Differentiation of adult pilocytic astrocytomas and hemangioblastomas using texture analysis / 实用放射学杂志

Objective To investigate the diagnostic value of the texture analysis in differentiating adult pilocytic astrocytomas (PA)from hemangioblastomas(HB).Methods 22 adult patients with PA and 20 patients with HB which were confirmed by postoperative pathological were retrospectively reviewed.The conventional MRI features and texture parameters were analyzed.Eight texture parameters were extracted using run-length matrix(RLM),and the differences of texture parameters of the two groups were analyzed by independent-samples t test.Results The short run emphasis(SRE),grey-level non-uniformity(GLNU),run-length non-uniformity(RLNU),high grey-level run emphasis(HGRE)and short run high grey-level emphasis(SRHGE)were higher in adult PA than in HB.The long run emphasis(LRE),low grey-level run emphasis(LGRE)and short run low grey-level emphasis(SRLGE)were lower in adult PA than in HB.The eight texture parameters had significant differences between the two groups(P<0.05).Conclusion Texture analysis can provide reliably objective basis for differentiating adult PA from HB.

Clinical analysis of intracranial hemangioblastomas treated by microsurgery combined with preoperative in-terventional embolization and its related problems / 中华显微外科杂志

Objective To investigate the clinical effect of microsurgical resection combined with preoperative interventional embolization in the treatment of solid intracranial hemangioblastomas, and to analyze the effect of pre-operative embolization on tumor resection,and to discuss the surgical technique and perioperative management of mi-crosurgery. Methods From September, 2010 to September, 2015, the clinical signs, preoperative embolization, mi-crosurgery and prognosis of 18 patients with solid hemangioblastomas were retrospectively analyzed. The patients were examined by CTA and MRI,18 patients underwent DSA tumor arterial embolization and tumor microsurgery. Results The tumor diameter was 2.5-4.0 cm, the proportion of preoperative embolization tumor was 100% in 4 cases, 80% -95% in 9 cases,60%-70% in 3 cases and 60% in 2 cases.16 cases(88.9%)were subtotal tumors,2 cases(11.1%) were subtotal resection, 1 case died. No patients with intraoperative blood transfusion.followed up for 2 years without tumor recurrence. Conclusion The risk of postoperative hemangioblastomas is high, and the intervention of em-bolization for tumor artery will reduce the risk of operation.Microsurgery is the preferred treatment,and skilled micro-surgery is the key to treatment.

Carcinomas renales múltiples como manifestación inicial del Síndrome de Von Hippel Lindau. Presentación de caso / Multiple renal carcinoma as a clinical manifestation of Von Hippel Lindau Syndrome. Case presentation

Introducción: El Síndrome de Von Hippel Lindau es una afección neoplásica multisistémica, heredada de manera autosómica dominante y con alta penetrancia. Su expresividad clínica es muy diversa,oscilando la incidencia entre 1/35000 y 1/36000 nacidos vivos. Esta enfermedad usualmente se diagnostica entre los 20 y 30 años, pero los síntomas pueden aparecer en la infancia. La lesión clínica inicial más común y precoz es el hemangioblastoma de la retina y/o del sistema nervioso central. Objetivo: Presentar un caso de un paciente con carcinomas renales múltiples como manifestación inicial de un Síndrome de Von Hippel Lindau. Presentación del Caso: Paciente masculino de 59 años, con antecedentes de salud, quien acude a urgencias por cuadro febril de 3 días de evolución, que fue interpretado como Dengue; se le realizó, dentro de los complementarios, ultrasonido abdominal, donde se descubrió masa sólida a nivel del polo superior del riñón derecho, asociado a existencia de otra en polo inferior de este mismo riñón, así como de 2 más en el contralateral. Además, se encontraron varios quistes pancreáticos y 2 renales izquierdos corticales. El paciente fue intervenido quirúrgicamente; se corroboró el diagnóstico de carcinomas renales de células claras. El examen oftalmológico reveló la presencia de un hemangioblastoma retiniano derecho; en tanto la tomografía computarizada simple de cráneo y la resonancia magnética espinal no mostraron alteraciones. Conclusiones: Este síndrome es una rara, pero grave afección genética, caracterizada por un alto riesgo de desarrollar enfermedades neoplásicas, lo que hace que sea aún más importante conocerlo, para poder identificar y tratar a tiempo sus temidas complicaciones(AU)

