Cavernous sinus hemangioma treated with gamma knife radiosurgery: usefulness of SPECT for diagnosis--case report.
(1/125)
A 79-year-old female presented with cavernous sinus hemangioma manifesting as double vision due to right oculomotor and trochlear nerve pareses. Computed tomography and magnetic resonance imaging revealed bony erosion and a right cavernous sinus tumor with "tail sign" after contrast medium administration. Thallium-201 (201Tl) single photon emission computed tomography (SPECT) showed low uptake within the tumor, and technetium-99m-human serum albumin-diethylenetriaminepenta-acetic acid SPECT disclosed high uptake within the tumor. 201Tl SPECT usually shows very high uptake in meningiomas and malignant tumors, so the tumor was considered to be an unrelated benign tumor. The patient underwent partial resection of the tumor. Histological examination of the specimen confirmed cavernous hemangioma. The oculomotor nerve paresis partially improved. Gamma knife radiosurgery was carried out 4 months after the operation. The tumor markedly shrank with full recovery of extraocular movement 6 months after radiosurgery. SPECT is useful for distinguishing cavernous sinus hemangiomas from other cavernous tumors. Radiosurgery should be performed after partial resection or biopsy for cavernous sinus hemangiomas and may be the initial treatment for patients with small cavernous sinus hemangioma if the diagnosis can be established based on neuroimaging. (+info)
Interaction between krit1 and icap1alpha infers perturbation of integrin beta1-mediated angiogenesis in the pathogenesis of cerebral cavernous malformation.
(2/125)
Cerebral cavernous malformation (CCM) is a common autosomal dominant disorder characterized by venous sinusoids that predispose to intracranial hemorrhage. CCM is genetically heterogeneous, with loci at 7q, 7p and 3q. Mutations in KRIT1 account for all cases linked to 7q (CCM1), but the pathogenesis of CCM is not understood. Krev Interaction Trapped 1 (krit1) was originally identified through its interaction with the Ras-family GTPase krev1/rap1a in a two-hybrid screen, inferring a role in GTPase signaling cascades. We demonstrated additional 5'-coding exons for krit1, extending the N-terminus by 207 amino acids compared to the previously reported protein. Remarkably, by two-hybrid analysis and co-immunoprecipitation, full-length krit1 fails to interact with krev1/rap1a but shows strong interaction with integrin cytoplasmic domain-associated protein-1 (icap1). Icap1 binds to a NPXY motif in the cytoplasmic domain of beta1 integrin and participates in beta1-mediated cell adhesion and migration. The novel N-terminus of krit1 contains a NPXY motif that it is required for icap1 interaction. Like beta1 integrin, krit1 interacts with the 200 amino acid isoform of icap1 (icap1alpha), but not a 150 amino acid form that results from alternative splicing (icap1beta). In a competition assay, induced expression of krit1 diminishes the interaction between icap1alpha and beta1 integrin. Taken together, these data suggest that beta1 integrin and krit1 compete for the same site on icap1alpha, perhaps constituting a regulatory mechanism. Loss-of-function KRIT1 mutations, as observed in CCM1, would shift the balance with predicted consequences for endothelial cell performance during integrin beta1-dependent angiogenesis. (+info)
Cavernous haemangioma in the interpeduncular cistern: case report and review of literature.
(3/125)
A rare case of a cavernous haemangioma in the interpeduncular cistern is reported. The patient, forty-five year old male presented with excruciating left sided trigeminal neuralgia and diplopia for the past one year. Examination revealed left third and fifth nerve paresis. Magnetic resonance imaging showed a well-defined, lobulated tumour in the interpeduncular cistern. The tumour was totally excised through a subtemporal route. Histology of the tumour revealed a cavernous haemangioma. Extracerebral location for a cavernous haemangioma is rare. An interpeduncular cavernous haemangioma has never been reported earlier in literature. The clinical and radiological features are discussed and relevant literature is briefly reviewed. (+info)
Radiation-induced cavernous hemangiomas of the brain: a late effect predominantly in children.
(4/125)
INTRODUCTION: The induction of cavernomas as a consequence of brain irradiation was first suspected in 1994 and has been controversial since that time. METHODS: Between 1986 and 2000, 189 cerebral cavernomas were diagnosed in the Neurosurgical Department of the University of Heidelberg; of those patients, 5 had received prior radiation therapy. The ages of these 5 patients were compared with those of the 184 others with naturally occuring cavernomas. In an examination of 40 patients with cavernomas occurring after radiation (the 5 mentioned above, plus 35 from the literature) the age distribution was investigated, and a possible relationship between radiation dosage and latency interval to diagnosis of cavernoma was examined. RESULTS: Almost one in four of the patients under 15 years of age diagnosed with a cerebral cavernoma in the Neurosurgical Department of the University of Heidelberg had received prior radiation. In 40 patients with cavernomas and prior radiation (5 from Heidelberg, 35 from the literature), there was a clear accumulation in the age group of 10-19 years (50%). Most of those patients had received radiation in the first 10 years of life. The accumulation of cavernomas after radiation in childhood could not be explained by a greater frequency of radiation exposure in children compared to adults. In children up to 10 years of age at the time of radiation therapy, a dose of 3000 cGy and higher was followed by a shorter latency interval to incidence of cavernoma (P = 0.0018). In patients older than 10 years at the time of radiation, postradiation cavernomas only occurred when dosage was 3000 cGy or greater. CONCLUSION: These results indicate a correlation between radiation and cavernoma, particularly in children under 10 years of age at the time of radiation therapy. In adults, cavernomas after radiation rarely occur, and then only after higher radiation dosages (3000 cGy or more). (+info)
Anterior transpetrosal approach for pontine cavernous angioma--case report.
