Multifocal micronodular pneumocyte hyperplasia (MMPH) in a patient with tuberous sclerosis-evidence for long term stability.
Multifocal micronodular pneumocyte hyperplasia (MMPH) is rare entity seen mostly in patients with the tuberous sclerosis complex (TSC). We present the case of a 50 year old woman with TSC (confirmedTSC2mutation) found to have multiple ground glass opacities with an upper lobe predominance on a screening chest CT. No abnormalities were detected in other viscera. A thoracoscopic lung biopsy obtained from right upper lobe confirmed the diagnosis of MMPH. There were no lesions suggestive of lymphangioleiomyomatosis (LAM) either on the chest CT or lung biopsy. A repeat CT chest obtained on follow up 9 years after initial diagnosis continued to show stability of all MMPH ground glass lesions. This case highlights the distinct patterns of lung involvement in TSC, with MMPH having a benign and stable nature as compared to LAM which is often relentlessly progressive with associated lung function decline.
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, United States.
Bibek S
BS
Pannu
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, United States.
Dinesh R
DR
Apala
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, United States.
Aditya
A
Kotecha
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, United States.
Jennifer M
JM
Boland
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, United States.
Vivek N
VN
Iyer