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Neurosurgery May 2017Despite many publications about cerebral cavernous malformations (CCMs), controversy remains regarding diagnostic and management strategies. (Review)
Review
Synopsis of Guidelines for the Clinical Management of Cerebral Cavernous Malformations: Consensus Recommendations Based on Systematic Literature Review by the Angioma Alliance Scientific Advisory Board Clinical Experts Panel.
BACKGROUND
Despite many publications about cerebral cavernous malformations (CCMs), controversy remains regarding diagnostic and management strategies.
OBJECTIVE
To develop guidelines for CCM management.
METHODS
The Angioma Alliance ( www.angioma.org ), the patient support group in the United States advocating on behalf of patients and research in CCM, convened a multidisciplinary writing group comprising expert CCM clinicians to help summarize the existing literature related to the clinical care of CCM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and radiology standards, (4) neurosurgical considerations, and (5) neurological considerations. The group reviewed literature, rated evidence, developed recommendations, and established consensus, controversies, and knowledge gaps according to a prespecified protocol.
RESULTS
Of 1270 publications published between January 1, 1983 and September 31, 2014, we selected 98 based on methodological criteria, and identified 38 additional recent or relevant publications. Topic authors used these publications to summarize current knowledge and arrive at 23 consensus management recommendations, which we rated by class (size of effect) and level (estimate of certainty) according to the American Heart Association/American Stroke Association criteria. No recommendation was level A (because of the absence of randomized controlled trials), 11 (48%) were level B, and 12 (52%) were level C. Recommendations were class I in 8 (35%), class II in 10 (43%), and class III in 5 (22%).
CONCLUSION
Current evidence supports recommendations for the management of CCM, but their generally low levels and classes mandate further research to better inform clinical practice and update these recommendations. The complete recommendations document, including the criteria for selecting reference citations, a more detailed justification of the respective recommendations, and a summary of controversies and knowledge gaps, was similarly peer reviewed and is available on line www.angioma.org/CCMGuidelines .
Topics: Advisory Committees; Central Nervous System Neoplasms; Consensus; Disease Management; Expert Testimony; Hemangioma, Cavernous; Hemangioma, Cavernous, Central Nervous System; Humans; Physical Therapy Modalities; Practice Guidelines as Topic; Stroke; United States
PubMed: 28387823
DOI: 10.1093/neuros/nyx091 -
Japanese Journal of Radiology Nov 2021To investigate and compare the CT and MRI features of hepatic sclerosed hemangioma (HSH) and sclerosing cavernous hemangioma (SCH).
PURPOSE
To investigate and compare the CT and MRI features of hepatic sclerosed hemangioma (HSH) and sclerosing cavernous hemangioma (SCH).
MATERIALS AND METHODS
Twelve HSH cases and 36 SCH cases were included, the imaging findings on CT (9 HSH and 34 SCH) and MRI (8 HSH and 10 SCH) were analyzed. Qualitative image analysis included the location, size, shape, capsular retraction, density, calcification, signal intensity on T-weighted image (TWI) and T-weighted image (TWI), presence of diffusion restriction, apparent diffusion coefficient (ADC) map, transient hepatic attenuation difference around the lesion, and the dynamic enhancement patterns.
RESULTS
The presence of liver cirrhosis in patients with HSH (3/12) was higher than SCH (1/36) (P = 0.043). The morphology appearance before enhancement showed no significant difference between HSH and SCH. Moreover, SCH had a stronger trend of centripetal enhancement patterns of cavernous hemangiomas (83.3%) compared to HSH (25%) (P < 0.001). Due to more frequent atypical enhancement features, containing rim-like enhancement, no enhancement, and peripheral heterogeneous enhancement, the misdiagnosis rate of HSH (75%) was significantly higher than that of SCH (16.7%) (P < 0.001). Furthermore, the ADC values of HSH and SCH were both higher than that of the surrounding liver parenchyma (P = 0.009, P = 0.002); however, there was no significant difference in ADC values between themselves (P = 0.613).
CONCLUSION
SCH showed the same trend of centripetal enhancement characteristics as typical hemangioma, while HSH exhibited atypical enhancement features due to complete sclerosis. Higher ADC values might contribute to the identification of atypical HSH and SCH from malignancies.
