PMID:Delayed manifestation of spinal metastasis: a special feature of hemangiopericytoma. 1286 May 7

Hemangiopericytoma is a rare tumor of the central nervous system and has seldom been reported intraventricularly. A 55-year-old woman presented with gradual onset of left side weakness, gait ataxia, and tendency to miss objects in the left visual field of uncertain duration. Magnetic resonance imaging with contrast showed a tumor with homogenous enhancement in the right lateral ventricle. The patient underwent right temporoparietal stealth-assisted craniotomy and surgical removal of the tumor. Histopathologic examination with ancillary tests confirmed hemangiopericytoma. Awareness that hemangiopericytoma can occur as an intraventricular tumor is important for clinicians and pathologists. Because of radiologic similarity, this tumor is not to be confused with intraventricular meningioma because the prognosis is different.
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PMID:Intraventricular hemangiopericytoma. 1561 39

Cervical hemangiopericytoma (HPC) is a rare tumor involving dura and bone. Because HPC shares some imaging features with meningioma, hemangioblastoma, schwannoma and solitary fibrous tumors; histology with appropriate immunohistochemistry is essential for its diagnosis and treatment. HPC is highly vascular and has a high rate of local recurrence following resection. Thus it can pose significant therapeutic challenges. To the best of our knowledge this is the 13th reported case of HPC of the cervical spine and the first case of cervical HPC treated using embolization with Onyx-18. In addition, a systematic review of the literature is presented describing previous experience with HPC of the cervical region. The case of a 61-year-old woman is reported who presented with a 7 month history of dizziness, mild ataxia and left-sided tinnitus when supine. MRI of the cervical spine demonstrated a gadolinium enhancing mass arising from the dorsal cervical dura, extending from the occiput to C2, with internal flow voids and extension into the C2 lamina and spinous process. Digital subtraction angiography demonstrated multiple corkscrew-shaped supplying vessels, marked hypervascularity, rapid arteriovenous shunting and delayed contrast washout. Super selective injection of Onyx-18 yielded approximately 90% embolization of the lesion, followed by subtotal resection and radiotherapy. Histology confirmed the presumptive diagnosis of HPC. Preoperative embolization with Onyx-18 should be considered for large HPC as it may decrease the risk of intraoperative hemorrhage. In this case, embolization with Onyx-18 did not preclude histopathologic diagnosis of HPC.
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PMID:Endovascular embolization of cervical hemangiopericytoma with Onyx-18: case report and review of the literature. 2199 Aug 49

A 22-yr-old female college athlete had noted ataxia during competitive gymnastic events. Magnetic resonance imaging revealed a large tumor compatible with preoperative diagnosis of meningioma arising from the petrotentorial region with marked brainstem compression. The technical challenges associated with this removal include safe dissection of cranial nerves IV to VIII and dissection from the brainstem and cerebellum, taking care to not interfere with blood supply to these structures. The vascular tumor was removed via standard suboccipital approach, with the trajectory above the seventh/eighth nerve complex. The attachment was at the petrotentorial junction, and the fourth nerve was intimately involved with the tumor as the tumor was emanating from the tentorial edge where the fourth nerve entered. In most instances, the nerves are displaced by the tumor but in this case injury to the fourth nerve ensued with dissection at the tumor attachment. After tumor resection, the interrupted fourth nerve was repaired microsurgically. A hemangiopericytoma was identified on pathological analysis, and the patient received postoperative radiation therapy, which has been given postoperatively or at recurrence in the literature. Technical nuances of removal are discussed. Patient consent was granted for publication of this video.
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PMID:Resection of Large Petrotentorial Tumor via a Retrosigmoid Approach: 3-Dimensional Operative Video. 2980 Mar 33