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冬眠瘤:一例罕见心脏肿瘤的病例报告

Hibernoma: a case report of a rare cardiac tumour.

作者信息

Cistulli David, Othman Farrah, Karim Rooshdiya, Puranik Rajesh

机构信息

Royal Prince Alfred Hospital, 50 Missenden Road, Camperdown, NSW, Australia.

New South Wales Health Pathology, Australia.

出版信息

Eur Heart J Case Rep. 2023 Dec 26;8(1):ytad612. doi: 10.1093/ehjcr/ytad612. eCollection 2024 Jan.

Abstract

BACKGROUND

A cardiac hibernoma is a rare phenomenon, with just a handful of reports in the literature. They are difficult to characterize with conventional imaging including echocardiography, computed tomography (CT), cardiac magnetic resonance (CMR), or positron emission tomography (PET). Their definitive diagnosis relies primarily on histopathology via either endovascular or surgical biopsy. Previous case reports have entailed surgical excision followed by histopathology; however, surgery may be unfavourable in some patients with increased perioperative risk.

CASE SUMMARY

We present the case of a 57-year-old woman who was referred to our cardiology service with an interatrial lipomatous mass found incidentally on chest CT for assessment of rib fractures. She had 6 months of unexplained syncope, which was attributed to superior vena cava (SVC) compression demonstrated by chest CT. The mass had benign characteristics on echocardiography, CT, and CMR but was glucose-avid on PET, which indicated a possible malignancy such as liposarcoma. Her comorbid and very significant airways disease precluded her from surgical excision, so instead, endovascular biopsy was performed. Histopathology showed brown fat which was negative for mouse double minute 2 amplification on fluorescence hybridisation testing; hence, a diagnosis was made of hibernoma, a rare benign tumour of brown fat. Given the benign diagnosis and her surgical risk with severe chronic obstructive pulmonary disease, a multidisciplinary recommendation was made favouring conservative management, with careful ongoing follow-up and the consideration of SVC stenting if symptoms progressed.

DISCUSSION

The definitive diagnosis of a cardiac hibernoma is complex and relies heavily on histopathology due to the contradictory findings on chest imaging. Careful consideration of management within a multidisciplinary team setting is essential to achieve a successful outcome.

摘要

背景

心脏冬眠瘤是一种罕见现象,文献中仅有少数报道。它们难以通过包括超声心动图、计算机断层扫描(CT)、心脏磁共振(CMR)或正电子发射断层扫描(PET)在内的传统成像进行特征性描述。其明确诊断主要依靠通过血管内或手术活检的组织病理学检查。既往病例报告均为手术切除后进行组织病理学检查;然而,手术对于一些围手术期风险增加的患者可能并不适宜。

病例摘要

我们报告一例57岁女性,因胸部CT偶然发现房间隔脂肪瘤性肿块以评估肋骨骨折而转诊至我们的心脏病科。她有6个月原因不明的晕厥,胸部CT显示这是由上腔静脉(SVC)受压所致。该肿块在超声心动图、CT和CMR上具有良性特征,但在PET上表现为葡萄糖摄取,提示可能为恶性肿瘤,如脂肪肉瘤。她合并严重的气道疾病,无法进行手术切除,因此改为进行血管内活检。组织病理学检查显示为棕色脂肪,荧光杂交检测显示小鼠双微体2扩增为阴性;因此,诊断为冬眠瘤,一种罕见的棕色脂肪良性肿瘤。鉴于诊断为良性且她因严重慢性阻塞性肺疾病存在手术风险,多学科团队建议采取保守治疗,密切持续随访,并在症状进展时考虑进行SVC支架置入术。

讨论

由于胸部影像学检查结果相互矛盾,心脏冬眠瘤的明确诊断较为复杂,且严重依赖组织病理学检查。在多学科团队环境下仔细考虑治疗方案对于取得成功的治疗结果至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a3c4/10790811/af322874a160/ytad612il2.jpg

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