Treatment of lumbar vertebra epithelioid hemangioma with radiation therapy: a case report.

BACKGROUND: Although epithelioid hemangiomas involving bone have been described in previous case reports and case series, the effects of radiation therapy on vertebral epithelioid hemangioma has not been fully reported. Here we provide a case report of tumor response to radiation therapy in a young adult with a large epithelioid hemangioma involving the fourth lumbar vertebrae. CASE PRESENTATION: A 27-year-old Latino man with a past medical history of type 1 diabetes and a 3-year history of low back pain presented to a hospital emergency department following acute worsening of back pain. On transfer to our tertiary medical center, he described the pain as "shock-like" which originated at the lateral aspect of his right hip and radiated down to his right knee. Paresthesia was also reported along the medial aspect of his lower right leg. Imaging included a computed tomography scan and magnetic resonance imaging which revealed fourth lumbar and right iliac lytic bone lesions. Image-guided biopsies of the lytic lesions were consistent with a diagnosis of epithelioid hemangioma and radiation therapy was recommended as the primary treatment. Our patient's low back and leg pain were initially managed with acetaminophen, oxycodone, pregabalin, and lidocaine patch 5%. He noted improvement in pain after his third fraction of radiation. Pain intensity continued to decline and oxycodone was discontinued. CONCLUSIONS: This case report demonstrates an unusual etiology of back and leg pain in a young man and elucidates the palliative effects of radiation therapy for epithelioid hemangioma involving the lumbar spine.

[Epithelioid hemangioendothelioma of the spine: A case report]. / Hémangioendothéliome épithélioïde rachidien : à propos d'un cas.

Epithelioid hemangioendothelioma is a rare vascular tumour, with features between hemangiomas and angiosarcomas. Evolution remains mainly local but may also metastasize. Here, we report a case of a patient with a spinal epithelioid hemangioendothelioma. After a first resection, local recurrence required a new excision and external radiotherapy. It was achieved by helical TomoTherapy® with intensity modulation. During the follow-up, a second inoperable spinal location necessitated a new sequence of radiotherapy in modulation of intensity with integrated boost. The patient was alive at 24 months of follow-up without evidence of local relapse or distance. This observation is followed by a discussion with review of the literature on other epithelioid hemangioendothelioma cases treated with radiation.

A rare case of intravascular epithelioid hemangioendothelioma of the cephalic vein treated with surgery and postoperative radiation therapy: a case report and review of the literature.

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is a rare endothelial tumor with an intermediate grade of malignancy. Few cases of primary vascular hemangioendothelioma have been described in the literature. Surgery is the treatment of choice, but radiation therapy and chemotherapy should also be considered in particular cases. CASE PRESENTATION: We present the case of a 44-year-old Caucasian woman affected by EHE of the cephalic vein, treated by complete surgical removal of the mass and postoperative local radiation therapy. At 5-year follow-up, our patient is alive, with no signs of local or distant relapse and with no late radiation-related effects. CONCLUSIONS: Postoperative radiotherapy may play a role in cases in which tumor margins are close or cannot be assessed or when high-risk features are present.

Pulmonary epithelioid hemangioendothelioma presenting with vertebral metastases: a case report.

INTRODUCTION: Epithelioid hemangioendothelioma is a rare vascular tumor that has an epithelioid and histiocytoid appearance, originates from vascular endothelial or pre-endothelial cells and comprises less than 1% of all vascular tumors. It was described for the first time in 1975 as pulmonary epithelioid hemangioendothelioma, because initially it was believed to be an aggressive form of bronchoalveolar cell carcinoma with a remarkable propensity to invade adjacent blood vessels and small airways. Only a few cases have been reported in the literature to date. Tumor cells expressing Fli-1 and CD31 have been identified as relatively specific endothelial markers. Epithelioid hemangioendothelioma may affect multiple organs and may vary considerably in its clinical and radiological presentation. More than 50% to 76% of pulmonary epithelioid hemangioendothelioma patients are asymptomatic. They are usually incidentally diagnosed on the basis of abnormal chest radiography during routine physical examinations. Hematologic and gastrointestinal disorders and weakness or numbness may also be observed, in addition to respiratory symptoms, in cases of disseminated pulmonary epithelioid hemangioendothelioma. Pain and swelling, pathological fractures, spine compression or paresthesia, loss of muscular strength and paraplegia may be present when bone metastases occur. Because of the rarity of this disease, there is no standard for treatment. CASE PRESENTATION: A 46-year-old Caucasian woman presented to our institution in November 2009 with metastases of pulmonary epithelioid hemangioendothelioma from the L3 and L4 vertebrae. A course of radiotherapy at a dosage of 3,000 cGy delivered in individual doses of 200 cGy/day for 5 days/wk to the L3 and L4 vertebrae led to the disappearance of the patient's lumbar pain without any detectable side effects. Percussion of the patient's vertebral spine was negative, and no radiological progression of bone disease was found at her 1-year follow-up examination. CONCLUSION: Since epithelioid hemangioendothelioma was first correctly defined, several research groups have reported their experiences with epithelioid hemangioendothelioma irradiation. Further studies are needed to establish a standard radiation dose to be used for such a complex and extremely rare disease. In our present case, a radiotherapy dosage of 3,000 cGy delivered in individual doses 200 cGy/day for 5 days/wk allowed us to reach our goals: local pain control with good tolerance and better quality of life by the 1-year follow-up examination.