(5/125)
A 58-year-old male patient presented with headache and unsteady gait. Magnetic resonance imaging revealed hemorrhage from a pontine cavernous angioma. The patient experienced stepwise aggravation of symptoms due to repeated hemorrhages. We decided to surgically remove the pontine cavernous angioma through an anterior transpetrosal approach, since the angioma and hematoma were located near the ventrolateral surface of the pons. The brain stem was incised at a site caudal to the trigeminal nerve and the hematoma and angioma were totally removed. No additional neurological deficits were observed following surgery. Brain stem cavernous angiomas are usually removed via a trans-fourth ventricle or lateral suboccipital approach. However, these approaches may not be appropriate if the angioma is located ventrally to the pons. We propose that the anterior transpetrosal approach is the method of choice for ventrally located pontine cavernous angioma. (+info)
Cognitive dysfunction after isolated brain stem insult. An underdiagnosed cause of long term morbidity.
(6/125)
Cognitive dysfunction adversely influences long term outcome after cerebral insult, but the potential for brain stem lesions to produce cognitive as well as physical impairments is not widely recognised. This report describes a series of seven consecutive patients referred to a neurological rehabilitation unit with lesions limited to brain stem structures, all of whom were shown to exhibit deficits in at least one domain of cognition. The practical importance of recognising cognitive dysfunction in this group of patients, and the theoretical significance of the disruption of specific cognitive domains by lesions to distributed neural circuits, are discussed. (+info)
Krit1/cerebral cavernous malformation 1 mRNA is preferentially expressed in neurons and epithelial cells in embryo and adult.
(7/125)
Cavernous malformations are capillaro-venous lesions mostly located within the central nervous system (CCM/OMIM#116860) and occasionally within the skin and/or retina. They occur as a sporadic or hereditary condition. Three CCM loci have been mapped, and the sole gene identified so far, CCM1, has been shown to encode KRIT1, a protein of unknown function. In an attempt to get some insight on the relationship between KRIT1 mutations and CCM lesions, we investigated Krit1 mRNA expression during mouse development from E7.5 to E20.5 and in adult tissues, of both mouse and human origin. A ubiquitous Krit1 mRNA expression was detected from E7.5 up to E9.5. Then, it became progressively restricted from E10.5 to E12.5, to become detectable later essentially in the nervous system and various epithelia. Strong labelling was observed in neurons in the brain, cerebellum, spinal cord, retina and dorsal root ganglia. In epithelia, Krit1 mRNA expression was detected in differentiating epidermal, digestive, respiratory, uterine and urinary epithelia. A similar pattern of expression persisted in mouse and man adult nervous system and epithelia. Unexpectedly, in vascular tissues, expression of Krit1 was detected only in large blood vessels of the embryo. (+info)
Intracranial extra-axial cavernous (HEM) angiomas: tumors or vascular malformations?
(8/125)
INTRODUCTION: Extra-axial cavernous hemangiomas or angiomas [(hem)angiomas] are relatively rare lesions. They usually arise in relation to the dura mater intracranially or at the spinal level. Most of these lesions have been described in the middle cranial fossa at level of the cavernous sinus. Controversy still exists regarding the exact nature of these extra-axial cavernous angiomas: vascular tumor versus vascular malformation similar to intra-axial cavernomas. It has been suggested that they could represent an adult form of the hemangioma of infancy. Extra-axial cavernous (hem)angiomas often mimic meningiomas and their clinical behavior and imaging appearance are quite different than those of intra-axial cavernous angiomas. SUBJECTS AND METHODS: Five patients ranging in age from 24 to 63 years with a histologically proven dural cavernous angioma were retrospectively included. The lesions were located at level of the cavernous sinus (4 cases) and falx. CT and MR scans were performed in all cases and angiography in three patients. Four patients underwent surgery and a biopsy was performed in one case. One lesion was embolized before biopsy. Histology was available in all patients. RESULTS: In the operated patients, the lesion was totally resected in 2 cases and partially in the other 2. No postsurgical complication was noted. Histology revealed a vascular malformation composed of large vascular channels lined by flat endothelium and separated by fibroconnective tissue stroma. The pathological diagnosis was cavernous angioma. CONCLUSION: On the basis of the analysis of the literature and of our cases, intra-cranial extra-cerebral so-called cavernous (hem)angiomas present findings suggesting that they are vascular malformative lesions, analogous to the intra-axial cavernous angioma. A relationship with the hemangiomas of infancy seems unlikely. Correct terms for extra-cerebral cavernous (hem)angiomas are cavernoma, cavernous angioma, or venous vascular malformation of cavernous type . The term hemangioma should be avoided and reserved for the common vascular tumor of infancy. (+info)