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Pulmonary lymphangiomyomatosis.

R L Shuman, R Engelman, C F Kittle

The Annals of Thoracic Surgery

|January 1, 1979

Summary

This summary is machine-generated.

Pulmonary lymphangiomyomatosis (PLAM) is a rare lung disease affecting women, causing breathing problems and fluid buildup. Estrogen may drive PLAM, suggesting hormone therapy could be beneficial.

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Area of Science:

Pulmonology
Pathology
Oncology

Background:

Pulmonary lymphangiomyomatosis (PLAM) is a rare, distinct clinical and pathological entity.
Characterized by hamartomatous proliferation of smooth muscle around lymphatic vessels in the lung, mediastinum, and retroperitoneum.

Observation:

Exclusively affects menstruating women and girls.
Manifests as spontaneous pneumothorax and chylous pleural or abdominal effusion.
Progressive lung involvement leads to pulmonary insufficiency and recurrent infections, often resulting in death.

Findings:

PLAM has not been previously reported in the surgical literature.
Thoracic surgeons play a role in diagnosis and palliation.
The disease is hypothesized to be estrogen-dependent.

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Implications:

Estrogen ablation is suggested for patients with positive estrogen receptors.
Highlights the need for increased awareness and reporting of PLAM in surgical contexts.
Suggests potential therapeutic avenues targeting hormonal pathways in PLAM management.