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El cavernoma cerebral es una malformación vascular de diagnóstico infrecuente. Se define como una malformación a nivel de la vasculatura microcerebral que, dependiendo a la ubicación y si existe la posibilidad de ruptura, conlleva a una emergencia que puede terminar en la muerte del paciente. En esta oportunidad se reporta el caso de un paciente con cavernoma cerebral asociado al síndrome de Evans. Se decide manejo quirúrgico de la lesión por aumento de intensidad de cefalea e intolerancia oral. Dada la coexistencia del Síndrome de Evans y la alta tasa de morbimortalidad es que se decide manejo quirúrgico mediante radiocirugía estereotáxica con gamma knife. El uso de dosis de margen bajo para tratamiento con gamma knife para uso en cavernomas cerebrales produce un manejo controlado para sintomatología de convulsiones y mejor expectativa de calidad de vida.
Cerebral cavernoma is an infrequently diagnosed vascular malformation. It is defined as a malformation at the level of the microcerebral vasculature that, depending on the location and if there is a possibility of rupture, leads to an emergency that can end in the death of the patient. On this occasion, we report a case of a patient with cerebral cavernoma associated with Evans syndrome. Surgical management of the lesion was decided due to increased intensity of headache and oral intolerance. Given the coexistence of Evans Syndrome and the high rate of morbidity and mortality, surgical management was decided by stereotaxic radiosurgery with a gamma knife. The use of low-margin doses for treatment with gamma knife for use in brain cavernomas produces controlled management for seizure symptoms and better quality of life expectancy.
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Spinal cavernous hemangiomas (SCH) represent a rare subtype of cavernous hemangiomas (CH). Existing literature predominantly indicates a higher prevalence of SCH in the thoracic region, although a comprehensive explanation for segmental distribution variations remains elusive. Current investigations suggest that the etiology of SCH may be linked to mutations in genes such as TEK, KRIT1, MGC4607, and PDCD10, and can also be precipitated by external factors including radiation, trauma, and infection. However, the specific impact of radiation dosage and modality on the incidence and severity of SCH warrants further in-depth study. The onset of this disease is concealed, but it can cause serious motor and sensory disorders. The symptoms mostly progress slowly or disappear gradually, but there are also cases of acute onset or sudden exacerbation. Recent research emphasizes the more prevalent magnetic resonance imaging (MRI) features of "flame-like" or "linear" intramedullary hemorrhages over the formerly identified "popcorn" or "mulberry-like" heterogeneous signals. Surgical intervention is generally recommended for patients with severe or progressively worsening symptoms, large lesion volumes, high risk of hemorrhage, or those with rapid increase in lesion size upon follow-up. Optimal outcomes are achieved when surgery is performed within three months of symptom onset. Surgical strategies typically involve laminectomy or laminoplasty via a posterior approach to achieve maximal lesion resection, although deep-seated lesions still pose considerable surgical challenges and risks. Ongoing clinical innovations are focusing on optimizing surgical approaches and techniques, such as employing anterior or dorsolateral approaches into the spinal cord via dorsal root entry zones and utilizing orthopedic robotic systems. Intraoperative monitoring technologies like neurophysiological monitoring and intraoperative ultrasound have been widely implemented, yet uniform warning criteria are yet to be established. A limited array of pharmacotherapies, including propranolol and sirolimus, have been clinically deployed, but their efficacy awaits validation from multicenter studies. In terms of disease management, patients with a history of spinal lesions, subarachnoid hemorrhages, or large lesions generally have poorer prognoses, while the impact of underlying comorbidities like hypertension and diabetes on hemorrhage risk remains unclear. The likelihood of re-hemorrhage in SCH patients increases over time, and tailored conservative treatment plans for diverse clinical scenarios are yet to be refined.
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Cerebral cavernous malformations (CCMs) are the common cerebrovascular malformation. Its incidence was 0.16%-0.5%. CCM can exist in both sporadic and familial forms, with the latter being inherited in an autosomal dominant manner. Its pathogenesis is associated with mutations in the CCM1, CCM2, and CCM3 genes. The somatic mutations of these genes are the basis for the occurrence of brain lesions. In order to explore the pathogenesis of CCM and identify therapeutic targets, various CCM animal models have been developed, providing assistance for the study of the pathological and physiological mechanisms of CCM. However, each CCM model has its own advantages, disadvantages, and applicability. Mice are the most commonly used animals to model CCM. Therefore, this article summarizes the characteristics and research progress of current murine CCM models.
