Extra and intradural spinal Hemangioblastoma Amato, Marcelo Campos Moraes; Matias, Caio César Marconato Simões; Assirati Junior, João Alberto ...
Coluna,
09/2012, Letnik:
11, Številka:
3
Journal Article
Odprti dostop
Hemangioblastomas of the central nervous system (CNS) are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are ...uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.
Hemangioblastomas del sistema nervioso central (SNC) son tumores altamente vascularizados, de grado bajo, que pueden ser esporádicos o vinculados a la enfermedad de Von Hippel-Lindau. Hemangioblastomas extradurales no son comunes, y aquellos localizados extra e intraduralmente son aún más raros. Este estudio usa un caso ilustrativo y la revisión de la literatura para analizar las dificultades cuanto a considerar el diagnóstico correcto y para seleccionar el mejor abordaje quirúrgico. Un paciente, hombre blanco de 57 años de edad, presentaba mielopatía con radiculopatía C5 derecha. Las imágenes mostraban lesión extra/intradural, muy acrecentada, con forma de ampolla y lobulada, la cual ocupaba el conducto espinal y ensanchaba el agujero intervertebral derecho C4-C5. La resección de la lesión intradural fue conseguida mediante un abordaje posterior, pero la parte extradural solamente pudo ser removida parcialmente. La mejoría completa fue observada después de cuatro meses de seguimiento y el tumor residual ha sido acompañado clínica y radiológicamente. Aunque la impresión preoperatoria era de schwannoma espinal, el examen histopatológico reveló hemangioblastoma grado I según la Organización Mundial de la Salud. A pesar de su rareza, los actuales exámenes complementarios permiten considerar, preoperativamente, el diagnóstico de hemangioblastoma. Esto es esencial para hacer un mejor planeamiento quirúrgico, teniendo en cuenta los aspectos quirúrgicos peculiares de esta lesión.
Hemangioblastomas do sistema nervoso central são lesões de baixo grau de malignidade, altamente vascularizadas, que podem se apresentar esporadicamente ou associadas com a doença de Von Hippel-Lindau. Hemangioblastomas extradurais são incomuns e os extra e intradurais são ainda mais raros. Este estudo usa um caso ilustrativo e revisão da literatura para discutir as dificuldades de considerar o diagnóstico correto e selecionar a melhor abordagem cirúrgica. Um paciente do sexo masculino, branco, com 57 anos de idade apresentou-se com mielopatia e radiculopatia de C5 à direita. As imagens mostraram lesão extra-intradural lobulada, em forma de ampulheta, com alta impregnação após contraste, que ocupava o canal vertebral e estreitava o forame intervertebral de C4-C5 à direita. A ressecção total da lesão intradural foi alcançada através de abordagem posterior, mas a porção extradural só pôde ser parcialmente removida. Melhora total dos sintomas foi observada após quatro meses e o tumor residual tem sido seguido clínica e radiologicamente. Embora a impressão pré-operatória tenha sido de um schwannoma espinal, o exame histopatológico revelou hemangioblastoma grau I, segundo a OMS. Apesar de sua raridade, exames complementares atuais permitem o correto diagnóstico pré-operatório. Isto é essencial para melhor programação cirúrgica, tendo em vista as características particulares desta lesão.
In the central nervous system, zinc is released along with glutamate during neurotransmission and, in excess, can promote neuronal death. Experimental studies have shown that metallothioneins I/II ...(MT-I/II), which chelate free zinc, can affect seizures and reduce neuronal death after status epilepticus. Our aim was to evaluate the expression of MT-I/II in the hippocampus of patients with temporal lobe epilepsy (TLE). Hippocampi from patients with pharmacoresistant mesial temporal lobe epilepsy (MTLE) were evaluated for expression of MT-I/II and for neuronal, astroglial, and microglial populations. Compared to control cases, MTLE group displayed widespread increase in MT-I/II expression, astrogliosis and reduced neuronal population. MT-I/II levels did not correlate with any clinical variables, but patients with secondary generalized seizures (SGS) had less MT-I/II than patients without SGS. In conclusion, MT-I/II expression was increased in hippocampi from MTLE patients and our data suggest that it may be associated with different seizure spread patterns.
The authors report their experience in the treatment of 12 symptomatic patients with intradural spinal neurocysticercosis.
