PULMONARY LYMPHANGIOLEIOMYOMATOSIS. A CASE REPORT AND REVIEW

Pulmunary LymphangioIeiomyomatosis (P-LAM ) is a rare disease that occurs mainly in women of childbearing age. The most common presenting symptom of the disease is spontaneous pneumothorax. Histologically, it is characterized by hamartomatous proliferation of smooth muscle cells around and within the pulmonary lymphatics, venules, and airways, which leads to cystic degeneration of the lung interstitium without predilection of any lung zone. Extrapulmonary manifestations of P-LAM are renal angiolipomas and enlarged retroperitoneal, paraaortic or pelvic lymph nodes; pathological findings are identical to those found in TSC (Tuberous Sclerosis Complex). TSCI and TSC2 are two suppressor genes: since the loss of heterozygosity of these genes occurs in P-LAM patients, some authors hypotized that PLAM and TSC have common genetic bases. The fact that pregnancy is reported to develop the disease process and the cases of postmenopausal women associated with estrogen replacement therapy suggests that a hormonal treatment could be used, although its results are largely ineffective. In recent years lung transplantation has become a therapeutic approach. Extensive pleural adhesions cause intraoperative problems, but the most important posttransplantation complications are pneumothorax in the native lung or chylothorax or recurrent P-LAM in the lung allograft, even in women who received a transplant from a male donor. With the improved understanding of genetic biology of PLAM, new therapies will soon become available. We report the case of a 35 years old woman, who developed spontaneous pneumothorax . P-LAM was suspected on the chest TC scans and was confirmed by a VATS lung biopsy.