Zhao S N, Jin Y, Xie H K, Wu C Y, Li Y, Zhang L P
Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Department of Pathology, Fudan University Shanghai Cancer Center, Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China.
Zhonghua Bing Li Xue Za Zhi. 2020 Dec 8;49(12):1282-1287. doi: 10.3760/cma.j.cn112151-20200305-00175.
To investigate the clinical pathological features of primary pulmonary and tracheal glomus tumors. The clinical and pathological features of 11 cases (4 cases from Shanghai Pulmonary Hospital, Tongji University School of Medicine, China and 7 cases from Fudan University Shanghai Cancer Center, China) of respiratory glomus tumor diagnosed from 2010 to 2019 were analyzed, and reviewed in light of the relevant literature. In the 11 cases, there were 5 males and 6 females, with the onset ages of 29‒66 years (median age of 43). Six tumors were located in the lung, and 5 in the trachea. The tumor diameters ranged 1.0‒7.5 cm, with the average diameter of 2.6 cm. At low magnification, the tumors were diffuse or lobulated in shape. The tumor cells composed of sheets of oval to short spindle cells, with sharply defined cell border and prominent branching thin-walled vessels. Among the 4 benign glomus tumors, one was classified as benign symplastic glomus tumor owing to the hyperchromatic or degeneration nuclei. Two cases were classified as glomus tumors of uncertain malignant potential, on the account of cellular atypia and rare atypical mitotic figures. Five cases were classified as malignant glomus tumors, owing to the tumor necrosis, vascular invasion, marked nuclear atypia, prominent nucleoli and brisk mitoses (2-20/10HPF) including pathological mitotic figures. The tumor cells showed strong immunostaining for SMA, vimentin, type Ⅳ collagen and caldesmon to different extents, while CD34, cytokeratin and S-100 stains were negative. One of the cases was positive for desmin, and one case positive for synaptophysin. Follow-up information was available in 8 patients with the duration ranging from 6 to 95 months. At the end of the follow-up, 6 patients were alive without recurrence or metastasis, and two of the patients with malignant glomus tumors died. Primary pulmonary and tracheal glomus tumors is rare. Among the reported cases, malignant glomus tumor is the most frequent, followed by benign glomus tumors and uncertain malignant potential glomus tumors. Glomus tumors show sheet-like growth pattern and clusters of round epithelioid cells with numerous vascular spaces. They can be easily misdiagnosed as carcinoid tumor. The final diagnosis should be combined with immunohistochemical staining, such as SMA, caldesmon and vimentin.
探讨原发性肺和气管球瘤的临床病理特征。分析了2010年至2019年诊断的11例（中国同济大学医学院附属上海肺科医院4例，复旦大学附属上海肿瘤医院7例）呼吸道球瘤的临床和病理特征，并结合相关文献进行复习。11例患者中，男性5例，女性6例，发病年龄29 - 66岁（中位年龄43岁）。6例肿瘤位于肺，5例位于气管。肿瘤直径1.0 - 7.5 cm，平均直径2.6 cm。低倍镜下，肿瘤呈弥漫性或分叶状。肿瘤细胞由成片的椭圆形至短梭形细胞组成，细胞边界清晰，有明显分支的薄壁血管。4例良性球瘤中，1例因细胞核深染或退变被分类为良性增生性球瘤。2例因细胞异型性和罕见的非典型有丝分裂象被分类为恶性潜能不确定的球瘤。5例因肿瘤坏死、血管侵犯、明显的核异型性、显著的核仁及活跃的有丝分裂（2 - 20/10HPF，包括病理性有丝分裂象）被分类为恶性球瘤。肿瘤细胞对SMA、波形蛋白、Ⅳ型胶原和钙调蛋白不同程度呈强免疫染色，而CD34、细胞角蛋白和S - 100染色阴性。1例结蛋白阳性，1例突触素阳性。8例患者有随访信息，随访时间6至95个月。随访结束时，6例患者存活，无复发或转移，2例恶性球瘤患者死亡。原发性肺和气管球瘤罕见。在已报道的病例中，恶性球瘤最为常见，其次是良性球瘤和恶性潜能不确定的球瘤。球瘤呈片状生长模式，有成群的圆形上皮样细胞和众多血管间隙。它们容易被误诊为类癌肿瘤。最终诊断应结合免疫组化染色，如SMA、钙调蛋白和波形蛋白。
