Abstract

The lymphatic abnormalities should be considered as entities with local and systemic involvement, of continuous spectrum, being in the majority of cases a diagnosis of exclusion, making the differential diagnosis between entities difficult.

Gorham Stout syndrome should be suspected in a patient with rapid and progressive osteolysis findings with cortical bone loss and presence of bilateral pleural effusion of chylous features. Considering the use of radiological studies and biopsy of the lesion as diagnostic methods. Prioritizing chylothorax treatment to be present.

Generalized lymphatic abnormality or lymphangiomatosis is suspected in similar symptoms, without progressive bone involvement.

Management is complicated, as there are no clinical trials. It is suggested to start with conservative management without the cynical situation of the patient allows it, emphasizing nutrition and the priority treatment of chylothorax if present, resorting to alternatives such as radiotherapy or surgery but does not achieve adequate control.

Keywords: Lymphangiomatosis; Heterogeneity; Sporadic malformations; PI3K/AKT/mTOR pathway; RAS pathway