Introduction: Von Hippel Lindau Syndrome is a multisystem neoplastic affection, which is inherited as an autosomal dominant trait, with high penetrance. Its clinical expressivity is very diverse, ranging its incidence between 1/35000 and 1/36000 born alive. This disease is usually diagnosed between the 20 and 30 years of age, but its symptoms can appear in childhood. The most common and early initial clinical lesion is the hemangioblastoma of the retina and/or central nervous system. Objective:To present a case of a patient with multiple renal carcinoma as initial manifestation of Von Hippel Lindau Syndrome. Case presentation: 59 years old male patient with a history of good health who comes to the Emergency Room because of febrile clinical state of 3 days´ evolution, that was interpreted as dengue. Abdominal ultrasound was included in the complementary studies, in which a solid mass at level of upper pole of right kidney was observed, associated with the existence of another one in lower pole of the same kidney, as well as two others in contralateral. Also, multiple pancreatic cysts and two left cortical renal ones were found. The patient underwent surgery, and the diagnosis of clear cells renal carcinoma was corroborated. The ophthalmological exam revealed the presence of a right retinal hemangioblastoma whereas the plain skull CT-scan, and the magnetic resonance imaging of the lumbar spine did not show any alterations. Conclusions:This syndrome is a rare, but a serious genetic affection, characterized by a high risk to develop neoplastic diseases; that´s one reason why it is very important to know about it in order to identify, and treat its feared complications in time(AU)

Abordagem cirúrgica dos hemangioblastomas intramedulares / Manejo quirúrgico de los hemangioblastomas intramedulares / Surgical management of intramedullary hemangioblastomas

OBJETIVO: descrever uma experiência e as técnicas para abordagem destas lesões com ênfase nos aspectos microcirúrgicos. MÉTODOS: foram relatados nove casos consecutivos de pacientes submetidos à ressecção de lesões intramedulares operados entre 2000 e 2008. A escala funcional de McCormick foi usada para avaliar o status funcional dos pacientes RESULTADOS: a idade dos pacientes variou de 13 a 45 anos (média de 27,1 anos). Quatro (44,4 por cento) pacientes eram homens e cinco (55,5 por cento), mulheres. Dois pacientes tinham a doença de von Hippel-Lindau, com outros hemangioblastomas associados. Conforme a localização, sete (77,7 por cento) pacientes tinham lesões na região cervical (dois deles com tumores na transição crânio-cervical) e dois (22,2 por cento) tinham lesões na região torácica. Todos os pacientes tiveram ressecção total das lesões, sendo que três apresentaram leve piora clínica no pós-operatório imediato. Após seis meses do procedimento cirúrgico, dois pacientes apresentaram melhora clínica, enquanto sete mantinham-se funcionalmente iguais ao período pré-operatório. CONCLUSÕES: o conhecimento anatômico e de técnicas microcirúrgicas adequadas permite a ressecação total destas lesões sem agregar morbidade adicional.

OBJECTIVES: to report an experience and to present a surgical technique to achieve total resection and cure. METHODS: nine consecutive cases of intramedullary haemangioblastomas, operated between 2000 and 2008 are presented. The functional scale proposed by McCormick was used to evaluate the patients' neurological status. RESULTS: age at presentation varied from 13 to 45 (average 27.1) years. Four (44.4 percent) patients were male and five (55.5 percent), female. Two patients had an associated von Hippel-Lindau disease, with others haemangioblastomas. According to the site of presentation, seven (77.7 percent) were localized at the cervical region (including two at the cervico-medullary junction), and two (22.2 percent) at the thoracic level. Total resection was achieved in all cases. Three patients had some functional worsening immediately after surgical procedure. After six months, there were no patients with functional worsening comparing with the pre-operative status and two patients had clinical improvement. CONCLUSIONS: adequate knowledge of anatomy and the correct use of microsurgical techniques allowed total resection of these tumors with minimal morbidity and maximum functional recovery.

OBJETIVOS: describir una experiencia y las técnicas para abordaje de esas lesiones, enfatizando los aspectos microquirúrgicos. MÉTODOS: fueron relatados nueve casos consecutivos de pacientes sometidos a resección de lesiones intramedulares, operados entre 2000 y 2008. La escala funcional de McCormick fue usada para la evaluación del status funcional de los pacientes RESULTADOS: la edad de los pacientes varió de 13 a 45 anos (media de 27,1). Cuatro (44,4 por ciento) pacientes eran hombres y cinco (55,5 por ciento), mujeres. Dos pacientes eran portadores del enfermedad de von Hippel-Lindau, con otros hemangioblastomas asociados. Conforme la localización, siete (77,7 por ciento) pacientes tenían lesiones en la región cervical (dos con tumores en la transición cráneo-cervical) y dos (22,2 por ciento) tenían lesiones en la región torácica. Todos los pacientes tuvieron resección total de los tumores, siendo que tres tuvieron deterioración clínica leve en el post-operatorio inmediato. Después de seis meses del procedimiento quirúrgico, dos pacientes presentaron mejoras clínicas, mientras seis se mantuvieron funcionalmente iguales al periodo pre-operatorio. CONCLUSIONES: el conocimiento anatómico y de técnicas microquirúrgicas adecuadas permite la resección total de estos tumores sin agregar morbididad adicional.

