Safe and effective use of nivolumab for treating lung adenocarcinoma associated with sporadic lymphangioleiomyomatosis: a rare case report

BMC Pulm Med. 2019 Jan 11;19(1):12. doi: 10.1186/s12890-018-0775-5.


Background: Sporadic lymphangioleiomyomatosis (LAM) is a rare form of diffuse parenchymal lung disease. PD-1 blocking antibodies constitute an essential treatment option for advanced non-small-cell lung cancer (NSCLC). The effect of immune checkpoint inhibitors in lymphangioleiomyomatosis patients with non-small cell lung cancer is unknown: concomitant symptomatic interstitial lung disease or the use of immunosuppressors was a key exclusion criterion in the original studies of immune checkpoint inhibitors, especially regarding the risk of interstitial lung disease exacerbation.

Case presentation: A 48-year-old female, active smoker (36 pack-years), diagnosed with sporadic LAM since 2004 suffered from metastatic adenocarcinoma of the lung. Third-line therapy with nivolumab was started in 2015, with a major partial response. Due to pulmonary function alterations, sirolimus was also reinitiated in 2017 in conjunction with nivolumab, without any undesirable effects and a major partial response continuing up to May 2018.

Conclusions: This case highlights the safe and effective use of nivolumab for managing metastatic lung adenocarcinoma that occurred in a patient with sporadic LAM. In the current case, immunotherapy proved highly successful in managing the NSCLC tumor that occurred upon LAM follow-up, with both a significantly prolonged partial response and acceptable safety profile.

Keywords: Adenocarcinoma; Immune checkpoint inhibitors; NSCLC; Nivolumab; Sporadic lymphangioleiomyomatosis.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma of Lung / complications
  • Adenocarcinoma of Lung / drug therapy*
  • Adenocarcinoma of Lung / secondary
  • Adrenal Gland Neoplasms / secondary
  • Antineoplastic Agents, Immunological / therapeutic use*
  • Female
  • Humans
  • Lung Neoplasms / complications
  • Lung Neoplasms / drug therapy*
  • Lung Neoplasms / pathology
  • Lymphangioleiomyomatosis / complications*
  • Middle Aged
  • Nivolumab / therapeutic use*
  • Treatment Outcome


  • Antineoplastic Agents, Immunological
  • Nivolumab