Zhang Xiaowei, Huang Bifei, Jiang Hongquan, Wei Hangping
Department of Pathology, Affiliated Dongyang Hospital of Wenzhou Medical University, Dongyang, China.
Department of Thoracic Surgery, Affiliated Dongyang Hospital of Wenzhou Medical University, Dongyang, China.
Front Med (Lausanne). 2022 Apr 18;9:836012. doi: 10.3389/fmed.2022.836012. eCollection 2022.
Hemangioblastoma-like clear cell stromal tumor (HLCCST) is a recently reported neoplasm of the lung. Only 13 cases have been reported in four recent studies. Because HLCCST is very rare, it has not been included in the 2021 WHO classification of lung tumors.
We report a case of HLCCST of the left lower lung in a 40-year-old female who was admitted to our hospital after pulmonary nodules were discovered. A plain chest CT scan showed a nodular high-density shadow measuring approximately 8 mm in diameter in the left lower lung. The lesion had clear borders, uneven internal density, and a low-density central vacuolar area. The left lower lung was partially resected by video-assisted thoracic surgery. Post-operative histopathologic diagnosis "hemangioblastoma-like clear cell stromal tumor" of the left lower lung.
The HLCCST is an extremely rare tumor and needs long-term follow-up after operation. Clinically, it may be easily confused with other benign and malignant tumors of the lung, and diagnosis is solely determined by histopathologic examination. This case suggests that immunohistochemical CD34 can be a strong positive marker.
血管母细胞瘤样透明细胞间质肿瘤（HLCCST）是一种最近报道的肺肿瘤。最近四项研究中仅报道了13例。由于HLCCST非常罕见，它未被纳入2021年世界卫生组织肺肿瘤分类。
我们报告一例40岁女性左下肺HLCCST病例，该患者在发现肺结节后入院。胸部平扫CT显示左下肺有一个直径约8mm的结节状高密度影。病变边界清晰，内部密度不均匀，中央有低密度空泡区。通过电视辅助胸腔镜手术对左下肺进行了部分切除。术后组织病理学诊断为左下肺“血管母细胞瘤样透明细胞间质肿瘤”。
HLCCST是一种极其罕见的肿瘤，术后需要长期随访。临床上，它可能容易与其他肺良恶性肿瘤混淆，诊断完全由组织病理学检查确定。该病例表明免疫组化CD34可为强阳性标记物。
