Hemangioma cavernoso / Cavernous Hemangioma

Introducción: El hemangioma cavernoso es una de las neoplasias benignas más frecuentes en la infancia. Objetivo: Describir el caso de un hemangioma cavernoso en edad pediátrica. Caso clínico: Lactante femenina de 6 meses de edad, de procedencia urbana, cuyo nacimiento tuvo lugar en el Hospital Materno Fe del Valle Ramos, del municipio Manzanillo. Al nacer se observa una lesión en forma placa eritematosa violácea infiltrada de ± 10 cm, de borde definido, de superficie liza, con aumento de temperatura al tacto, que se extendía desde la parte medial anterior y posterior hasta la superior de la pierna del miembro inferior izquierdo. Se decidió interconsultar con el Servicio de Angiología, el cual diagnostica la lesión como hemangioma cavernoso. Conclusiones: Se corroboró que el hemangioma cavernoso es una entidad frecuente en la infancia, su diagnóstico y tratamiento oportunos son altamente beneficiosos para la mejoría y la cura, por lo que se impone la realización de un correcto y exhaustivo examen físico, de lo que se deriva también la prevención de complicaciones a corto, mediano y largo plazo(AU)

Introduction: Cavernous hemangioma is one of the most frequent benign neoplasms in childhood. Objective: To describe the case of a cavernous hemangioma at pediatric age. Clinical case: A 6-month-old female infant of urban origin was born at Hospital Materno Fe del Valle Ramos, Manzanillo Municipality, Granma Province. At birth, a violaceous erythematous plaque-like infiltrated lesion was observed, of approximately 10 cm, with a defined border, smooth surface, increased temperature at hand contact, extending from the anterior and posterior medial part to the upper leg of the left lower limb. It was decided to do an interconsultation with the angiology service, which diagnosed the lesion as a cavernous hemangioma. Conclusions: Cavernous hemangioma was corroborated to be a frequent entity in childhood, whose timely diagnosis and treatment are highly beneficial for improvement and cure, so it extremely necessary to carry out a correct and exhaustive physical examination, also deriving the prevention of complications in the short, medium and long terms(AU)

Giant Cavernous Hemangioma of the Liver in a Patient with Autosomal Dominant Polycystic Kidney Disease.

BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) is frequently associated with liver cysts, but an association with giant cavernous liver hemangioma is not mentioned in the literature. CASE REPORT We report the case of a 41-year-old man with ADPKD, secondary arterial hypertension, and stage 4 chronic kidney disease who presented with a 2-week history of persistent pain at the base of the right hemithorax and in the right hypochondrium. An ultrasound examination and a contrast-enhanced computed tomography scan revealed a giant cavernous liver hemangioma. Surgery was intially taken into account (however, twice delayed because of the COVID-19 pandemic) but later refused because it would have left the patient with dangerously few liver parenchyma. CONCLUSIONS To our knowledge, this is the first reported case of ADPKD associated with cavernous liver hemangioma. Vascular endothelial growth factor could be the pathophysiological link between the 2 conditions. Further research may unravel the molecular biology that underlies this possible association, pointing to new therapeutic avenues for ADPKD.

Radiological Analysis of Orbital Cavernous Hemangiomas: A Review and Comparison Between Computed Tomography and Magnetic Resonance Imaging.

Cavernous hemangiomas are the most common benign orbital tumors in the orbit, but radiological differentiation from other solitary orbital masses can still be challenging at times. While there have been previous studies describing the radiological characteristics of cavernous hemangiomas on computed tomography (CT) and magnetic resonance imaging (MRI), there have not been any studies comparing the 2 imaging modalities. The purpose of our study was to evaluate CT and MRI findings of orbital cavernous hemangiomas and compare both modalities.We performed a cross-sectional study of patients with a histopathological diagnosis of cavernous hemangioma over a 20-year period from January 1997 to December 2016 in a single tertiary institution.Our study included 77 patients; mean age was 46.6 ±â€Š11.2 years, and females comprised 68.8%. The lateral orbit (23.4%) was the most common location. The masses were well-defined, with 55.8% being ovoid, 27.3% round, and 16.9% lobulated. The most common enhancement pattern on CT was a small point starting in the periphery, progressing to heterogeneous filling in the late phase. The most common enhancement pattern on MRI was multiple patchy starting points, widespread across the tumor, with a final homogeneous filling in the late phase.Significant differences between CT and MRI were found in terms of area, location, and number of starting points of contrast enhancement in the early phase. We also found that the use of MRI is limited in differentiating fast-filling cavernous hemangiomas from other orbital tumors and in such cases, contrast-enhanced CT would be helpful.

