Abstract
This study reports the case of a 39-year-old woman with tuberous sclerosis complex (TSC) combined lymphangioleiomyomatosis (LAM), multifocal micronodular pneumocyte hyperplasia (MMPH), and clear-cell micronodules. She had repetitious bilateral pneumothoraces, and chest radiography revealed diffuse thin-walled cysts and multiple nodules. Bilateral renal angiomyolipomas (AMLs) and cerebral periventricular spots were also present. Lung biopsy revealed dilated airspace with HMB-45-positive spindle cell infiltration and aggregation indicative of LAM. Randomly distributed nodules of proliferative type II pneumocytes were consistent with MMPH. Clear-cell micronodules comprised nodular aggregation of HMB-45-positive polygonal cells with clear cytoplasm. Abnormal migration and proliferation of HMB-45 immunoreactive perivascular epithelioid cells (PEComas) is posited to indicate LAM and AML histogenesis. Clear-cell micronodules are also believed to be one of the families of PEComas and are reported in association with TSC. These behaviors of perivascular epithelioid cells may play a role in TSC.
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