Extraaxial Cavernous Hemangiomas Research Articles

GNA14 and GNAQ somatic mutations cause spinal and intracranial extra-axial cavernous hemangiomas

Extra-axial cavernous hemangiomas (ECHs) are complex vascular lesions mainly found in the spine and cavernous sinus. Their removal poses significant risk due to their vascularity and diffuse nature, and their genetic underpinnings remain incompletely understood. Our approach involved genetic analyses on 31 tissue samples of ECHs employing whole-exome sequencing and targeted deep sequencing. We explored downstream signaling pathways, gene expression changes, and resultant phenotypic shifts induced by these mutations, both invitro and invivo. In our cohort, 77.4% of samples had somatic missense variants in GNA14, GNAQ, or GJA4. Transcriptomic analysis highlighted significant pathway upregulation, with the GNAQ c.626A>G (p.Gln209Arg) mutation elevating PI3K-AKT-mTOR and angiogenesis-related pathways, while GNA14 c.614A>T (p.Gln205Leu) mutation led to MAPK and angiogenesis-related pathway upregulation. Using a mouse xenograft model, we observed enlarged vessels from these mutations. Additionally, we initiated rapamycin treatment in a 14-year-old individual harboring the GNAQ c.626A>G (p.Gln209Arg) variant, resulting in gradual regression of cutaneous cavernous hemangiomas and improved motor strength, with minimal side effects. Understanding these mutations and their pathways provides a foundation for developing therapies for ECHs resistant to current therapies. Indeed, the administration of rapamycin in an individual within this study highlights the promise of targeted treatments in treating these complex lesions.

Somatic GJA4 mutation in intracranial extra-axial cavernous hemangiomas

ObjectiveExtra-axial cavernous hemangiomas (ECHs) are sporadic and rare intracranial occupational lesions that usually occur within the cavernous sinus. The aetiology of ECHs remains unknown.MethodsWhole-exome sequencing was performed on ECH lesions...

Intralesional Injection of Cyanoacrylate Adhesive by Percutaneous Direct Puncture for Removal of Intracranial Extraaxial Cavernous Hemangioma: Two-case Reports

Intralesional Injection of Cyanoacrylate Adhesive by Percutaneous Direct Puncture for Removal of Intracranial Extraaxial Cavernous Hemangioma: Two-case Reports

Multidisciplinary management of an intra-sellar cavernous hemangioma: Case report and review of the literature

Extra-axial cavernous hemangiomas (ECH) are rare vascular lesions with a tendency to grow within the medial structures of the middle cranial fossa. This pathological entity lacks specific symptoms, and falls into the category of differential diagnosis of space occupying lesions in the cavernous sinus (CS) with or without sellar involvement, including those of tumoral, vascular and inflammatory nature. Of note, ECH can also be indolent, and is at times discovered incidentally during autopsy investigations. On radiological studies, ECH with sellar extension are frequently mistaken at first for pituitary adenomas. Total removal of intrasellar-CS ECH is technically demanding and burdened by remarkable morbidity and mortality rates, mostly related to the complex neuroanatomy of the CS-sellar region (i.e., peri and postoperative bleeding, and transitory or permanent nerve palsies, hormonal deficits). Consequently, only a few cases of successful total removal have been reported so far in the literature. Surgical debulking with cranial nerve decompression followed by stereotactic radiosurgery is currently considered the best alternative to total removal when the latter carries excessive perioperative risks. We present a rare case of a mainly located intrasellar ECH extending to the left CS discussing its clinical features and focusing on the most relevant aspects of the surgical management along with a review of the pertinent literature.