The mean age of the 12 patients was 33 years. There were eight female and ...four male patients. Cysticercosis was present in association with hydrocephalus in nine cases. In nine of 12 patients the spinal lesion was confined to the thoracic or lumbar spinal cord, and in three the cysticerci occupied the cervical region. In all patients with hydrocephalus nerve root symptoms developed seven to 48 months later (mean 27.6 +/- 15.5 months). In one patient hydrocephalus was absent, but he presented with cysticercal meningitis 24 months before spinal cord compression developed. Presenting symptoms suggesting spinal cysticercosis included muscular weakness (67%), pain (67%), and sphincter disturbance (25%). Neurological examination demonstrated a motor deficit in nine patients, sensory deficit in four, and radicular pain in three. The prognosis was worse in patients with moderate-to-severe arachnoiditis and spinal cord compression compared with those with isolated nerve roots involvement in whom outcome was favorable. Ten patients underwent laminectomy, after which neurological status improved in 44%, remained unchanged in 33%, or worsened in 22.2%.
The authors discuss their findings in these cases. Additionally they briefly review the literature, pathophysiology, and therapeutic and/or surgical strategies involved in this disease.
Neurocysticercosis (NCC) is the most common parasitic disease of the human central nervous system and a major health problem for most developing countries. The most common clinical manifestations of ...NCC are epileptic seizures. Whenever epilepsy and NCC coexist in the same patient, an uncertainty may rise about a causal relationship between them.
We described a female patient with disseminated calcified NCC lesions and intractable epilepsy. Her medical history included cysticercotic meningoencephalitis and status epilepticus caused by active NCC. Fundoscopy showed the ocular presence of parasite; computed tomography of the brain showed evidence of cystic lesions with the scolex and calcified lesions; enzyme-linked immunosorbent assay of the cerebrospinal fluid was positive for cysticercosis. Epileptic seizures started after an 8-year silent period. Magnetic resonance imaging showed left hippocampal sclerosis. Plain x-ray film showed calcifications in muscles and subcutaneous tissue. Video-electroencephalography and ictal and interictal single-photon emission computed tomography disclosed left mesial temporal lobe epilepsy. The patient underwent left temporal lobectomy and has been seizure free since surgery, for a follow-up of 4 years.
This case report highlights and supports surgical therapy in patients with epileptic seizures and calcified NCC, even when there are several calcifications, provided that clear localization of epilepsy has been determined by means of a presurgical workup.
The clinical course of 69 patients with neurocysticercosis who underwent surgery to control increased intracranial pressure (ICP) or cyst removal is analyzed. Increased ICP was caused by ...hydrocephalus in 63 patients, by cerebral edema in four, and by giant cysts in two. Skull x-ray films showed calcifications in 14% and signs of elevated ICP in 46%. Examination of cerebrospinal fluid (CSF) revealed pleocytosis with eosinophils in 52% of cases and a positive complement fixation test for cysticercosis in 66%. Ventriculography allowed localization of the CSF obstruction and ventricular cysts, and generally differentiated between an obstruction due to cysts and an inflammatory process. Computerized tomography showed cysts in the cerebral parenchyma and ventricular dilatation. Ventricular cysts were best seen when intraventricular metrizamide was used. Intracranial shunting and posterior fossa exploration were less effective in the treatment of hydrocephalus than was ventriculoatrial (VA) or ventriculoperitoneal (VP) shunting, although VA or VP shunting was associated with a high percentage of complications. Quality of survival was good in 87% of the cases in the first 3 postoperative months and in 93% of patients who survived 2 years after surgery. Forty-seven patients (68%) were readmitted one or more times for CSF shunt revision; 14 of them for shunt infection (meningitis). The early operative mortality rate was 1.8% for patients with VA or VP shunt placement and 5.3% for those with posterior fossa exploration. The authors conclude that placement of CSF shunts is indicated in the treatment of hydrocephalus, and cyst removal is indicated only when the cyst exhibits tumor-like behavior. Surgical exploration is also indicated when the diagnosis is uncertain.