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Objective:To study the clinical features of pure spinal epidural cavernous hemangioma in order to improve the diagnosis and treatment ability.Methods:The clinical data of 7 patients with pure spinal epidural cavernous hemangioma from January 2013 to November 2022 in Xiangyang Central Hospital were analyzed retrospectively.Results:Among the 7 patients, 2 males and 5 females, and the average age was 49.4 years old. Location of the lesion: cervical spine 1 case, thoracic spine 5 cases, and lumbar spine 1 case. One patient had pure radicular symptoms, 4 patients had pure spinal cord symptoms, and the other 2 patients had both myelopathy symptoms and radicular symptoms. T 1 and T 2 weighted images showed equal or slightly longer signals, and the enhanced scans showed uniform enhancement. Before operation, 1 case was misdiagnosed as meningioma, and the patient′s lesion was not carefully identified during operation, so the lesion was mistaken for oozing hemorrhage caused by operation; 2 cases were misdiagnosed as schwannoma. All patients underwent preoperative localization and microsurgical resection of epidural lesions through the posterior median approach. The lesions were completely removed and no significant complications were observed during hospitalization. Before operation, the neurological function Frankel grade C was in 1 case, grade D in 3 cases, grade E in 3 cases; the patients were followed up for 1 to 117 months, at the last follow-up, neurological function Frankel grade D was in 3 cases, grade E in 4 cases, no recurrence was found. Conclusions:The pure spinal epidural cavernous hemangioma is very likely to be misdiagnosed as the schwannoma and meningioma in imaging, the preoperative imaging should be carefully observed. It is recommended to inject methylene blue into the spinous process of the lesion segment before operation and locate the photos to help with precise intraoperative positioning. Once the disease is considered, special attention should be paid when opening the vertebral lamina to remove the epidural fat during the operation. The lesion is prone to bleeding, and is mistakenly believed to be absorbed by the aspirator or bitten together with the epidural fat. Total resection is an effective treatment for this disease, and it should be treated as soon as possible to avoid the influence of acute hemorrhage on the prognosis.
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RESUMEN Introducción: Los tumores de partes blandas son un grupo heterogéneo de lesiones tanto benignas como malignas. El origen histológico es diverso y entre ellos se encuentra el vascular como el hemangioma. Objetivo: Conocer un paciente con hemangioma cavernoso de partes blandas del pie derecho. Caso clínico: Paciente masculino de 21 años de edad, blanco sin antecedentes mórbidos de salud, acude a la consulta externa de Ortopedia y Traumatología por presentar una bola a nivel de la planta del pie derecho acompañada de dolor. Apareció hace dos años, pero ha incrementado su tamaño de forma rápida en los últimos tres meses. Mediante la exploración física se observó la tumoración en la cara plantar e interna del pie derecho, de consistencia dura, fija, mayor a 8 cm, de bordes irregulares y pobremente definidos con aumento de la temperatura local. Se realizaron exámenes complementarios imagenológicos y hematológicos. Al analizar el resultado de la exploración física y los complementarios se decidió la intervención quirúrgica consistente en la resección de la tumoración. Conclusiones: El hemangioma cavernoso es una enfermedad que afecta por lo general a niños y adolescentes sin predilección por el sexo, sus síntomas y signos más encontrados son el aumento de volumen y el dolor. El tratamiento consiste en la resección del tumor a través de un margen de seguridad y su principal complicación es la recidiva.
ABSTRACT Introduction: Soft tissue tumors are a heterogeneous group of both benign and malignant lesions. The histological origin is diverse and among them is the vascular one such as hemangioma. Objective: To present a patient with a soft tissue cavernous hemangioma of the right foot. Case report: A 21-year-old white male, without morbid antecedents, attended at the Orthopedics and Traumatology outpatient clinic for presenting a ball at the level of the sole of the right foot accompanied by pain. It appeared two years ago, but has grown rapidly in size in the last three months. The physical examination revealed that the tumor was on the plantar and inner side of the right foot, it was of a hard, fixed consistency, greater than eight centimeters, with irregular and poorly defined edges, with an increase in local temperature. Complementary imaging and hematological examinations were performed. Upon analyzing the results of the physical examination and the complementary ones, a surgical intervention consisting of resection of the tumor was decided. Conclusions: Cavernous hemangioma is an entity that generally affects children and adolescents without predilection for sex, its most common symptoms and signs are increased volume and pain. Treatment consists of resection of the tumor through a safety margin and its main complication is recurrence.