Extra Axial Cavernous Hemangioma of the Cerebellopontine Angle With Intratumor Hemorrhage: Case Report and Review

Extraaxial cavernous hemangiomas are rare and benign vascular lesions that have a propensity to bleed. Unusual locations of these vascular malformations can cause misdiagnoses. A 16-year-old female had only headaches for 1 year and had 1 month of increasing headaches without neurologic deficit. Magnetic resonance imaging studies revealed a right extraaxial cerebellopontine angle lesion with enhancement after gadolinium showing intratumoral hemorrhage. Preoperatively, a cavernous hemangioma was suspected, and surgery was carried out through a right unilateral suboccipital craniectomy. Histopathologically, the lesion was a cavernous hemangioma. The postoperative period was uneventful. In the literature, cerebellopontine angle cavernous hemangiomas are very rare. We did not find any presented cases with associated intratumoral hemorrhage. Although the localization of this case was unusual with respect to those earlier published, it was without neurologic deficit, despite the intratumoral hemorrhage. Correct preoperative diagnosis is very important; it is advisable to consider cavernous hemangioma in the differential diagnosis of cerebellopontine angle lesions.

Imaging Findings of Cavernous Sinus Hemangiomas

Extra-axial cavernous sinus hemangiomas are rare and account for less than 1% of parasellar tumors. These lesions have characteristic radiological features but the differential diagnosis from parasellar meningiomas and schwannomas can be difficult. Preoperative diagnosis is important due to the risk of severe intraoperative bleeding and the complex neurovascular structures of the cavernous sinus. We describe two cases of cavernous sinus hemangiomas and review the imaging findings of these lesions.

CAVERNOUS HEMANGIOMA OF THE CAVERNOUS SINUS, SKIN, AND RETINA

There are several reports concerning cavernous hemangiomas of the skin and central nervous system. Additional retinal involvement has also been reported. The authors report a 69-year-old woman with a giant extra-axial cavernous hemangioma of the right cavernous sinus involving the supra- and parasellar region, retina, and skin. Shrinkage of its cutaneous part lead to subsequent increase of the volume of the intracranial part. Owing to compression of the optic and the oculomotor nerves, oculomotor disturbances, ptosis, and visual impairment to 0.2 occurred. Via a pterional approach microsurgical removal of the tumor except for a remnant of the intracavernous part was performed. Hemodynamic connection between cutaneous, retinal, and intracranial hemangiomas should be considered.

Unusual locations for cavernous hemangiomas: report of two cases and review of the literature

Cavernous hemangiomas are most commonly found within the subcortical neural parenchyma near the fissura Rolandi, in the basal ganglia, or in the brain stem. Because of advancing neuro-imaging technology and thus resulting in a higher incidence of cavernous hemangiomas they have rising impact in neurosurgery. We present two unusual cases of extra-axial cavernous hemangiomas: one located at the frontal falx, the other within the bone of the right frontal bone. We discuss these and other cases in the literature with respect to the more common differential diagnoses and the appropriate therapy regimen for cavernous hemangiomas in these locations.

Commentary

Background: Extraaxial cavernous hemangiomas (cavernomas) are very rare lesions, and less than 20 descriptions of these lesions outside the middle fossa have been reported. In this report, we describe a dural cavernous angioma involving the posterior sagittal sinus and discuss the clinical, radiological, operative, and histological features of this very uncommon lesion. Case Description: A 31-year-old right-handed male presented with headache and decreasing visual acuity. Severe bilateral papilledema was found on fundoscopic examination. Neurological examination demonstrated a minor right temporal field cut. Brain magnetic resonance imaging with contrast demonstrated a 2.5 x 2.5 cm hyperintense enhancing mass in the midline, which was contiguous with the posterior margin of the falx cerebri. The patient underwent a bilateral occipital craniotomy centered on the lesion. The histological features were consistent with cavernous angioma. Conclusion: This report demonstrates that although extra axial cavernomas are quite rare, they must be included in the differential diagnosis of enhancing lesions along the posterior sagittal sinus. The operative removal of these lesions can be quite treacherous and usually requires a careful reapproximation of the patent sinus after lesion excision.

