Persistent fever may be a sign of dissemination to extrathoracic sites.

It can be congenital or acquired, and extrathoracic or intrathoracic (inside or outside the thoracic cavity.

For patients with either diffuse pulmonary involvement or severely ill patients with extrathoracic disseminated disease, amphotericin B is the preferred initial therapy (AII) (636).

In the early 1990s, Southall led a study which pioneered continuous negative extrathoracic pressure therapy, a treatment for breathing difficulties in young children involving the application of negative pressure to the patients' chests.

Additionally, Tazelaar, et al., described a pattern of histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed "diffuse pulmonary lymphangiomatosis" (DPL).

Stage III: Local extension of disease into the following areas, e.g., chest wall or mediastinum, heart or through the diaphragm or peritoneum, with or without extrathoracic or contralateral (N3) lymph node involvement.