Lymphangitic carcinomatosis of the lung: Pathologic, diagnostic and therapeutic considerations

A review of the tumor registry files of 269 breast cancer patients revealed 24 patients who had lymphangitic carcinomatosis; 17 patients had autopsies. In addition to permeation of the pulmonary lymphatics by tumor, most patients also had pulmonary or pleural nodules, hilar or mediastinal lymph node metastases or tumor emboli in small arteries and arterioles. In four patients only lymphangitic metastases were observed. The clinical diagnosis usually was made from chest X-ray findings of a diffuse interstitial pattern. Perfusion lung scan findings of an irregular peripheral perfusion defect were helpful in raising the suspicion of lymphangitic carcinomatosis in patients with normal or atypical chest X-ray findings. Patients who were managed by supportive care had a very short survival. Patients who were managed solely with either (single agent) chemotherapy, endocrine ablation surgery or whole lung irradiation failed to respond to treatment and also had a very short survival. Patients who were managed by whole lung irradiation and combination chemotherapy usually had a favorable response. Dyspnea improved and survival increased.