[A case of micronodular pneumocyte hyperplasia, lymphangiomyomatosis associated with tuberous sclerosis].
We report a case of tuberous sclerosis (TS) associated with renal angiolipomas, subungual fibroma, adenoma sebaceum, pulmonary lymphangiomyomatosis (LAM), and micronodular pneumocyte hyperplasia (MNPH). A CT scan of the chest demonstrated multiple minute nodules present throughout both lung fields. Histologically, the nodules were well demarcated, and were composed of thickened, fibrotic, alveolar septa lined by pleomorphic, type II pneumocytes. This type of MNPH is extremely rare, and appears to be a hamartomatous proliferation occurring in patients with TS.
Kawashima
M
M
Department of Saga Medical School, 5-1-1 Nabeshima, Saga, Japan.
Kobayashi
H
H
Tominaga
M
M
Yathunami
J
J
Hayashi
S
S
D000328:Adult; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008168:Lung / Q000473:pathology*; D008175:Lung Neoplasms / Q000150:complications / Q000473:pathology*; D018192:Lymphangioleiomyomatosis / Q000150:complications / Q000473:pathology*; D014402:Tuberous Sclerosis / Q000150:complications*