Topics: Hemangioma; Hemangioma, Cavernous; Humans; Liver Neoplasms; Magnetic Resonance Imaging; Sclerosis
PubMed: 34041675
DOI: 10.1007/s11604-021-01139-z -
World Journal of Surgical Oncology Dec 2019The cavernous hemangioma of mediastinum (CHM) is a rare benign lesion caused by congenital vascular dysplasia. However, its incidence is extremely low, and patients... (Review)
Review
BACKGROUND
The cavernous hemangioma of mediastinum (CHM) is a rare benign lesion caused by congenital vascular dysplasia. However, its incidence is extremely low, and patients often lack relevant clinical symptoms. So we analyzed retrospectively some cases to investigate the imaging features of cavernous hemangioma of mediastinum (CHM) and improve the diagnostic accuracy.
METHODS
The CT/MRI imaging features and clinical information of 19 patients with CHM were analyzed retrospectively.
RESULTS
The lesions of 18 CHM patients were single. Twelve cases in the anterior mediastinum and 8 in the posterior mediastinum. The diameter of CHM ranges from 2.0 to 7.0 cm. Thirteen cases were oval-shaped or round, 4 cases were lobulated, and 2 cases were irregular. Phleboliths or nodular calcification were identified in four cases. High signal of T2WI lipid suppression in two cases and blood vessel shadows were observed in two cases. After contrast-enhanced scan, the nodular enhancement of arteries were identified in 14 cases and contrast agent was further filled of the venous phase, where "fast in and slow out" feature was performed. One case showed inhomogeneous enhancement, one case performed "fast in and slow out" feature of multiphase-enhanced MRI. Besides, aberrant veins can be seen in or around the lesion among five cases.
CONCLUSIONS
CHM is more frequently located at the anterior mediastinum than at the posterior mediastinum. The performance of phleboliths, high signal on T2WI fat suppression and DWI, the nodular enhancement of the artery, venous and delayed phase filling, enhanced "fast in and slow out," and aberrant veins in the lesion are helpful for the diagnosis and differential diagnosis. Multiple period contrast-enhanced CT and MRI scan is helpful for the diagnosis of CHM.
Topics: Adult; Aged; Female; Follow-Up Studies; Hemangioma, Cavernous; Humans; Magnetic Resonance Imaging; Male; Mediastinal Neoplasms; Middle Aged; Prognosis; Retrospective Studies; Tomography, X-Ray Computed; Young Adult
PubMed: 31801558
DOI: 10.1186/s12957-019-1742-1 -
BMJ Case Reports Apr 2011Adrenal haemangioma is a rare, benign, non-functioning neoplasm. Haemangiomas are tumours mainly affecting the liver. In 1955, Johnson and Jeppesen described the first...
Adrenal haemangioma is a rare, benign, non-functioning neoplasm. Haemangiomas are tumours mainly affecting the liver. In 1955, Johnson and Jeppesen described the first adrenal cavernous haemangioma. Here the authors report a large adrenal haemangioma presenting in a 75-year-old woman who had experienced left flank pain for 5 months. Laboratory examinations and the plasma levels of tumour markers in the patient were within normal limits. Imaging with ultrasound and CT showed a heterogeneous 15×14×18 cm mass located in the left adrenal gland. The tumour showed irregular peripheral enhancement after bolus intravenous injection of contrast medium. The mass was removed surgically and histopathology revealed infracted cavernous haemangioma. No signs of malignancy were detected. Although rare, haemangioma should be included in the differential diagnosis of adrenal neoplasms.
Topics: Adrenal Gland Neoplasms; Aged; Female; Hemangioma, Cavernous; Humans
PubMed: 22701011
DOI: 10.1136/bcr.12.2010.3604 -
The Journal of Thoracic and... May 2020
Topics: Adult; Aorta; Heart Neoplasms; Hemangioma, Cavernous; Humans; Male; Treatment Outcome; Vascular Neoplasms
PubMed: 31160112
DOI: 10.1016/j.jtcvs.2019.04.020 -
Annals of Cardiac Anaesthesia 2020Benign cardiac hemangiomas are rare tumors that may present in or out side of the heart, epicardium being the most common site. Echocardiography is the method of choice...
Benign cardiac hemangiomas are rare tumors that may present in or out side of the heart, epicardium being the most common site. Echocardiography is the method of choice in diagnosing cardiac masses and though 3D TEE may seem to add exact information about the location, the time constraint in doing a comprehensive examination along with 3D rendering inside operation room may become an hindrance.
