Pancreatic masses are extremely rare in pediatric patients, with limited data available. This lack of data makes the diagnosis and management of these tumors in children extremely challenging. Therefore, we aimed to describe the presentations, clinical course, and outcomes of children with pancreatic tumors at our center. A retrospective analysis was performed of all pediatric patients diagnosed with pancreatic masses between 2003 and 2022 in an academic freestanding children\'s hospital. Data including demographics, clinical presentation, workup, management, and subsequent morbidity and mortality were collected and aggregated. Furthermore, we reviewed cases of pancreatic tumor resections in the National Surgical Quality Improvement Program - Pediatric (NSQIP-P) database to identify common adverse outcomes and measures for quality improvement. In total, 17 patients were identified at our institution. Diagnoses included solid pseudopapillary (n = 9), gastrinoma (n = 1), rhabdomyosarcoma (n = 2), pancreatoblastoma (n = 2), and insulinoma (n = 1). Two patients did not have a histopathologic diagnosis and were excluded from subsequent analysis. Overall, 12 patients underwent surgical intervention, with the most common procedures being pancreaticoduodenectomy and distal pancreatectomy, and all 12 were known to be alive at last contact. There were 3 deaths, all due to complications related to metastatic disease. Furthermore, 30-day postoperative outcomes in the NSQIP-P dataset for pancreatic surgeries in pediatric patients are excellent, with negligible morbidity and no mortalities after the index surgery.
CONCLUSIONS: Children with pancreatic tumors amenable to surgical resection appear to have adequate long-term survival. Short-term outcomes at diagnosis are excellent and mainly appear to be influenced by the presence of metastatic disease at initial presentation.
BACKGROUND: • Pancreatic masses are a rare entity in children with limited data on their presentation, management and surgical outcomes. • Solid Pseudopapillary tumors are one of the most common pancreatic tumors in children with a fair prognosis after surgical intervention.
BACKGROUND: • Surgical management of pediatric patients with pancreatic tumors is safe and effective in patients who do not have aggressive tumor types or metastatic disease. • Our case series provides a notable cohort of these pancreatic tumors with insight into the presentation, management and outcomes of five of these tumor types.

We demonstrate robot-assisted treatment of a patient with benign pancreatic insulinoma. A 31-year-old patient suffered from attacks of weakness, numbness of the fingertips and «turbidity of consciousness» for 2 years. These symptoms occurred on an empty stomach and regressed after eating. We found pancreatic insulinoma. The patient underwent robotic enucleation of pancreatic tumor. Surgery time was 145 min. Postoperative period proceeded without complications. Hyperglycemia up to 10.5 mmol/l on the first postoperative day was followed by normalization after 4 days. The patient was discharged in 6 days after surgery. Minimally invasive robotic enucleation of insulinoma minimizes surgical trauma and provides precise resection of tumor. The key aspect of safe enucleation is localization of tumor at a distance of at least 2 mm from the pancreatic duct.
Продемонстрирован опыт лечения пациента с инсулиномой поджелудочной железы методом робот-ассистированной энуклеации опухоли. Пациент, 31 год, в течение 2 лет отмечал приступы слабости, онемение кончиков пальцев на руках, «помутнения сознания», которые возникали натощак и купировались приемом пищи. При клинико-инструментальном обследовании обнаружена опухоль в теле-хвосте поджелудочной железы, выставлен диагноз инсулиномы. Пациенту выполнена робот-ассистированная энуклеация опухоли поджелудочной железы, длительность вмешательства составила 145 мин. Послеоперационный период протекал без осложнений. В первые послеоперационные сутки наблюдалась гипергликемия до 10,5 ммоль/л с последующей полной компенсацией гликемии до нормальных значений к 4 сут послеоперационного периода. Пациент выписан на 6-е сутки после операции. Применение робот-ассистированного мини-инвазивного доступа при выполнении энуклеации инсулином поджелудочной железы позволяет прецизионно удалить опухоль и минимизировать операционную травму. Ключевым моментом возможности безопасного выполнения энуклеации является расположение опухоли на расстоянии не менее 2 мм от протока поджелудочной железы.