Cervical spine epithelioid hemangioendothelioma: case report.

UNLABELLED: Epithelioid Hemangioendothelioma (EHE) is a rare aggressive vascular tumor and can occur in almost all locations. Spine location is rare. There are just a few cases published in the literature and the longer series included only five cases. CASE REPORT: We report a case of a 58-year-old woman who presented with anemia and cervical pain without neurological deficit. Radiological diagnosis revealed a C2-C3 mass with vertebral artery involvement and liver metastases. Partial resection and arthrodesis followed by radiotherapy and chemotherapy were performed. Local control of the diseases was achieved but distance metastasis appears two years after surgery. CONCLUSIONS: EHE of the spine is extremely rare in clinic as a primary aggressive vascular tumor. Metastatic disease is the most important factor to predict prognosis. It is not clear in the literature which is the best surgical approach in this patients and it mainly depends of the location and systemic illness. En bloc resection or expanded resection supported with adjunct radiation therapy may present with acceptable results of local control of the tumor. Systemic disease control must be achieved with chemotherapy.

Epithelioid hemangioendothelioma of the spine treated with RapidArc volumetric-modulated radiotherapy.

Radiotherapy for epithelioid hemangioendothelioma (EHE) using volumetric intensity-modulated arc radiotherapy (VMAT). A 48-year-old woman was referred for curative irradiation of a vertebral EHE after failure of surgery. A comparison between VMAT and conventional conformal tridimensional (3D) dosimetry was performed and potential advantage of VMAT for sparing critical organs from irradiation's side effects was discussed. The total delivered dose on the planning target volume was 54 Gy in 27 fractions. The patient was finally treated with VMAT. The tolerance was excellent. There was no acute toxicity, including no increase in pain. With a follow-up of 18 months, no delayed toxicity was reported. The clinical response consisted of a decrease in the dorsal pain. The Dmax for the spinal cord was reduced from 55 Gy (3D-radiotherapy [RT]) (which would be an unacceptable dose to the spine because of the risk of myelopathy) to 42.8 Gy (VMAT), which remains below the recommended dose threshold (45 Gy). The dose delivered to 20% of organ volume (D20) was reduced from 47 Gy (3D-RT) to 3 Gy (VMAT) for the spinal cord. The study shows that VMAT allows the delivery of curative treatment for vertebral EHEs because of critical organ sparing.

Radiotherapy as an effective primary treatment for epithelioid haemangioendothelioma of the cervical spine.

Epithelioid haemangioendothelioma (EHE), first described in 1982, is an uncommon malignancy comprising of around 1% of all vascular tumors and accurate diagnosis relies on specialist review of histology. Cervical spinal primaries are very rare and standard treatment for localised disease is surgery. Data regarding management of localized but unresectable tumors are limited. We undertook a literature review in order to highlight its clinico-pathological features and we describe a first case of an unresectable cervical EHE, successfully treated with radical radiotherapy.

Selective intra-arterial Y-90 microsphere therapy in hemangioendothelioma.

Epithelioid hemangioendothelioma is a malignant soft tissue tumor originating from the endothelial cell. Its clinical course varies from benign hemangioma to angiosarcoma. The therapy procedure remains unclear. Although resection of the tumor is the most frequently suggested treatment, due to its multinodular type, transplantation is frequently performed. Therapy regimens other than transplantation and resection are not effective and the median survival in hemangioendothelioma with unresectable lesions is approximately eight months. In this case, we report the therapeutic effect of selective intra-arterial radionuclide yttrium (Y)-90 microspheres and the value of F-18 fluorodeoxyglucose positron emission tomography/computed tomography to evaluate therapy response in hemangioendothelioma.

