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肝移植后恶性胰岛素瘤患儿的长期生存情况

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

作者信息

Moszczyńska Elżbieta, Wydra Arnika, Zasada Klaudia, Baszyńska-Wilk Marta, Majak Dorota, Śliwińska Anna, Grajkowska Wiesława

机构信息

The Children’s Memorial Health Institute, Department of Endocrinology and Diabetology, Warsaw, Poland

Bielański Hospital, Center of Postgraduate Medical Education, Department of Endocrinology, Warsaw, Poland

出版信息

J Clin Res Pediatr Endocrinol. 2024 Mar 11;16(1):106-110. doi: 10.4274/jcrpe.galenos.2022.2022-3-5. Epub 2022 Sep 1.

Abstract

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 Ga-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.

摘要

胰岛素瘤是胰腺神经内分泌肿瘤(PanNET)之一,在儿童中极为罕见。该肿瘤因胰岛素过度释放导致严重低血糖。我们报告一例患有恶性胰岛素瘤的儿科患者,因其胰岛素瘤肝转移而接受了肝移植(LT)。一名13岁女孩因高胰岛素血症出现低血糖症状。计算机断层扫描(CT)显示胰腺头部有一个多囊性病变和肿大的淋巴结。实施了改良的惠普尔手术,组织学检查确诊为PanNET。CT还显示肝脏肿大并有多处转移。成功进行了同种异体肝移植。患者22岁时使用镓-多胺多乙酸标记的生长抑素类似物(SSA)进行的正电子发射断层扫描-CT证实完全代谢缓解。该患者目前仍在接受免疫抑制和抗增殖治疗。在这例转移性恶性胰岛素瘤病例中,多次手术干预、肝移植联合SSA以及免疫抑制药物治疗被证明是有效的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/987f/10938515/82363e5b5097/JCRPE-16-106-g1.jpg

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