Intracranial epithelioid hemangioendothelioma.

Post Graduate Institute of Neurological Surgery, ALNC, VHS Hospital, Taramani, Chennai, 600 113, India.
Child s Nervous System (Impact Factor: 1.24). 08/2008; 24(7):863-8. DOI: 10.1007/s00381-008-0634-4
Source: PubMed

ABSTRACT INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is an uncommon soft-tissue vascular neoplasm. Although a well-defined entity outside the neuraxis, its intracranial occurrence is rare. Literature review revealed 35 reported cases till date. CASE REPORT: The authors report a case of intracranial EHE in a 15-year-old girl, who presented with a short history and unusual radiology. Following radical removal, the lesion recurred within 1 month and progressed to terminal stages. This tumor is thought to have an indolent intermediate malignancy potential and such rapid progression has not been reported so far.

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    ABSTRACT: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival. A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols. Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival. AS histology was the most important negative prognostic factor for overall survival and event-free survival. Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis. Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses. Response to systemic treatment was poor (44%) and did not prevent local and distant relapses. The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS. RTX and delayed surgery should be performed more frequently and earlier in the disease course. An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy. The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.
    Annals of Surgical Oncology 03/2010; 17(7):1878-89. · 4.12 Impact Factor
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    ABSTRACT: Spindle cell hemangioendothelioma is a rare benign vascular tumor that is not known to involve the sacrum. The authors describe the case of a 31-year-old woman presenting with low-back and radicular pain without weakness or bowel or bladder dysfunction. Admission CT and MRI studies revealed a large S1-3 lytic sacral lesion. The patient initially underwent a nondiagnostic percutaneous biopsy. She subsequently underwent an open biopsy, during which the lesion was found to be highly vascular. Histological investigation revealed a vasoformative lesion consistent with spindle cell hemangioendothelioma. Preoperative embolization followed by resection via intralesional currettage resulted in resolution of symptoms up to 9 months postintervention. Despite the authors' recommendation, the patient became pregnant at that time and requested no additional follow-up imaging. The authors present the first reported case of a spindle cell hemangioendothelioma of the sacrum and review the current literature.
    Journal of neurosurgery. Spine 05/2014; · 1.61 Impact Factor
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    ABSTRACT: Epithelioid hemangioendothelioma is an extremely rare intracranial tumor and is regarded as a low-proliferation tumor. We present two cases of primary intracranial epithelioid hemangioendothelioma and give an overview of the English literature pertaining to this disease. We described two new cases of primary intracranial epithelioid hemangioendothelioma and performed a search of MEDLINE (PubMed) using the words "epithelioid hemangioendothelioma". Only cases in the English language that were intracranially located and contained clinical information pertinent to the analysis were included. The tumor in case 1 originated from the right temporal bone and invaded the surrounding cranium, dura and temple muscles. The tumor in case 2 was located in the petroclival bone and had also invaded the surrounding cranium. Both tumors were well vascularized. The tumors were totally (case 1) or subtotally (case 2) removed with moderate blood loss. A total of 36 cases of intracranial epithelioid hemangioendothelioma were found in the literature. The tumor was typically diagnosed in young adults and infants. There was no sex predominance in adult patients, while in children, males were more frequently affected (M/F ratio, 3.5:1). Surgical removal was the main therapeutic protocol, and adjuvant therapy included radiotherapy or chemotherapy. Including the 2 patients presented here, a total of 38 patients were analyzed: 32 % showed local invasion, 24 % suffered a recurrence, 15 % had metastases, and the mortality rate was 15 %. Intracranial epithelioid hemangioendothelioma is a rare, low-proliferation tumor, but it exhibits some clinically malignant behaviors, such as local invasion, recurrence and metastasis. Total resection is mandatory where possible, and radiotherapy and/or chemotherapy are otherwise required. Preoperative feeding-artery embolization is recommended.
    Journal of Neuro-Oncology 08/2012; 110(1):119-27. · 3.12 Impact Factor

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