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 .

CAVERNOUS HEMANGIOMA OF THE RETINA: A Comprehensive Review of the Literature (1934-2015).

PURPOSE: Cavernous hemangioma of the retina (CHR) is a rare retinal vascular hamartoma. The objective of this study was to summarize the current knowledge of this rare condition based on a detailed review of 96 documented cases. METHODS: The incidence, clinical and histological appearance, differential diagnosis, treatment, and prognosis of CHR were analyzed by evaluating clinical data obtained from 96 cases in the literature since the first case report in 1934. RESULTS: More than 90% of cases of CHR have been reported in white individuals with even gender distribution. A majority of patients presented a unilateral unifocal lesion without positive family history. Our analysis showed bilateral cases were strongly correlated with family history, which suggests the importance of screenings for family members in bilateral CHR cases. Intracranial involvement was reported in 14% of CHR cases. Most patients were asymptomatic, but in patients with history of headaches, transient visual disturbance, or seizures 80% had intracranial involvement. CONCLUSION: It is important to screen family members in bilateral CHR cases, and it is critical to exclude cerebral vascular abnormalities in view of relative high incidence of intracranial involvement and high morbidity.

[Surgical treatment of liver haemangiomas in a third level hospital in south-east Mexico]. / Tratamiento quirúrgico de los hemangiomas hepáticos en una unidad médica de alta especialidad del sureste de México.

BACKGROUND: Liver haemangiomas are the most common benign tumours, commonly presented in women and considered giant when their diameter surpasses 4cm. They are mostly asymptomatic and incidental findings. They manifest with abdominal pain and mass effect. These tumours can be managed by observation, enucleation, resection, and embolisation. OBJECTIVE: To determine the experience in our unit as regards the treatment and post-surgical outcomes of patients with liver haemangiomas. MATERIALS AND METHODS: A retrospective study was performed on 14 patients with a histopathological diagnosis of liver haemangioma. An analysis was made using the sociodemographic, tumour-related and surgical related variables, as well as any complications. RESULTS: Of the 14 patients analyse, there were 7 males and 7 females, with a median age of 43.43±15.03 years, and a mean tumour size of 6.86±3.5cm. Eight (51.7%) of the tumours were located in the right lobe, 3 (21.4%) in the left lobe, and 3 (21.4%) in the caudate lobe. Resection was performed in 7 patients (50%), enucleation in 5 patients (35.7%), and biopsy in 2 patients (14.3). No relationship was found between sex, pathology, or tumour location. No morbidity or mortality was found. CONCLUSIONS: Liver haemangiomas in our unit have similar characteristics to those described in other studies. Surgical treatment in our hospital offers a positive outcome.

[Adult orbital tumors]. / Tumeurs de l'orbite de l'adulte.

Orbital tumors are a rather frequent pathology. Their diagnosis and treatment may be difficult. They can be benign or malignant. All the tissues of the orbit can give rise to a tumor, resulting in their large number. Among the benign tumors, we have meningiomas and cavernous hemangiomas, and for the malignant tumors, lymphomas, metastasis, ENT tumors and lacrimal gland tumors in the adult. Usually the signs are nonspecific, with proptosis, oculomotor disturbance, inflammatory signs, pain and sometimes a mass. Imaging (CT, MRI and color Doppler ultrasound) shows the tumor, its location, extent and possible metastases. Biopsy and anatomic and cytopathologic examination confirm the type of benign or malignant tumor. Based on these three elements: clinical appearance, imaging and histology, the tumor will be treated, usually by a surgical approach according to the recommendations of a multidisciplinary tumor conference. Radiation therapy and chemotherapy may supplement the treatment.

