Multicentric Epithelioid Hemangioendothelioma Involving the Same Lower Extremity: A Case Report and Review of Literature

Liu, Qingjun; Miao, Jianyun; Lian, Kejian; Huang, Lianshui; Ding, Zhenqi

doi:10.7150/ijms.8.558

Cited by 16 publications

(25 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This results in an unpredictable, yet oftentimes, indolent clinical course, and patients can survive for decades even with Fig. 1 Axial, non-contrast, CT scan demonstrating a complex mass filling the right maxillary sinus with erosion of the medial (arrow) and posterior maxillary sinus walls (asterisk) multi-organ disease [11,12]. Mentzel et al [12] noted 20 % of tumors metastasized and 17 % of patients died in their study.…”

Section: Histologymentioning

confidence: 99%

Epithelioid Hemangioendothelioma of the Maxillary Sinus

Avadhani

Loftus

Meltzer

et al. 2015

Head and Neck Pathol

View full text Add to dashboard Cite

The clinical course and pathologic features of a 72 year old female who presented with epistaxis are presented. Radiographic findings were notable for a large, soft tissue lesion filling the maxillary sinus with significant bony erosion and expansion. The patient was ultimately diagnosed with epithelioid hemangioendothelioma (EHE) and underwent endoscopic resection. She has no evidence of local, regional or distant recurrence 14 months postsurgery. The rarity of this neoplasm, the unusual anatomic location and non-specific symptoms present diagnostic and management challenges. Epithelioid vascular tumors encompass a spectrum of benign and malignant tumors. EHE itself is thought to have an intermediate malignant behavior pattern, though cases with indolent behavior have been reported. Differentiation of EHE from other lesions has historically based on histopathology. Additionally, recent studies have described a recurrent genetic fusion WWTR1-CAMTA1 in EHE, involving t(1;3) (p36;q25). This represents the second reported case of EHE arising in a paranasal sinus. The histopathologic findings of this lesion are reviewed.

show abstract

Section: Histologymentioning

confidence: 99%

Epithelioid Hemangioendothelioma of the Maxillary Sinus

Avadhani

Loftus

Meltzer

et al. 2015

Head and Neck Pathol

View full text Add to dashboard Cite

show abstract

“…Mainly located in the long tubular bones of the lower extremities and pelvis, the tumor is rarely found in the upper extremities and flat bones (5)(6)(7)(8)(9). Therefore, the formation of a definitive diagnosis of EH is difficult and the tumor is easily misdiagnosed.…”

Section: Introductionmentioning

confidence: 98%

“…Previous studies have indicated that multifocal EH may exhibit a better prognosis than unifocal EH, however, the pathogenesis remains controversial and the number of cases reported is limited (8,16). Various mechanisms have been described, including metastatic spread, malignant transformation and multifocal formation.…”

Section: Case Reportmentioning

confidence: 99%

See 1 more Smart Citation

Bone multicentric epithelioid hemangioendothelioma of the lower and upper extremities with pulmonary metastases: A case report

Zhang

2015

Oncology Letters

View full text Add to dashboard Cite

Abstract. The present study reports a rare case of bone multicentric epithelioid hemangioendothelioma (EH) involving the upper and lower extremities simultaneously, with visceral involvement of the lung. Osteolytic lesions were first identified in the right distal femur and proximal tibia. Slight increased radionuclide uptake was observed in the right shoulder joint on bone scintigraphy, however, this was ignored, as no clinical symptoms were present. The pa tient was initially misdiagnosed with multifocal chondroblastoma, and an extra-articular curettage of lesions was performed in the proximal tibia and medial femoral condyle, which was filled with bone cement. The histopathological diagnosis was corrected post-operatively following immunohistochemical analysis, which indicated EH, and subsequently, an amputation of the right leg at thigh level was performed. In addition, multiple lytic lesions in the right shoulder joint and pulmonary metastases were identified on whole-body radiological examination. Radiotherapy was administered to the right shoulder joint, however, the patient re fused chemotherapy or further surgery. At 15 months after the initial surgery, the patient currently remains alive. This case indicates that an improved understanding with regard to the clinical features of this disease may prevent misdiagnosis and improve EH treatment.

show abstract

“…Therefore, some authors began to believe the hypothesis that EHE does not represent a distinct entity, but rather is an intermediate state of endothelial dedifferentiation with a highly variable and unpredictable prognosis [5]. …”

Section: Introductionmentioning

confidence: 99%

Pulmonary epithelioid hemangioendothelioma presenting with vertebral metastases: a case report

et al. 2014

View full text Add to dashboard Cite

IntroductionEpithelioid 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 presentationA 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 3,000cGy delivered in individual doses of 200cGy/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.ConclusionSince 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 3,000cGy delivered in individual doses 200cGy/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.

show abstract

Multicentric Epithelioid Hemangioendothelioma Involving the Same Lower Extremity: A Case Report and Review of Literature

Cited by 16 publications

References 29 publications

Epithelioid Hemangioendothelioma of the Maxillary Sinus

Epithelioid Hemangioendothelioma of the Maxillary Sinus

Bone multicentric epithelioid hemangioendothelioma of the lower and upper extremities with pulmonary metastases: A case report

Pulmonary epithelioid hemangioendothelioma presenting with vertebral metastases: a case report

Contact Info

Product

Resources

About