Nishida Chinatsu, Yatera Kazuhiro, Kido Takashi, Noguchi Shingo, Akata Kentaro, Hanaka Minako, Yamasaki Kei, Hoshino Teppei, Shimono Masayuki, Ishimoto Hiroshi, Sakamoto Noriho, Mukae Hiroshi
Department of Respiratory Medicine, School of Medicine, University of Occupational and Environmental Health.
Department of Internal Medicine, Kitakyushu Municipal Yahata Hospital.
J UOEH. 2017;39(2):133-141. doi: 10.7888/juoeh.39.133.
Multifocal micronodular pneumocyte hyperplasia (MMPH) is pathologically characterized by multifocal nodular hyperplasia of type Ⅱ pneumocyte-like cells. MMPH is usually complicated with tuberous sclerosis complex (TSC). MMPH patients tend to be asymptomatic or only slightly symptomatic. MMPH tends to progress slowly and needs no treatment. We herein describe two cases of MMPH with its characteristic radiological features and clinical manifestations of TSC. Case 1: a 20-year-old female with definitive TSC in infancy. Chest CT at the age of 18 revealed multiple nodular opacities and ground-glass attenuations in a scattered and random distribution in the bilateral lungs. Case 2: a 44-year-old female with probable TSC at 36 years of age. Chest CT at the age of 43 showed random areas of small ground-glass attenuations, predominantly in the upper lung fields. Case 1 and Case 2 have had no respiratory symptoms or radiographic changes in the recent two years and four years, respectively. Although pathological examinations of the lung were not performed because consent for surgical lung biospies was unobtainable, we considered that these pulmonary manifestations were most likely MMPH with TSC because of these characteristic radiographical findings of multiple nodular opacities and ground-glass attenuations of 10 mm or less in size and their scattered distribution, and because there have been no abnormal laboratory data or changes in their chest radiological findings for years. Neither patient is under treatment for pulmonary lesions. Although MMPH is a rare disease, multiple nodules and ground-glass attenuations on lung imaging findings should be considered as pulmonary manifestations in patients with TSC.
多灶性微小结节性肺细胞增生症（MMPH）的病理特征为Ⅱ型肺细胞样细胞的多灶性结节性增生。MMPH通常与结节性硬化症（TSC）相关。MMPH患者往往无症状或仅有轻微症状。MMPH往往进展缓慢，无需治疗。我们在此描述两例MMPH病例，及其特征性的影像学表现和TSC的临床表现。病例1：一名20岁女性，婴儿期确诊为TSC。18岁时胸部CT显示双侧肺野散在随机分布的多个结节状阴影和磨玻璃样密度影。病例2：一名44岁女性，36岁时可能患有TSC。43岁时胸部CT显示随机分布的小片磨玻璃样密度影，主要位于上肺野。病例1和病例2在最近两年和四年中分别无呼吸道症状或影像学改变。尽管由于无法获得手术肺活检的同意而未进行肺的病理检查，但考虑到这些肺部表现最可能是伴有TSC的MMPH，因为有多个结节状阴影和大小在10mm或更小的磨玻璃样密度影的特征性影像学表现及其散在分布，且多年来实验室数据无异常或胸部影像学表现无变化。两名患者均未接受肺部病变的治疗。尽管MMPH是一种罕见疾病，但对于TSC患者，肺部影像学检查发现的多个结节和磨玻璃样密度影应被视为肺部表现。
