Zhao Jikai, Shao Jinchen, Zhu Lei, Yu Keke, Zhao Ruiying, Ding Wenjie, Zhang Jie, Han Yuchen
Department of Pathology, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai 200030, China.
Department of Biobank, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai 200030, China.
Pathol Res Pract. 2018 Nov;214(11):1885-1891. doi: 10.1016/j.prp.2018.09.014. Epub 2018 Sep 20.
Solitary pulmonary capillary hemangioma (SPCH) is an extraordinarily rare capillary derived mesenchymal neoplasm. Although routine morphology and immunohistochemistry are adequate for the diagnosis of classical SPCH in surgical specimens, true gross appearance identification of most tumor themselves and diagnosis for some exceptional cases are still very difficult. Furthermore, preoperative imaging and frozen diagnosis remain a challenge.
We reported nine original cases of solitary pulmonary capillary hemangioma and summarized the clinical characteristics of twenty-one reported lesions. Imaging materials were reviewed by the image experts of our hospital. Quick hematoxylin-eosin stained intraoperative frozen sections and routine histological diagnosis were re-confirmed by 3 specialist pathologists with at least 10 years of diagnostic experience in our department. Immunohistochemistry analysis was performed on formalin fixed archival tissue. The surgical methods, following up information and prognosis were retrospectively analyzed.
In imaging, three tumors showed solid nodules, three cases displayed mix ground glass nodules, two nodules were pure ground glass density, and one case was a cystic-solid mass. Macroscopically, solitary pulmonary capillary hemangiomas were ill-defined soft hemorrhagic lesion with pale yellow or dark brownish cut surface. Two cases had a clear boundary and seven lesions were poorly demarcated. Typical morphological features were densely proliferating thin-walled capillaries composing of single layer of flatten or cuboidal endothelial cells within the thickened alveolar septa. One case was mistaken for a histiocytogenic lesion during freezing. The cystic-solid lesion showed a hyperplasia capillary network along the submucosal interstitium of bronchioles. Immunohistochemically, tumor endothelial cells were positive for ERG, Fli-1, CD31, CD34 and Vimentin and negative for CK, α-SMA, TTF-1, HMB45, S-100 and CD68. Lobectomy was performed on seven cases, wedge resection and segmentectomy were proceeded in two patients respectively. Follow up information showed no evidence of complication or recurrence.
Solitary pulmonary capillary hemangioma has special imaging and various histological features and must be distinguished from small benign lung lesions and preinvasive cancer. Although the prognosis of this tumor is good after surgical resection, the correct interpretation of the gross appearance and radiographic findings are still important. Choosing appropriate resection mode depends on accurate evaluation preoperative and intraoperative.
孤立性肺毛细血管瘤（SPCH）是一种极其罕见的源自毛细血管的间叶性肿瘤。尽管常规形态学和免疫组织化学足以诊断手术标本中的经典SPCH，但大多数肿瘤本身的真实大体外观识别以及一些特殊病例的诊断仍然非常困难。此外，术前影像学检查和冷冻诊断仍然是一项挑战。
我们报告了9例孤立性肺毛细血管瘤的原始病例，并总结了21例已报道病变的临床特征。影像学资料由我院影像专家进行回顾。快速苏木精-伊红染色的术中冷冻切片和常规组织学诊断由我科3名具有至少10年诊断经验的专科病理学家再次确认。对福尔马林固定的存档组织进行免疫组织化学分析。对手术方法、随访信息和预后进行回顾性分析。
在影像学上，3个肿瘤表现为实性结节，3例表现为混合磨玻璃结节，2个结节为纯磨玻璃密度，1例为囊实性肿块。大体上，孤立性肺毛细血管瘤是边界不清的软血性病变，切面呈淡黄色或暗褐色。2例边界清晰，7个病变分界不清。典型的形态学特征是在增厚的肺泡间隔内由单层扁平或立方内皮细胞组成的密集增生的薄壁毛细血管。1例在冷冻时被误诊为组织细胞源性病变。囊实性病变在细支气管黏膜下间质可见增生的毛细血管网。免疫组织化学显示，肿瘤内皮细胞ERG、Fli-1、CD31、CD34和波形蛋白阳性，细胞角蛋白、α-平滑肌肌动蛋白、甲状腺转录因子-1、HMB45、S-100和CD68阴性。7例行肺叶切除术，2例分别行楔形切除术和肺段切除术。随访信息显示无并发症或复发迹象。
孤立性肺毛细血管瘤具有特殊的影像学表现和多样的组织学特征，必须与肺部小的良性病变和浸润前癌相鉴别。尽管该肿瘤手术切除后预后良好，但对大体外观和影像学表现的正确解读仍然很重要。选择合适的切除方式取决于术前和术中的准确评估。
