Abstract
BackgroundCastleman's disease or angiofollicular lymphoid hyperplasia is a rare benign lymph node hyperplasia usually presenting as an asymptomatic mediastinal mass in children. The disease can present at any extra thoracic site with lymphoid tissue such as retroperitoneal, mesentery, axilla, and pelvis. Hepatic localization castleman disease is very rare in children. Herein, we reported a case of Castleman's disease arising from the lymph node in hilum of liver.Case presentationA 5 -year-old girl with chief complaint of abdominal pain for two months which exaggerated in last three days was referred to the hospital. On routine physical examination, only a generalized abdominal pain was noticed. Routine laboratory investigations and Chest X-Ray were normal. Abdominal Sonography revealed a 3.7 × 3.1 cm solid mass in the hilum of the liver. On the MRI images, a lobulated mass in the portal hepatic associated with mass effect on the portal vein was visible. Histological examination revealed expansion of mantle zone in lymphatic nodules accompanied by burnt out germinal centers. This pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease. The patient underwent a laparotomy. The patient had an uneventful postoperative course.ConclusionThis pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease.
Highlights
Castleman’s disease, first described in 1956, is a rare lymphoproliferative disorder, which commonly found in mediastinum and lung hila
Only a generalized abdominal pain was noticed, and the family only complained of nocturia during the period of the disease
Kujat et al in 1990 described one patient with a lesion arising in the spleen, that described by [5], in which histological examination showed a mixture of both hyalinevascular and plasma cell types
Summary
Castleman’s disease, first described in 1956, is a rare lymphoproliferative disorder, which commonly found in mediastinum and lung hila. Extrathoracic site of disease is uncommon but not unknown It is divided in two types: a localized form, which is usually asymtomatic and presented as a mass or swelling, and a multicentric type characterized by fever with chills, anaemia, generalized lymphadenopathy and hepatosplenomegaly [1]. Abdominal sonography revealed a 3.7 × 3.1 cm solid mass in the hilum of the liver that shifted the kidney to the right. The mass was low signal intensity on T1 and high signal intensity on T2 weighted images. This was similar to a lymph node. The patient underwent a laparotomy that revealed a solid, homogenous mass adjacent to the right lobe of liver.
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