[A case of micronodular pneumocyte hyperplasia, lymphangiomyomatosis associated with tuberous sclerosis].

Abstract

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.

• Authors: M Kawashima, H Kobayashi, M Tominaga, J Yathunami, S Hayashi
• Journal: Nihon Kokyuki Gakkai zasshi = the journal of the Japanese Respiratory Society (Nihon Kokyuki Gakkai Zasshi) Vol. 39 Issue 4 Pg. 277-80 (Apr 2001) ISSN: 1343-3490 [Print] Japan
• PMID: 11481828 (Publication Type: Case Reports, English Abstract, Journal Article)

Topics

• Adult
• Female
• Humans
• Hyperplasia
• Lung (pathology)
• Lung Neoplasms (complications, pathology)
• Lymphangioleiomyomatosis (complications, pathology)
• Tuberous Sclerosis (complications)