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

IF 1.5 4区 医学 Q4 ENDOCRINOLOGY & METABOLISM Journal of Clinical Research in Pediatric Endocrinology Pub Date : 2024-03-11 Epub Date: 2022-09-01 DOI:10.4274/jcrpe.galenos.2022.2022-3-5
Elżbieta Moszczyńska, Arnika Wydra, Klaudia Zasada, Marta Baszyńska-Wilk, Dorota Majak, Anna Śliwińska, Wiesława Grajkowska
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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 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.

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一名患恶性胰岛素瘤的儿童肝移植后的长期存活。
胰岛素瘤属于胰腺神经内分泌肿瘤,在儿童中极为罕见。这种肿瘤会因胰岛素释放过多而导致低血糖的严重后果。我们报告了一名患有恶性胰岛素瘤的儿童患者,该患者因肝脏转移而接受了肝移植手术。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,胰腺头部有多环病变,淋巴结肿大。患者接受了改良Whipple手术,组织学检查证实为胰腺神经内分泌肿瘤。全身 CT 显示肝脏肿大,并有大量转移灶。成功进行了异体肝移植。22 岁时,使用 68Ga-DOTA 标记的体生长抑素类似物进行的正电子发射断层扫描-计算机断层扫描(PET/CT)证实,患者的代谢完全缓解。患者目前仍在接受免疫抑制和抗增生治疗。多种手术干预、肝移植结合使用体生长激素类似物以及免疫抑制药物对恶性胰岛素瘤可能有效。
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来源期刊
Journal of Clinical Research in Pediatric Endocrinology
Journal of Clinical Research in Pediatric Endocrinology ENDOCRINOLOGY & METABOLISM-PEDIATRICS
CiteScore
3.60
自引率
5.30%
发文量
73
审稿时长
20 weeks
期刊介绍: The Journal of Clinical Research in Pediatric Endocrinology (JCRPE) publishes original research articles, reviews, short communications, letters, case reports and other special features related to the field of pediatric endocrinology. JCRPE is published in English by the Turkish Pediatric Endocrinology and Diabetes Society quarterly (March, June, September, December). The target audience is physicians, researchers and other healthcare professionals in all areas of pediatric endocrinology.
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