Le syndrome de Kasabach-Merritt (SKM) est une affection rare et grave qui associe une lésion vasculaire (hémangiome), un syndrome hématologique dominé par une thrombopénie, souvent profonde, exposant ...à un syndrome hémorragique et une coagulopathie de consommation plus ou moins marquée ; le traitement est souvent difficile. Nous rapportons le cas d’un nouveau-né admis à j6 de vie pour un SKM compliquant un hémangiome cutané inopérable. Le traitement reposant initialement seulement sur la corticothérapie n’a amené aucune amélioration, puis l’association de la vincristine a permis une augmentation rapide du taux sanguin des plaquettes ainsi qu’une nette régression du volume de l’hémangiome. Par son action antimitotique inhibitrice de l’angiogenèse, la vincristine semble être un traitement efficace et bien toléré du SKM ; elle permet de contrôler la coagulopathie et d’éradiquer la tumeur vasculaire.
Abstract
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.
Abstract Objective Although hemangiomas are common lesions of the head and neck, sinonasal hemangiomas are rare. The purpose of this study was to analyze the clinical features (sex, age, symptoms, ...and size and anatomical location of the lesion) and the histological findings of sinonasal hemangioma cases, to assess preoperative transarterial embolization, and to evaluate the outcome (recurrence or no recurrence) of endoscopic sinus surgery. Methods Clinical records of 31 patients who underwent endoscopic sinus surgery for resection of sinonasal hemangioma between January 2010 and June 2015 were retrospectively reviewed. Results The study group consisted of 19 men and 12 women. Mean age was 53.3 ± 15.9 years. The principal symptom was epistaxis (81%). Mean tumor size was 12.6 ± 8.2 mm. The most common origin of hemangioma was the inferior turbinate (45%), followed by the nasal septum (39%), and, in both locations, the origin had a tendency to be located in the anterior portion. Thirty-one specimens were histologically categorized as 9 cavernous hemangiomas and 22 capillary hemangiomas. Preoperative transarterial embolization was performed in 2 cases. Only one recurrence was observed among 31 cases. In the recurrent case, the hemangioma of the nasal septum was resected during pregnancy. Conclusion According to our results, the transnasal endoscopic approach can be useful for the resection of sinonasal hemangioma. However, sinonasal hemangioma in connection with pregnancy must be addressed with care to decide the appropriate time for treatment.
Intramuscular capillary-type haemangiomas (ICTH) are rare vascular anomalies that can easily be misdiagnosed as other entities. A systematic review was performed of all cases of ICTH in the ...literature since its first description in 1972. An adjudication committee reviewed cases to include only ICTHs. Among 1,143 reports screened, 43 were included, involving 75 patients. The most frequent differential diagnosis was intramuscular venous malformations. The mean age of patients at diagnosis was 21.2 years. ICTH was mainly described as a gradually increasing mass (81.8%), painless (73.9%), that could occur anywhere in the body but most frequently on the head and neck (44.0%). Magnetic resonance imaging (MRI) was mainly used for diagnosis (69.1%) and displayed specific features. The most frequent treatment was complete surgical removal (73.9%), which could be preceded by embolization, and led to complete remission without recurrence in all but 1 case.
Giant cavernous haemangioma in cavernous sinus Rabelo, Nícollas Nunes; Chaves, Pedro Henrique Silveira; Oliveira, Marco Antônio de ...
British journal of neurosurgery
ahead-of-print, Številka:
ahead-of-print
Journal Article
Recenzirano
The aim of this article is show the neuroimaging, the pathological analysis and makes a brief review regarding to a giant cavernous haemangioma located in cavernous sinus in a 72 years old patient. A ...brief review was made in the literature searching for the key words "hemangioma" and "cavernous sinus" in the databases PubMed and Scielo for the last ten years. The images addressed were obtained by magnetic resonance imaging (MRI) in FLAIR, T1 and T1-weighted contrast-enhanced. The intracranial cavernous haemangiomas are rare conditions that comprise from 0,1 to 4% of intracranial vascular malformations. Diagnosis is made by MRI, when available SPECT (99mTc) is used to confirm and the treatment is done surgically with complement of radiotherapy and radiosurgery. The reported neuroimaging and pathological analysis show a giant cavernous hemangioma in cavernous sinus, a benign neoplasm involving the left internal carotid artery and maintaining contact with the contralateral internal carotid artery formed by abundant vascular structures, but without the presence of a muscular tunic.
