Category: Other - Neuroendocrine tumors

Monitor: 23

23 - Proinsulinoma Liver Metastasis and Tumor Recurrence After Diagnosis and Management of a Malignant Insulinoma

Thursday, Apr 25
12:30 PM – 1:00 PM

Objective :

Pancreatic neuroendocrine tumors (PNETs) can overproduce insulin, and cause signs and symptoms of hypoglycemia. While 95% of insulinomas are benign, in this abstract, we present a rare case of metastatic proinsulinoma, likely progressed from benign insulinoma, which initially presented with fasting hypoglycemia.


Methods : n/a


Results : n/a


Discussion :

A 74-year-old male with PMH of prostate cancer, hypertension, and hyperlipidemia presented with severe hypoglycemia, glucose level 38 mg/dL. MRI and CT scan of the abdomen showed a 2.5 x 5.0 x 2.3 cm solid, heterogeneous mass in the pancreatic tail. Endoscopic biopsy showed a well-differentiated, pancreatic, neuroendocrine neoplasm with Ki-67 index of   Further obtained bloodwork showed a fasting glucose of 96 mg/dl.  PET CT was performed and revealed focal FDG uptake of the suspected metastatic liver lesion and another FDG uptake at the splenic hilum. Both are highly suspicious for disease recurrence. Neuroendocrine tumor markers were found elevated:  chromogranin A (5 nmol/L), pancreastatin (64 pg/mL), and neurokinin A (88 pg/mL). Further studies showed elevated levels of proinsulin (59.9 pmol/L), insulin (5.2 mU/ml), C peptide (1.9 ng/mL), and glucose (95 mg/dL), which are consistent with proinsulinoma. This patient is currently undergoing liver microwave ablation at another institution, monthly injections of lanreotide, and planning for resection of recurrent tumor with possible splenectomy. He is active and has no significant pain or complaints except loose bowel movements which are controlled with lanreotide and pancreatic enzymes.


Conclusion :

Pancreatic neuroendocrine tumors account for about 6% of all pancreatic tumors. Although Insulinomas are generally associated with high cure rate (surgical cure rate 77%-100%) and a benign clinical course, our case represents PNETs can possibly relapse in malignant form with metastasis (in our case, proinsulinoma with liver metastasis).  They can also lose the original biochemical feature. In this case, the recurrence of proinsulinoma has no hypoglycemia as its initial presentation. This evolutionary development emphasizes the clinical importance of imaging studies as surveillance tools, as biochemical markers can change and may not reflect the original clinical presentation.  

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Jana Havranova

Resident
St.Luke's University Hospital
Bethlehem, Pennsylvania

Resident

Matthew D. Krinock

Resident
St.Luke's University Hospital
Bethlehem, Pennsylvania

Resident

Gary G. Lu

Attending physician
St.Luke's University Hospital
Bethlehem, Pennsylvania

Attending physician

David W. Leh

Attending physician
St.Luke's University Hospital
Bethlehem, Pennsylvania

Attending physician

Thomas Gallagher

Endocrinologist
St. Luke's University Hospital
Bethlehem, Pennsylvania

Endocrinologist at St. Luke's University Health Network in Bethlehem, PA.

Mohammad Ishaq Arastu

Physician
St.Luke's University Hospital
Bethlehem, Pennsylvania

Physician