Case presentation of 8-year follow up of recurrent malignant duodenal Insulinoma and lymph node metastases and literature review of malignant Insulinoma management.

Autor: Walker MP; Department of Endocrinology, University of Pennsylvania, 3400 Civic Center Boulevard, West Pavilion, 4th Floor, Philadelphia, PA, 19104, USA. michelle.walker@pennmedicine.upenn.edu., Shenoy V; Department of Endocrinology, Christiana Care, 4735 Ogletown Stanton Road, Suite MAP2, Newark, DE, 19713, USA., Metz DC; Department of Gastroenterology, University of Pennsylvania, 3400 Civic Center Boulevard, South Pavilion, 4th Floor, Philadelphia, PA, 19104, USA., Stanley CA; Department of Endocrinology and Metabolism, Children's Hospital of Philadelphia, 3516 Civic Center Boulevard #802, Philadelphia, PA, 19104, USA., Fraker D; Department of Endocrine and Oncologic Surgery, University of Pennsylvania, 3400 Civic Center Boulevard, West Pavilion, 3rd Floor, Philadelphia, PA, 19104, USA., Chandrasekhara V; Department of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street. SW, Rochester, MN, 55905, USA., Amaro A; Department of Endocrinology, University of Pennsylvania, 3400 Civic Center Boulevard, West Pavilion, 4th Floor, Philadelphia, PA, 19104, USA.
Jazyk: angličtina
Zdroj: BMC endocrine disorders [BMC Endocr Disord] 2022 Dec 09; Vol. 22 (1), pp. 310. Date of Electronic Publication: 2022 Dec 09.
DOI: 10.1186/s12902-022-01219-9
Abstrakt: Background: Insulinoma is an uncommon insulin-secreting neuroendocrine tumor that presents with severe recurrent hypoglycemia. Although cases of extrapancreatic insulinomas have been reported, the majority of insulinomas occur in the pancreas. The number of reported cases of ectopic insulinomas with follow-up assessments is limited and they do not report disease recurrence. The current report presents the first documented case of recurrent extrapancreatic insulinoma with 8 years of follow-up, provides relevant literature review, and proposes surveillance and treatment strategies.
Case Presentation: We describe an insulinoma localized in the duodenal wall of a 36-year-old female who presented in 2013 with weight gain and Whipple's triad and was successfully managed with duodenotomy and enucleation. She presented again in 2017 with recurrent Whipple's triad and was found to have metastatic disease localized exclusively to peripancreatic lymph nodes. Primary pancreatic insulinoma was not evident and her hypoglycemia resolved following lymph node dissection. Eight years after initial presentation continuous glucose monitoring (CGM) showed a trend for euglycemia, and PET-CT Gallium 68 DOTATATE scan evaluation indicated absence of recurrent disease.
Conclusion: Insulinomas are rare clinical entities and extrapancreatic insulinomas are particularly uncommon. Follow-up evaluation and treatment strategies for ectopic insulinoma recurrence presents a significant clinical challenge as the condition has hitherto remained undescribed in the literature. Available evidence in the literature indicates that lymph node metastases of intrapancreatic insulinomas likely do not change prognosis. Given the absence of long-term data informing the management and monitoring of patients with extrapancreatic insulinoma, we suggest patient education for hypoglycemic symptoms, monitoring for hypoglycemia with CGM, annual imaging, and a discussion with patients regarding treatment with octreotide or alternative somatostatin receptor analog therapies.
(© 2022. The Author(s).)
Databáze: MEDLINE
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