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Abstract Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple well-encapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon.
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Resumen Las malformaciones venosas son lesiones vasculares benignas infrecuentes que se presentan en el útero. Están conformadas por venas anormales, de diferentes tamaños y proporciones, con configuración espongiforme y disposición al azar. En la literatura, han sido previamente reportados algunos casos, usando el término "hemangioma cavernoso", pero según los cambios recientes en la terminología, aprobados por Sociedad Internacional para el Estudio de las Anormalidades Vasculares (ISSVA), se desaconseja el uso de este término y se sugiere el de "Malformación venosa", si se cumplen los hallazgos histopatológicos al momento de hacer el diagnóstico. Presentamos el caso de una mujer de 44 años, con cuadro de hemorragia vaginal anormal y diagnóstico clínico de miomatosis y mioma abortado por el orificio cervical interno, el estudio histopatológico reveló la presencia de una malformación venosa que comprometía el miometrio y endometrio, con formación subsecuente de un pólipo.
Abstract Venous malformations are benign vascular lesions that rarely appear in the uterus. They are made up of abnormal veins, of different sizes and proportions, with spongiform configuration and random disposition. In the literature, some cases have been previously reported, using the term "cavernous hemangioma", but according to recent changes in terminology, approved by the International Society for the Study of Vascular Abnormalities (ISSVA), the use of this term is discouraged, and the diagnosis of Venous malformation is suggested, if the histopathological findings are met. We present the case of a 44-year-old woman, with abnormal vaginal bleeding and a clinical diagnosis of myomatosis and myoma aborted by the internal cervical orifice, in whom the histopathological study revealed the presence of a venous malformation that compromised the myometrium and endometrium, with subsequent formation of a polyp.
Subject(s)
Humans , Female , Adult , Uterus , Vascular Malformations , Hemangioma, Cavernous , Uterus/pathology , Vascular System Injuries , Hemangioma , MorphogenesisABSTRACT
Objective:To investigate the clinical manifestations and pathogenic gene mutation sites of familial cavernous hemangioma by a pedigree study of this disease.Methods:A family of cerebral cavernous hemangioma who was admitted to the Department of Neurology of the First Affiliated Hospital of Henan University of Science and Technology in April 2019 was diagnosed as cerebral cavernous hemangioma type 1 based on clinical manifestations and head magnetic resonance imaging (MRI), diffusion weighted imaging and susceptibility weighted imaging screening. According to Zabramski classification criteria, the family′s clinical data were collected and genes were sequenced.Results:A 58-year-old female proband had dizziness and headache as the main symptoms, her daughter and son had no clinical symptoms, and her granddaughter had clinical manifestations of cerebral hemorrhage and seizures. The proband and her family members showed multiple cavernous hemangioma on cranial MRI,and the p.L436fs mutation in the KRIT1 gene of familial cerebral cavernous malformation type 1 was confirmed through genetic examination, which was consistent with the Zabramski typing results based on head MRI. The mutation site of the familial spongiform malformation type 1 pathogenic gene was found to be p.L436fs in KRIT1 gene, which has not been reported in familial cerebral cavernous hemangioma type 1 until now.Conclusion:A new p.L436fs mutation of KRIT1 gene was found in familial cerebral cavernous malformation type 1, which expands understanding of the clinical manifestations and pathogenic gene mutation sites of familial cavernous hemangioma.
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Los hemangiomas hepáticos, también denominados hemangiomas cavernomatosos, son los tumores hepáticos más comunes. Se caracterizan por ser lesiones solitarias, pequeñas y benignas que se diagnostican frecuentemente de forma incidental. Suelen ser lesiones asintomáticas, si bien los síntomas se presentan más frecuentemente en aquellas lesiones mayores de 5 cm, también conocidas como hemangiomas gigantes. Presentamos el caso de una mujer de 43 años, pauci-asintomática que presenta un hemangioma gigante de 16x16x27 cm, así como se realiza una revisión sistemática de la literatura.