Dural cavernous angioma of the posterior sagittal sinus: case report

Background Extraaxial cavernous hemangiomas (cavernomas) are very rare lesions, and less than 20 descriptions of these lesions outside the middle fossa have been reported. In this report, we describe a dural cavernous angioma involving the posterior sagittal sinus and discuss the clinical, radiological, operative, and histological features of this very uncommon lesion. Case Description A 31-year-old right-handed male presented with headache and decreasing visual acuity. Severe bilateral papilledema was found on fundoscopic examination. Neurological examination demonstrated a minor right temporal field cut. Brain magnetic resonance imaging with contrast demonstrated a 2.5 × 2.5 cm hyperintense enhancing mass in the midline, which was contiguous with the posterior margin of the falx cerebri. The patient underwent a bilateral occipital craniotomy centered on the lesion. The histological features were consistent with cavernous angioma. Conclusion This report demonstrates that although extra axial cavernomas are quite rare, they must be included in the differential diagnosis of enhancing lesions along the posterior sagittal sinus. The operative removal of these lesions can be quite treacherous and usually requires a careful reapproximation of the patent sinus after lesion excision.

Cavernous hemangioma extending to extracranial, intracranial, and orbital regions

Extraaxial cavernous hemangiomas are rare intracranial lesions that can be located in different cranial compartments. Extension across different tissue planes such as the subcutaneous tissue, skull, orbital cavity, intracranial dura mater, and extracranial trigeminal divisions within the same patient has not been previously reported. This 32-year-old woman suffered left exophthalmos, left sixth nerve palsy, and trigeminal neuropathy. Magnetic resonance imaging studies revealed an extensive multicompartmental lesion, with enhancement following Gd administration. A left orbitopterional approach allowed removal of several cavernomatous lesions located in the orbit, frontotemporal dura, and lateral wall of the cavernous sinus. A histologically based diagnosis of extraaxial cavernous hemangioma was made. In the postoperative period the patient experienced a regression of her symptoms. The authors report on a case of cavernous hemangioma with a unique extension to different intracranial/extracranial compartments. Although radical removal of the lesion was not feasible, partial excision allowed for satisfactory clinical control of the patient's symptoms.

Sclerosing Cavernous Hemangioma in the Cavernous Sinus: Case Report.

Cavernous hemangiomas of the cavernous sinus belong to a well-distinguished entity of extra-axial cavernous hemangiomas located in the cavernous sinus and have a typical appearance on magnetic resonance imaging (MRI). Severe intraoperative bleeding has been described during the excision of these lesions that is probably associated with their pathological features. An atypical case of a sclerosing lesion with distinct MRI features is described. In these particular cases, especially with giant hemangiomas where en bloc excision would be difficult, safe internal decompression and resection can be achieved.

Merits of intralesional fibrin glue injection in surgery for the cavernous sinus cavernous hemangiomas

✓ In planning surgical treatment for extraaxial cavernous hemangiomas, care should be taken to control severe tumor bleeding. The authors present a case of a large cavernous hemangioma of the cavernous sinus, which was completely removed with the aid of multiple intratumoral injections of fibrin glue. This novel method is very effective for preventing excessive blood loss during surgery for this type of lesion.

Extra-Axial Cavernous Hemangioma: Two Case Reports

Two patients with extra-axial cavernous hemangioma who presented with headache and oculovisual disturbances were investigated with computed tomography and magnetic resonance imaging. The lesions masqueraded as basal meningioma, but this diagnosis was not supported by magnetic resonance spectroscopy in one patient. Cerebral angiography with embolization was indicated in one patient, but embolization was not justified in the other. Both patients underwent a pterional craniotomy. The lesions were extradural and highly vascular, necessitating excessive transfusion in one patient in whom gross total resection was achieved, and precluding satisfactory removal in the other. There was no mortality. Transient ophthalmoplegia, the only complication in one patient, was due to surgical manipulation of the cavernous sinus; it resolved progressively over 3 months. Extra-axial skull base cavernous hemangiomas are distinct entities with clinical and radiological characteristics that differ from those of intraparenchymal cavernous malformations. They can mimic meningiomas or pituitary tumors. In some cases, magnetic resonance spectroscopy may narrow the differential diagnoses. Surgical resection remains the treatment of choice, facilitated by preoperative embolization to reduce intraoperative bleeding and by the application of the principles of skull base surgery. Fractionated radiotherapy is an alternative in partial or difficult resections and in high-risk and elderly patients.

