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Chylothorax and Sternal Destruction Secondary to Lymphangioma (Gorham’s Syndrome)

St. Elizabeth’s Medical Center, Boston, MA

INTRODUCTION: Neoplasms of lymphatic vessels have varied presentations; a case of lymphangioma causing chylothorax and bone destruction, known as Gorham’s syndrome, will be discussed.

CASE PRESENTATION: 22 year-old male with 8 months of progressive retrosternal discomfort and dry cough. He had no medical or surgical problems, or allergies. Denied toxic exposures, chest trauma or weight loss.

He was well-built, hemodinamically stable and afebrile. Decreased breath sounds were noted in the right hemithorax; the sternum appeared to sink on inspiration. Routine labs were normal; chest x-ray revealed a large right-sided pleural effussion. Thoracentesis yielded four liters of brown fluid; analysis showed normal lactic dehydrogenase and protein, no organisms, negative culture and cytology, and a triglyceride level of 1450.

Tuberculin test, autoimmune workup, and human immunodeficiency virus assay were negative. Galium scan, blood flow cytommetry, and bone marrow biopsy were normal. Thoracoscopy was unremarkable. A lymphatic scan showed no activity in the area of thoracic duct.

Chest CT scan (fig.1 ) showed irregularity and heterogeneity of the sternum. Sternal biopsy revealed destruction of the manubrium, and replacement by a fibrous mass. Pathology revealed sponge-like channels lined by factor VIII (+) endothelial cells (fig. 2) and destruction of adjacent bone, compatible with lymphangioma.

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DISCUSSION: Trauma and neoplasms are the most frequent causes of chylothorax (12).

Lymphangiomas typically occur in children, but may develop at any age. Symptoms arise from compression of adjacent structures (3, 4).

Radiologically, lymphangiomas appear as cystic masses; mediastinal widening and pleural effussions are common (5). Lymphangiogram may show lesions of the thoracic duct and dylated lymphatics (6).

Histologically, there are large, irregular vascular spaces lined by well-differentiated endothelial cells (7) that stain positive for Factor VIII and CD31 (8).

The association between localized bone destruction and chylothorax with lymphangiomas was described by Gorham and Stout in 1955 (9).

Surgical exploration and resection, with low, right-sided ligation of the thoracic duct and pleurodesis is recommended. Treatment of bone destruction consists of resection, bone implants, and/or radiotherapy (4). Adjuvant use of clodronate and alfa 2b interferon has been reported (10).

CONCLUSION: Chylothorax in association with localized bone destruction suggests the presence of lymphangioma. The manifestations of these tumors will depend on the structures affected. Multiple therapeutic interventions are recommended.

DISCLOSURE: E. Velez, None.

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