Topics: Echocardiography; Female; Heart Atria; Heart Neoplasms; Hemangioma, Cavernous; Humans; Middle Aged; Tomography, X-Ray Computed
PubMed: 32687093
DOI: 10.4103/aca.ACA_58_19 -
Asian Journal of Surgery Feb 2022
Topics: Hemangioma, Cavernous; Humans; Ribs
PubMed: 34961724
DOI: 10.1016/j.asjsur.2021.11.056 -
Open Biology Nov 2020Cerebral cavernous malformations (CCMs) are neurovascular abnormalities characterized by thin, leaky blood vessels resulting in lesions that predispose to haemorrhages,... (Review)
Review
Cerebral cavernous malformations (CCMs) are neurovascular abnormalities characterized by thin, leaky blood vessels resulting in lesions that predispose to haemorrhages, stroke, epilepsy and focal neurological deficits. CCMs arise due to loss-of-function mutations in genes encoding one of three CCM complex proteins, KRIT1, CCM2 or CCM3. These widely expressed, multi-functional adaptor proteins can assemble into a CCM protein complex and (either alone or in complex) modulate signalling pathways that influence cell adhesion, cell contractility, cytoskeletal reorganization and gene expression. Recent advances, including analysis of the structures and interactions of CCM proteins, have allowed substantial progress towards understanding the molecular bases for CCM protein function and how their disruption leads to disease. Here, we review current knowledge of CCM protein signalling with a focus on three pathways which have generated the most interest-the RhoA-ROCK, MEKK3-MEK5-ERK5-KLF2/4 and cell junctional signalling pathways-but also consider ICAP1-β1 integrin and cdc42 signalling. We discuss emerging links between these pathways and the processes that drive disease pathology and highlight important open questions-key among them is the role of subcellular localization in the control of CCM protein activity.
Topics: Animals; Biomarkers; Carrier Proteins; Disease Management; Disease Susceptibility; Genetic Predisposition to Disease; Hemangioma, Cavernous, Central Nervous System; Humans; Intracellular Space; Mutation; Protein Binding; Protein Interaction Domains and Motifs; Protein Interaction Mapping; Protein Interaction Maps; Protein Transport; Signal Transduction
PubMed: 33234067
DOI: 10.1098/rsob.200263 -
Medicine Jul 2018Cavernous hemangioma is a congenital venous malformation with the potential to develop in all tissues of the body. However, cavernous hemangioma of the thymus is... (Review)
Review
RATIONALE
Cavernous hemangioma is a congenital venous malformation with the potential to develop in all tissues of the body. However, cavernous hemangioma of the thymus is extremely rare.
PATIENT CONCERNS
The present study describes the case of an asymptomatic, 30-year-old female who presented with a cavernous hemangioma in the thymus during a physical examination. Enhanced computed tomography of the chest revealed a 2.3 × 1.7 × 1.3 cm mass in the thymus.
DIAGNOSES
Histopathological examination revealed that the tumor exhibited the typical histological findings of a cavernous hemangioma.
INTERVENTIONS
The patient underwent surgical resection due to the uncertain diagnosis and the possibility that the mass was a thymoma or teratoma.
OUTCOMES
One-year post surgery, the patient was alive with no evidence of tumor recurrence.
LESSONS
Cavernous hemangioma of the thymus is a very rare disease. Complete surgical resection may be a critical therapeutic option.
Topics: Adult; Diagnosis, Differential; Female; Hemangioma, Cavernous; Humans; Teratoma; Thoracic Surgery, Video-Assisted; Thymoma; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 30045327
DOI: 10.1097/MD.0000000000011698 -
Cirugia Y Cirujanos 2021Diffuse cavernous hemangioma (DCH) is a rare benign vascular lesion. The rectosigmoid colon is the most common site of this disease. It affects mainly young adults. The...
Diffuse cavernous hemangioma (DCH) is a rare benign vascular lesion. The rectosigmoid colon is the most common site of this disease. It affects mainly young adults. The most frequent symptom is chronic rectal bleeding which is painless, often begins in the infancy and sometimes is uncontrollable. Endoscopy is the diagnose method of choice and complete surgical excision with sphincter-saving procedure is the primary mode of treatment. A high index of suspicion and a correct diagnose is necessary. We present a 34-year-old male with a DCH of the rectum and anus who required an APR because of affection of dentate line.
Topics: Adult; Anal Canal; Colon; Hemangioma, Cavernous; Humans; Male; Rectal Neoplasms; Rectum; Young Adult
PubMed: 34851591
DOI: 10.24875/CIRU.20000746