ABSTRACT
The association between vascular tumors and thrombocytopenia is rare. Kasabach-Merritt Syndrome is seen in childhood and is characterized by hemangiomas and thrombocytopenia. A 42 years-old man with a cerebellar hemangioblastoma and thrombocytopenia, admitted with a subarachnoid hemorrhage is reported. The patient was operated and required a splenectomy to manage the thrombocytopenia. After the splenectomy the patient developed a subdural hematoma that was operated. Despite the surgical treatment, the patient died.
Subject(s)
Humans , Male , Adult , Thrombocytopenia/complications , Cerebellar Neoplasms/complications , Hemangioblastoma/complications , Thrombocytopenia/pathology , Thrombocytopenia/therapy , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Cerebellar Neoplasms/pathology , Cerebellar Neoplasms/therapy , Hemangioblastoma/pathology , Hemangioblastoma/therapy , Fatal Outcome , Diagnosis, Differential , Kasabach-Merritt Syndrome/pathology , Hematoma, Subdural/complications , Hematoma, Subdural/pathologyABSTRACT
Hemangioblastoma (HBL) in the pituitary stalk is extremely rare. Only 16 such cases have been reported in the past and 5 cases have been treated with surgical procedure. Here, we report surgical case of HBL in the pituitary stalk diagnosed in a 34-year-old woman. The patient underwent a gross-total resection via the modified lateral supra-orbital approach. No recurrence was observed in two years after surgery. To our knowledge, this is the 17th case of HBL in the pituitary stalk and the 6th surgical case. If the tumor is symptomatic and the volume is over 5 cubic centimeters as in our case, we recommend that the surgical resection of the HBL in the pituitary stalk is a more safe and reasonable than radiotherapy.
Subject(s)
Adult , Female , Humans , Cerebellar Neoplasms/pathology , Hemangioblastoma/pathology , Pituitary Gland/pathology , Treatment OutcomeABSTRACT
Computerized tomography (CT) scan and operative observations, and histolopathogical findings of 25 cases of intracranial hemangioblastoma were correlated. Solid hemangioblastomas showed a large number of thin-walled capillaries and abundant stromal cells with eosinophilic cytoplasm. Tumors with a cystic component and a mural nodule had a large number of stromal cells with vacuolated cytoplasm and microcysts.
Subject(s)
Adolescent , Adult , Aged , Cerebellar Neoplasms/pathology , Child , Female , Hemangioblastoma/pathology , Humans , Male , Middle Aged , Tomography, X-Ray ComputedABSTRACT
Von Hippel Lindau (VHL) disease is a rare autosomal dominant condition manifested by central nervous system hemangioblastoma, retinal angiomas, cysts of pancreas, kidney and epididymis, pheochromocytomas and renal cell carcinoma. We present such a case in a 45 years old male patient.
Subject(s)
Carcinoma, Renal Cell/pathology , Hemangioblastoma/pathology , Humans , Kidney Neoplasms/pathology , Male , Middle Aged , Supratentorial Neoplasms/pathology , von Hippel-Lindau Disease/pathologyABSTRACT
Intramedullary spinal hemangioblastoma is well known to be accompanied by syringomyelia. However, holocord secondary syringomyelia is uncommon. We present 2 cases of spinal hemangioblastoma, one in the conus medullaris and the other in midthoracic region, accompanied by holocord syrinx. In both the cases the secondary syrinx resolved following successful total tumor excision with good neurological recovery.
Subject(s)
Adolescent , Adult , Hemangioblastoma/pathology , Humans , Magnetic Resonance Imaging , Male , Spinal Cord Neoplasms/pathology , Syringomyelia/pathologyABSTRACT
Relatamos os achados clínicos, epidemiológicos e anatomopatológicos de 14 casos de hemangioblastoma. Sessenta e quatro por cento ocorreram em pacientes do sexo masculino, com idades variando de 16 a 60 anos, com média de 34,4 anos. Nove localizaram-se no cerebelo. Os sintomas mais comuns foram cefaléia (n=7) e tontura (n=7), com período médio de evolução de 70 dias. O diagnóstico de síndrome de von Hippel-Lindau (vHL) foi feito em 3 pacientes. Onze pacientes foram submetidos a ressecção cirúrgica total e 3 a exérese parcial. Evidenciou-se recidiva em 28 ppor cento dos casos, em 3 anos de acompanhamento. Os pacientes com vHL apresentaram recidiva em 66 por cento dos casos. Estes achados aproximam-se dos encontrados na literatura, enfatizando a morbidade deste tumor quando associado à vHL.