A retrospective and consecutive analysis of the epidemiology and management of spinal cavernomas over the last 20 years in a single center.

Spinal cavernous malformations (SCM) are rare lesions often presenting with acute onset of symptoms and progressive neurological deterioration due to hemorrhage into the spinal cord. With the aid of modern techniques, their surgical removal became much safer. The present study was undertaken to analyze the outcome of our series of surgically and conservatively treated patients with SCM. Over a period of 20 years, 20 surgically treated and 5 conservatively managed patients with spinal cavernous malformations were identified and enrolled into this analysis. Demographic data, clinical symptoms, localization and extension of the cavernoma, as well as pre- and postoperative neurological status were obtained. The clinical status was assessed using the Frankel score. Patients were followed up clinically and by MRI. Before surgery, 90% (18/20) of our surgical patients were classified as Frankel D (93.8%), whereas two patients (10%) were graded C. None of the patients had a worse Frankel score at the time of discharge. Eighty percent of them (16 cases) remained unchanged, and 20% (4 patients) improved during the first follow-up (mean 6.3 months, range 2-17 months). All improved patients had a superficially located SCM and were operated early (≤3 months). No worsening was observed during extended follow-up (range 9-134 months, mean 44.7 months). Five nonsurgically treated patients showed no significant clinical deterioration over a period of 6.7 years (mean, range 2.9-8 years). SCM localization and number of involved segments had no influence on outcome. Our data show that SCM can be resected with favorable neurological outcome by using intraoperative neuromonitoring. Within the follow-up period, patients treated conservatively remained in a stable neurological condition.

Revisión de cavernomatosis múltiple: a propósito de una familia / Multiple cavernomatosis: A review and presentation of a familial case

No disponible

[Management of orbital cavernous hemangioma - evaluation of surgical approaches: report of 43 cases]. / Prise en charge des hémangiomes caverneux orbitaires - évaluation des voies d'abord chirurgicales: à propos de 43 cas.

Cavernous hemangioma is the most frequent benign orbital tumor in adults. The purpose of this study was to examine its clinical features, to define surgical indications, and to determine the roles of the various surgical approaches praticed in ophthalmology: transconjunctival (increasingly utilized), anterior transcutaneous, and lateral orbitotomy. The records of all patients treated for orbital cavernous hemangioma (OCH) since 2004 at the Fondation Rothschild (Paris, France) were retrospectively reviewed. Forty-three patients were treated for orbital cavernous hemangioma. Fifty-eight percent were women, mean age 50.2; 79 % of the tumors were intraconal. Among those patients, 36 underwent surgical removal, 5 were followed periodically, and 2 were lost to follow-up. The main surgical indications were: optic nerve compression (26 patients), proptosis (24 patients) and diplopia (3 patients). Transconjunctival, anterior transcutaneous and Kronlein approaches were used in 16, 12 and 4 patients respectively. Four patients had intrapalpebral hemangiomas easily reached transcutaneously. Two patients demonstrated transient partial 3rd nerve palsy (one with the lateral orbitotomy approach and one with the transconjunctival approach), one patient with the lateral orbitotomy approach developed a palsy of the superior branch of the 3rd nerve, and one patient with the transcutaneous anterior approach developed mydriasis. Surgical excision of OCH's is required in the presence of clinical complications. The transconjunctival approach is a safe technique which can lead to complete resection of the tumor in most cases.

Characterizing the relationship between functional MRI-derived measures and clinical outcomes in patients with vascular lesions.

