Bolasco Giulia, Capriati Teresa, Grimaldi Chiara, Monti Lidia, De Pasquale Maria Debora, Patera Ippolita Patrizia, Spada Marco, Maggiore Giuseppe, Diamanti Antonella
Hepatology, Gastroenterology and Nutrition Unit, "Bambino Gesù" Children's Hospital, IRCCS, Rome 00165, Italy.
Division of Hepatobiliopancreatic Surgery, Liver and Kidney Transplantation, "Bambino Gesù" Children's Hospital, IRCCS, Rome 00165, Italy.
World J Clin Cases. 2021 Sep 6;9(25):7340-7349. doi: 10.12998/wjcc.v9.i25.7340.
Pancreatic neoplasms are uncommon in children and in most cases they are benign or have low malignant potential. Pancreatoblastoma and solid pseudopapillary tumor are the most frequent types in early and late childhood, respectively. Complete resection, although burdened by severe complications, is the only curative treatment for these diseases. Pancreatic surgery may result in impaired exocrine and endocrine pancreatic function. However, limited data are available on the long-term pediatric pancreatic function following surgical resection.
To investigate endocrine and exocrine pancreatic function and growth after oncological pancreatic surgery in a pediatric series.
A retrospective analysis of all pediatric patients who underwent surgery for pancreatic neoplasm in our Institution from January 31, 2002 to the present was performed. Endocrine and exocrine insufficiency, auxological and fat-soluble vitamin status (A, D, E and clotting tests) were assessed at diagnosis and at every follow-up visit. Exocrine insufficiency was defined as steatorrhea with fecal elastase-1 < 200 µg/g stool, while endocrine insufficiency was identified as onset of Diabetes or Impaired Glucose Tolerance. Growth was evaluated based on body mass index (BMI) -score trend.
Sixteen patients (12 girls and 4 boys, mean age 10.7 ± 5.3 years), were included. Nine patients (56%) had a neoplasm in the pancreatic head, 4 in the body/tail, 2 in the tail and 1 in the body. Histological findings were as follows: Solid pseudopapillary tumor in 10 patients (62.5%), insulinoma in 2 patients, neuroendocrine tumor in 2 patients and acinar cell carcinoma in 2 patients. The most frequent surgery was pancreaticoduodenectomy (50%). Exocrine failure occurred in 4 patients (25%) and endocrine failure in 2 patients (12.5%). Exocrine insufficiency occurred early (within 6 mo after surgery) and endocrine insufficiency later (8 and 10 years after surgery). Mean BMI -score was 0.36 ± 1.1 at diagnosis and 0.27 ± 0.95 at the last assessment. Vitamin D was insufficient (< 30 ng/mL) in 8 of the 16 patients during the follow-up period. Vitamins A, E and clotting test were into the normal ranges in all patients.
Careful and long-term monitoring should follow any pancreatic surgery, to recognize and promptly treat exocrine and endocrine pancreatic insufficiency, which can occur after surgery.
胰腺肿瘤在儿童中并不常见，大多数情况下为良性或恶性潜能较低。胰母细胞瘤和实性假乳头状瘤分别是儿童早期和晚期最常见的类型。尽管存在严重并发症，但完整切除是这些疾病唯一的治愈性治疗方法。胰腺手术可能导致胰腺外分泌和内分泌功能受损。然而，关于手术切除后儿童胰腺功能的长期数据有限。
在一组儿科患者中研究胰腺肿瘤手术后的内分泌和外分泌胰腺功能及生长情况。
对2002年1月31日至目前在我院接受胰腺肿瘤手术的所有儿科患者进行回顾性分析。在诊断时及每次随访时评估内分泌和外分泌功能不全、生长发育及脂溶性维生素状态（维生素A、D、E及凝血检查）。外分泌功能不全定义为粪便弹性蛋白酶-1<200μg/g粪便且伴有脂肪泻，而内分泌功能不全定义为糖尿病或糖耐量受损的发生。根据体重指数（BMI）评分趋势评估生长情况。
纳入16例患者（12例女孩和4例男孩，平均年龄10.7±5.3岁）。9例患者（56%）肿瘤位于胰头，4例位于体/尾部，2例位于尾部，1例位于体部。组织学检查结果如下：10例患者（62.5%）为实性假乳头状瘤，2例为胰岛素瘤，2例为神经内分泌肿瘤，2例为腺泡细胞癌。最常见的手术是胰十二指肠切除术（50%）。4例患者（25%）出现外分泌功能衰竭，2例患者（12.5%）出现内分泌功能衰竭。外分泌功能不全发生较早（术后6个月内），内分泌功能不全发生较晚（术后8年和10年）。诊断时平均BMI评分0.36±1.1，最后一次评估时为0.27±0.95。随访期间16例患者中有8例维生素D不足（<30ng/mL）。所有患者的维生素A、E及凝血检查均在正常范围内。
任何胰腺手术后都应进行仔细的长期监测，以识别并及时治疗手术后可能出现的胰腺外分泌和内分泌功能不全。
