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

Bone multicentric epithelioid hemangioendothelioma of the lower and upper extremities with pulmonary metastases: A case report
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下肢和上肢骨多中心上皮样血管内皮瘤伴肺转移:病例报告。

DOI:
10.3892/ol.2015.3018
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发表时间:
2015-05-01
期刊:
影响因子:
2.9
通讯作者:
Ye, Zhaoming
Ye, Zhaoming
中科院分区:
医学4区
文献类型:
--
作者:
Zhang, Hua;Fu, Yanbiao;Ye, Zhaoming

文献摘要

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本研究报告一罕见的骨多中心上皮样血管内皮瘤(EH),同时累及上肢和下肢,并累及肺部内脏。溶骨性病变首先在右侧股骨远端和胫骨近端被发现。在骨显像上观察到右肩关节的放射性核素摄取略有增加,然而,由于没有临床症状,这一点被忽略了。患者最初被误诊为多灶性软骨母细胞瘤,在胫骨近端和股骨内侧髁处进行了关节外病变刮除,并用骨水泥填充。在免疫组织化学分析提示EH后,组织病理学诊断得到纠正,随后,在大腿水平进行了右腿截肢。此外,在全身放射学检查中发现右肩关节多发性溶解病灶和肺转移瘤。右肩关节接受了放射治疗,然而,患者拒绝化疗或进一步手术。在最初的手术后15个月,患者目前仍然活着。这一病例表明,对这种疾病的临床特征有更好的认识可能会防止误诊和改善EH治疗。
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 patient 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 refused 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.