Department of General Surgery, Kawasaki Medical School, Okayama, Japan.
Department of Pathology 1, Kawasaki Medical School, Okayama, Japan.
Thorac Cancer. 2022 Apr;13(8):1227-1231. doi: 10.1111/1759-7714.14374. Epub 2022 Mar 17.
Pulmonary hypoplasia is diagnosed during the perinatal period and is a cause of death in newborns. However, these developmental abnormalities are diagnosed in adulthood in some cases. A 70-year-old male smoker was diagnosed with stage IIIA pulmonary adenocarcinoma in the right upper lobe with right middle lobe hypoplasia. He subsequently underwent right upper and middle lobectomy with lymph node dissection by video-assisted thoracoscopic surgery. In addition to an invasive adenocarcinoma in the right upper lobe, pathological examination of the hypoplastic lobe revealed neuroendocrine hyperplasia, as well as tumorlets and a typical carcinoid. Eight cases of pulmonary neuroendocrine tumors that developed from pulmonary hypoplasia have been reported to date. Interestingly, all but one case occurred in the right middle lobe. Neuroendocrine cell hyperplasia has been reported to develop in hypoplastic lungs postnatally; therefore, we speculated that the lesion was the origin of these neuroendocrine tumors. Moreover, the pathological findings suggested that atelectasis was involved in the pathogenesis of this rare condition. In adults, when lobar hypoplasia is diagnosed, neuroendocrine tumors should be anticipated.
肺发育不全是在围产期诊断出来的，是新生儿死亡的原因之一。然而，在某些情况下，这些发育异常在成年期才被诊断出来。一名 70 岁的男性吸烟者，因右侧中上肺叶伴有右中叶发育不全被诊断为 IIIA 期肺腺癌。随后，他接受了右侧中上肺叶切除术和淋巴结清扫术，采用了电视辅助胸腔镜手术。除了右上叶的浸润性腺癌外，发育不全叶的病理检查还显示出神经内分泌增生，以及肿瘤细胞和典型类癌。迄今为止，已有 8 例源自肺发育不全的肺神经内分泌肿瘤的报道。有趣的是，除了 1 例外，所有病例均发生在右中叶。据报道，出生后肺发育不全的肺神经内分泌细胞会增生；因此，我们推测病变是这些神经内分泌肿瘤的起源。此外，病理发现提示，肺不张参与了这种罕见疾病的发病机制。在成人中，当诊断出肺叶发育不全时，应预测到神经内分泌肿瘤的存在。
