Kolik Ali Kubilay, Bakkaloğlu Doğu Vurallı, Yilmaz Ismail, Cakir Mehmet Semih, Yegen Gulcin, Kara Murat, Özlük Yasemin
Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Department of Pathology, Sultan Abdulhamid Han Training & Research Hospital, University of Health Sciences, Istanbul, Turkey.
Int J Surg Pathol. 2025 May;33(3):700-705. doi: 10.1177/10668969241271372. Epub 2024 Sep 14.
We hereby report a patient with ALK-positive histiocytosis with localized lung involvement. A 47-year-old woman presented with a solitary pulmonary nodule in the left upper lobe, 7 months after COVID infection. A well-defined 15 mm yellow mass was found in trisegmentectomy specimen. Histopathological examination revealed that the mass was composed of epithelioid and spindle cells with foamy cytoplasms. No necrosis, pleomorphism, or nuclear atypia was detected. The cells were positive for CD68, CD163, PU.1, ALK and negative for KRT, smooth muscle actin (SMA), S100, Melan-A, CD34, STAT6, and BRAF VE1. Flourescence in situ hybridization demonstrated ALK gene rearrangement, and next generation sequencing confirmed fusion. Lung involvement in ALK-positive histiocytosis is characterized by the presence of pulmonary nodules, which can be seen in all forms of the disease. However, lung involvement is rarely seen in single-system ALK-positive histiocytosis. Our report represents the fourth documented instance of localized lung involvement in ALK-positive histiocytosis, an exceedingly rare occurrence, and it is the third instance with available molecular data.
我们在此报告一例ALK阳性组织细胞增多症伴局限性肺部受累的患者。一名47岁女性在感染新冠病毒7个月后,左上叶出现一个孤立性肺结节。在肺叶切除标本中发现一个边界清晰的15毫米黄色肿块。组织病理学检查显示,该肿块由具有泡沫状细胞质的上皮样细胞和梭形细胞组成。未检测到坏死、多形性或核异型性。细胞CD68、CD163、PU.1、ALK呈阳性,而细胞角蛋白、平滑肌肌动蛋白(SMA)、S100、Melan-A、CD34、STAT6和BRAF VE1呈阴性。荧光原位杂交显示ALK基因重排,二代测序证实存在融合。ALK阳性组织细胞增多症的肺部受累表现为肺结节,可见于该病的所有形式。然而,单系统ALK阳性组织细胞增多症很少见肺部受累。我们的报告是ALK阳性组织细胞增多症局限性肺部受累的第四例记录病例,极为罕见,也是第三例有可用分子数据的病例。