Splenic and adrenal lymphangiomatosis

Lymphangiomatosis is a rare syndrome that can affect different organs, although simultaneous splenic and adrenal involvement is exceptional. We report the case of a young female with a symptomatic massive splenomegaly and no-nhypersecreating bilateral adrenal masses incidentally discovered that represented a diagnostic challenge. The management of adrenal incidentalomas is controversial, as the presence of large sized bilateral masses are highly indicative of malignancy. Despite the different diagnostic techniques available, it is sometimes impossible to reach an accurate preoperative diagnosis. Elective splenectomy and left adrenalectomy were performed, preserving the functioning of the right adrenal gland, radiologically similar to the left one, to prevent the development of definitive adrenal insufficiency. Postoperative evolution has been favourable without initial increase of the size of the right adrenal lesion. However, the persistence of this mass obligated long-term follow-up and shall assist us in better understanding the behavior of this bening lesion.