Lymphangioleiomyomatosis and metastatic adenocarcinoma of the lung

Today Dr. Govert presented an interesting case of recurrent pneumothorax with CT findings suggestive of bullous emphysema/cystic lung disease. Biopsy ultimately demonstrated lymphangioleiomyomatosis (LAM), shout out to Anmol for the mic drop diagnosis upon entering the room. Ken Lyles would have been proud. Anyways, we briefly discussed the use of sirolimus for LAM. I’ve attached the NEJM RCT comparing sirolimus v placebo in 89 women with LAM. In short, those treated with sirolimus had less decline in FEV1 compared to placebo. They also had lower levels of VEGF-D and improved scores on a quality of life and functional performance survey. During a 12 month observational period of withholding sirolimus (after those randomized to sirolimus were treated), the decline in lung function resumed in the sirolimus group at a similar rate to those in the placebo group.

Nick presented an interesting case of RUL adenocarcinoma of the lung that presented without pulmonary symptoms, but instead with LLQ pain, back pain, anorexia, 25 lb weight loss, and nystagmus/dizziness. Imaging ultimately left adrenal mass and cerebellar mass. We discussed what would likely be first line treatment option, and assuming NGS testing was negative for an actionable mutation (such as EGFR), he would likely be a candidate for chemo + pembrolizumab. In the KEYNOTE-189 phase 3 trial, 616 patients with nonsquamous NSCLC were randomized to pemetrexed + platinum-based chemo plus pembrolizumab or placebo. There was superior OS at 12 months in the pembrolizumab containing arm (69.2% versus 49.4%), and improved PFS (8.8 months v. 4.9 months).

As a fun crossover, I also attached a case report in which a patient with LAM who developed metastatic adenocarcinoma of the lung was successfully treated with nivolumab.

Literature references:

Sirolimus_in_LAM

Pembro+ChemoNSCLC

LAM+NSCLC