Epithelioid Hemangioendothelioma of the Mastoid: Resection for Recurrence and Adjuvant Radiation with 8-Year Followup

Drazin, Doniel; Gandhi, Ravi; Slodkowska, Elzbieta; Boulos, Alan S.

doi:10.1155/2013/469201

Cited by 15 publications

(13 citation statements)

References 22 publications

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“… 12-14 , 20 Table 3 summarizes the clinical aspects of EHE localized at other sites, different from lung: the clinical presentation of this rare vascular tumor is as heterogeneous as its clinical localization can be. 2 , 10 , 47 , 49 , 51 , 53-58 , 60-69 In particular, when bone metastases of EHE involve more than 50% of the cortex, there is a serious risk for pathological fractures, and if they arise in vertebrae they might cause spine compression and therefore paresthesia, loss of muscular strength and paraplegia. 57 , 60 , 61 Hemolytic anemia and consumption coagulopathy have been rarely described.…”

Section: Clinical Aspectsmentioning

confidence: 99%

Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

Sardaro¹,

Bardoscia²,

Petruzzelli³

et al. 2014

Oncol Rev

207

315

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Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease.

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Section: Clinical Aspectsmentioning

confidence: 99%

Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

Sardaro¹,

Bardoscia²,

Petruzzelli³

et al. 2014

Oncol Rev

207

315

View full text Add to dashboard Cite

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“…Adjuvant radiotherapy is used to control residual disease for patients with localized epithelioid hemangioendothelioma. Chemotherapy with interferon-2α or carboplatin plus etoposide is the preferred therapy for patients with widespread disease, but the benefits are unclear [ 4 , 13 , 14 ].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary epithelioid hemangioendothelioma misdiagnosed as a benign nodule

et al. 2015

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Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of borderline malignancy that originates from endothelial cells. Chest computed tomography (CT) performed during a routine cancer screening revealed multiple small pulmonary nodules in a 50-year-old man who had previously undergone endoscopic submucosal dissection of early gastric cancer. To rule out metastatic nodules, a wedge resection of the left upper lobe was performed and the frozen biopsy reported a benign fibrotic nodule. Using immunohistochemistry, the final pathology was indicated to be PEH, and consecutive surgery for the right-side nodules was planned and performed.

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“…Intensity modulated radiation therapy (IMRT) has been used to avoid radiation exposure to normal surrounding tissues 14. Drazin et al 13 used an adjuvant dose of 33 fractions of a total dose of 5940 cGy for a mastoid EHE with 8-year disease free survival. Yim et al 15 used a dose of 55 Gy in 32 fractions over 43 days to achieve remission for an unresectable cervical EHE.…”

Section: Discussionmentioning

confidence: 99%

A trimodality approach in the management of metastatic low-grade epithelioid hemangioendothelioma of the bone

et al. 2015

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SUMMARYA 29-year-old man presented with a 2-week history of severe pain in the left foot with no preceding history of trauma. A left foot radiograph demonstrated a cortical lucency in the mid-distal shaft of the third metatarsal bone. MRI of the left foot showed an expansile lesion in the same location. A staging bone scan showed a focal uptake in the known lucency in the left third metatarsal and in the proximal left femur. A subsequent left hip radiograph demonstrated a lucency in the intertrochanteric region. CT scan of the chest, abdomen and pelvis was unremarkable. A biopsy of the left third metatarsal expansile lesion performed during an incision and curettage procedure revealed an epithelioid haemangioma (EHE) of the bone. MRI of the left hip performed in response to the findings on the bone scan showed metastatic disease in the left intertrochanteric region. A prophylactic left hip fixation surgery with an interlocking intramedullary femoral nail was therefore undertaken to avoid a pathological fracture of the left hip from the metastatic disease. Simultaneously, a left hip biopsy was performed, which also revealed an EHE. The patient underwent external beam radiation to the left femoral head and neck. This was followed by fractionated radiosurgery to the left third metatarsal. Once the left foot wound had healed, the patient subsequently received four cycles of doxorubicin and ifosfamide. A restaging positron emission tomography CT carried out after completion of therapy showed no metabolic evidence of residual primary tumour or metastasis. More than 2 years after completing his trimodality therapy, the patient remains fully functional and symptom free. BACKGROUND

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Epithelioid Hemangioendothelioma of the Mastoid: Resection for Recurrence and Adjuvant Radiation with 8-Year Followup

Cited by 15 publications

References 22 publications

Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

Pulmonary epithelioid hemangioendothelioma misdiagnosed as a benign nodule

A trimodality approach in the management of metastatic low-grade epithelioid hemangioendothelioma of the bone

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