Subject . | Gender . | Age of diagnosis . | WHO grading/Ki67 index . | Primary tumor and metastases at diagnosis. Subsequent progression to other sites . | Synchronous or metachronous liver metastases . | Germline testing . | Hypoglycemia at presentation? yes or no. If no, when did hypoglycemia occur? Glucose mg/dL/insulin mU/L/proinsulin pmol/L levels at diagnosis. . | Treatments provided during follow-up . | Order and timing of treatment in relation to original diagnosis of insulinoma. Treatments that led to control of hypoglycemia. . | Follow up duration . |
---|---|---|---|---|---|---|---|---|---|---|
1 | M | 54 | 2; Ki67 11.6% | CT abdomen 3.1 × 1.9 × 2.1 cm lesion in pancreas. > 20 liver metastases. No skeletal metastases | Synchronous | Invitae cancer genetics panel (130 genes tested, 2018). Pathogenic variant in NBN: c.657_661delACAAA (p.Lys219Asnfs*16). | Yes, at diagnosis. 45/51.2/700. | Surgeries X 2 TACE x 3 Everolimus | Distal pancreatectomy; enucleation large liver lesion at 2 months. TACE at 3 months, 7 months; 12 months. Everolimus at 8 months for a total of 7 months. Hepatectomy at 34 months. Hypoglycemia resolved after 4 months following surgery and TACE x 2. | 4.9; alive |
2 | M | 65 | 2; Ki67 15%; | CT abdomen: 2 cm lesion in pancreas. Liver metastases noted 9 years later. No skeletal metastases | Metachronous | NA | Yes, at diagnosis. 47/34.2/93 | Surgery TAE Lanreotide | Surgery at diagnosis. TAE 9 years later. Hypoglycemia controlled by lanreotide. | 10.9; alive |
3 | F | 55 | 2; Ki67 5% | MR abdomen up to 10 cm in pancreas, liver metastases. Octreoscan noted lesions in liver, pancreas, porta hepatis and spleen near hilum; lung nodules; L2 vertebral body. Subsequently development of more metastatic disease in skeleton & lungs | Synchronous | (Athena diagnostics MEN 1 mutation analysis, 2013). Negative MEN1 screen | Yes, at diagnosis. 40/35/297 . | Octreotide Everolimus PRRT x3 Lanreotide Capecitabine/ temozolomide cabozantinib Nivolumab/ ipilimumab SIRT SBRT | No surgery. Octreotide at diagnosis. Everolimus at 2 months after diagnosis. PRRT 7 months,10 months 12 months after diagnosis. Switched to lanreotide at 1.6 years. SIRT and SBRT (liver) at 2 yrs. Capecitabine/temozolomide at 2.6 years. Cabozantinib at 3.8 years. Briefly participated in a nivolumab/ipilimumab clinical trial before death (at 4.5 years after diagnosis). Hypoglycemia resolved after PRRT treatment. | 4.7; died |
4 | F | 65 | 2; Ki-67 7.3% | CT abdomen: pancreatic mass 3.6 cm; 4 liver metastases. 8 years later developed periportal and retroperitoneal nodes; cervical spine body lesion | Synchronous | Invitae cancer genetics panel 71 genes tested, 2016. VUS FLCN gene(c.748C > A, p.Leu250Met) | Yes, at diagnosis. 47/181/657 | octreotide TAE x 4 Surgery TACE x 1 microwave ablation SBRT FOLFOX | First treated with octreotide. TAE x 2 at 5 yrs. Distal pancreatectomy and left hepatectomy at 5 yrs. TAE 5 yrs. TACE 5.5 yrs. TAE, 5.6 yrs., Microwave ablation 5.7 yrs. Briefly given sunitinib but stopped because of intratumoral bleed. SBRT to hepatic mets at 6.9 yrs. FOLFOX at 7 yrs. Octreotide controlled hypoglycemia. TAE temporarily reduced need for octreotide to control hypoglycemia. | 8.2; died |
