Long-term Survival in a Child with Malignant Insulinoma After Liver Transplantation

被引:1
作者
Moszczynska, Elzbieta [1 ]
Wydra, Arnika [2 ]
Zasada, Klaudia [1 ]
Baszynska-Wilk, Marta [1 ]
Majak, Dorota [3 ]
Sliwinska, Anna [4 ]
Grajkowska, Wieslawa [5 ]
机构
[1] Childrens Mem Hlth Inst, Dept Endocrinol & Diabetol, Warsaw, Poland
[2] Bielanski Hosp, Ctr Postgrad Med Educ, Dept Endocrinol, Warsaw, Poland
[3] Childrens Mem Hlth Inst, Dept Diagnost Imaging, Warsaw, Poland
[4] Childrens Mem Hlth Inst, Dept Nucl Med, Warsaw, Poland
[5] Childrens Mem Hlth Inst, Dept Pathol, Warsaw, Poland
关键词
Hypoglycemia; insulinoma; liver transplantation; children; MANAGEMENT; TUMORS;
D O I
10.4274/jcrpe.galenos.2022.2022-3-5
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Insulinoma is one of the pancreatic neuroendocrine tumors (PanNET) and is exceptionally rare in children. The tumor leads to severe hypoglycemia caused by excessive insulin release. We report a pediatric patient with malignant insulinoma who underwent liver transplantation (LT) due to liver metastases of the insulinoma. A 13 -year -old girl presented with symptoms of hypoglycemia due to hyperinsulinism. On computed tomography (CT), a polycystic lesion in the head of the pancreas and enlarged lymph nodes were revealed. A modified Whipple's operation was performed, and histological examination confirmed PanNET. CT also showed an enlarged liver with numerous metastases. Allogeneic LT was carried out successfully. Positron emission tomography -CT using 68Ga-DOTA-labeled somatostatin analogs (SSAs) at the age of 22 years confirmed complete metabolic remission. The patient currently remains under immunosuppressive and anti -proliferative treatment. Multiple surgical interventions, LT combined with SSAs, and immunosuppressive medication proved effective in this case of metastatic malignant insulinoma.
引用
收藏
页码:106 / 110
页数:5
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