Hepatic hemangiomas, also referred to as cavernous hemangiomas, are the most common benign mesenchymal hepatic tumors. They are often solitary, small lesions that have an excellent safety-prognosis and were commonly incidentally detected. Hepatic hemangiomas are frequently asymptomatic, although symptoms are more likely in those lesions larger than 5 cm also referred to as giant hemangiomas. We present a case of a pauci-asymptomatic 43 year-old woman with an uncommon 16x16x27 cm giant hemangioma and perform a review of the literature.
Subject(s)
Adult , Female , Humans , Hemangioma, Cavernous/diagnosis , Liver Neoplasms/diagnosis , Tumor Burden , Hemangioma, Cavernous/pathology , Liver Neoplasms/pathologyABSTRACT
ObjectIve To evaluate the safety and efficacy of Gamma knife radiosurgery for the treatment of cavernous sinus cavernous hemangioma. Methods From June 2010 to July 2016, 35 consecutive patients with cavernous sinus cavernous hemangioma treated with Gamma knife radiosurgery at the Department of Neurosurgery,West China Hospital,Sichuan University were enrolled retrospective, including 22 females and 13 males. All patients were diagnosed by MRI before procedure. They were confirmed by postoperative histopathology or further verification by MRI. The irradiation dose of Gamma knife around tumors were 11-16 Gy (mean 14. 3 ± 1. 1 Gy),covered by 45%-50% isodose line and ensured that the exposure dose of the optic cross was less than 8 Gy. In the first year after operation,they were followed up at 3,6,9,and 12 months,and after that,the clinical efficacy,surgical complications and tumor volume were evaluated once a year. Results Thirty-five patients were followed up for 12-77 months (mean 36 ± 4 months). No surgery-related optic nerve injury and new cranial nerve injury occurred in all cases . Symptomatic patients were 28 (80%)and symptomatic improvement rate was 96. 4%(n=27),among them,the symptoms were improved in 15 cases (53. 6%),and the symptoms disappeared in 12 cases (42. 9%),did not have any change in 1 (3. 6%)case,and no symptom deterioration occurred. The symptom improvement time was 2-7 months after surgery (mean 3. 7 ± 0. 3 months). Imaging follow-up revealed that in 35 patients with cavernous sinus cavernous hemangioma treated by gamma knife radiosurgery,the tumor volume was significantly reduced after treatment compared with that before treatment,and the difference wasstatisticallysignificant(8.8±0.9cm3vs.32.6±3.6cm3,t=8.492,P<0.01).Theratesoftumor volume change were ≤25%,26%-50%,51%-75%,and ≥75%. There were 3 patients (8. 6%), 2 (5. 7%),8 (22. 9%),and 22 (62. 9%),respectively. Conclusions Gamma knife radiosurgery is safe and effective in the treatment of cavernous sinus cavernous hemangioma. It can be used as the preferred treatment modality for this disease,but long-term follow-up is needed to observe its long-term efficacy.
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Objective@#To investigate the clinical features and surgical treatment effect of patients with cardiac cavernous hemangioma.@*Methods@#Clinical data of 9 patients (5 male, aged from 4 to 53 years old) with cardiac cavernous hemangioma, who underwent surgical treatment from November 2002 to March 2015 and the diagnosis of cardiac cavernous hemangioma was confirmed by postoperative histological examination, were retrospectively analyzed. Effects of surgical treatment were analyzed.@*Results@#Four patients were asymptomatic (heart murmur presented in 3 patients during physical examination). Three patients presented with palpitation, chest distress, and short of breath. One patient presented with epigastric discomfort and another patient presented with intermittent fever for more than 10 months. ST and T wave changes of electrocardiogram were found in 2 patients, cardiac mass was detected in the right heart chamber in 5 patients by echocardiography, and no cardiac mass was detected the rest 4 patients. Cardiac masses were resected en bloc, then the adjacent tissues were repaired in 7 patients, and mass was partially resected due to the involvement with adjacent heart structure. No cardiac mass was found during operation in 1 case, impaired mitral valve structure was excised and postoperative pathologically confirmed as cardiac valve cavernous angioma on the excised mitral valve structure. No signs of recurrence or enlargement of cardiac cavernous hemangioma were found during the 11(10, 11)years follow up.@*Conclusions@#There is no specific clinical feature for patients with cardiac cavernous hemangioma. It is difficult to detect the cardiac valve cavernous angioma by echocardiography before surgery. Individualized surgical treatment is associated with good clinical outcome in this patient cohort. However, the clinical features and surgical treatment effect of patients with cardiac cavernous hemangioma still need to investigate in large sample trial.
