Hemangioendothelioma of the skull: A case report (original) (raw)

A Rare Case of a Giant Hemangioendothelioma of Neck

Bengal Journal of Otolaryngology and Head Neck Surgery, 2015

Introduction Hemangioendothelioma is a rare variety of vascular soft tissue neoplasm, classified as a borderline tumour with diverse modality of clinical presentation. It is seen mostly at sites like the liver, lungs and bones while very few head and neck cases have been reported. Case Report A rare case of an unusually large tumour mass presenting as an anterolateral neck mass with extensions to right parapharyngeal space, posterior triangle and prevertebral space is presented here. The entire tumour was removed en-masse surgically. Histopathological examination confirmed the mass to be an epitheloid hemangioendothelioma, the enormous size of which in the neck makes it unique and unusual.

Multiple epithelioid hemangioedothelioma of the skull in a child: A case report

Medicine, 2016

Epithelioid hemangioedothelioma (EHE) is a rare vascular tumor characterized by neoplastic proliferation of epithelioid or histiocytoid epithelial cells. EHE of bone constitutes <1% of primary malignant bone tumor. EHE in the skull is an extremely rare case. Here, we report a case of multiple neoplasm of the skull in a 15-month-old boy who presented with gradual facial swelling for 2 months. On computed tomography (CT) scan, multiple irregular osteolytic lesions were seen on the right maxillary, sphenoid, left zygoma, and roof of the left orbit. Excisional surgery of the lesion was planned. Histopathological and immunohistochemical examination of excised specimen suggest it to be epithelioid hemangioedothelioma. Follow-up for 6 months showed no recurrence. Epithelioid hemangioedothelioma is a locally aggressive tumor with metastatic potential. CT imaging could help in assessment of lesion, but final diagnosis is possible only with histopathology. Complete surgical resection at th...

Aggressive intracranial and extra-cranial epithelioid hemangioendothelioma: A case report and review of the literature

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.

Hemangioendothelioma of soft tissue: Cytological dilemma in two cases at unusual sites

Journal of Cytology, 2012

Hemangioendothelioma is a rare vascular tumor of intermediate malignancy. Cytologically, it can simulate a non-vascular malignant tumor. We report two cases of this tumor, which were misdiagnosed at cytology. In the first case, a 27-year-old man presented with an anterior abdominal wall tumor. Fine needle aspiration cytology (FNAC) of the tumor showed polygonal cells with vacuolated cytoplasm in clusters having moderate nuclear atypia in a background of necrosis. A diagnosis of metastatic carcinoma was made. The histological examination showed features of epithelioid hemangioendothelioma. In the second case, a 13-year-old female child presented with unilateral enlargement of the right tonsil. At ultrasound-guided FNAC, a diagnosis of, 'small round cell tumor, could be consistent with alveolar rhabdomyosarcoma,' was made. The histological examination showed features of papillary intralymphatic angioendothelioma (Dabska's tumor). We conclude that epithelioid hemangioendothelioma should be considered in the differential diagnosis of metastatic carcinoma and small round cell tumor even at unusual sites.

Epithelioid Hemangioendothelioma of the Infundibular–Hypothalamic Region: Case Report and Literature Review

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.

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.

Osseous hemangiopericytomas of unsuspected intracranial origin

Skeletal Radiology, 1998

Two cases of osseous hemangiopericytoma are presented that were initially diagnosed as primary in origin, but later reclassified as metastases, after a history of resection for an intracranial tumor was discovered. An intracranial source should be excluded before an isolated osseous tumor is determined to be a primary hemangiopericytoma.

A Rare Case of Intraosseous Papillary Hemangioma of the Head and Neck

Objectives Papillary hemangioma (PH) is a novel variant of intravascular capillary 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 PH involving the frontal bone. Methods Brain imaging in a 69-year-old male with a slowly enlarging swelling on the right frontal area following an accidental fall demonstrated a 4.5 cm x 1.7 cm x 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. Results 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 and vimentin. AE1/AE3, EMA, PR, D2-40, inhibin and S100 stains were negative. Ki-6...