Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis
- Radiologic Findings
- HRCT images (Fig. 1-2) show a random distribution of multiple and variable-sized ground glass opacity nodules in both lungs. Abdomen CT images (Fig. 3-4) show lobulated osteosclerotic lesion involving the lumbar spine and a well-defined nodule containing fat attenuation and lacy, enhancing area in the left kidney. VATS biopsy was performed and pathologic diagnosis was multifocal micronodular pneumocyte hyerplasia (MMPH). These findings suggest tuberous sclerosis complex (TSC) of the patient with MMPH, TS-associated osteosclerotic lesion, and an angiomyolipoma of kidney.
- Brief Review
- TSC is an autosomal dominant syndrome characterized by hamartomatous growth in the skin, eyes, kidneys, and central nervous system. Pulmonary involvement occurs in 1% of cases and usually manifests as lymphangioleiomyomatosis (LAM). MMPH is another, extremely rare pulmonary manifestation of TSC, which was first described by Popper et al. in 1991. MMPH is a hamartomatous process of the lung that exhibits multiple tiny pulmonary nodules. High-resolution CT in MMPH reveals nodules 1
- References
- Ross L. Ristagno, Paul W. Biddinger, Elsira M. Pina, Cris A. Meyer. Multifocal Micronodular Pneumocyte Hyperplasia in Tuberous Sclerosis. AJR 2005;184:S37-39.
2. Yoshihiro Kobashi, Tadaaki Sugiu, Keiji Mouri, Tsutomu Irei. Clinicopathological analysis of multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis in Japan. Respirology. 2008;13:1076-1081.
- Keywords
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Lung, Multiple organ, Interstitial lung disease, ILD,