To Be or Not To Be a Neoplasm: What Is Lymphangioleiomyomatosis? Are We Calling It What It Really Is?

摘要

As an academic pulmonologist who cares for women with lymphangioleiomyomatosis (LAM) and directs a LAM Clinic, I find it provocative to propose, as McCormack and colleagues (1) have, that LAM should be called a "low grade, destructive, metastasizing neoplasm." The idea that LAM is a neoplasm, however, is not new and has surfaced in the literature for more than two decades. Yet I am faced with a level of uneasiness and confusion with the current declaration of LAM as a cancer. The authors express their own confusion through a series of questions that conclude by suggesting that the conventional labels, "benign" or "malignant," be accompanied by the descriptive modifiers of a neoplasm. Does this make LAM a "cancer" or not if it is a destructive and metastasizing process? Does the statement, "LAM cells have growth-promoting DNA mutations, evidence of clonal origins, invasive and metastatic potential, and metabolic profiles" make LAM "entirely consistent with a neoplastic process"? Does the fact that LAM results in "remote tissue destruction, progressive respiratory failure and death or need for lung transplantation" further support LAM as a neoplastic process? I would propose that the jury is undecided.