The diagnosis of lymphangioleiomyomatosis (LAM) and histiocytosis X (Hx) need to be histologically proven?

Sergio Harari, M. Barberis, E. De Juli, F. Colombo, G. Cimino, L. Sabolla, E. Soresi

Research output: Contribution to journalArticlepeer-review


Purpose: LAM and Hx are two cystic nodular lung diseases with peribronchial distribution. The rareness of both diseases make difficult the diagnosis to be made in early stages, even if the clinical ground is typical. In advanced diseases videoassisted thoracoscopic biopsy (VAT) can present some risks. Methods: Therefore we retrospectively evaluated our experience in the diagnosis of the 20 patients (pts) presenting radiological findings of cystic nodular lung disease. Results: Pulmonary function test (PFT) showed an obstructive or mixed pattern while a pure restrictive one was observed only in initial disease cases presenting reticular pattern at high definition computed tomography (HRCT). The clinical radiological suspicion of Hx was made in 13 cases while in 7 LAM was suspected. Diagnosis in Hx pts were made as follow: 3 VAT (2 in pts with > 3% of GD1 a positive cells in BAL), 1 TBB, 1 fine needle aspiration, 5 BAL (more than 3% of CD1 a pos cell and 2 Birbeck granules). 3 pts presented bone eosinophilic granuloma, 2 diabete insipidus. Only 4 pts had a clinical-radiological diagnosis, in 1 case confirmed of autopsy. In LAM pts diagnosis was made in 3 cases by TBB, 5 out of 7 pts presented renal angiomyolipoma. 4 clinical radiological-diagnosis were confirmed in 3 cases by the explanted lungs for transplantation. VAT were performed in 2 LAM and 3 Hx: all procedures have been complicated by prolonged airway leakages. Conclusions: In our opinion in specialized centers clinical-radiological diagnosis of LAM and Hx may be acceptable.

Original languageEnglish
Issue number4 SUPPL.
Publication statusPublished - Oct 1996

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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