5 | M | 48 | NA | CT abdomen and PET/CT scan: pancreatic and subcentimeter liver lesions. 4.2 years later, mass right iliac bone multiple foci retroperitoneal nodes and widespread metastases in skeleton | Synchronous | NA | Yes, at diagnosis. 36/37/160 | Diazoxide Octreotide TACE Capecitabine/ temozolomide Everolimus Sunitinib SBRT | Initially treated with diazoxide but had persistent hypoglycemia. Unclear if patient took drug consistently. Octreotide 6 months after diagnosis. TACE at 3 yrs. - octreotide held for one year before restarting. Capecitabine/temozolomide at 4.5 yrs. for progression. SBRT to right iliac bone at 4.6 yrs. Everolimus at 4.9 yrs. Hypoglycemia better after TACE. Then had mild hypoglycemia which resolved with octreotide therapy. | 5.4; died |
6 | F | 58 | 1; Ki 67 < 2% (1.3%) | DOTATE PET CT scan—pancreatic tail lesion and liver metastases.. One year later widespread skeletal metastases.. | Synchronous | (Ambry genetics 49 panel 2015). Negative MEN1 screen | Yes, at diagnosis. 38/79.6/700 | Surgery TACE x 2 Octreotide Everolimus Carboplatin/ etoposide PRRT x 4 SBRT x3 | Diazoxide and octreotide at diagnosis. TACE at 2 weeks and 2 months. Hypoglycemia resolved and diazoxide discontinued. Distal pancreatectomy, hepatectomy at 1.9 yrs. External radiation spine for metastatic disease at 2.3 yrs. Everolimus initiated. External radiation left hip and femur 3. 8 years. Everolimus discontinued. PRRT 4 cycles at 4 years. External radiation the sacrum, spine, rib. Restart everolimus 4.5 years. Recurrent hypoglycemia and started on carboplatin/etoposide at 4.8 years. Died 5 yrs. after diagnosis. Hypoglycemia controlled after TACE, octreotide and surgery. Recurrent hypoglycemia with progression and controlled with carboplatin/etoposide. | 5.1; died |
7 | F | 67 | 2; Ki 67 16%; | CT abdomen 2.8 cm pancreatic body lesion & hepatic masses. 10 years later lung nodules measuring up to 2.6 cm. | synchronous | NA | No, hypoglycemia occurred 8 months after diagnosis. 42/104/260 | Octreotide TACE x 1 PRRT x 5 carboplatin/ etoposide capecitabine/ temozolomide SBRT | Hypoglycemia 8 months after diagnosis. PRRT two cycles eight months later. Also, TACE. Hypoglycemia controlled on octreotide. PRRT two cycles at 3.5 yrs. Carboplatin etoposide at 4 years. Switched to capecitabine/temozolomide for 1.8 yrs. and discontinued for progression. Remained on octreotide. External radiation to liver mets at 8.6 yrs. One more dose PRRT at 9.5 yrs. Died 10.4 yrs. after diagnosis. Hypoglycemia controlled after PRRT and octreotide. | 10.4; died |
8 | M, | 73 | 1; Ki67 1.3% | CT abdomen showed 6.4 cm pancreatic lesion and lymph node involvement. 2.5 years, later liver metastases. Subsequently developed retroperitoneal nodes; osseous metastatic disease; small degree of lung disease | Metachronous | Cancer susceptibility multigene panel, 2015. Ambry Cancer Next Panel 2017. Negative for MEN1 mutations. VUS MRE11A gene | Yes, at diagnosis. 39/NA/NA | Surgery x 3 TAE x4 Octreotide Diazoxide Everolimus Capecitabine/ temozolomide PRRT x 4 | Distal pancreatectomy at diagnosis with resolution of hypoglycemia. Liver metastases 2.5 years after diagnosis. Started Octreotide at 2.6 years. TAE 2.6 years; 3.3 years. Diazoxide at 3.5 years with mild improvement hypoglycemia. Resection liver metastasis with LN dissection at 4 yrs. - significant improvement in hypoglycemia. Everolimus at 4.8 yrs. with immediate resolution of hypoglycemia. TAE at 5.3 yrs.; 5.5 yrs. Partial hepatectomy & LN dissection 5.8 yrs. Everolimus & octreotide discontinued. Interval progression of metastatic disease. Restarted octreotide at 6.3 yrs. PRRT 6.4, 6.5. 6.8. 7 yrs. Capecitabine/temozolomide at 11 yrs. for progression of disease. Also, on octreotide. Hypoglycemia controlled with surgeries and everolimus. | 12.3; alive |