OBJECT: Functional MRI (fMRI) has proven to be an effective component of pretreatment planning in patients harboring a variety of different brain lesions. The authors have recently reported significant relationships concerning distances between brain tumor borders and areas of functional activation (lesion-to-activation distance; LAD) with regard to patient morbidity and mortality. This study further examines the relationship between LAD, focusing on a host of vascular lesions and pre- and posttreatment morbidity. METHODS: This study included a sample population of patients with vascular lesions (n = 106), primarily arteriovenous malformations (AVMs) and cavernomas. These patients underwent pretreatment fMRI-based motor mapping (n = 72) or language mapping (n = 84). The impact of LAD and other variables derived from the patient medical record were analyzed with respect to functional deficits in terms of morbidity (weakness and/or aphasia). RESULTS: In patients with no pretreatment deficits, there was trend for a significant relationship between the Wernicke area LAD and posttreatment language deficits. In patients with or without pretreatment deficits, a trend toward significance was observed between sensorimotor LAD and posttreatment motor deficits. Additionally, lesion type (AVMs or cavernomas) affected posttreatment deficits, with more patients with cavernomas showing posttreatment language deficits than patients with AVMs. However, this difference was not observed for posttreatment motor deficits. CONCLUSIONS: These findings suggest that the proximity of a vascular lesion to sensorimotor and language areas is a relevant parameter in estimating patient prognosis in the perioperative period. Additionally, vascular lesion type and existence of pretreatment deficits play a significant role in outcomes.

An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011.

PURPOSE: To evaluate the frequencies of orbital space-occupying lesions seen at single orbital unit in a period of 35 years. METHODS: In this retrospective case series, the authors reviewed the medical records of 2,480 consecutive patients referred to the authors' Orbital Unit for evaluation of an orbital mass between 1976 and 2011. The final diagnosis in each case was established by a combination of history, ocular findings, diagnostic imaging, and histopathologic analysis, when available. The number and percentage of benign and malignant tumors were determinated, also according to the age of the patients and the tumor location within the orbit. This study adhered to the principles of the Declaration of Helsinki. RESULTS: Of the 2,480 lesions, 1,697 (68%) were benign and 783 (32%) were malignant. The most frequent benign tumors were dermoid cyst (14%) and cavernous hemangioma (9%). The most common malignant tumors were non-Hodgkin lymphoma (12%), basal cell carcinoma (3%), and orbital metastases (3%). In patients younger than 60 years, benign tumors are more frequent, whereas in patients older than 60 years, the frequency of malignant tumors increased. Regarding the distribution in the orbit, the most common tumors were dermoid cyst (206 cases) in the upper-outer quadrant, mucocele (155 cases) in the upper-inner quadrant, basal cell epithelioma (35 cases) in the lower-inner quadrant, cavernous hemangioma (68 cases) in the lower-outer quadrant, and meningioma (90 cases) in central space. Most of the tumors were located in the upper-outer quadrant. In the lower-inner quadrant, malignant tumors were more frequent than benign tumors. CONCLUSIONS: The authors' findings highlight the distinctive nature of the orbital oncology experience at an ocular oncology department.

Critical infantile hepatic hemangioma: results of a nationwide survey by the Japanese Infantile Hepatic Hemangioma Study Group.

BACKGROUND: The current survey aimed to describe the clinical features of critical infantile hepatic hemangioma (IHH) and the implications of recent treatments. MATERIALS AND METHODS: A nationwide survey of critical IHH patients treated between 2005 and 2010 was performed in all 117 registered pediatric surgical hospitals in Japan. As a result, 19 patients were identified and reviewed using a statistical analysis. RESULTS: Abdominal distention (47.4%), high-output cardiac failure (47.4%), coagulopathy (42.1%), and respiratory distress (31.6%) were the major symptoms. Three patients died (1 of coagulopathy, 1 of cardiac failure, and 1 of both). An accompanying portovenous shunt was also highlighted. Infantile hepatic hemangioma was totally insensitive to steroid treatment in 3 (23.1%) of the 13 patients, and 9 (47.4%) of the 19 patients required other treatments. Surgical resection and ß-blocker improved the hematologic data, whereas hepatic arterial ligation and embolization seemed to produce a limited effect. Among the dead patients, several hematologic parameters were significantly worse: the thrombocyte count (pretherapeutic: 73,000 vs 300,000/mm(3), dead vs survivor, respectively [P < .03]; posttherapeutic: 66,000 vs 388,700/mm(3) [P < .003]) and the prothrombin time (posttherapeutic, 35.0 vs 12.1 seconds [P < .0001], dead vs survivor, respectively). CONCLUSION: For critical IHH cases with steroid-insensitive hematologic disorders, alternative treatments including ß-blocker therapy, surgery, and liver transplantation should be considered.