Papillary hemangioma is a novel variant of intravascular hemangioma. It is more common in adults and has a male predominance. Most tumors reported so far are solitary and cutaneous. Here we present a ...rare case of an intraosseous papillary hemangioma involving the frontal bone. Brain imaging in a 69-year-old man with a slowly enlarging swelling on the right frontal area following an accidental fall demonstrated a 4.5 cm × 1.7 cm × 4.2 cm mass originating from the right frontal bone, with a tiny defect on the orbital roof. A malignant process was favored, and the mass was removed. Histopathology revealed a vascular lesion showing intraosseous distribution with foci of extension into the fibrous connective tissue. There were areas of plump endothelial cells with intracytoplasmic hyaline globules arranged in papillary configuration. The lesional cells were immunoreactive with CD34. AE1/AE3, EMA, PR, D2-40, inhibin, and S100 stains were negative. Ki-67 was low. This is the first intraosseous and second noncutaneous papillary hemangioma. Clinically it differs from other cases by the presence of trauma as a preceding event. Since its prognosis is unknown such patients should be monitored for recurrence or malignant transformation.
Background Congenital hemangiomas have been divided into 2 major subtypes based on clinical behavior: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH). ...Objective We describe a clinical subtype of congenital hemangioma that begins as a RICH but fails to completely involute and persists as a NICH-like lesion. We propose the term “partially involuting congenital hemangioma” for this lesion with overlapping features. Methods A review of the medical charts, serial clinical photographs, imaging, and biopsies performed on children with a diagnosis of partially involuting congenital hemangioma between 2001 and 2012 at Centre Hospitalier Universitaire Sainte-Justine pediatric dermatology/vascular anomalies clinic was performed. Results Eight full-term, healthy infants presented at birth with vascular lesions typical of RICH. Affected locations included the head and neck, trunk, or extremities. Size varied from 2.0 × 1.5 cm to 13.0 × 8.5 cm. All had rapid involution during the first 12 to 30 months of life before stabilizing in size and appearance. Limitations Only a small number of cases were identified. Conclusion Partially involuting congenital hemangiomas are congenital hemangiomas with a distinct behavior, evolving from RICH to persistent NICH-like lesions. Their recognition and study will help us better understand whether RICH and NICH are indeed separate entities or simply part of a spectrum.
Cavernous hemangioma is a benign vascular tumor occurring in all parts of the urinary system, including the kidney, bladder, prostate, ureter, and rarely urethra. Urethral cavernous hemangiomas are ...mostly seen in male patients, and only a few cases of female urethral hemangiomas are reported. Herein, we present the management and follow-up course of 3 cases of female urethral cavernous hemangioma. All 3 cases were menopause women complaining of lower urinary tract symptoms. Definitive diagnosis is made by histopathologic evaluation. In case of large or pedunculated masses, initial surgical resection is highly recommended. Regular follow-up of patients in order to prevent any recurrence is suggested.
Almost all of the epidural hemangiomas reported are cavernous hemangiomas. Purely extradural spinal capillary hemangiomas are very rare. Capillary hemangiomas are hamartomatous malformations that ...result from proliferations of vascular endothelial cells. Only ten cases have been reported in the English literature, treated with surgical excision.
A case of a dorsal extradural spinal capillary hemangioma is described. A total surgical removal has been performed after spinal angiography and embolization. Complete surgical removal should always be the goal in these lesions. Embolization did not show to reduce bleeding during the surgical procedure in this case.
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