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Cavernous hemangiomas rarely involve the female genital tract. It is difficult to identify vascular malformations when these lesions are concealed in the vagina or deep vulva area. We present a rare case of vaginal cavernous hemangioma in a 30-year-old primiparous woman with an early severe postpartum hemorrhage (PPH) and delayed continuous bleeding from the episiotomy site. She was treated successfully with transarterial embolization of the left vaginal artery. To our knowledge, this is the first reported case of PPH caused by rupture of a vaginal hemangioma during vaginal delivery in English literature.
Subject(s)
Adult , Female , Humans , Arteries , Episiotomy , Hemangioma , Hemangioma, Cavernous , Hemorrhage , Postpartum Hemorrhage , Postpartum Period , Rupture , Vagina , Vascular Malformations , VulvaABSTRACT
Tracheal hemangioma is a rare benign vascular tumor in adults. We reported a case of massive hemoptysis caused by a cavernous hemangioma in a 75-year-old man. This is the first report, to our knowledge, of a tracheal cavernous hemangioma that presented with massive hemoptysis. The lesion was removed with a CO2 laser under rigid laryngoscopy. Endovascular tumors, such as tracheobronchial hemangiomas, should be considered a diagnostic option in cases of massive hemoptysis without a significant underlying lung lesion.
Subject(s)
Adult , Aged , Humans , Hemangioma , Hemangioma, Cavernous , Hemoptysis , Laryngoscopy , Lasers, Gas , Lung , TracheaABSTRACT
Cavernous hemangiomas of the gastrointestinal tract are extremely rare. In particular, the diagnosis of small bowel hemangiomas is very difficult in children. A 13-year-old boy presented at the outpatient clinic with dizziness and fatigue. The patient was previously diagnosed with iron-deficiency anemia at 3 years of age and had been treated with iron supplements continuously and pure red cell transfusion intermittently. Laboratory tests indicated that the patient currently had iron-deficiency anemia. There was no evidence of gross bleeding, such as hematemesis or bloody stool. Laboratory findings indicated no bleeding tendency. Gastroduodenoscopy and colonoscopy results were negative. To obtain a definitive diagnosis, the patient underwent capsule endoscopy. A purplish stalked mass was found in the jejunum, and the mass was excised successfully. We report of a 13-year-old boy who presented with severe and recurrent iron-deficiency anemia caused by a cavernous hemangioma in the small bowel without symptoms of gastrointestinal bleeding.
Subject(s)
Adolescent , Child , Humans , Male , Ambulatory Care Facilities , Anemia, Iron-Deficiency , Capsule Endoscopy , Colonoscopy , Diagnosis , Dizziness , Fatigue , Gastrointestinal Tract , Hemangioma , Hemangioma, Cavernous , Hematemesis , Hemorrhage , Iron , JejunumABSTRACT
Background Orbital cavernous hemangiomas (OCH) is a common benign orbital tumor in adult,and accurate localization and diagnosis before operation supports the significant premise for surgical safety and success of tumor extraction.Objective This study was to research the clinical characteristics,preoperative diagnosis,the selection for different surgical approaches,therapeutic effectiveness and complication prevention of OCH.Methods The clinical data of 117 eyes of 117 patients with OCH who received surgery were retrospectively analyzed.The patients received surgery in Henan Eye Institute,Henan Eye Hospital from January 2011 to December 2014 and followed-up for 3 months to 5 years.The visual acuity,exophthalmos,ocular movement,orbital A/B ultrasound,color Doppler image,CT and MRI were examined before and after surgery.Results The primary clinical manifestations of OCH were gradual exophthalmos and impaired vision.The accordance rate of preoperative diagnosis and pathological diagnosis was 100% in the group of patients.The surgical approachs included conjunctival approach in 52.14% (61/117),lateral orbitectomy in 30.77% (36/117),anterior approach in 16.24% (19/117) and lateral combined with medial approach in 0.85% (1/117).At the end of followed-up,visual acuity was significantly improved in 30.77% (36/117),declined in 8.55% (10/117) and unchanged in 60.68% (71/117).Temporary complications after surgery were pupil dilatation in 14.53% (17/117),emorrhoea in 1.71% (2/117),ocular motility disorders in 16.24% (19/117) and ptosis in 4.27% (5/117).The permanent complications after operation were pupil dilatation in 2.56% (3/117),visual loss in 0.85% (1/117) and permanent abduction imitation in 0.85% (1/117).Conclusions Accurate qualitative and site-specific diagnosis and correct choice of surgeries for OCH depend on clinical and iconographical examinations.Suitable surgical approach and operative skill are helpful to the therapeutic outcome and safety of OCH.
