Primary epithelioid hemangioendothelioma in the clival region: A case report and literature review (original) (raw)
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Acta Clinica Croatica, 2018
-Epithelioid hemangioendothelioma is a rare vascular brain tumor. It develops from endothelial cells, usually in the liver, lung, bone and soft tissue. Primary localization of this tumor in the intracranial space is very uncommon; only 47 cases have been described in the literature. Th is tumor was initially classifi ed as grade I (benign) in the World Health Organization (WHO) 2007 classifi cation. In 2016, this tumor was re-classifi ed as grade III (malignant). Herein, the fi rst case report of epithelioid hemangioendothelioma in the cerebellum of a male patient is presented. Complete surgical excision was done. No adjuvant therapy was administered. Magnetic resonance imaging performed 2 years after the surgery continued to show no recurrence of the tumor. To our knowledge, this is the fi rst report of cerebellar location of this rare tumor. In addition, the authors report drastic re-classifi cation of the epithelioid hemangioendothelioma from the benign tumor (WHO 2007) to a malignant one (2016), which signifi cantly changes postoperative management and follow up of this brain neoplasm.
Neuropathology, 2006
Epithelioid hemangioendothelioma is a rare tumor of intermediate malignancy. Twelve intracranial cases have been well documented. We report an intra-and extracranial single mass epithelioid hemangioendothelioma. We describe the case of a 27-year-old male who presented a left temporal and retroauricular pain and an intra and extracranial mass extending to the left infratemporal fossa. The neoplasm was isointense with gray matter in both T1 and T2-weighted images, showing marked nodular gadolinium enhancement. About 90% of the tumor was surgically removed. The neoplasm was fibrous, well defined and arose from the left temporal artery branches. Recovery was uneventful. The histological diagnosis was epithelioid hemangioendothelioma. Enhanced magnetic resonance scan was repeated at 3 and 6 months after surgery, showing an increasing mass volume extending to the surrounding tissues, including intracranially, infratemporal fossa and left orbit. The patient died 8 months after the initial diagnosis. Epithelioid hemangioendotheliomas can be very aggressive tumors, presenting initially at an intra-and extra-cranial location, due to its fast growth. From our review of the literature, they seem to have an equal gender distribution (M : F = 7 : 6) and tend to affect people under the age of 30 (10/13). We report a unique and extremely aggressive tumor. The rarity of reports and the continuous spectrum of differentiation, ranging from borderline to highly malignant are obstacles to identifying initial therapeutic protocols and the adjunctive therapy after surgery.
Epithelioid hemangioendothelioma of the craniocervical junction; case report and review
ACTA ORTHOPAEDICA et TRAUMATOLOGICA TURCICA, 2016
Epithelioid hemangioendotheliomas are uncommon vascular neoplasms and their spinal location is even rarer. We report clinical course of a 31-year-old man with an epithelioid hemangioendothelioma at the cranio-cervical junction. A cervical magnetic resonance imaging revealed tumor that caused posterior cervical cord compression. C1,2,3 total laminectomy and surgical excision of the tumor was performed. Postoperative external beam radiation was performed on the surgical field especially around the right vertebral artery. At 2-year follow-up there was no neurological deficit and no tumor recurrence.
Journal of Neuro-Oncology, 2000
Epithelioid hemangioendothelioma (EHE) is an uncommon vascular neoplasm. Its intracranial occurrence is rare, and the literature review revealed only 23 cases (14 adults and 9 children). To our knowledge, this is the first case of EHE arising in the infundibular-hypothalamic region. A 53-year-old man presented with headaches and loss of libido. Magnetic resonance imaging revealed a supra-sellar mass, which homogeneously absorbed the contrast agent. A trans-sphenoidal craniotomy and a biopsy were performed. Adjuvant radiotherapy was administered due to intolerance to interferon. Epithelioid hemangioendothelioma is a hemorrhagic tumor. Total resection is mandatory where possible, otherwise radiotherapy seems necessary. The prognosis of intracranial location has not yet been well defined, despite the favorable outcome noted in the majority of cases.
Aggressive multifocal form of epithelioid hemangioendothelioma--case report
Collegium antropologicum, 2011
Epithelioid hemangioendothelioma (EHE) is a rare tumor of the vascular origin. It was first described in its pulmonary form by Dail and Leibow in 1975 and named "intravascular bronchioalveolar tumor" (IVBAT). Since then, reports of occurrences of the tumor have been made for number of locations, but most often tumor can be found in soft tissues, liver, lungs, bone and skin. It is considered to be a low or borderline malignant tumor with, usually, slow progression, but aggressive forms have been described. We here report a case of a 46-year old female patient with multifocal malignant tumor spreading to lungs, liver, spleen and with synchronous involvement of lumbal vertebrae, illiac bones and central nervous system dissemination. To the best of the authors knowledge, no case of malignant EHE with multiorgan involvement of this proportions and synchronous central nervous system and bone involvement in one patient has been reported to this date in English-speaking literature.
An unusual case of aggressive malignant spread of epithelioid hemangioendothelioma
Rare Tumors, 2021
Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm which typically originates from liver, lung, or bone. Due to the low incidence of disease, the most effective treatment is not easily studied and much of the information known about EHE has been learned through case reports and case series. In this case, we will present an uncommon form of primary soft tissue EHE with local recurrence, bone metastasis, and lymphangitic spread to the lungs leading to respiratory failure. Imaging of the chest was atypical for EHE with intraseptal thickening and hilar lymphadenopathy. Respiratory failure was progressive despite aggressive multimodal treatment. This case highlights an unusually aggressive recurrence and metastasis of primary soft tissue EHE with atypical pulmonary imaging findings.
Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma
Oman Medical Journal, 2015
pithelioid hemangioendothelioma (EHE) is a rare vascular tumor with an estimated prevalence of less than one in one million. It has an intermediate malignant potential and clinically behaves in an intermediate way between a benign hemangioma and a highly malignant angiosarcoma. It usually involves the liver, bone, lungs, skin, and other soft tissue. 1,2 Single organ involvement is more common than multi-organ involvement (64%). 2 Together the liver and lung (51%) were the most commonly involved organs in those who presented with multi-organ disease, as reported in a series of 264 patients of EHE. 2 The distribution of EHE involving soft tissue as described in a series of 49 cases are extremities (n=32), head and neck (n=6), mediastinum and trunk (n=4, each), genitals (n=2), and the retroperitoneum (n=1).
ESMO Open, 2021
Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.