Korean J Med.
2000 Feb;58(2):234-239.
A case of systemic lymphangiomatosis with splenic involvement
- Affiliations
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- 1Department of Internal Medicine, College of Medicine, Institute of Digestive Diseases and Nutrition, Korea Universitiy, Seoul, Korea.
Abstract
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Lymphangiomatosis is a very rare and slow-growing benign tumor generally accepted to be the result of a
congenital malformation of the lymphatic system. It is most commonly found in the neck, axilla and less commonly
retroperitoneum, mediastinum, mesentery, omentum, pelvis, bone, skin, scrotum, and spleen. We present a patient
who had systemic lymphangiomatosis with splenic involvement. She was presented with the cystic neck mass suspected
to be a cystic hygroma during the first decade. She complained of the mass on left upper quadrant of abdomen at the age
of 28. Under full investigations, we had diagnosed the lymphangiomatosis involving spleen and right adrenal gland, and
splenectomy was done. We present a case of systemic lymphangiomatosis with emphasis on diagnosis, management,
and prognosis.