关键词: Chondromyxoid neoplasm Malignant sarcomatous transformation Pulmonary hamartoma

Mesh : Humans Hamartoma / pathology surgery diagnosis Female Middle Aged Lung Diseases / pathology surgery diagnosis Incidental Findings Lung Neoplasms / pathology surgery diagnosis

来  源:   DOI:10.1186/s13000-024-01506-0   PDF(Pubmed)

Abstract:
BACKGROUND: Pulmonary hamartomas are benign lung lesions. Histopathologically, pulmonary hamartoma is composed of varying amounts of mesenchymal elements, including chondroid tissue, mature adipose tissue, fibrous stroma, smooth muscle, and entrapped respiratory epithelium. Most pulmonary hamartoma cases are asymptomatic and found incidentally during imaging. They usually appear as well-circumscribed lesions with the largest dimension of less than 4 cm. Asymptomatic giant pulmonary hamartomas that more than 8 cm are rare.
METHODS: In the current case report, a 12.0 × 9.5 × 7.5 cm lung mass was incidentally noticed in a 59-year-old female during a heart disease workup. Grossly, the lesion was lobulated with pearly white to tan-white solid cut surface and small cystic areas. Microscopically, representative tumor sections demonstrate a chondromyxoid appearance with relatively hypocellular stroma and entrapped respiratory epithelium at the periphery. No significant atypia is noted. No mitosis is noted, and the proliferative index is very low (< 1%) per Ki-67 immunohistochemistry. Mature adipose tissue is easily identifiable in many areas. Histomorphology is consistent with pulmonary hamartoma. A sarcoma-targeted gene fusion panel was further applied to this case. Combined evaluation of microscopic examination and sarcoma-targeted gene fusion panel results excluded malignant sarcomatous transformation in this case. The mediastinal and hilar lymph nodes are histologically benign. After surgery, the patient had an uneventful postoperative period.
CONCLUSIONS: Giant pulmonary hamartoma is rare; our case is an example of a huge hamartoma in an asymptomatic patient. The size of this tumor is concerning. Thus, careful and comprehensive examination of the lesion is required for the correct diagnosis and to rule out co-existent malignancy.
摘要:
背景:肺错构瘤是肺部良性病变。组织病理学,肺错构瘤由不同数量的间充质成分组成,包括软骨组织,成熟脂肪组织,纤维基质,平滑肌,并截留了呼吸道上皮。大多数肺错构瘤病例无症状,在成像过程中偶然发现。它们通常表现为界限清楚的病变,最大尺寸小于4厘米。超过8厘米的无症状巨大肺错构瘤很少见。
方法:在当前的病例报告中,在心脏病检查中,偶然发现一名59岁女性的肺部肿块为12.0×9.5×7.5厘米。严重的,病变呈小叶状,白色至棕白色固体切面和小的囊性区域。微观上,有代表性的肿瘤切片显示软骨粘液样外观,周围有相对低细胞的基质和包裹的呼吸上皮。没有注意到显著的非典型性。没有注意到有丝分裂,根据Ki-67免疫组织化学,增殖指数非常低(<1%)。成熟的脂肪组织在许多领域很容易识别。组织形态学与肺错构瘤一致。将肉瘤靶向的基因融合组进一步应用于这种情况。在这种情况下,显微镜检查和肉瘤靶向基因融合组结果的联合评估排除了恶性肉瘤转化。纵隔和肺门淋巴结在组织学上是良性的。手术后,患者术后度过了一个平稳的时期.
结论:巨大的肺错构瘤是罕见的;我们的病例是无症状患者的巨大错构瘤的一个例子。这个肿瘤的大小令人担忧。因此,为了正确诊断和排除共存的恶性肿瘤,需要对病变进行仔细和全面的检查。
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