Lymphangiomatosis

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Lymphangiomatosis is a condition where a lymphangioma is not present in a single localised mass, but in a widespread or multifocal manner. It is a rare neoplasm which results from an abnormal development of the lymphatic system. This may involve one or several organs.

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[edit] Synonyms

Lymphangiomatosis is known under the eponym Gorham's disease, vanishing / disappearing bone disease, Gorham-Stout syndrome, idiopathic / progressive massive osteolysis, essential osteolysis, phantom bone disease, essential osteolysis, and combinations of the previous.

Lymphangiomatosis should not be confused with lymphangioleiomyomatosis, which is a disease resulting from the proliferation of a cell type in the lymphatics that is similar (phenotypically) to a smooth muscle cell.

[edit] Signs and symptoms

Lymph vessels growing into bones may cause pathological fractures, and acute or chronic pain. In children it may cause bone deformity. When detected early, radiographic evidence of decreased bone mass can be found.

Pericardial or pleural effusion can also be a symptom. Lymphangiomatosis can involve tissues like the lung.

[edit] Diagnosis

The symptoms and typical radiographical findings can hint the diagnosis. A biopsy is mandated.

[edit] External links