Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

Although most lesions that occur in the chest have a nonspecific soft-tissue appearance, fat-containing lesions are occasionally encountered at cross-sectional computed tomography (CT) or magnetic resonance imaging. The various fat-containing lesions of the chest include parenchymal and endobronchial lesions such as hamartoma, lipoid pneumonia, and lipoma. Endobronchial hamartoma usually appears at CT as a lesion with a smooth edge, focal collections of fat, or fat collections that alternate with foci of calcification. Mediastinal fat-containing lesions include germ cell neoplasms, thymolipomas, lipomas, and liposarcomas. The most frequent CT manifestation of the germ cell neoplasm teratoma is a heterogeneous mass with soft-tissue, fluid, fat, and calcium attenuation. Cardiac lesions with fat content include lipomatous hypertrophy of the interatrial septum and arrhythmogenic right ventricular dysplasia. Diagnosis of the former is made with CT when a smooth, nonenhancing, well-marginated fat-containing lesion is identified in the interatrial septum. Finally, fat may herniate into the chest at several characteristic locations. When such a lesion is identified, the time required for differential diagnosis is significantly reduced, often allowing a definitive radiologic diagnosis. Sagittal and coronal reformatted images can add valuable information by showing diaphragmatic defects and hernia contents.

Lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH) produce cystic and nodular disease, respectively, in the lungs of patients with tuberous sclerosis. The objective of this study was to prospectively characterize the prevalence, clinical presentation, and genetic basis of lung disease in TSC. We performed genotyping and computerized tomographic (CT) scanning of the chest on 23 asymptomatic women with tuberous sclerosis complex (TSC). Cystic pulmonary parenchymal changes consistent with LAM were found in nine patients (39%). These patients tended to be older than cyst-negative patients (31.9 +/- 7.6 yr versus 24.8 +/- 11.6 yr, p = 0.09). There was no correlation between presence of cysts and tobacco use, age at menarche, history of pregnancy, or estrogen-containing medications. Three of the cyst-positive patients had a prior history of pneumothorax. Pulmonary function studies revealed evidence of gas trapping but normal spirometric indices in the cyst-positive group. All nine cyst-positive patients had angiomyolipomas (AML), which were larger (p < 0.05) and more frequently required intervention (p = 0.08) than cyst-negative patients (8 of 14 with AMLs, p < 0.05). Ten patients (43%) had pulmonary parenchymal nodules. Pulmonary nodules were more common in women with cysts (78% versus 21%, p < 0.05), and 52% of all patients had either cystic or nodular changes. TSC2 mutations were identified in all cyst-positive patients who were tested (n = 8), whereas both TSC1 and TSC2 mutations were found in patients with nodular disease. Correlation of the mutational and radiographic data revealed one pair of sisters who were discordant for cystic disease, two mother- daughter pairs who were discordant for nodular disease, and no clear association between cyst development and a specific mutational type. This prospective analysis demonstrates that cystic and nodular pulmonary changes consistent with LAM and MMPH are common in women with TSC.

Pneumocystis jiroveci pneumonia is a common opportunistic infection affecting immunosuppressed patients. High-resolution CT may be indicated for evaluation of immunosuppressed patients with suspected pneumonia and normal chest radiographic findings. The most common high-resolution CT finding of Pneumocystis jiroveci pneumonia is diffuse ground-glass opacity. Consolidation, nodules, cysts, and spontaneous pneumothorax also can develop.

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HRCT is an important modality in the evaluation of interstitial lung disease to include cystic lung disease. Although most commonly associated with lymphangioleiomyomatosis or Langerhans cell histiocytosis, cystic lung disease is increasingly being recognized as a feature of other entities. Awareness of the spectrum of HRCT findings associated with these diseases may help the trained observer narrow the differential diagnosis.

Comparison of tumor volumes at serial CT examinations reveals a very wide range of growth rates. Some tumors grow so slowly that biopsy is required to prove they are malignant.