title: Lymphangioleiomyomatosis, multifocal micronodular pneumocyte hyperplasia, and clear-cell micronodules in a 39-year-old woman with tuberous sclerosis: a case report.
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.
authors.affiliation: Far Eastern Memorial Hospital, Taipei, Taiwan. magieva_tw@hotmail.com
authors.forename: Min-hsiang
authors.initials: MH
authors.lastname: Chang
authors.forename: Hsu
authors.initials: H
authors.lastname: Yao-Peng
mesh_terms: D000328:Adult; D056809:Alveolar Epithelial Cells / Q000473:pathology*; D018207:Angiomyolipoma / Q000150:complications / Q000473:pathology; D005260:Female; D006801:Humans; D006965:Hyperplasia / Q000150:complications / Q000473:pathology; D007680:Kidney Neoplasms / Q000150:complications / Q000473:pathology; D018192:Lymphangioleiomyomatosis / Q000150:complications* / Q000473:pathology; D054973:Perivascular Epithelioid Cell Neoplasms / Q000150:complications / Q000473:pathology; D014402:Tuberous Sclerosis / Q000150:complications* / Q000473:pathology