9 | F | 62 | 2; Ki 67 3.5 % | Initially only pancreas. Portocaval nodes; liver nodules noted 3.75 yrs. Later | Metachronous | Invitae Genetics 79 gene panel 2016. Negative MEN1 screen | Yes, at diagnosis. 56/105.8/294 | Surgery Octreotide SBRT | Pancreatectomy at diagnosis. Recurrent hypoglycemia 3 years later. Liver metastases noted at 3.75 yrs. Octreotide therapy at 4 yrs. SBRT at 5 yrs. Died from cirrhosis related to NASH. Hypoglycemia controlled once patient went on octreotide. | 5.8;died |
10 | F | 20 | 2, Ki 67 20% | CT 3.9 cm lesion splenic hilum; hepatic lesions; breast, L1 lesion; osseous mets. Rt internal mammary node | Synchronous | UC500 germline panel negative | No, hypoglycemia documented 1.5 years after diagnosis, 48/45.1/195. | Capecitabine/ temozolomide Pembrolizumab PRRT Lanreotide | capecitabine/Temozolomide at diagnosis for 1 year. TACE at 1.4 years; than pembrolizumab + PRRT clinical trial (2 cycles) at 1.6 years. Also started lanreotide at 1.6 years Hypoglycemia controlled with combination of pembrolizumab, PRRT and lanreotide | 1.9 alive |
11 | M | 71 | 3, Ki 67 80% (high-grade neuroendocrine neoplasm) | CT abdomen 4.9 cm mass pancreatic head; liver metastases; skeleton | Synchronous | Invitae 62 gene germline panel negative | Yes, at diagnosis, 60/49/664.2 | Surgery Octreotide, Lanreotide, pembrolizumab PRRT | Octreotide soon after diagnosis; Whipple procedure + RFA 2 months later. carboplatin + etoposide 6 cycles started 4 months later. pembrolizumab + PRRT clinical trial (2 cycles) 10 months later. Lanreotide 10 months later Hypoglycemia controlled after PRRT and lanreotide | 1.2 alive |
12 | M | 50 | 2, Ki67 8% | MR abdomen 4 × 2.5 cm mass distal body/tail of pancreas; multiple metastases in liver | Synchronous | ---- | No, hypoglycemia 5 years after diagnosis 53/34/96.5 | Microwave ablation liver metastases. Surgery octreotide PRRT | Microwave ablation liver metastases 2 months after diagnosis.; Y90 SIRT at 5 months; Resection pancreatic tumor and lymph node dissection at 9 months. Octreotide at 1.4 years. PRRT x 2 cycles at 7 years. Hypoglycemia controlled after PRRT and octreotide | 7.3 alive |
13 | F | 64 | 2, Ki67 17.3% | CT abdomen 4.5 × 3.5 × 3.3 cm mass pancreatic tail and multiple metastases in liver | Synchronous | UC500 germline panel negative. | No, hypoglycemia 1.7 years after diagnosis. 36/18.1/320 | Octreotide Capecitabine/ temozolomide PRRT SBRT Pembrolizumab FOLFOX Carboplatin/etoposide | Octreotide at 2 months after diagnosis. Capecitabine/Temozolomide at 6 months. PRRT x 4 cycles at 1 year. SBRT to acetabulum and L4 met at 1.4 years. Pembrolizumab one dose + Y90 radioembolization at 1.6 years clinical trial.; FOLFOX at 1.8 years; carboplatin/etoposide 3 cycles at 2 years. Hypoglycemia controlled after treatment with FOLFOX | 2.5 died |
