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顶骨血管外皮细胞瘤/孤立性纤维性肿瘤:一例报告

Hemangiopericytoma/Solitary Fibrous Tumor of the Parietal Bone: A Case Report.

作者信息

Neto Ana Rita Dias, Leite Regina, Rothwell David, Roda Domingos, Sousa Claudia, Henriques Mónica, Alves Paula

机构信息

Department of Radiotherapy, Portuguese Institute of Oncology of Coimbra, Coimbra, Portugal.

Department of Radiotherapy, Algarve Radiation Oncology Unit - Joaquim Chaves Saúde (JCS), Faro, Portugal.

出版信息

Case Rep Oncol. 2022 Nov 10;15(3):1021-1026. doi: 10.1159/000525724. eCollection 2022 Sep-Dec.

Abstract

Hemangiopericytoma/solitary fibrous tumor (HPC/STF) is a rare tumor arising from Zimmerman's pericytes and it is characterized by an aggressive malignancy, with a high tendency for local and distant recurrence. The authors report the case of a middle-aged woman with HPC/SFT of the right parietal bone, which is an extremely rare primary location of involvement. The patient presented with a painful deformity of insidious growth at the right parietal region. Assessment with cranial computed tomography scan and magnetic resonance imaging revealed an expansive lesion at the right parietal bone, with exocranial extension and 27 mm of maximal diameter. Craniotomy with gross tumor removal, duraplasty, and cranioplasty was performed, and the diagnosis of HPC/SFT, WHO grade III, was established by pathological and immunohistochemical analysis. The patient was then evaluated for adjuvant radiation therapy and received a dose of 60 Gy (2 Gy/fraction) with 3D conformal radiotherapy to the surgical bed. The adjuvant treatment was uneventful and, after 8 months of follow-up, there was no suspected local or distant recurrence. The rarity of this diagnosis, its aggressive behavior, and the lack of published data posed several challenges for the treatment management.

摘要

血管外皮细胞瘤/孤立性纤维性肿瘤(HPC/STF)是一种起源于齐默尔曼周细胞的罕见肿瘤,其特征为具有侵袭性恶性,局部和远处复发倾向高。作者报告了一例右顶骨发生HPC/SFT的中年女性病例,这是极其罕见的原发受累部位。患者表现为右顶区隐匿性生长的疼痛性畸形。头颅计算机断层扫描和磁共振成像评估显示右顶骨有一个膨胀性病变,向颅外扩展,最大直径为27毫米。进行了开颅肿瘤全切、硬脑膜成形术和颅骨成形术,通过病理和免疫组化分析确诊为WHO III级HPC/SFT。然后对患者进行辅助放疗评估,并对手术床给予60 Gy(2 Gy/分次)的三维适形放疗剂量。辅助治疗过程顺利,随访8个月后,未发现可疑的局部或远处复发。这种诊断的罕见性、其侵袭性行为以及缺乏已发表的数据给治疗管理带来了诸多挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efea/9830289/555e39e257fd/cro-0015-1021-g01.jpg

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