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盆腔复发性恶性孤立性纤维瘤:病例报告与治疗方法。

Recurrent malignant solitary fibrous tumor of pelvis: A case report and treatment approach.

机构信息

Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.

出版信息

Medicine (Baltimore). 2023 Aug 4;102(31):e34520. doi: 10.1097/MD.0000000000034520.

Abstract

RATIONALE

Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that typically arise from the pleura but can also occur in various locations throughout the body. Although there are increasingly more reports of extra-pleural SFTs, reports of SFTs in bone are very rare, and a malignant SFT accompanying recurrence of pelvis has not yet been reported.

PATIENT CONCERNS

We herein present the case of a 15-year-old male who experienced intermittent numbness in his right buttock, accompanied by radiating pain in his lower limbs for 6 months. Radiological examinations revealed an expansive, heterogeneous enhanced mass in the sacral and iliac regions, with a branch of the right internal iliac artery feeding the tumor.

DIAGNOSES

The histological examination suggest a diagnosis of a malignant SFT with high proliferation activity.

INTERVENTIONS

The sacral mass was surgically excised.

OUTCOMES

Following the surgery, the patient experienced a local recurrence of the tumor at 9 months and was administered adjuvant imatinib treatment. Recent magnetic resonance imaging contrast-enhanced displayed shrinkage of the tumor, which may provide certain evidence for chemotherapy for the treatment of recurrence of malignant SFTs in the pelvic region.

LESSONS

Complete surgical excision is the recommended treatment for this rare disease entity, and the role of adjuvant therapies is controversial due to their rarity. Our case underscores the challenges in managing recurrent malignant SFTs and highlights the importance of a thorough diagnostic workup. Further research is needed to establish the role of adjuvant therapies in the management of these tumors.

摘要

背景

孤立性纤维瘤(SFT)是一种罕见的间叶性肿瘤,通常起源于胸膜,但也可发生于全身各部位。尽管越来越多的报道涉及胸膜外 SFT,但骨骼中的 SFT 非常罕见,且伴随骨盆复发的恶性 SFT 尚未见报道。

病例报告

我们在此报告了 1 例 15 岁男性病例,其间断出现右侧臀部麻木,伴有下肢放射痛 6 个月。影像学检查显示骶骨和髂骨区域有一个膨胀性、异质性增强肿块,右侧髂内动脉的一支分支为肿瘤供血。

诊断

组织学检查提示为高增殖活性的恶性 SFT。

干预措施

手术切除骶骨肿块。

结果

术后 9 个月患者出现肿瘤局部复发,给予辅助伊马替尼治疗。最近的磁共振成像增强显示肿瘤缩小,这可能为化疗治疗骨盆区恶性 SFT 复发提供一定依据。

教训

对于这种罕见疾病实体,完整的手术切除是推荐的治疗方法,由于其罕见性,辅助治疗的作用存在争议。我们的病例强调了管理复发性恶性 SFT 的挑战,并强调了彻底诊断评估的重要性。需要进一步研究以确定辅助治疗在这些肿瘤管理中的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/006c/10403011/4bdc938d1ce2/medi-102-e34520-g001.jpg

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