Department of Anatomic Pathology, General University Hospital of Udine, Udine, Italy.
Adv Anat Pathol. 2013 Mar;20(2):130-5. doi: 10.1097/PAP.0b013e318286245d.
The authors report 2 cases of an apparently unpublished stromal tumor of the lung characterized by a predominantly endobronchial growth pattern and benign-appearing clear cells. Both tumors were discovered incidentally in adult patients during routine workups for other medical reasons and treated with lobectomy. On gross inspection there was no evidence of infiltration of the adjacent lung tissue. Microscopically, both lesions featured monotonous oval-shaped to spindle-shaped cells growing in a vaguely nested pattern. The cytoplasm was slightly vacuolated or granular. In 1 case there was a variable admixture with mature fat. Immunohistochemistry was negative for markers of epithelial and stromal differentiation except for vimentin. A focal reaction for CD34 was seen in 1 case. No mutation of coding sequence of VHL gene was seen in one case. Medical follow-up at 1 year was negative for tumor recurrence or metastases. The broad differential diagnosis within the spectrum of stromal lung tumor is discussed. Owing to distinctive microscopic features such as the nesting of clear cells within a vascularized background, both tumors appeared similar to hemangioblastoma, although the expected immunohistochemical profile of the latter was not fully expressed. Because of pattern of growth seen in both lesions we believe that the appellation of endobronchial, hemangioblastoma-like clear cell stromal tumor may be provisionally designed.
作者报告了 2 例显然尚未发表的肺基质肿瘤，其特征为主要的支气管内生长模式和良性表现的透明细胞。这两个肿瘤都是在成年患者因其他医疗原因进行常规检查时偶然发现的，并通过肺叶切除术进行治疗。大体检查没有证据表明相邻肺组织的浸润。显微镜下，两个病变均具有单调的椭圆形至梭形细胞，呈模糊的巢状生长。细胞质略带空泡或颗粒状。在 1 例中，存在可变的成熟脂肪混合物。除了波形蛋白外，上皮和间质分化标志物的免疫组织化学均为阴性。1 例中可见 CD34 的局灶性反应。1 例中未发现 VHL 基因突变。1 年内的医疗随访未发现肿瘤复发或转移。讨论了在肺间质肿瘤范围内广泛的鉴别诊断。由于透明细胞在血管化背景中的巢状排列等独特的显微镜特征，这两个肿瘤与血管母细胞瘤相似，尽管后者预期的免疫组织化学特征并未完全表达。由于在两个病变中观察到的生长模式，我们认为可以暂时采用支气管内、血管母细胞瘤样透明细胞基质肿瘤的名称。