14 | M | 30 | 1, Ki67 < 1% | 5 cm tumor pancreatic head. Liver metastases first noted 3.4 years later | Metachronous | Germline testing UC500 germline panel MSH2 pL556S (c. 1667T > C, pLeu556Ser)—missense variant in the MSG2 DNA mismatch (MMR) protein—likely pathogenic for hereditary colorectal cancer | No, hypoglycemia 3.5 years after diagnosis. 41/20/229.7 | Surgeries x 2 Lanreotide Sunitinib Pembrolizumab Diazoxide Capecitabine/ temozolomide | Whipple procedure at diagnosis; Lanreotide at 1.8 years; sunitinib at 6 years Liver resection and microwave ablation at 7.4 years. Pembrolizumab at 8. 3 years (for high tumor mutation burden). Diazoxide at 9 years. Capecitabine/temozolomide 9.1 years Hypoglycemia controlled with food and diazoxide | 9.1 alive |
Subject . | Gender . | Age of diagnosis . | WHO grading/Ki67 index . | Primary tumor and metastases at diagnosis. Subsequent progression to other sites . | Synchronous or metachronous liver metastases . | Germline testing . | Hypoglycemia at presentation? yes or no. If no, when did hypoglycemia occur? Glucose mg/dL/insulin mU/L/proinsulin pmol/L levels at diagnosis. . | Treatments provided during follow-up . | Order and timing of treatment in relation to original diagnosis of insulinoma. Treatments that led to control of hypoglycemia. . | Follow up duration . |
---|---|---|---|---|---|---|---|---|---|---|
1 | M | 54 | 2; Ki67 11.6% | CT abdomen 3.1 × 1.9 × 2.1 cm lesion in pancreas. > 20 liver metastases. No skeletal metastases | Synchronous | Invitae cancer genetics panel (130 genes tested, 2018). Pathogenic variant in NBN: c.657_661delACAAA (p.Lys219Asnfs*16). | Yes, at diagnosis. 45/51.2/700. | Surgeries X 2 TACE x 3 Everolimus | Distal pancreatectomy; enucleation large liver lesion at 2 months. TACE at 3 months, 7 months; 12 months. Everolimus at 8 months for a total of 7 months. Hepatectomy at 34 months. Hypoglycemia resolved after 4 months following surgery and TACE x 2. | 4.9; alive |
2 | M | 65 | 2; Ki67 15%; | CT abdomen: 2 cm lesion in pancreas. Liver metastases noted 9 years later. No skeletal metastases | Metachronous | NA | Yes, at diagnosis. 47/34.2/93 | Surgery TAE Lanreotide | Surgery at diagnosis. TAE 9 years later. Hypoglycemia controlled by lanreotide. | 10.9; alive |
3 | F | 55 | 2; Ki67 5% | MR abdomen up to 10 cm in pancreas, liver metastases. Octreoscan noted lesions in liver, pancreas, porta hepatis and spleen near hilum; lung nodules; L2 vertebral body. Subsequently development of more metastatic disease in skeleton & lungs | Synchronous | (Athena diagnostics MEN 1 mutation analysis, 2013). Negative MEN1 screen | Yes, at diagnosis. 40/35/297 . | Octreotide Everolimus PRRT x3 Lanreotide Capecitabine/ temozolomide cabozantinib Nivolumab/ ipilimumab SIRT SBRT | No surgery. Octreotide at diagnosis. Everolimus at 2 months after diagnosis. PRRT 7 months,10 months 12 months after diagnosis. Switched to lanreotide at 1.6 years. SIRT and SBRT (liver) at 2 yrs. Capecitabine/temozolomide at 2.6 years. Cabozantinib at 3.8 years. Briefly participated in a nivolumab/ipilimumab clinical trial before death (at 4.5 years after diagnosis). Hypoglycemia resolved after PRRT treatment. | 4.7; died |