Cavernous Hemangioma of the Cavernous Sinus: A Case Report

Intracranial extra-axial cavernous hemangioma of the cavernous sinus is a very rare vascular malformation. It usually appears as a round non-encapsulated mass with well-defined borders, mimicking meningioma. We describe a case of cavernous hemangioma of the cavernous sinus, including the radiologic imaging findings, and also review the literature.

Cavernous hemangiomas in the cavernous sinus. Case reports

BACKGROUND Extra-axial cavernous hemangiomas are rare and have a propensity to develop within the cavernous sinus. Total removal of these vascular tumors is difficult due to the risk of severe intraoperative bleeding and the complicated neurovascular structures of the cavernous sinus. Only a small number of cases have been reported to be successfully totally removed. METHODS Retrospective studies were done in three cases of extra-axial cavernous hemangiomas located in the cavernous sinus. All three patients presented with clinical symptoms common to other tumors located in the region, such as headache and impairment of cranial nerve function. Their preoperative MRI results showed significant hyperintensity on T2-weighted images and marked enhancement with gadolinium-DTPA that delineated a sharp tumor margin. RESULTS All three patients underwent total tumor removal, with an uneventful postoperative course. There was no postoperative neurological deficit in one patient, and a complete ophthalmoplegia and diminished sensation in the V 1 distribution in two patients. Three months after operation, follow-up MRI or CT scan showed no residual tumor. CONCLUSION Surgical resection of these lesions was possible but difficult because of severe bleeding. Avoiding piecemeal removal before the main feeding arteries are interrupted can minimize intraoperative bleeding.

Homonymous Hemianopsia Due to a Dural Cavernous Hemangioma

The clinical and radiographic features of extra-axial cavernous hemangiomas are described, and a case of homonymous visual field loss due to a dural-based occipital cavernous hemangioma is reported. A patient presented with a homonymous hemianopsia due to an enhancing tentorial mass lesion. The preoperative clinical and magnetic resonance imaging features were suggestive of meningioma. The patient underwent gross total resection of the lesion and the final pathologic examination was consistent with cavernous hemangioma. There was complete resolution of the visual field defect after surgery. Extra-axial cavernous hemangiomas differ from intra-axial cavernous hemangiomas in their clinical and radiographic features. The former lesions may mimic meningioma and should be considered in the differential diagnosis of a dural-based mass. Early recognition of the lesion is important because surgical removal of cavernous hemangiomas may be associated with a higher morbidity and mortality rate than meningiomas.

Extra-axial cavernous hemangiomas involving the dural sinuses

Extra-axial cavernous hemangiomas are rare lesions previously associated with unacceptable mortality and morbidity rates that precluded surgical resection. The authors analyze the clinical presentation, surgical results, and histology of eight intrasinus cavernous hemangiomas: six located in the cavernous sinus, one in the petrosal sinus, and one in the torcula. Magnetic resonance imaging is the best radiographic test for surgical planning. Successful tumor removal was achieved in six cases with no mortality and low morbidity. In the remaining two patients, only subtotal resection was achieved because of massive hemorrhage in one and the misdiagnosis of a pituitary adenoma leading to a transsphenoidal approach in the other. For hemangiomas arising within the cavernous sinus, extradural removal of the sphenoid bone facilitated preservation of the neurovascular structures. Since the clinical and histological characteristics of these lesions are distinct from intra-axial cavernous hemangiomas, a more appropriate term may be "sinus cavernoma" to indicate that these lesions are primarily intrasinus in origin.