Korean J Radiol.  2002 Jun;3(2):130-132. 10.3348/kjr.2002.3.2.130.

Mediastinal Lymphangioma and Chylothorax: Thoracic Involvement of Gorham's Disease

• Yoo SY
• Goo JM
• Im JG

• KMID: 754072
• DOI: http://doi.org/10.3348/kjr.2002.3.2.130

Abstract

We report a case of mediastinal lymphangioma associated with Gorham's disease in a 38-year-old man who had suffered recurrent clavicular fractures during a seven-year period. Mediastinal widening associated with osteolysis of the clavicles and the sternal manubrium was revealed by chest radiography, while computed tomography demonstrated a cystic anterior mediastinal mass infiltrating mediastinal fat and associated with osseous destruction of the clavicles and manubrium. Chylothorax recurred during the course of the disease.

Keyword

Computed tomography (CT); Lymphangioma; Mediastinum

MeSH Terms

Adult
Case Report
Chylothorax/*complications
Human
Lymphangioma/*complications/radiography
Male
Mediastinal Neoplasms/*complications/radiography
Osteolysis, Essential/*complications/pathology/radiography
Thoracic Vertebrae/*pathology
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Gorham's disease in a 38-year-old man with mediastinal lymphangioma. A. Posteroanterior chest radiograph shows widening of the mediastinum and osteolysis of the proximal clavicles. B, C. Contrast-enhanced CT scan demonstrates an anterior mediastinal mass of low attenuation, which infiltrates mediastinal fat and envelops adjacent structures. Lysis of the manubrium (arrow in B) is apparent and follow-up CT (C) after three months shows left pleural effusion. D. Irregular-shaped dilated lymphatic spaces lined by attenuated endothelium are embedded in connective tissue stroma, and small lymphoid aggregates are dispersed in the stroma. These findings are compatible with cavernous lymphangioma (original magnification, ×250; hematoxylin-eosin staining).

Reference

1. Jackson JBS. A boneless arm. Boston Med Surg J. 1838. 18:368–369.

2. Fisher KL, Pogrel MA. Gorham's syndrome (massive osteolysis): A case report. J Oral Maxillofac Surg. 1990. 48:1222–1225.

3. Halliday DR, Dahlin DC, Pugh DG, Young HH. Massive osteolysis and angiomatosis. Radiology. 1964. 82:637–644.

4. Pedicelli G, Mattia P, Zorzoli AA, Sorrone A, De Martino F, Sciotto V. Gorham's syndrome. JAMA. 1984. 252:1449–1451.

5. Tie ML, Poland GA, Rosenow EC. Chylothorax in Gorham's syndrome: A common complication of a rare disease. Chest. 1994. 105:208–213.

6. Nicolas C, Philippe C, Gil F, Gabrielle V, Pierre-Yves B. Chylothorax complicating Gorham's disease. Ann Thorac Surg. 2001. 72:937–939.

7. Brown LR, Reiman HM, Rosenow EC, Gloviczki PM, Divertie MB. Intrathoracic lymphangioma. Mayo Clin Proc. 1986. 61:882–892.

8. Michael B. Mediastinal lymphangioma. AJR. 1992. 159:218–219.

9. Pilla TJ, Wolverson MK, Sundaram M, Heiberg E, Shields JB. CT evaluation of cystic lymphangioma of the mediastinum. Radiology. 1982. 144:841–842.