MRI Diagnosis and Differential Diagnosis of Hemangioblastoma of Central Nervous System / 中国医学影像学杂志

Purpose:To discuss the MRI features and differential diagnosis of hemangioblastomas in the central nervous system.Materials and Methods: The MRI features of 22 patients with hemangioblastomas confirmed histopathologically were analyzed retrospectively.Results: In this group there were 50 lesions in 22 patients.Multiple lesions were revealed in 5 cases.The lesions located in the cerebellar hemisphere and vermis ( n = 40),medulla oblongata and spinal cord ( n.= 9 ),cerebral hemisphere ( n = 1).Among the 50 lesions,12 appeared as a large cyst with mural nodule,36 as a solid mass,2 as a simple cyst.Of large cyst with mural nodule lesions,the content of the cyst was hy-pointense signal on T1WI,and hyperintense signal on T2WI.The mural nodules were slightly hypointense signal or isointense signal on Tl WI,and hyperintense signal on T2WI.The solid masses were isointense signal on Tl WI,slightly hyperintense signal and hyperintense signal on T2WI.On contrast enhanced scans,all mural nodules and solid tumors were showed marked homogeneous enhancement.On PWI the mural nodules and solid tumors were demonstrated marked hyperperfusion.Conclusion:Hemangioblastomas have distinctive manifestation,MRI enhanced scans and PWI play an important role in the diagnosis and differential diagnosis of hemangioblastomas.

Treatment Strategy of Multiple Hemangioblastomas

OBJECTIVE: Hemangioblastomas are highly vascular and benign neoplasm of the central nervous system(CNS). They can often be found as multiple lesions, as is commonly observed in von Hippel-Lindau(VHL) disease. The aim of this study is to determine the proper management for multiple hemangioblastomas. METHODS: Since 1990, 78cases of hemangioblastoma have been encountered. Among these, 9cases were multiple hemangioblastomas that were treated with surgical resection with or without radiosurgery. The medical, radiological, surgical and histological records were reviewed retrospectively and analyzed statistically. RESULTS: Nine patients presented with multiple hemangioblastomas and were diagnosed as VHL disease. The mean follow-up duration was 75.7months (6.6~159.2months) after the first surgical treatment. Three patients were treated with surgical resection alone and six patients were treated by both surgical resection and radiosurgery. Twenty-one surgical procedures (13 surgical resections and 8 radiosurgery) were performed. One patient required ventriculoperitoneal shunt and a posterior fossa decompressive craniectomy because of post-radiation brain swelling. Another patient refused additional treatment for the newly developed lesions after the successful treatment of initial lesions. The other patient who presented with numerous lesions in the whole brain and spine underwent cranio-spinal irradiation. Remaining patients showed good results. CONCLUSION: The surgical outcomes for the patients with a single lesion of the CNS hemangioblastoma are favorable. However, the treatment of multiple hemangioblastoma is more difficult, and should be treated by surgical resection and radiosurgery with careful consideration.

MR Imaging Study of Spinal Hemangioblastoma / 实用放射学杂志

Objective To study the MRI findings of spinal hemangioblastomas.Methods The MRI findings of spinal hemangioblastomas proved pathologically in 29 cases (20 males,9 females) were retrospectively reviewed.Results The lesions were single in 12 cases (41.4%) and multiple in 17 cases(58.6%).Seven patients combined with intracranial hemangioblastomas.On unenhanced MRI,36 tumors were isointense on T 1WI and T 2WI,16 were mixed iso-hypointense on T 1WI and mixed iso-hyperintense on T 2WI,one was hypointense on T 1WI and hyperintense on T 2WI.Small hemangioblastomas tended to be isointense on T 1WI and T 2WI.After contrast injection,all tumors enhanced markedly and homogenously with clear borders.21 tumors in 17 patients had associated vascular flow voids on T 2WI.40 tumors in 27 patients were associated with syringes or cysts.Conclusion Hemangioblastomas of the spinal cord are often multiple and in company with intracranial hemangioblastomas.The marked contrast enhancements with clear borders and associated vascular flow voids are the most important signs on MRI.