Hemangioblastomas of the central nervous system (CNS) are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are ...uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.Hemangioblastomas del sistema nervioso central (SNC) son tumores altamente vascularizados, de grado bajo, que pueden ser esporádicos o vinculados a la enfermedad de Von Hippel-Lindau. Hemangioblastomas extradurales no son comunes, y aquellos localizados extra e intraduralmente son aún más raros. Este estudio usa un caso ilustrativo y la revisión de la literatura para analizar las dificultades cuanto a considerar el diagnóstico correcto y para seleccionar el mejor abordaje quirúrgico. Un paciente, hombre blanco de 57 años de edad, presentaba mielopatía con radiculopatía C5 derecha. Las imágenes mostraban lesión extra/intradural, muy acrecentada, con forma de ampolla y lobulada, la cual ocupaba el conducto espinal y ensanchaba el agujero intervertebral derecho C4-C5. La resección de la lesión intradural fue conseguida mediante un abordaje posterior, pero la parte extradural solamente pudo ser removida parcialmente. La mejoría completa fue observada después de cuatro meses de seguimiento y el tumor residual ha sido acompañado clínica y radiológicamente. Aunque la impresión preoperatoria era de schwannoma espinal, el examen histopatológico reveló hemangioblastoma grado I según la Organización Mundial de la Salud. A pesar de su rareza, los actuales exámenes complementarios permiten considerar, preoperativamente, el diagnóstico de hemangioblastoma. Esto es esencial para hacer un mejor planeamiento quirúrgico, teniendo en cuenta los aspectos quirúrgicos peculiares de esta lesión.Hemangioblastomas do sistema nervoso central são lesões de baixo grau de malignidade, altamente vascularizadas, que podem se apresentar esporadicamente ou associadas com a doença de Von Hippel-Lindau. Hemangioblastomas extradurais são incomuns e os extra e intradurais são ainda mais raros. Este estudo usa um caso ilustrativo e revisão da literatura para discutir as dificuldades de considerar o diagnóstico correto e selecionar a melhor abordagem cirúrgica. Um paciente do sexo masculino, branco, com 57 anos de idade apresentou-se com mielopatia e radiculopatia de C5 à direita. As imagens mostraram lesão extra-intradural lobulada, em forma de ampulheta, com alta impregnação após contraste, que ocupava o canal vertebral e estreitava o forame intervertebral de C4-C5 à direita. A ressecção total da lesão intradural foi alcançada através de abordagem posterior, mas a porção extradural só pôde ser parcialmente removida. Melhora total dos sintomas foi observada após quatro meses e o tumor residual tem sido seguido clínica e radiologicamente. Embora a impressão pré-operatória tenha sido de um schwannoma espinal, o exame histopatológico revelou hemangioblastoma grau I, segundo a OMS. Apesar de sua raridade, exames complementares atuais permitem o correto diagnóstico pré-operatório. Isto é essencial para melhor programação cirúrgica, tendo em vista as características particulares desta lesão.
Abstract Background Inflammatory diseases stand out among sensory neuronopathies because, in their active phase, they can be treated with immunosuppressive agents. Immunosuppressive therapy may ...present severe adverse effects and requires previous inflammatory activity confirmation. Sensory neuronopathies are diagnosed based on clinical and EMG findings. Diagnostic confirmation and identification of inflammatory activity are based on sensory ganglion histopathological examination. We describe the surgical technique used for dorsal root ganglionectomy in patients with clinical/EMG diagnosis of sensory neuronopathies. Methods The sensory ganglion was obtained from 15 patients through a small T7-T8 hemilaminectomy and foraminotomy to expose the C7 root from its origin to the spinal nerve bifurcation. In 6 patients, the dural cuff supposed to contain the ganglion was resected en bloc; and in 9 patients, the ganglion was obtained through a longitudinal incision of the dural cuff and microsurgical dissection from the ventral and dorsal roots and radicular arteries. All ganglia were histopathologically examined. Results No ganglion was found in the dural cuff in 2 patients submitted to en bloc removal, and the ganglion was removed in all patients who underwent microsurgical dissection. All but 2 patients that had ganglion examination presented a neuronopathy of nerve cell loss, 3 with mononuclear inflammatory infiltrate. These patients underwent immunosuppressive therapy, and 2 of them presented clinical improvement. No surgical complications were observed. Conclusions Microsurgical dorsal root ganglionectomy for diagnosing inflammatory sensory ganglionopathies was effective and safe. Although safe, en bloc resection of the proximal dural cuff was not effective for this purpose.
Parasagittal meningiomas: follow-up review Colli, Benedicto Oscar; Carlotti, Carlos Gilberto; Assirati, João Alberto ...
Surgical neurology,
01/2006, Letnik:
66
Journal Article
Parasagittal meningioma is one that fills the parasagittal angle, with no brain tissue between the tumor and the SSS. Invasion of the SSS is a challenge for complete removal and, consequently, for ...recurrence of these tumors. The objective of this study was to analyze the factors that influenced the clinical outcome of patients with parasagittal tumors surgically treated.