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Objective To retrospectively evaluate the safety ,technical success rate and long‐term efficacy of the hepatic mul‐tiple cavernous hemangioma with super selective arterial cheoembolization .Methods 6 cases multiple hepatic cavernous hemangio‐ma by clinical diagnosed between 2004-2011 years in our hospital ,Through arterial super selective and completely filling cheoem‐bolization by Pingyang mycin lipiodol emulsion(PYM‐Lip) ,To assess the long‐term efficacy .by multi slice spiral CT enhanced scan‐ning and carry on relevant statistics processing in postoperative 6 ,12 ,36months .Results 26 lesions were embolismed in 6 cases multiple hepatic cavernous hemangioma ,Among the number of successful embolization were 15 of 1 cases ,2 of 4 cases ,3 of 1 cases , respectively .26 lesions was decreased with different degrees ,which the diameter of lesions were reduced with embolismed by CT enhanced scanning in postoperative 6 ,12 ,36months and diameter reduced> 50% ,diameter reduced≤50% ,lesions disappear was 38% (10/26) ,54% (14/26) ,8% (2/26) ,62% (16/26) ,23% (6/26) ,15% (4/26) ,69% (18/26) ,12% (3/26) ,19% (5/26) .Technical operation success rate 100% ,not serious complications occurred .There are statistically significant differences in the size of lesions before and after operation(P<0 .01) .Conclusion The technique success rate was high ,minimally invasive ,the complications was less ,the curative efficacy was obvious by transcatheter arterial super selective cheoembolizaton with hepatic multiple cavernous he ‐mangioma .
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Os cavernomas são lesões classificadas como malformações vasculares, juntamente com malformações arteriovenosas, desenvolvimento venoso anômalo e telangiectasias capilares. Podem ocorrer de forma esporádica ou familiar e acometer qualquer área do sistema nervoso central, sendo a região supratentorial sua localização mais frequente. Neste artigo, é descrita uma série de seis pacientes com cavernoma na fossa posterior. Cinco cavernomas localizavam-se no tronco encefálico e um no cerebelo. A faixa etária dos pacientes variou de 14 a 50 anos. A abordagem cirúrgica diferiu entre os casos. Quatro casos apresentaram piora no pós-operatório imediato, com melhora subsequente. O seguimento dos pacientes variou de quatro meses a sete anos. Não houve óbito na série. Os cavernomas da fossa posterior requerem abordagem individualizada e técnica cirúrgica acurada. Embora possa ocorrer piora momentânea no pós-operatório imediato, a melhora subsequente é a regra, sendo o óbito infrequente.
Cavernomas are lesions classified as vascular malformations, along with arteriovenous malformations, developmental venous anomalies and capillary telangiectasia. They can occur in a sporadic or familial form and can affect any area of the central nervous system, being the region supratentorial the most frequent location. In this article, we describe a series of six cases of posterior fossae cavernoma. Five cavernomas were located in the brainstem and one in cerebellum. The ages ranged from 14 to 50 years old. The surgical approach differed between cases. Four cases worsened in the immediate postoperative period, with subsequent improvement. The follow-up ranged from four months to seven years. There were no deaths. Posterior fossae cavernomas require individualized approach and accurate surgical technique. Although momentary worsening may occur in the immediate postoperative period, the subsequent improvement is the rule, and the death is infrequent.
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Cranial Fossa, Posterior/abnormalities , Central Nervous System Vascular MalformationsABSTRACT
CONTEXT: Cavernous hemangiomas of the adrenal gland are rare benign neoplastic tumors. The clinical presentation of adrenal hemangiomas is usually vague, and they are often discovered incidentally through imaging examination s performed for other reasons. CASE REPORT: We report the case of a non-functional adrenal hemangioma found incidentally in a 37-year-old man with a one-year history of headache and hypertension. A right adrenal mass was detected by means of magnetic resonance imaging. Physical examination and all laboratory values were unremarkable. The patient underwent laparoscopic right adrenal gland resection. Histopathological evaluation confirmed adrenal cavernous hemangioma. CONCLUSIONS: Most occurrences of cavernous hemangiomas of the adrenal gland are non-functional and often discovered incidentally. Although rare, these unusual benign adrenal masses should form part of the differential diagnosis of adrenal neoplasms. The proper treatment for adrenal cavernous hemangioma is surgical removal. .
