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一名36岁复发性左侧胸腔积液女性:纵隔淋巴管瘤罕见病例

A 36-Year-Old Female with Recurrent Left Sided Pleural Effusion: A Rare Case of Mediastinal Lymphangioma.

作者信息

Swarnakar Rajesh N, Hazarey Jetendra D, Dhoble Chetan, Vaghani Bhavesh, Ainsley Alaine S, Khargie James F, Likaj Lorena

机构信息

Department of Pulmonology, Getwell Hospital and Research Institute, Nagpur, India.

Department of Thoracic Surgery, Getwell Hospital and Research Institute, Nagpur, India.

出版信息

Am J Case Rep. 2016 Oct 28;17:799-804. doi: 10.12659/ajcr.895258.

Abstract

BACKGROUND Lymphangioma is an atypical non-malignant, lymphatic lesion that is congenital in origin. Lymphangioma is most frequently observed in the head and neck, but can occur at any location in the body. About 65% of lymphangiomas are apparent at birth, while 80-90% are diagnosed by two years of age. Occurrence in adults is rare, as evidenced by less than 100 cases of adult lymphangiomas reported in the literature. CASE REPORT A 36-year-old Indian woman with a medical history of recurrent pleural effusions presented with chief complaints of dyspnea on exertion for one year and a low-grade fever for one month. A thorax CT revealed left-sided pleural effusion with thin internal septations. Thoracoscopy revealed a large cystic lesion arising from the mediastinum from the hilum surrounding the mediastinal great vessels. The diagnosis of lymphangioma was confirmed via histopathologic examination of the cyst. It was managed with partial cystectomy along with the use of a sclerosing agent (talc). CONCLUSIONS The size and location of lymphangiomas can vary, with some patients presenting with serious problems like respiratory distress, while others may be asymptomatic. Complete cyst resection is the gold standard treatment for mediastinal cystic lymphangioma. Partial cyst resection along with the use of sclerosing agents can be an effective option when complete cystectomy is not possible. Although lymphangioma is a rare patient condition, it should be included in the differentials for patients presenting with pleural effusions. Also, a biopsy should be done at the earliest opportunity to differentiate lymphangioma from other mediastinal malignant tumors.

摘要

背景

淋巴管瘤是一种非典型的、非恶性的先天性淋巴病变。淋巴管瘤最常发生于头颈部,但也可出现在身体的任何部位。约65%的淋巴管瘤在出生时即可发现,80 - 90%在两岁前被诊断。成人发病较为罕见,文献报道的成人淋巴管瘤病例不足100例。

病例报告

一名36岁的印度女性,有复发性胸腔积液病史,主要症状为活动时呼吸困难1年,低热1个月。胸部CT显示左侧胸腔积液伴内部薄分隔。胸腔镜检查发现一个大的囊性病变,起源于纵隔,位于围绕纵隔大血管的肺门处。通过对囊肿的组织病理学检查确诊为淋巴管瘤。采用部分囊肿切除术并使用硬化剂(滑石粉)进行治疗。

结论

淋巴管瘤的大小和位置各不相同,一些患者会出现如呼吸窘迫等严重问题,而另一些患者可能无症状。完整囊肿切除术是纵隔囊性淋巴管瘤的金标准治疗方法。当无法进行完整囊肿切除时,部分囊肿切除并使用硬化剂可能是一种有效的选择。尽管淋巴管瘤是一种罕见的病症,但对于出现胸腔积液的患者,应将其纳入鉴别诊断范围。此外,应尽早进行活检,以将淋巴管瘤与其他纵隔恶性肿瘤区分开来。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d85/5087561/839601ac21be/amjcaserep-17-799-g001.jpg

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