Ectopic insulinoma diagnosed by 68Ga-Exendin-4 PET/CT

Zhang, Xiaona; Jia, Hongwei; Li, Fengao; Fang, Chunyun; Zhen, Jinyang; He, Qing; Liu, Ming

doi:10.1097/md.0000000000025076

Cited by 12 publications

(11 citation statements)

References 20 publications

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“…In patients 1 and 3, early diagnosis was possible because of a family history of MEN1. Insulinomas can develop in the extrapancreatic tissues and are more likely to be multifocal in younger patients [24,25]. As in our case, differentiating between insulinoma and accessory spleen can be challenging [26,27].…”

Section: Discussionmentioning

confidence: 57%

Impact of Early Diagnostic and Therapeutic Interventions and Clinical Course in Children and Adolescents with Multiple Endocrine Neoplasia Types 1 and 2

Kim,

Lee,

Hwang

et al. 2023

Exp Clin Endocrinol Diabetes

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Objective: Multiple endocrine neoplasia types 1 (MEN1) and 2 (MEN2) are inherited endocrine tumor syndromes caused by mutations in the MEN1 or RET genes. This study aimed to investigate clinical outcomes and molecular characteristics among children with MEN. Methods: This study included 8 patients from 7 unrelated families. Data on clinical course, biochemical findings, and radiologic studies were collected by retrospective chart review. All diagnoses were genetically confirmed by Sanger sequencing of MEN1 in three MEN1 patients and RET in four patients with MEN2A and one patient with MEN2B. Results: Three patients with MEN1 from two families presented with hypoglycemia at a mean age of 11 ± 2.6 years. Four patients with MEN2A were genetically diagnosed at a mean of 3.0 ± 2.2 years of age by family screening; one of them was prenatally diagnosed by chorionic villus sampling. Three patients with MEN2A underwent prophylactic thyroidectomy from 5 to 6 years of age, whereas one patient refused surgery. The patient with MEN2B presented with a tongue neuroma and medullary thyroid carcinoma at 6 years of age. Subsequently, he underwent a subtotal colectomy because of bowel perforation and submucosal ganglioneuromatosis at 18 years of age. Conclusion: This study described the relatively long clinical course of pediatric MEN with mean follow-up duration of 7.5 ± 3.8 years. Insulinoma was the first manifestation in children with MEN1. Early diagnosis by family screening during the asymptomatic period enabled early intervention. The patient with MEN2B exhibited the most aggressive clinical course.

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Section: Discussionmentioning

confidence: 57%

Impact of Early Diagnostic and Therapeutic Interventions and Clinical Course in Children and Adolescents with Multiple Endocrine Neoplasia Types 1 and 2

Kim,

Lee,

Hwang

et al. 2023

Exp Clin Endocrinol Diabetes

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“…Insulinomas are best treated with surgical excision. [ 11 ] During the procedure, the entire abdomen must be examined, and liver metastases must be ruled out. The pancreas should be mobilized by dividing the gastrocolic ligament and entering the lesser sac.…”

Section: Discussionmentioning

confidence: 99%

“…Pancreatic neuroendocrine tumors (PNETs) are an uncommon sub-type of pancreatic tumors with a reported frequency of 4 occurrences per million per year, with the majority being insulinomas. [ 1 ] The vast majority of insulinomas (90%) are benign and solitary, with only 10% being malignant. Insulinomas are sporadic in 90% of cases, whereas 10% of them are linked to the MEN-1 disease.…”

Section: Introductionmentioning

confidence: 99%

Insulinoma: Presenting as hypoglycemia in a non-diabetic patient: A rare case report

