# Spontaneous Hydropneumothorax After Pleurodesis in a Patient With Pulmonary Lymphangioleiomyomatosis With Associated Endometrial Cancer

## TL;DR

A rare case of a patient with LAM and endometrial cancer developed a pneumothorax after pleurodesis, highlighting the need for close monitoring and careful treatment in high-risk patients.

## Contribution

Reports a rare case of LAM and endometrial cancer co-occurring with a post-pleurodesis hydropneumothorax.

## Key findings

- LAM patients are at high risk for recurrent pneumothorax despite pleurodesis.
- LAM and endometrial cancer may co-occur due to shared mTOR pathway activation.
- Pleurodesis in high-risk LAM patients should prioritize procedures with the lowest recurrence rates.

## Abstract

Lymphangioleiomyomatosis (LAM) is a rare cystic disease that occurs due to the abnormal proliferation of smooth muscle-like cells. It primarily affects the lungs but can also have extrapulmonary manifestations such as lymphangioleiomyoma and angiomyolipomas. It is more common in young women of childbearing age, with female sex hormones contributing to the disease course. LAM can develop either through sporadic mutations or through genetic inheritance of the tuberous sclerosis complex (TSC) genes. TSC, LAM, and endometrial cancer are associated with mTOR pathway activation, which can explain why these diseases can co-exist, although the co-existence of LAM and endometrial cancer in the same patient is very rare. Due to the cystic nature of LAM, pneumothorax most often occurs at least once during the course of the disease, and most times, it is the first manifestation observed in LAM. These patients are also at high risk for recurrent pneumothorax, and when that occurs, pleurodesis is indicated. Unfortunately, pleurodesis still does not preclude a pneumothorax from occurring. We present the case of a female patient with LAM and endometrial cancer who was found to have an incidental spontaneous hydropneumothorax after pleurodesis. Patients with LAM should be closely monitored for the possible development of other mTOR-associated diseases. Moreover, when performing pleurodesis for recurrent pneumothorax in very high-risk patients, the procedure with the lowest recurrence rate should be utilized.