Lymphangitic Carcinomatosis

Lymphangitis carcinomatosis is the malignant infiltration and inflammation of lymphatic vessels secondary to the metastatic spread of malignancy from a primary site. The carcinomatous infiltration of the lymphatic vessels almost always occurs in the pulmonary interstitial lymphatics (pulmonary lymphangitic carcinomatosis) though rare reports of lymphangitic carcinomatosis of skin, duodenum & kidney(non-pulmonary lymphangitic carcinomatosis) have been described in the literature. Our focus of attention in this review is about pulmonary lymphangitic carcinomatosis, which almost always represents the clinical form of lymphangitic carcinomatosis. The terms pulmonary lymphangitic carcinomatosis & pulmonary tumor embolism represent end-stage manifestations of malignancies and have been used synonymously in the literature given highly similar clinical & radiological features though the former represents a pathology predominantly restricted to the interstitium with absent tumor cells in pulmonary arteries and or capillaries, whereas the latter demonstrates the presence of tumor cells inside the pulmonary vessels. Dr. Gabriel Andral was the first to describe pulmonary lymphangitic carcinomatosis in 1829 in a case of uterine malignancy. Subsequently, Troissier & Reynaud made a detailed description of the gross anatomy & histopathology in 1874. Although pulmonary lymphangitic carcinomatosis usually represents an end-stage malignancy with poor life expectancy, reports of increased survival have been observed in recent literature. The commonest causes for lymphangitic carcinomatosis include breast, lung, stomach, prostate, pancreas, colon, cervix, & uterine malignancies.