Intracranial Epithelioid Hemangioendothelioma Research Articles

Surgical treatment of intracranial epithelioid hemangioendothelioma: a case report

Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular neoplasm with an intermediate pattern of malignancy between benign neoplasms and angiosarcomas. Only 46 cases of intracranial localization of primary EHE in adults have been published.Case report: A 47-year-old male patient developed pronounced speech disorder of sensory and amnestic aphasia type, right-sided pyramidal hemiparesis within 3–4 days. Magnetic resonance imaging revealed a delimited left sided brain islet neoplasm with signs of hemorrhage and small perifocal edema. Intraoperatively, the neoplasm was represented by a conglomerate of pathologic vessels, with involvement of the terminal branches of the M2 segment of the middle cerebral artery, which prevented radical resection of the neoplasm. Based on histopathological and immunohistochemistry analysis of the neoplasm (positive expression of CD31, CD34, proliferative activity index Ki-67 10%) the morphologic diagnosis of EHE was established. 3 months after surgery, continued growth of the residual part of the neoplasm was noted. Temozolomide chemotherapy was clinically ineffective. The patient died due to the development of dislocation syndrome after 9 months.Discussion. The malignant characteristics of EHE include invasive growth, recurrence, and metastasis, which is more common in intracranial localization of the neoplasm. If EHE is suspected, radical surgical removal of the neoplasm should be sought.

Epithelioid hemangioendothelioma of the liver and pregnancy (Literature review and clinical case)

The article is devoted to the analysis of scientific publications about the etiology, pathogenetic mechanisms, and management of the patients with epithelioid hemangioendotheliomas.Epithelioid hemangioendothelioma is the vascular tumor that affects liver, spleen, lungs, and various other organs and tissues. The tumor is malignant with high metastatic potential and variable course. The frequency of it is less than 0.1 per 100,000 population. The etiology is unknown. The role of the molecular and genetic mechanisms is studied, and today an association of the WWTR1-CAMTA1 protein in the development of epithelioid hemangioendothelioma was determined.Visual examination, computed tomography and magnetic resonance imaging, which are characterized by typical signs: «white target sign», «lollipop sign» are used for the diagnosis. There are no treatment standards for epithelioid hemangioendothelioma. Chemotherapy, radiofrequency ablation, surgery, and liver transplantation are usedOnly 8 cases of epithelioid hemangioendothelioma during pregnancy have been described in the medical literature, three cases of which are connected with one woman. Among them: progressive liver tumor with negative outcomes – the perinatal and maternal mortality; epithelioid hemangioendothelioma of the liver with the birth of a live, full-term baby; multifocal malignant hemangioendothelioma of the liver with premature birth by cesarean section and the development of sepsis in the mother; palate tumor involving the oropharynx and the birth of a child in physiological terms; presacral tumor, the diagnosis of which was confirmed after pregnancy; recurrent course of intracranial epithelioid hemangioendothelioma with liver and heart damage and three obstetric cases.The article describes a clinical case of epithelioid hemangioendothelioma of the liver in a pregnant woman with a history of resection of the left lobe of the liver. The management of this case with timely diagnosis, delivery at 35 weeks by cesarean section of the child in a satisfactory state is presented. The woman delivered a male infant weighing 2500 g, height 48 cm in satisfactory condition. Thromboprophylaxis was performed for 10 days, lactation was excluded.

Suprasellar epithelioid hemangioendothelioma: Case report and review of the literature.

Background:Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin, which is clinically and histologically intermediate between benign hemangioma and angiosarcoma. It is most commonly found in the liver, lung, and bone, however, 46 intracranial cases have been reported in the literature, of which this is the fifth reported suprasellar tumor.Case Description:A 45-year-old woman developed progressive lethargy, somnolence, and memory decline over the course of 6 months. On computed tomography (CT), she was found to have a large hypothalamic mass and underwent subtotal resection via a bifrontal craniotomy.Conclusions:While primary intracranial EHE is an uncommon presentation of a rare tumor, the suprasellar region does not seem to be an unusual location when it does occur. Prognosis is generally good, and may be better for primary intracranial disease than that for EHE originating elsewhere. Surgery is the first line of therapy, with variable benefit from adjuvant chemotherapy or radiation when total resection is not possible. Chemotherapeutic approaches in current use are directed at preventing endothelial proliferation.

Primary intracranial epithelioid hemangioendothelioma: a low-proliferation tumor exhibiting clinically malignant behavior

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.

Intracranial epithelioid hemangioendothelioma

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. 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.

Meningel intracranial epithelioid hemangioendothelioma: case report and literature review

Intracranial epithelioid hemangioendothelioma (EH) is a very rare vascular neoplasm. We describe a case of meningeal intracranial tumor in a 58 year old man, located in the greater wing of the sphenoid bone. The tumor was completely excised via pterional approach and the pahological analysis showed a hemorrhagic lesion with capillary-sized vessels lined by atypical-appearing endothelial cells that expressed CD31, CD34 and Factor VIII antigens. A diagnosis of EH was established, and one year after surgery the patient is symptom-free, without tumor recurrence or residual tumor. A review of the literature disclosed other 34 cases of intracranial EH. In these tumors, total resection is mandatory if possible, otherwise radiotherapy is recommended. Outcome is favorable in the majority of cases.

Intracranial epithelioid hemangioendothelioma arising at site of previously excised atypical meningioma

Epithelioid hemangioendothelioma is an uncommon vascular tumor which, in most cases, pursues a clinical course intermediate between hemangioma and angiosarcoma. Only four completely documented cases of central nervous system involvement by this tumor appear in the literature. We present an additional case, which is remarkable in its occurrence at the site of a previously excised atypical meningioma and in its unusually aggressive clinical course. To our knowledge this is the first report of intracranial epithelioid hemangioendothelioma with postmortem documentation. The patient initially presented with a 7 cm right post frontal tumor; an atypical meningioma was excised and removal appeared complete on immediate post-operative scans. Seven months later the patient re-presented with tumor recurrence at the previous operative site; a second craniotomy was performed and, on this occasion, the excised tumor had the histological features of an epithelioid hemangioendothelioma without evidence of meningioma: both morphologically and immunohistochemically the two tumors were quite distinctive. Further recurrence prompted radiotherapy but the patient continued to deteriorate and died five months later. Massive recurrence of intracranial tumor was found at postmortem examination.

Recurrent Intracranial Epithelioid Hemangioendothelioma Associated with Multicentric Disease of Liver and Heart

EPITHELIOID HEMANGIOENDOTHELIOMA IS an unusual vascular neoplasm with prominent cytoplasmic vacuolization representing primitive lumen formation. A case is presented of this unique vascular neoplasm in a woman with a seizure disorder who had cardiac, hepatic, and recurrent nervous system lesions. To our knowledge, this is the third known case of intracranial epithelioid hemangioendothelioma. Emphasis is placed on the indolent course of this rare neoplasm, with a recommendation for aggressive surgical treatment and diligent follow-up.