Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue: A rare case report.
Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue: A rare case report.
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累及肺、肺外淋巴结及周围软组织的淋巴管瘤病罕见病例报告
DOI：
10.1097/md.0000000000009032
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发表时间：
2017-12
期刊：
影响因子：
1.6
通讯作者：
Yi X
中科院分区：
文献类型：
--
作者：
Fang X;Huang Z;Zeng Y;Zhu X;Wang S;Yu X;Li X;Wu C;Yi X
弥漫性肺淋巴管瘤病（DPL）主要累及肺和胸膜。很少有肺损伤伴有肺外淋巴结和周围软组织弥漫性受累的病理报告。肺内和肺外病变共存的临床病理意义尚不清楚。在这里，我们报告一个16岁的男性患者。对锁骨上淋巴结、软组织及肺活检标本进行病理学观察和免疫组化染色。回顾文献并讨论临床和影像学结果。患者咳嗽咳痰1年，间断咯血4个月。超声显示双侧颈部、左腋窝和耻骨联合淋巴结肿大。胸部CT扫描显示弥漫性网格状和线状阴影，双侧胸膜增厚，结节形成。多发性肿大淋巴结主要位于双侧肺门、纵隔、主动脉旁、小弯和腹膜后。锁骨上淋巴结活检证实了关节囊和周围软组织中的淋巴增生和扩张。胸腔镜检查发现左胸有血性乳糜胸。肺活检示胸膜及邻近肺组织淋巴管增生扩张。免疫组化染色显示淋巴管内皮细胞呈D2-40和CD 31阳性。根据上述组织学检查结果，诊断为肺和肺外淋巴结及周围软组织淋巴管瘤病。浅表淋巴结淋巴管瘤病主要累及淋巴结包膜及其周围软组织。淋巴结活检所获得的信息可以提示和辅助DPL的诊断。
Diffuse pulmonary lymphangiomatosis (DPL) mainly affects the lung and pleura. There are very few pathological reports of lung damage accompanied by diffuse involvement of the extrapulmonary lymph nodes and surrounding soft tissue. The clinicopathological significance of coexistence of pulmonary and extrapulmonary lesions is unknown. Here, we report a 16-year-old male patient. The pathological specimens of the supraclavicular lymph node and soft tissue together with the lung biopsy were analyzed by pathological observation and immunohistochemical staining. Literatures were reviewed and clinical and imaging findings were discussed. The patient presented with coughing and expectoration for 1 year and intermittent hemoptysis for 4 months. Ultrasound revealed swollen lymph nodes in bilateral neck, left armpit, and pubic symphysis. Chest CT scan showed diffuse grid and linear shadows, bilateral pleural thickening, and nodule formation. Multiple enlarged lymph nodes were mainly investigated in bilateral hilar, mediastinal, para-aortic, lesser curvature, and retroperitoneal. Supraclavicular lymph node biopsy confirmed the lymphatic hyperplasia and expansion in the capsule and surrounding soft tissue. The thoracoscopic examination found bloody chylothorax on the left chest. And lung biopsy showed the lymphatic vessel hyperplasia and expansion on the pleura and adjacent lung tissue. Immunohistochemical stains showed that the lymphatic endothelial cells were positive for D2–40 and CD31. Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue was diagnosed based on the aforementioned histological findings. Lymphangiomatosis of superficial lymph node mainly involves the capsule of lymph nodes and its surrounding soft tissue. The information obtained from the lymph node biopsy can prompt and assist the diagnosis of DPL.