Hui Li Yan Sandra, Cheng Xin Min, Soo Ing Xiang, Low Su Ying
Department of Respiratory and Critical Care Medicine Changi General Hospital Singapore.
Department of Anatomical Pathology Singapore General Hospital Singapore.
Respirol Case Rep. 2021 Oct 28;9(12):e0870. doi: 10.1002/rcr2.870. eCollection 2021 Dec.
Multifocal micronodular pneumocyte hyperplasia (MMPH) is the lesser known pulmonary manifestation of tuberous sclerosis. It manifests radiologically as diffuse small ground-glass and solid nodules. Accurate diagnosis is essential as it can be mistaken for miliary tuberculosis or malignant lesions which necessitates specific treatment. Constellation of radiological features such as multicentric disease at onset and stability over time can help to distinguish MMPH from its differentials. Histologically, MMPH is characterized by hamartomatous proliferation of type II pneumocytes with a lack of high nuclear to cytoplasmic ratio. MMPH confers a benign prognosis unlike its differentials. Therefore, accurate diagnosis is paramount in ensuring appropriate care is delivered. Here, we describe the radiological and histological features of MMPH in a patient with genetically proven tuberous sclerosis complex and co-existing lymphangioleiomyomatosis.
多灶性微小结节性肺细胞增生症(MMPH)是结节性硬化症鲜为人知的肺部表现。其影像学表现为弥漫性小磨玻璃结节和实性结节。准确诊断至关重要,因为它可能被误诊为粟粒性肺结核或恶性病变,而这需要特定的治疗。一些影像学特征,如发病时的多中心性疾病以及随时间的稳定性,有助于将MMPH与其鉴别诊断区分开来。组织学上,MMPH的特征是II型肺细胞的错构瘤样增生,且核质比不高。与其他鉴别诊断不同,MMPH预后良好。因此,准确诊断对于确保提供适当的治疗至关重要。在此,我们描述了一名经基因证实患有结节性硬化症复合体且并存淋巴管平滑肌瘤病患者的MMPH的影像学和组织学特征。