肺隔离症中发生的原发性血管外皮细胞瘤：是巧合还是两种罕见疾病并存？
Primary Hemangiopericytoma Arising in Extralobar Pulmonary Sequestration: A Coincidence or Two Rare Disorders?
机构信息
Department of Pathophysiology, University of Athens, Medical School, Athens, Greece.
Second Department of Propedeutic Surgery, Laiko General Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
出版信息
In Vivo. 2019 Nov-Dec;33(6):2141-2145. doi: 10.21873/invivo.11715.
BACKGROUND/AIM: Extralobar pulmonary sequestration (EPS) is an unusual congenital defect characterized by the presence of non-functioning lung tissue receiving arterial supply from the systemic arteries. Primary hemangiopericytoma (HPC) is an uncommon potentially malignant tumor of vascular origin that usually involves the soft tissue of the extremities or retroperitoneum, but extremely rarely affects the lung. We present the rare case of a primary pulmonary HPC arising in an EPS.
CASE REPORT
A 65-year-old woman, with dyspnea and pleuritic chest pain, was referred for further investigation. Radiological evaluation demonstrated a well-circumscribed mass above the right hemidiaphragm, receiving its arterial supply from the descending thoracic aorta. The patient underwent a right posterolateral thoracotomy and a middle lobectomy. The intraoperative finding was a well-encapsulated solid mass. The histological evaluation described HPC.
RESULTS
The patient remains fit and healthy.
CONCLUSION
Pulmonary HPC can arise in EPS. Surgical excision is the treatment of choice.
背景/目的：肺隔离症（EPS）是一种罕见的先天性畸形，其特征是存在由体循环动脉供血的无功能肺组织。原发性血管外皮细胞瘤（HPC）是一种起源于血管的罕见的潜在恶性肿瘤，通常累及四肢或腹膜后软组织，但极罕见于肺部。我们报告了一例罕见的肺隔离症中发生的原发性肺 HPC。
病例报告
一名 65 岁女性，有呼吸困难和胸膜炎性胸痛，被转介进一步检查。影像学评估显示右膈肌上方有一个边界清楚的肿块，其动脉供应来自降主动脉。患者接受了右后外侧开胸和中叶切除术。术中发现为一个包膜完整的实性肿块。组织学评估描述为 HPC。
结果
患者身体状况良好。
结论
肺 HPC 可发生于肺隔离症。手术切除是首选的治疗方法。
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