Two Cases of Multiple Hemangioblastomas in a Von Hippel-Lindau Family

Hemangioblastoma is uncommon, accounting for 1 to 2.5% of all primary neoplasms of the central nervous system. Ten to 20% of hemangioblastomas occur as part of Von Hippel-Lindau disease(VHL). Multiple hemangioblastomas are seen only with VHL and these are seen in up to half of VHL-associated hemangioblastomas. We have treated two cases of multiple hemangioblastomas associated with VHL disease in a family, sister(case I, 48/female) and her brother(case II, 41/male). Both patients had renal and pancreatic cysts in addition to CNS hemangioblastomas. Their hemangioblastomas were removed totally, and then their neurological symptoms had Improved. With a review of the literature, the authors present a family of VHL-associated multiple hemangioblastomas.

Cerebellar Hemangioblastoma: Clinical Presentations and Surgical Outcome

In order to analyze clinical presentations and surgical outcome, we studied eleven cases of cerebellar hemangioblastomas from among a series of 421 intracranial tumors treated over a period of seven years. The proportion of male patients was 2.7 times that of females and the age peak appeared between 30 and 50 years(mean age: 37.7 years old). The common clinical presentations were headache(73%), dizziness(55%), unilateral cerebellar dysfunction(45%), and nausea or vomiting(36%). In our series there were two cases of cranial nerve palsy associated with von Hippel-Lindau(VHL) disease. Polycythemia that normalized postoperatively appeared in two of eleven cases. Radiologic studies showed nine cystic tumors, six in the cerebellar hemisphere and three in the cerebellar vermis, and three solid tumors extended into the brain stem. After aspiration of the cyst, careful removal of the mural nodule gave good results in more than 70% of patients. Because the tumor extended into the brain stem, however, three solid lesions were resected subtotally. Postoperative complications developed in four patients, and in three of these, the lesion extended into the brain stem. In conclusion, major clinical features were due to increased intracranial pressure, and varying expression of cerebellar and cranial nerve deficits was characteristic. To determine the resectability of lesions and to minimize surgical morbidity, extremely careful clinical and neuroradiological observation is essential.

2 Cases of Multifocal and Recurrent Hemangioblastomas with Von Hippel-Lindau Syndrome: Case Report

Hemangioblastomas are benign tumors that most commonly occur in the posterior fossa around the 4th ventricle. Recurrent mutifocal hemangioblastomas are often found as a part of a systemic autosomal dominant disease, the Von Hippel-Lindau syndrome. Surgical removal of recurrent multifocal tumors are technically more difficult and challenging than solitary ones due to their proximity to critical structures, disturbed anatomical landmark and adehesion to the surrounding tissue, all of which may lead to high postoperative morbidity and mortality. Authors have experienced 2 cases of the multiple and recurrent hemangioblastomas with Von Hippel-Lindau syndrome. The patients of each of the cases underwent surgery for tumor removal at least 2 times. In the first patient, a 42-year-old male, a solitary cerebellar hemangioblastoma had been removed 6 years prior to recurrence. On followup MRI, more than three solid, homogenously enhanced nodules were found in the cerebellum. These tumors were operated on through the previous craniectomy site, but only two of them could be removed. This patient was discharged without additional neurologic deficit. The 2nd patient, a 40-year-old-male, had undergone three prior operations for cerebellar hemangioblastomas. On followup examination, he presented with progressive ataxia and confusion. Brain MRI showed multiple recurrent lesions of homogenously strongenhanced masses in the 4th ventricle and cerebellar vermis surrounded by cystic lesions, accompanied by obstructive hydrocephalus. No additional surgery of tumors were attempted, and only ventriculoperitoneal shunt was performed. He was discharged with improved neurological symtomes and sign.

