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一例罕见的左臀部上皮样血管内皮瘤病例报告:诊断与治疗挑战

A rare case report of epithelioid hemangioendothelioma of the left buttock: Challenges in diagnosis and management.

作者信息

Choi Jong Yun, Park Hee Yang, Moon Suk-Ho, Oh Deuk Young, Jun Young-Joon, Choi Jangyoun

机构信息

Department of Plastic and Reconstructive Surgery, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, South Korea.

出版信息

Medicine (Baltimore). 2025 Aug 29;104(35):e44137. doi: 10.1097/MD.0000000000044137.

Abstract

RATIONALE

Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy which poses significant diagnostic and therapeutic challenges due to its variable course and lack of standardized treatment protocols. In this case, the lesion's benign appearance and ambiguous histology delayed definitive diagnosis.

PATIENT CONCERNS

A 46-year-old woman presented with a recurrent soft tissue mass in the left buttock. Initial impression with partial biopsy favored a benign neoplasm, complicating the diagnosis.

DIAGNOSES

Histopathological examination confirmed EHE, characterized by epithelioid endothelial cells and positive immunohistochemical staining for vascular markers.

INTERVENTIONS

Complete surgical resection was performed, with no adjuvant therapy administered postoperatively.

OUTCOMES

Surveillance over 24 months revealed no evidence of local recurrence or distant metastasis. Follow-up computed tomography on 18 month revealed no evidence of recurrence.

LESSONS

Comprehensive histopathological evaluation are critical for proper diagnosis of EHE. Surgical resection is the first line treatment for localized EHE. Long-term monitoring is essential due to the unpredictable metastatic potential.

摘要

理论依据

上皮样血管内皮瘤(EHE)是一种罕见的血管恶性肿瘤,由于其病程多变且缺乏标准化治疗方案,给诊断和治疗带来了重大挑战。在本病例中,病变的良性外观和不明确的组织学表现延误了明确诊断。

患者情况

一名46岁女性,左臀部出现复发性软组织肿块。初步印象及部分活检结果倾向于良性肿瘤,使诊断变得复杂。

诊断

组织病理学检查确诊为EHE,其特征为上皮样内皮细胞以及血管标志物免疫组化染色阳性。

干预措施

进行了完整的手术切除,术后未给予辅助治疗。

结果

24个月的随访未发现局部复发或远处转移的证据。18个月时的随访计算机断层扫描未发现复发迹象。

经验教训

全面的组织病理学评估对于EHE的正确诊断至关重要。手术切除是局限性EHE的一线治疗方法。由于转移潜力不可预测,长期监测必不可少。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6659/12401398/0d3c02f3cf32/medi-104-e44137-g001.jpg

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