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Cerebellar Neoplasms/pathology , Hemangioblastoma/pathology , Spinal Cord Neoplasms/pathology , Cerebellar Neoplasms/surgery , Hemangioblastoma/surgery , Recurrence , Spinal Cord Neoplasms/surgery , Syndrome , von Hippel-Lindau Disease/complications , von Hippel-Lindau Disease/diagnosisABSTRACT
El hemangioblastoma cerebeloso es un tumor raro que tiene una incidencia de 1 a 2 por ciento entre los tumores cerebelosos. Se presenta generalmente en hombres jóvenes, de edad media entre 30 y 40 años. Es un tumor formado por numerosos vasos sanguíneos de tipo capilar con células endoteliales, pericitos y células estromales claras vacuoladas. En el Hospital Juárez de México se estudiaron cuatro casos de hemangioblastomas cerebelosos, de los cuales dos eran hombres y dos mujeres todos ellos presentaron síndrome de hipertención endocraneana y síndrome cerebeloso. El diagnóstico radiológico se dio en tres casos de cisticercosis y uno de hemangioblastoma. A todos ellos se les operó inicialmente realizándose cirugía descompresiva. El diagnóstico histológico de hemangioblastoma se realizó en el estudio definitivo. Dicho diagnóstico es de por sí difíil, por lo que requiere tinciones especiales; en tanto, el diagnóstico diferencial debe hacerse con carcinoma renal o astrocitoma pilocítico
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Cerebellar Diseases/diagnosis , Cerebellar Diseases/pathology , Hemangioblastoma/diagnosis , Hemangioblastoma/pathology , Hemangioblastoma/surgery , Tomography, X-Ray ComputedABSTRACT
Os autores relatam os achados de autópsia de pacientes de uma mesma família e o diagnóstico por biópsia de hemangioblastoma de um terceiro membro desta mesma família. O primeiro paciente tinha 34 anos por ocasiao do óbito e os achados de necrópsia mostraram hemangioblastoma de retina, cerebelo, bulbo e medula espinhal, além de carcinoma renal, feocromocitoma, lesoes císticas de rim e pâncreas, hidromielia e meningiomas atípicos. Seu irmao morreu com 30 anos de idade e a autópsia revelou hemangioblastomas de cerebelo, carcinoma renal e cistoadenoma de células de células claras de epidídimo. A terceira paciente era filha do primeiro paciente e apresentou cefaléia e ataxia. A tomografia computadorizada mostrou lesao cerebelar cística e a biópsia confirmou tratar-se de hemangioblastoma. Sao feitas consideraçoes epidemiológicas sobre lesoes viscerais e do sistema nervoso mais comumente encontradas, além de discutir critérios diagnósticos.
Subject(s)
Humans , Male , Female , Adolescent , Adult , von Hippel-Lindau Disease/pathology , Carcinoma, Renal Cell , Cerebellar Neoplasms/pathology , Cerebral Angiography , Hemangioblastoma/pathology , Hemangioblastoma/surgery , Kidney Neoplasms , Meningioma/pathology , Meningeal Neoplasms/pathology , von Hippel-Lindau Disease/geneticsABSTRACT
Os autores relatam um caso de paciente masculino, 47 anos, com hidrocefalia e malformaçao vascular da fossa posterior, indicadas por arteriografia. O paciente foi submetido à drenagem ventrículo-peritoneal em duas ocasioes, ocorrendo obstruçao da válvula em ambas. Quatro meses após o surgimento dos sintomas, o paciente faleceu, sendo demonstrada, pela autópsia, uma neoplasia cística ocupando o bulbo, cujo diagnóstico histopatológico foi hemangioblastoma. O tumor apresentou reaçoes de imuno-histoquímica positivas para vimentina, proteína S100, enolase e positividade discutível para proteína ácida fibraglial (GFAP).