4 | F | 65 | 2; Ki-67 7.3% | CT abdomen: pancreatic mass 3.6 cm; 4 liver metastases. 8 years later developed periportal and retroperitoneal nodes; cervical spine body lesion | Synchronous | Invitae cancer genetics panel 71 genes tested, 2016. VUS FLCN gene(c.748C > A, p.Leu250Met) | Yes, at diagnosis. 47/181/657 | octreotide TAE x 4 Surgery TACE x 1 microwave ablation SBRT FOLFOX | First treated with octreotide. TAE x 2 at 5 yrs. Distal pancreatectomy and left hepatectomy at 5 yrs. TAE 5 yrs. TACE 5.5 yrs. TAE, 5.6 yrs., Microwave ablation 5.7 yrs. Briefly given sunitinib but stopped because of intratumoral bleed. SBRT to hepatic mets at 6.9 yrs. FOLFOX at 7 yrs. Octreotide controlled hypoglycemia. TAE temporarily reduced need for octreotide to control hypoglycemia. | 8.2; died |
5 | M | 48 | NA | CT abdomen and PET/CT scan: pancreatic and subcentimeter liver lesions. 4.2 years later, mass right iliac bone multiple foci retroperitoneal nodes and widespread metastases in skeleton | Synchronous | NA | Yes, at diagnosis. 36/37/160 | Diazoxide Octreotide TACE Capecitabine/ temozolomide Everolimus Sunitinib SBRT | Initially treated with diazoxide but had persistent hypoglycemia. Unclear if patient took drug consistently. Octreotide 6 months after diagnosis. TACE at 3 yrs. - octreotide held for one year before restarting. Capecitabine/temozolomide at 4.5 yrs. for progression. SBRT to right iliac bone at 4.6 yrs. Everolimus at 4.9 yrs. Hypoglycemia better after TACE. Then had mild hypoglycemia which resolved with octreotide therapy. | 5.4; died |
6 | F | 58 | 1; Ki 67 < 2% (1.3%) | DOTATE PET CT scan—pancreatic tail lesion and liver metastases.. One year later widespread skeletal metastases.. | Synchronous | (Ambry genetics 49 panel 2015). Negative MEN1 screen | Yes, at diagnosis. 38/79.6/700 | Surgery TACE x 2 Octreotide Everolimus Carboplatin/ etoposide PRRT x 4 SBRT x3 | Diazoxide and octreotide at diagnosis. TACE at 2 weeks and 2 months. Hypoglycemia resolved and diazoxide discontinued. Distal pancreatectomy, hepatectomy at 1.9 yrs. External radiation spine for metastatic disease at 2.3 yrs. Everolimus initiated. External radiation left hip and femur 3. 8 years. Everolimus discontinued. PRRT 4 cycles at 4 years. External radiation the sacrum, spine, rib. Restart everolimus 4.5 years. Recurrent hypoglycemia and started on carboplatin/etoposide at 4.8 years. Died 5 yrs. after diagnosis. Hypoglycemia controlled after TACE, octreotide and surgery. Recurrent hypoglycemia with progression and controlled with carboplatin/etoposide. | 5.1; died |
7 | F | 67 | 2; Ki 67 16%; | CT abdomen 2.8 cm pancreatic body lesion & hepatic masses. 10 years later lung nodules measuring up to 2.6 cm. | synchronous | NA | No, hypoglycemia occurred 8 months after diagnosis. 42/104/260 | Octreotide TACE x 1 PRRT x 5 carboplatin/ etoposide capecitabine/ temozolomide SBRT | Hypoglycemia 8 months after diagnosis. PRRT two cycles eight months later. Also, TACE. Hypoglycemia controlled on octreotide. PRRT two cycles at 3.5 yrs. Carboplatin etoposide at 4 years. Switched to capecitabine/temozolomide for 1.8 yrs. and discontinued for progression. Remained on octreotide. External radiation to liver mets at 8.6 yrs. One more dose PRRT at 9.5 yrs. Died 10.4 yrs. after diagnosis. Hypoglycemia controlled after PRRT and octreotide. | 10.4; died |