Review of data on 53 patients with diagnosis of parasagittal meningiomas surgically treated from 1984 to 2004. Thirty-four (64.2%) were female and 19 (35.8%) were male; age ranged from 18 to 81 years old (mean, 54.98 ± 5.80). Follow-up ranged from 2 to 261 months (mean, 93.71 ± 68.45). The patients were operated on using microsurgical techniques. Tumors in the anterior third (9) or occluding the SSS (5) were removed with the sinus; tumors touching/pouching the SSS (20) were removed and its dural attachment coagulated; tumors invading one sinus wall (10) were removed with partial excision and reconstruction of the sinus wall, and tumors invading more than one sinus wall in the posterior two thirds of the SSS (7) had a subtotal removal. No attempt at sinus resection and reconstruction was performed for tumors placed in the posterior two thirds of the SSS. Analysis of the patient outcome was done using survival and RFS Kaplan-Meier curves. The
χ
2, Fisher exact, log-rank, Mann-Whitney, and Kruskall-Wallis ANOVA tests were used for comparing demographic data, survival curves, proportions, and medians, respectively.
Total and subtotal resection were achieved in 85% and 13.1%, respectively. Males had better survival than females (
P = .0252). Total RF rates were 10%, 25%, and 100% at 5 years and 100%, 50%, and 100% at 10 years for patients with meningiomas WHO grades I, II, and III, respectively. The RF survival curve was better for patients with grade I meningioma (grades I vs II vs III,
P = .0001). There was no difference between the RF survival curves according to age, histopathologic WHO grade, location along or invasion of the SSS, and extent of resection. Males (
P = .0401), WHO grade I (
P < .0001), total resection (
P = .0139), and less sinus invasion (
P = .0308) had better RFS curves. Operative, surgery-related, and overall mortality were 1.9%, 5.4%, and 26.4%, respectively.
Recurrence of parasagittal meningiomas predominated in males, in grades II/III tumors, after subtotal resection, and with more invasion of the SSS. Subtotal or total resections without sinus resection were considered adequate for treating these patients.
Two cases of intramedullary paracoccidioidomycosis are reported. Paracoccidioidomycosis is a systemic disease that involves the buccopharyngeal mucosa, lungs lymph nodes and viscera and infrequently ...the central nervous system. Localization in the spinal cord is rare. Case 1: a 55-year old male admitted with crural pararesis, tactile/painful hypesthesia and sphincter disturbances of 15 days duration. Cutaneous-pulmonary blastomycosis was diagnosed 17 years ago. Myelotomography showed a blockade of T3-T4 (intramedullary lesion). The lesion surgically removed was a Paracoccidioides brasiliensis granuloma. Treatment with sulfadiazine was started after the surgery. Follow-up of 15 month showed an improvement of the clinical signs. Case 2: a 57-year old male was admitted elsewhere 6 months ago and, with a radiologic diagnosis of pulmonary paracoccidioidomycosis, was treated with amphotericin B. He progressively developer paresthesia and tactile/pain anaesthesia on the left side, sphincter disturbances and tetraparesis with bilateral extensor plantar response and clonus of the feet. Myelotomography showed a blockade of C4-C6 (intramedullary lesion). The lesion was not found during surgical exploration and the patient deteriorated and died. Post-mortem examination revealed an intramedullary tumor above the site of the mielotomy (Paracoccidioides brasiliensis granuloma). The preoperative diagnosis of intramedullary paracoccidioidomycotic granulomas is difficult because the clinical and radiologic manifestations are uncharacteristic. Clinical suspicion was possible in our cases based on the history of previous systemic disease. Contrary to intracranial localizations, paracoccidioidomycotic granulomas causing progressive spinal cord compression may require early surgery because response to clinical treatment is slow and the reversibility of neurological deficits depends on the promptness of the decompression.
The surgical treatment of intracranial aneurysms by clipping is recognized as effective and definitive. However some cases that suffered a new subarachnoid hemorrhage (SAH) some time after they were ...submitted to aneurysm clipping have raised doubts about the concept of "cure" after this treatment. Eleven patients previously submitted to aneurysm clipping who presented a new SAH were analyzed. The time elapsed from surgery to SAH varied from 3 to 10 years. After SAH four patients had a poor outcome. The new episode of SAH occurred due to intrinsic factors of the cerebral vasculature: 1. a weak point of the vessel wall near the previous aneurysm, 2. a weak point of another vessel far from the previous aneurysm, 3. a previous infundibular dilation of the posterior communicating artery; and due to technical problems: 1. aneurysm not identified during the previous treatment, 2. aneurysm deliberately left untreated, 3. persistence of the aneurysm due to inappropriate surgery, 4. persistency of part of the aneurysm neck after clipping and 5. slipping of the clip from the neck of the aneurysm. The measures to prevent new SAH after surgery start with adequate preoperative angiographic studies, a careful inspection of the position of the clip and emptying of the aneurysm. Early angiography studies may reveal a persistent neck and later ones may reveal newly developed aneurysms. In conclusion, SAH after aneurysm clipping is a late and severe phenomenon and the concept of "cure" after this surgery should be interpreted with caution.
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