Diagnosis and management of extensive vascular malformations of the lower limb: part I. Clinical diagnosis.

There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermatologist's role in the diagnosis of these lesions. At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. This article highlights the differences in clinical appearance and discusses the differential diagnosis of extensive vascular malformations in an attempt to ensure earlier diagnosis and better outcomes for these patients.

Association of hepatic hemangiomatosis with giant cavernous hemangioma in the adult population: prevalence, imaging appearance, and relevance.

OBJECTIVE: The purpose of our study was to evaluate the association of hepatic hemangiomatosis with giant cavernous hemangioma (GCH) and describe the imaging appearances and clinical relevance. MATERIALS AND METHODS: Forty-one patients who had undergone CT or MRI with reported GCH (> 8 cm) between 1997 and 2009 were identified retrospectively. Three readers interpreted 27 MRI studies, 36 CT studies, and 16 ultrasound studies of these patients. Prevalence, extent, and imaging appearance of coexistent hemangiomatosis in the surrounding liver parenchyma were evaluated. RESULTS: Forty-two GCHs were identified in 41 patients and hemangiomatosis was present in 18 of 41 patients (44%) with GCH. Twelve patients had a diffuse pattern of hemangiomatosis (67%), and six patients showed a nodular pattern consisting of multiple coalescent nodules measuring < 5 mm (33%). There was no association between the size of the GCH and presence and extent of hemangiomatosis. The common hepatic artery was enlarged (> 5 mm) in 14 patients with GCH, of whom 12 had associated hemangiomatosis. There was a statistically significant association between the size of the hepatic artery and presence of hemangiomatosis (p < 0.001). CONCLUSION: Hemangiomatosis is not rare in the liver parenchyma adjacent to a GCH. The presence and extent of hemangiomatosis must be specifically communicated to referring physicians. Surgical candidates have to be carefully selected to avoid complications, such as excessive blood loss and diminished risk of postoperative liver decompensation from apparent overestimation of functional residual volume due to oversight of involved liver areas by hemangiomatosis.

[Intramedullary spinal cord cavernous hemangiomas: clinical features and surgical treatment].

Intramedullary spinal cord cavernous hemangiomas (angiomas) are occult vascular malformations characterized by rare lesions consisting of closely packed capillary-like vessels. In general, patients with this disease become symptomatic because of hemorrhage leads to progressive neurological deficits. Therefore, surgical tumor resection should be considered for symptomatic patients should be considered a surgical tumor resection.

Radiation-induced tumors in children irradiated for brain tumor: a longitudinal study.

BACKGROUND: Radiation-induced tumors (RIT) are increasingly recognized as delayed complications of brain irradiation during childhood. However, the true incidence is not established, their biology is poorly understood, and few guidelines exist regarding the long-term follow-up of irradiated children. METHODS: We studied retrospectively patients irradiated for brain tumor under 18 years and followed in our institution since 1970. RIT were defined as new masses, different from the original tumor, occurring after delay in irradiated areas, and not related to phacomatosis. RESULTS: Among 552 irradiated patients, 42 (7.6%) developed one or more RIT, 26 months to 29 years after irradiation (mean 12.8 years). The cumulated incidence was 2.0% at 5 years and 8.9% at 10 years. Of the patients, 73.8% were adult at the time of diagnosis of RIT, and 75% were diagnosed within 18.1 years after irradiation. We identified 60 cavernomas, 26 meningiomas, 2 malignant gliomas, 1 meningosarcoma, and 6 thyroid tumors. Compared with meningiomas, cavernomas appeared earlier, in children irradiated at an older age, and with a male predominance. Although RIT were correlated with higher irradiation doses, 80.9% of these occurred at some distance from the maximum irradiation field. Twenty-five lesions were operated in 20 patients; three patients died because of progression of the RIT. CONCLUSION: A significant number of patients undergoing irradiation for brain tumor during childhood develop a RIT, often during adulthood. Our data suggest that radiation-induced cavernomas result from angiogenetic processes rather than true tumorigenesis. Protracted follow-up with MRI is warranted in children irradiated for brain tumor.