Kumar

Chatterjee

Kishore

et al. 2022

Journal of Family Medicine and Primary Care

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PNETs (pancreatic neuroendocrine tumors) are a rare sub-type of pancreatic tumors, with the majority of them being insulinomas. The vast majority of insulinomas (90%) are benign and solitary, with only 10% being malignant. It has a wide range of clinical manifestations and requires a high level of suspicion to diagnose. Surgical excision has long been the gold standard for treating localized PNET and is still the therapy of choice. Recurrent hypoglycemia is usual in diabetic patients, but this is a rare finding in non-diabetic individuals. Here, we are presenting a rare case of insulinoma who was non-diabetic and presented with recurrent hypoglycemic episodes. A 61-year-old non-diabetic male presented with multiple episodes of hypoglycemia in the past. On thorough workup, there was an increased fasting insulin level with the fasting blood glucose level ranging from 60 to 90 mg/dl. His C-peptide and proinsulin were markedly elevated. His abdominal ultrasound failed to pick up any abnormality. His DOTANOC scan revealed a 2 × 2 cm sized lesion in the distal pancreas suggestive of neuroendocrine pathology. He subsequently underwent spleen preserving distal pancreatectomy, following which his blood sugar levels remained normal, and continued to be free of symptoms on follow-up. Our instance emphasizes the need for evaluating insulinoma as a cause of recurrent hypoglycemia in people who are not diabetic. A high index of suspicion in hypoglycemic individuals who do not respond to standard treatment or whose symptom pattern changes will lower the likelihood of insulinoma diagnosis being delayed.

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“…In patients with "hypoglycemic unawareness" caused by repeated episodes of hypoglycemia, a continuous glucose monitoring system may be useful for diagnosis. Advances in treatment include endoscopic-ultrasound-guided radiofrequency ablation for benign insulinomas [82].…”

Section: Insulinomasmentioning

confidence: 99%

A Comprehensive Review on Neuroendocrine Neoplasms: Presentation, Pathophysiology and Management

Sultana

Kar

Verma

et al. 2023

JCM

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This review focuses on neuroendocrine neoplasms (NENs), which represent a heterogeneous group of relatively uncommon tumors that arise from cells of the endocrine and nervous systems. Although diverse, these tumors share common features, such as an alike histological appearance, the presence of secretory granules and, often, the generation and release of hormones or biogenic amines. NENs have been categorized based on their clinical behavior, histology, anatomic location and proliferation rate—factors relevant to their treatment and prognosis. Previously, chiefly diagnosed by their clinical features, their presence incidental to widespread imaging techniques appears to be on the rise in the US and several other countries. In this light, this article summarizes their pathophysiology, staging and recent changes to clinical practice pertinent to their diagnosis and treatment. Neuroendocrine neoplasms (NENs) are a group of heterogeneous tumors with neuroendocrine differentiation that can arise from any organ. They account for 2% of all malignancies in the United States. A significant proportion of NEN patients experience endocrine imbalances consequent to increased amine or peptide hormone secretion, impacting their quality of life and prognosis. Over the last decade, pathologic categorization, diagnostic techniques and therapeutic choices for NENs—both well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs)—have appreciably evolved. Diagnosis of NEN mostly follows a suspicion from clinical features or incidental imaging findings. Hormonal or non-hormonal biomarkers (like serum serotonin, urine 5-HIAA, gastrin and VIP) and histology of a suspected NEN is, therefore, critical for both confirmation of the diagnosis and classification as an NET or NEC. Therapy for NENs has progressed recently based on a better molecular understanding, including the involvement of mTOR, VEGF and peptide receptor radionuclide therapy (PRRT), which add to the growing evidence supporting the possibility of treatment beyond complete resection. As the incidence of NENs is on the rise in the United States and several other countries, physicians are more likely to see these cases, and their better understanding may support earlier diagnosis and tailoring treatment to the patient. We have compiled clinically significant evidence for NENs, including relevant changes to clinical practice that have greatly updated our diagnostic and therapeutic approach for NEN patients.

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Ectopic insulinoma diagnosed by 68Ga-Exendin-4 PET/CT

Cited by 12 publications

References 20 publications

Impact of Early Diagnostic and Therapeutic Interventions and Clinical Course in Children and Adolescents with Multiple Endocrine Neoplasia Types 1 and 2

Impact of Early Diagnostic and Therapeutic Interventions and Clinical Course in Children and Adolescents with Multiple Endocrine Neoplasia Types 1 and 2

Insulinoma: Presenting as hypoglycemia in a non-diabetic patient: A rare case report

A Comprehensive Review on Neuroendocrine Neoplasms: Presentation, Pathophysiology and Management

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