OBJECTIVE: This retrospective study evaluates the value of 68Ga-DOTATATE PET/CT in the diagnosis and localization of insulinomas, whether sporadic, malignant or MEN-1 associated insulinoma.
METHODS: The study included 43 patients, having clinical (symptomatic hypoglycemia) and/or laboratory suspicion of having insulinoma (72 h fasting test with serum insulin ≥18 pmol/L), with available pre-operative 68Ga-DOTATATE PET/CT and CE-CT, and diagnosed with insulinoma confirmed by post-operative histopathology. Preoperative imaging was retrospectively analyzed by two radiologists who were blinded to the final diagnosis and to the results of other imaging modalities. Histopathology of specimen was considered the reference standard, and head-to-head comparison of preoperative CE-CT and PET imaging findings. Findings were classified as true positive (TP), true negative (TN), false positive (FP), and false negative (FN) for each modality. Based on these results, sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of CE-CT, and 68Ga-DOTATATE PET/CT for the detection of insulinoma were calculated.
RESULTS: 43 patients (N = 43 patients, L = 56 lesions), out of these, 37 patients had benign sporadic insulinoma (N = 37, L = 42), only 3 patients had malignant sporadic insulinoma (N = 2, L = 9), and 3 patients had MEN-1 syndrome associated insulinoma (N = 3, L = 5). There was no significant statistical difference in sensitivity (P = 0.3058) and PPV (P = 0.5533) for insulinoma localization in the overall cohort with 68Ga-DOTATATE PET/CT (87.5 %, 90.74 %) compared to CE-CT (80.36 %, 93.75 %).
CONCLUSIONS: 68Ga-DOTATATE PET/CT is a non-invasive imaging modality that can identify most insulinomas. Still, it offers limited additional information when the tumor is localized by other anatomic imaging studies, so should be used as an adjunct when imaging studies fail to localize the tumor in insulinoma patients, especially when minimally invasive surgical is intended.

Non-somatostatin receptor expressing hypovascular insulinomas can be challenging to prove through imaging. This case highlights the utility of a structured approach to molecular imaging in patients with confirmed endogenous hyperinsulinemia. A 54-year-old woman was admitted because of a sudden loss of consciousness. Her relative reported that she complained of dizziness, intense sweating, blurry vision, and upper extremity tingling before becoming unresponsive for 20 min, after which the patient had little recollection of the event. She experienced similar episodes of shorter duration, trouble recalling everyday events, and unintentional weight gain of over 10 kg during the previous two years. Abdominal magnetic resonance imaging (MRI) and multidetector computerized tomography (MDCT) were unremarkable. Selective arterial calcium stimulation significantly increased hepatic venous insulin concentrations when the superior mesenteric and gastroduodenal arteries were stimulated. Technetium-99m (99mTc) octreotide single-photon emission computed tomography (SPECT) did not localize the lesion. Gallium-68 DOTA-Exendin-4 PET/CT acquisition was performed. A single intense 2 cm hyperperfused pancreatic lesion was located anteriorly in the head of the pancreas. Earlier targeted PET/CT imaging and recognition of significant neuropsychiatric symptoms attributable to the patient\'s hypoglycemic state might have accelerated the resolution of her condition and obviated the need for unnecessary testing.

Insulinomas can present with neuroglycopenic symptoms suggesting neuropsychiatric disorders, delaying diagnosis and treatment. We recently treated a 65-year-old woman with insulinoma who was misdiagnosed at her nearby psychiatric clinic as having schizophrenia because of personality changes and memory impairment; she was treated with brexpiprazole, which was discontinued due to persistence of the symptoms. Despite her relatively low casual plasma glucose (70 mg/dL), the physician at the psychiatric clinic did not investigate the possibility of hypoglycemia, partly because her HbA1c level (5.2%) was within normal range. After skipping lunch one day, she was found by her family to be unable to communicate properly. She was transported to the emergency room of our hospital, where intermittently scanning continuous glucose monitoring (isCGM) use permitted detection of the hypoglycemia and led to a diagnosis of insulinoma and successful resection. A 72-h fasting test established hyperinsulinemic hypoglycemia. Contrast-enhanced computed-tomography and endoscopic ultrasonography together with selective arterial calcium stimulation test revealed an insulin-secreting tumor in the tail of the pancreas. Surgical resection of the tumor corrected her glucose and insulin levels as well as eliminated the insulinoma neuropsychiatric symptoms. Pathological examination showed that the tumor was positive for chromogranin A, synaptophysin and insulin. It is, therefore, important for physicians to be aware that insulinomas can manifest as neuroglycopenic symptoms and to consider the possibility of hypoglycemia by careful medical interview and isCGM, especially when patients suspected of psychiatric disorders do not show the expected response to antipsychotic drugs.
UNASSIGNED: The online version contains supplementary material available at 10.1007/s13340-024-00722-9.

UNASSIGNED: Insulinoma during pregnancy is a rare condition with vague clinical symptoms, making diagnosis challenging. The standard treatment for insulinoma is surgical tumor removal, preferably using a minimally invasive method. However, there have been no recorded examples of employing a robotic platform in pregnant women with insulinoma. In this report, we present the first successful case of robotic enucleation for insulinoma during pregnancy.
UNASSIGNED: A 30-year-old pregnant woman presented with recurrent hypoglycemic symptoms throughout her first trimester that were relieved by food intake. After confirming endogenous hyperinsulinemia, an abdominal magnetic resonance imaging scan was performed to locate the tumor. A well-defined 2-cm mass was found in the pancreatic body. Robotic enucleation was performed at week 18 of gestation, and the patient experienced relief from hypoglycemic episodes postoperatively. Her blood glucose levels returned to normal, and she had an uneventful pregnancy. The patient eventually delivered a healthy baby via cesarean section without any complications.
UNASSIGNED: For a subset of pregnant individuals with insulinoma, a minimally invasive approach as robotic-assisted surgery is safe and feasible. This innovative technique has the potential to both mothers and fetuses.

