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Case Nine - Lymphangiomyomatosis

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Clinical History: A 39 year old female non-smoker who presented with increasing shortness of breath. Status post resection of angiomyolipoma in childhood. History of use of oral contraceptives.

Findings: Diffuse, numerous thin-walled cysts of various sizes scattered throughout all lung zones.

Diagnosis: Lymphangiomyomatosis

Discussion: Lymphangiomyomatosis is a rare disorder affecting women of child-bearing age (17-50 years). The disease is characterized by gradually progressive diffuse interstitial lung disease, recurrent chylous effusions and recurrent pneumothoraces (1). Pathologically there is proliferation of atypical smooth muscle cells in pulmonary lymphatic chanels, blood vessels and airways. This results in airway obstruction, pulmonary edema and lymphatic obstruction with subsequent chylous effusion (2). Patients present with increasing shortness of breath, hemophysis (30-40%) and chylaptysis. Pulmonary function tests show severe airway obstruction. The disease may be associated with Tuberous Sclerosis (3). Symptoms may be worsened by use of birth control pills
Classic radiographic signs include (4):

Coarse reticular interstital pattern
Recurrent large chylothorax (50-75%, uni- or bilateral)
Recurrent pneumothorax (40%)
Increasing lung volumes
Kerley-B lines
Pulmonary cysts/honeycombing.

CT findings include (5).

Numerous randomly scattered thin-walled cysts
.5 to 5 cm in size (most 2 cm),surrounded by normal parenchyma by normal parenchyma.
Bronchovascular bundles at periphery of cyst walls.

Prognosis is bleak, most patients die within 10 years. Differential diagnosis should include Histiocytosis (cyst walls may be thicker with associated interstitial nodularity) and emphysema.

References:
1. Cornog JL, Enterline HT. Lymphangiomyoma, a benign lesion of chyliferous lymphatics
synonymous with lymphangiopericytoma. Cancer 1966; 19:1909.
2. Corrin B. Liebow AA, Friedman PJ. Pulmonary lymphangiomyomatosis. Am J Pathol
1975; 79:347.
3. Vaieris QJ. Pulmonary lymphangiomyoma, a probable Form Frost of Tuberous
Sclerosis. A case report and survey of the literature. Am Rev Respir Dig 1973; 108: 1411.
4. Silverstein EF, Ellis K. Wolff M et al. Pulmonary lymphangiomyomatosis. Am J
Roentgenol 1974; 120: 832.
5. Rappaport DC, Weisbrod GL, Hermon SJ et al. Pulmonary lymphangioleiomyomatosis:
High-resolution CT findings in four cases. Am J Roentgenol 1989; 152: 961.

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Submitted by:
Terrence J. Lewis, M.D.
Virginia L. Lampert, M.D.