Table 2.
Characteristics of cystic lung diseases
| Disorder | Age, yr | CT findings (cyst) |
Biopsy | Clinical | Treatment for cyst | ||
|---|---|---|---|---|---|---|---|
| Shape | Distribution Other | ||||||
| LAM | 35 | Round, smooth | Diffuse | Effusion | LAM cells, HMB-45 (+) | TSC, AML, meningioma, F >> M | mTOR inhibitor |
| PLCH | 2-40 | Bizarre, irregular | Upper/middle lobe | Nodules, cavitation | Langerhans cell, CD1a (+) | Smoker, F = M, BRAF mutation | Immunosuppression, BRAF inhibitor |
| BHD | 3-40 | Round, lentiform | Basilar, medial, subpleural | Nonspecific, alveoli within an alveolus | Skin, renal tumor, FLCN mutation, AD | ||
| LIP/FB | 4-70 | Internal structure (+) | Random, perivascular | GGO, centrilobular nodules, septal thickening | Lymphocytic infiltration | Autoimmune disorder, F > M | Immunosuppression |
| Amyloidosis | Round | Random | Nodules, calcified cavitation | Apple-green birefringence appearance on Congo Red stain | MM, lymphoma, Sjogren’s disease | Immunosuppression | |
CT, computed tomography; LAM, lymphangioleiomyomatosis; HMB-45, human melanoma-block 45; TSC, tuberous sclerosis complex; AML, angiomyolipomas; F, female; M, male; mTOR, mammalian target of rapamycin; PLCH, pulmonary Langerhans cell histiocytosis; BRAF, v-Raf murine sarcoma viral oncogene homolog B; BHD, Birt-Hogg-Dube; FLCN, folliculin; AD, autosomal dominant; LIP, lymphocytic interstitial pneumonia; FB, follicular bronchiolitis; GGO, ground glass opacity; MM, multiple myeloma.