UNASSIGNED: Insulinomas are rare, usually sporadic, and typically benign pancreatic neuroendocrine tumours. Pre-operative localization is challenging and evidence on comparative analysis of anatomic and scintigraphic modalities for pre-operative tumour localization is limited, even in contemporary series.
UNASSIGNED: The current study was designed to study the clinical features and management challenges of insulinomas managed at a tertiary care centre. Clinical features, diagnosis, imaging techniques, surgical procedures, and outcomes details were collated. Pre-operative imaging techniques (CT/MRI, nuclear scintigraphy) were compared with intraoperative and histopathological findings to assess their accuracy of localization.
UNASSIGNED: Thirty-seven patients (15 females [42%]; median age 36 years [IQR 28-49]) were included in the study. In four patients (10.8%), the tumour occurred in the setting of multiple endocrine neoplasia type 1 (MEN 1) while the remaining were sporadic. The sensitivity of pre-operative localization was 61.5% (multiphasic CT), 66.6% (multiphasic MRI), 100% (68Ga Exendin-4 PET-CT), and 91.6% (EUS). Three patients with normal multiphasic CT had localization on 68Ga Exendin-4 PET-CT. The positive predictive value (PPV) of both Exendin-PET-CT and EUS was similar at 91.6% and 91.6%, respectively. All patients (except one with nesidioblastosis), who underwent enucleation or partial pancreatic resection, were cured.
UNASSIGNED: 68Ga Exendin-4 PET-CT based is a non-invasive imaging modality that has high sensitivity and PPV and can be used as a first-line imaging modality. The overall prognosis of these tumours is good with high cure rates attained following surgical resection.

Aims/Background: Insulinoma is an extremely rare condition in pediatric patients. This study aims to examine the pathological and clinical characteristics of pediatric insulinoma. Methods: A retrospective, single-center study was conducted involving five pediatric patients diagnosed with insulinoma. The study involved evaluating the postoperative status of the patients during follow-up and analyzing their clinical manifestations, diagnostic work-up, pathological findings, and therapeutic approaches. Results: The study cohort comprised four males and one female, aged between 4 and 9 years. Common symptoms included dizziness and fatigue. The insulinomas were located in various parts of the pancreas: two in the head, one in the neck, one in the body, and one in the tail. After undergoing subtotal pancreatectomy, four patients experienced no side effects during a follow-up period of 41 to 153 months. One patient, who underwent an incomplete pancreatic resection, required ongoing postoperative treatment with 150 mg Creon due to pancreatic enzyme deficiency. Postoperative pathological results indicated that all cases were low-grade neuroendocrine tumours, classified as grade 1 (G1) or grade 2 (G2). Two cases exhibited capsule invasion, and one case showed microvascular invasion. Despite these invasions, no recurrences or metastases have been observed to date. Conclusion: Surgical resection is a viable treatment option for pediatric insulinoma, yielding a favorable prognosis. The presence of capsular and microvascular invasions does not seem to affect the overall prognosis in these cases.

Insulinoma, a rare neuroendocrine tumor of the pancreas, often presents diagnostic challenges due to its diverse clinical manifestations. We present the case of a 25-year-old female with recurrent hypoglycemic seizures and neuroglycopenic symptoms, ultimately diagnosed with insulinoma. Despite an initial asymptomatic period, the patient experienced progressively worsening symptoms over three years, culminating in eight episodes of generalized tonic-clonic seizures per week. Biochemical investigations during hypoglycemic episodes revealed elevated C-peptide and insulin levels, consistent with endogenous hyperinsulinemia. Imaging studies, including contrast-enhanced computed tomography (CECT) and Ga-DOTATATE scan, confirmed a hyper-enhancing lesion in the distal body of the pancreas, indicative of insulinoma. Histopathological examination (HPE) further corroborated the diagnosis. Prompt recognition and surgical excision led to the complete resolution of symptoms and improved long-term prognosis. This case underscores the importance of considering insulinoma in young individuals presenting with recurrent hypoglycemic episodes and highlights the significance of early diagnosis and intervention in preventing morbidity and mortality associated with this condition.

Insulinoma is the most common functional neuroendocrine tumor of the pancreas, with the main clinical symptom being hypoglycemia. The standard treatment is surgery, but some patients are not eligible for surgery, while in those operated on, the risk of perioperative complications is up to 30%. Diazoxide treatment to prevent hypoglycemia is effective only in 50% of patients. To prevent tumor growth and hormonal excess, stereotactic radiotherapy may be an alternative to surgical treatment. In our paper, we present two cases of patients with insulinoma treated successfully with stereotactic body radiation therapy (SBRT).