53. Lymphangioleiomyomatosis

Definition

Lymphangioleiomyomatosis (LAM) affects women during the childbearing years. It is characterized by proliferation of smooth muscle phenotypic neoplastic cells in the lungs, kidneys, and axial lymphatics. The proliferation results in cystic destruction of the lung with progressive pulmonary dysfunction and abdominal tumors.

Incidence

The incidence of LAM is unknown. More than 500 cases had been documented in the United States as of 2006.

Etiology

The etiology of LAM is unknown. The disease occurs predominately in premenopausal women and seems to be exacerbated by elevated estrogen states, suggesting that female hormones may have a prominent role in the cause.

Signs and Symptoms

• Chest pain

• Chylothorax

• Chylous ascites

• Chyluria (chyle in the urine)

• Cough

• Dyspnea

• Lymphedema

• Pleural effusions

• Pneumoperitoneum

• Pneumothorax(-aces)

Medical Management

Generalized medical care of the patient with LAM is directed toward treatment of specific symptoms.

Pleural effusions may be treated with chemical pleurodesis or surgical obliteration of the pleural space. Pulmonary dysfunction is treated with good, generalized pulmonary care incorporating bronchodilators and supplemental oxygen. In extreme circumstances, lung transplantation may be considered.

Ascites may be treated with paracentesis to remove the fluid.

Hormonal manipulation has been advocated. Medroxyprogesterone and gonadotropin-releasing hormone agonists have been advocated, but case reports are inconclusive. More recent experimental drug therapies include sirolimus, doxycycline, and octreotide.

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