CONTEXTO: Hemangiomas cavernosos da glândula adrenal são tumores neoplásicos raros, benignos. A apresentação clínica dos hemangiomas adrenais é geralmente vaga e muitas vezes eles são descobertos acidentalmente por exame imagiológico realizado por outras razões. RELATO DE CASO: Nós relatamos um caso de hemangioma adrenal não funcional encontrado por acaso em um homem de 37 anos de idade com histórico de um ano de dor de cabeça e hipertensão. Foi detectada massa adrenal direita por meio de ressonância magnética. Exame físico e todos os valores de laboratoriais estavam normais. O paciente foi submetido a cirurgia laparoscópica para ressecção da glândula adrenal direita. Avaliação histopatológica confirmou hemangioma adrenal cavernoso. CONCLUSÃO: A maioria dos hemangiomas cavernosos de glândula adrenal são não funcionais e muitas vezes descobertos por acaso. Embora raras, essas massas adrenais benignas devem ser parte de um diagnóstico diferencial de neoplasias suprarrenais. O tratamento adequado para o hemangioma adrenal cavernoso é a remoção cirúrgica. .
Subject(s)
Adult , Humans , Male , Adrenal Gland Neoplasms/diagnosis , Hemangioma, Cavernous/diagnosis , Rare Diseases/diagnosis , Incidental Findings , Laparoscopy , Magnetic Resonance Imaging , Tomography Scanners, X-Ray ComputedABSTRACT
El propósito de esta revisión es resaltar la epidemiología, presentación clínica y características imaginológicas de las malformaciones cavernosas del sistema nervioso central, especialmente en sus diferentes localizaciones, con el objetivo de brindarle al radiólogo claves para el diagnóstico de esta patología potencialmente curable. De las bases de datos de nuestras instituciones recolectamos casos de malformaciones cavernosas. Las imágenes de TC, MR y angiografía fueron evaluadas, y el tamaño, la localización, las características imaginológicas y los hallazgos asociados fueron registrados. Adicionalmente, se revisó la literatura científica pertinente a este tema para proveer una manera práctica de hacer una aproximación diagnóstica de esta malformación. Las malformaciones cavernosas son lesiones vasculares congénitas compuestas por vasos sinusoidales que forman una masa compacta. Pueden ocurrir en cualquier sitio del encéfalo y de la médula espinal. Imaginológicamente pueden presentar ciertas características alarmantes que pueden simular otras patologías más agresivas e incluso malignas. El conocimiento de los hallazgos característicos según su localización es esencial para evitar tratamientos innecesarios. Las malformaciones cavernosas del SNC pueden presentar ciertos rasgos que se sobreponen con patologías más malignas. Complicaciones asociadas como son la hemorragia y el edema perilesional pueden afectar aún más el diagnóstico adecuado. Por esta razón, es indispensable que el radiólogo conozca el comportamiento imaginológico de estas lesiones.
The purpose of this revision is to highlight the demographics, clinical presentation, and imaging features of cavernous malformations (CA) of the central nervous system, especially in its different locations, with the purpose of providing the radiologist clues regarding the diagnosis of this potentially curable pathology. We collected cases of cavernous malformations from the databases at our institutions. CT, MR and angiographic studies were evaluated and lesion size, location, imaging characteristics, multiplicity, and associated findings were recorded. Additionally, the scientific literature pertinent to the subject was reviewed in a practical manner in order to provide a practical manner of making a diagnostic approach of this malformation. Cavernous malformations are congenital vascular lesions composed of sinusoid- type blood vessels that assemble into a compact mass. They may occur in any location in the brain and in the spinal cord. In terms of imaging, they may present certain alarming characteristics that may mimic more aggressive or malignant entities. Associated complications such as hemorrhage and perilesional edema may affect adequate diagnosis at an ever larger degree. For this reason, it is essential that the radiologist is familiar with the imaging behavior of these lesions.