Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Splenic and adrenal lymphangiomatosis. 1451 Jun 33

Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified.
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PMID:Selected case from the Arkadi M. Rywlin international pathology slide series: lymphangiomatosis of the spleen associated with ipsilateral abdominopelvic and lower extremity venolymphatic malformations: a case report and review of the literature. 2491 Dec 54