Multiple Hemangioblastomas in Cerebellum, Medulla Oblongata and Spinal Cord

Hemangioblastomas comprise 1 to 2% of all intracranial neoplasm, and 8 to 12% of tumors within the posterior fossa. They are composed of admixtures of three different cell types; endothelial cells, pericytes and stromal cells. Although most hemangioblastomas arise sporadically, they are associated with von Hippel-Lindau disease in about 20% cases. We have experienced a case of multiple hemangioblastomas occuiing in the cerebellum, medulla oblongata and cervical spinal cord simultaneously in a 55-year-old male. He had complained of headache, dizziness, generalized weakness and gait disturbance for 2 weeks. The patient had neither specific family history nor increased hematocrit. MRI showed a nonenhancing cystic lesion with an enhancing mural nodule in the right cerebellar hemisphere and two separate enhancing nodules in the medulla oblongata and dorsal cervical spinal cord at the 5-6th. Grossly, the excised mass of the cerebellum, 2.5 x 2 x 1.8cm, was solid to partly cystic, and that of spinal cord, lcm in diameter, was mostly solid. Microscopically, the tumor was composed of thin-walled blood vessels in variable size and interspersed stromal cells. The stromal cells revealed dimorphic cytoplasm that were either homogeneous and eosinophilic, or clear and vacuolated. Immunohistochemically, the endothelial cells reacted positively for glial fibrfllaty acidic protein(GFAP) and vimentin. The stromal cells reacted diffusely positively for vimentin, focally positively for GFAP and S-100 protein near the periphery of the tumor, focally positivel for neuro specipic enolase(NSE), and negatively for lysozyme, desmin and chromogranin. Ultrastructurally, the stromal cells contained numerous microfilaments and lipid droplets.

Multiple Hemangioblastomas in Cerebellum, Medulla Oblongata and Spinal Cord

Hemangioblastomas comprise 1 to 2% of all intracranial neoplasm, and 8 to 12% of tumors within the posterior fossa. They are composed of admixtures of three different cell types; endothelial cells, pericytes and stromal cells. Although most hemangioblastomas arise sporadically, they are associated with von Hippel-Lindau disease in about 20% cases. We have experienced a case of multiple hemangioblastomas occuiing in the cerebellum, medulla oblongata and cervical spinal cord simultaneously in a 55-year-old male. He had complained of headache, dizziness, generalized weakness and gait disturbance for 2 weeks. The patient had neither specific family history nor increased hematocrit. MRI showed a nonenhancing cystic lesion with an enhancing mural nodule in the right cerebellar hemisphere and two separate enhancing nodules in the medulla oblongata and dorsal cervical spinal cord at the 5-6th. Grossly, the excised mass of the cerebellum, 2.5 x 2 x 1.8cm, was solid to partly cystic, and that of spinal cord, lcm in diameter, was mostly solid. Microscopically, the tumor was composed of thin-walled blood vessels in variable size and interspersed stromal cells. The stromal cells revealed dimorphic cytoplasm that were either homogeneous and eosinophilic, or clear and vacuolated. Immunohistochemically, the endothelial cells reacted positively for glial fibrfllaty acidic protein(GFAP) and vimentin. The stromal cells reacted diffusely positively for vimentin, focally positively for GFAP and S-100 protein near the periphery of the tumor, focally positivel for neuro specipic enolase(NSE), and negatively for lysozyme, desmin and chromogranin. Ultrastructurally, the stromal cells contained numerous microfilaments and lipid droplets.

MRI Diagnosis and Differential Diagnosis of Hemangioblastomas / 实用放射学杂志

Objective To study the MRI manifestations of hemangioblastomas.Methods MRI data of 27 cases of hemangioblastomasproved by surgery and pathology were analyzed retrospectively.Results Hemangioblastomas were often located in hemispherium of cerebellum.All cases could be divided into three types : cyst-nodule type cyst-solid type and purely solid mass type.The large cyst and small nodule sign were characteristic manifestations of the cyst-nodule type,the signals of the fluid of the cyst were higher than that ofcerebrospinal fluid on MRI.The nodules or solid potions of the mass were showed as equal or slight long T_1 and slight long T_2 signalintensity,and were obviously enhanced.The blood vessel flow-void-sign around and inside the solid parts of the masses could be seen,and had a close relation with surrounding meninges.Conclusion MRI is a effective method in diagnosing hemangioblastomas,but the atypical cases should be differentiated from cystic astrocytoma,meningioma and metastesis.

Posterior Fossa Hemangioblastoma

Hemangioblastomas are histologically benign tumors, which generally occur at the posteriro fossa and rarely in supratentorial region. The authors analyzed 18 cases of posterior fossa hemangioblastoma which have been operated at the Department of Neurosurgery, Seoul National University Hospital from 1982 to 1989. The clinico-pathological and radiological features were as follows : 1) Among 18 cases, 13 cases were cystic type with or without mural nodule and 5 cases were solid type. 2) 2 cases were compatible with Von Hippel Lidau's complex, one of which was associated with retinal angiomatosis and the other with mulitple cysts in the pancreas and kidney. In 3 cases, hemoglobin level was above 18g/dl, implying polycythemia, but returned to normal level postoperatively. 3) Mural nodules were not visible in the enhanced CT scan in the 2 cases, in one of which mural nodule was visible in the angiography and so, angiography was more helpful than the CT scan in the detection and the localization of the mural nodule. 4) Among 18 cases, 2 cases(11%) recurred at 34 months and 48 months postperatively.