F-18 FDG PET/CT monitoring of radiation therapeutic effect in hepatic epithelioid hemangioendothelioma.

We report the F-18 FDG PET/CT findings in a 59-year-old man with a rare hepatic tumor of malignant epithelioid hemangioendothelioma, who was treated with radiotherapy (RT). This patient had multifocal discrete hepatic tumors, and PET/CT images showed intense FDG uptake in these nodules, regardless of poor enhancement on contrast-enhanced computed tomography. Seven months after RT to the 2 relatively large nodules, the intense FDG uptake disappeared in these nodules, while the remaining untreated nodule showed persistently intense uptake. RT is one of the options for treatment of hepatic epithelioid hemangioendothelioma. F-18 FDG PET/computed tomography appears to be useful for monitoring the effect of RT in this neoplasm.

Epithelioid hemangioendothelioma mimicking a nerve sheath tumor clinically and on MR imaging.

Epithelioid hemangioendothelioma is a tumor of the soft tissues arising from the vascular endothelium. It is considered an intermediate grade malignancy. A 42-year-old female patient presented with pain and tingling down her right arm and a mass at the right medial upper extremity. MRI revealed an oblong mass along the course of the neurovascular bundle. Given the clinical and MR findings, a nerve sheath tumor was suspected. At surgery, the mass was adherent to both the brachial artery medially and the median nerve posteriorly. Pathology revealed epithelioid hemangioendothelioma. The imaging characteristics of epithelioid hemangioendothelioma on ultrasound, CT, and MRI are reviewed. Epithelioid hemangioendothelioma can mimic a nerve sheath tumor clinically and radiologically and should be considered in the differential diagnosis of tumors involving or adjacent to a neurovascular bundle.

Successful removal after radiotherapy and vascular embolization in a huge tentorial epithelioid hemangioendothelioma: a case report.

Intracranial epithelioid hemangioendothelioma (E-HE) is an extremely rare, vasoformative tumor with variable biological behavior. A huge E-HE confined to the upper side of the tentorium has not been documented previously. Recognizing E-HE as a specific vascular tumor is important, and complete tumor removal guarantees long-term survival. Here, we report an intracranial E-HE of a 24-year-old woman with the initial symptoms of progressive headache over several years. Magnetic resonance imaging revealed a huge tumor attached to the upper side of the right tentorium, with tentorial and tonsillar herniation, and secondary syringomyelia in the upper cervical spinal cord. The initial surgical attempt to remove the lesion was abandoned due to profuse bleeding from the tumor. One year after irradiation and 5 days after vascular embolization, the tumor could be removed totally. The final definitive diagnosis as E-HE was made by special immunohistochemical and electron microscopic study. The patient is free from the tumor 9 years after the second operation. Thus, E-HE is a highly vascular-rich tumor to excise, and we stress need for preoperative embolization and/or irradiation to remove huge tumor completely.

Mediastinal epithelioid hemangioendothelioma in a patient with type IV Ehlers-Danlos syndrome: a case report and review of the literature.

Ehlers-Danlos syndrome (EDS) is a collection of inherited connective tissue disorders with at least 10 types, differentiated on clinical and genetic grounds. Malignancy has been described only rarely in association with the syndrome. Epithelioid hemangioendothelioma (EH) is a rare endothelial tumor, which displays clinical behavior intermediate between that of hemangioma and angiosarcoma. A case report of a 50-year-old man with type IV EDS who was extensively investigated for several years for multiple mediastinal nerve palsies and chest pain. Magnetic resonance imaging (MRI) demonstrated an anterior mediastinal mass, which at biopsy showed EH. Subsequent metastatic spread to liver and lungs is unique among reported cases of mediastinal EH. The patient experienced significant symptomatic improvement from external beam radiotherapy (RT) to the mediastinum. After metastatic disease developed, multiagent chemotherapy was administered, but without response. The literature is reviewed regarding treatment of EH and the potential problems associated with EDS. Although there appears to be no etiological association between EDS and EH, the connective tissue disease clearly contributed to a delay in diagnosis and raised concerns regarding RT tolerance. The potential predisposition to aggressive tumor invasion remains a possibility. In addition, mediastinal EH has the potential to metastasize, and in this case demonstrated resistance to a broad range of chemotherapy agents.