8 | M, | 73 | 1; Ki67 1.3% | CT abdomen showed 6.4 cm pancreatic lesion and lymph node involvement. 2.5 years, later liver metastases. Subsequently developed retroperitoneal nodes; osseous metastatic disease; small degree of lung disease | Metachronous | Cancer susceptibility multigene panel, 2015. Ambry Cancer Next Panel 2017. Negative for MEN1 mutations. VUS MRE11A gene | Yes, at diagnosis. 39/NA/NA | Surgery x 3 TAE x4 Octreotide Diazoxide Everolimus Capecitabine/ temozolomide PRRT x 4 | Distal pancreatectomy at diagnosis with resolution of hypoglycemia. Liver metastases 2.5 years after diagnosis. Started Octreotide at 2.6 years. TAE 2.6 years; 3.3 years. Diazoxide at 3.5 years with mild improvement hypoglycemia. Resection liver metastasis with LN dissection at 4 yrs. - significant improvement in hypoglycemia. Everolimus at 4.8 yrs. with immediate resolution of hypoglycemia. TAE at 5.3 yrs.; 5.5 yrs. Partial hepatectomy & LN dissection 5.8 yrs. Everolimus & octreotide discontinued. Interval progression of metastatic disease. Restarted octreotide at 6.3 yrs. PRRT 6.4, 6.5. 6.8. 7 yrs. Capecitabine/temozolomide at 11 yrs. for progression of disease. Also, on octreotide. Hypoglycemia controlled with surgeries and everolimus. | 12.3; alive |
9 | F | 62 | 2; Ki 67 3.5 % | Initially only pancreas. Portocaval nodes; liver nodules noted 3.75 yrs. Later | Metachronous | Invitae Genetics 79 gene panel 2016. Negative MEN1 screen | Yes, at diagnosis. 56/105.8/294 | Surgery Octreotide SBRT | Pancreatectomy at diagnosis. Recurrent hypoglycemia 3 years later. Liver metastases noted at 3.75 yrs. Octreotide therapy at 4 yrs. SBRT at 5 yrs. Died from cirrhosis related to NASH. Hypoglycemia controlled once patient went on octreotide. | 5.8;died |
10 | F | 20 | 2, Ki 67 20% | CT 3.9 cm lesion splenic hilum; hepatic lesions; breast, L1 lesion; osseous mets. Rt internal mammary node | Synchronous | UC500 germline panel negative | No, hypoglycemia documented 1.5 years after diagnosis, 48/45.1/195. | Capecitabine/ temozolomide Pembrolizumab PRRT Lanreotide | capecitabine/Temozolomide at diagnosis for 1 year. TACE at 1.4 years; than pembrolizumab + PRRT clinical trial (2 cycles) at 1.6 years. Also started lanreotide at 1.6 years Hypoglycemia controlled with combination of pembrolizumab, PRRT and lanreotide | 1.9 alive |
11 | M | 71 | 3, Ki 67 80% (high-grade neuroendocrine neoplasm) | CT abdomen 4.9 cm mass pancreatic head; liver metastases; skeleton | Synchronous | Invitae 62 gene germline panel negative | Yes, at diagnosis, 60/49/664.2 | Surgery Octreotide, Lanreotide, pembrolizumab PRRT | Octreotide soon after diagnosis; Whipple procedure + RFA 2 months later. carboplatin + etoposide 6 cycles started 4 months later. pembrolizumab + PRRT clinical trial (2 cycles) 10 months later. Lanreotide 10 months later Hypoglycemia controlled after PRRT and lanreotide | 1.2 alive |
12 | M | 50 | 2, Ki67 8% | MR abdomen 4 × 2.5 cm mass distal body/tail of pancreas; multiple metastases in liver | Synchronous | ---- | No, hypoglycemia 5 years after diagnosis 53/34/96.5 | Microwave ablation liver metastases. Surgery octreotide PRRT | Microwave ablation liver metastases 2 months after diagnosis.; Y90 SIRT at 5 months; Resection pancreatic tumor and lymph node dissection at 9 months. Octreotide at 1.4 years. PRRT x 2 cycles at 7 years. Hypoglycemia controlled after PRRT and octreotide | 7.3 alive |
13 | F | 64 | 2, Ki67 17.3% | CT abdomen 4.5 × 3.5 × 3.3 cm mass pancreatic tail and multiple metastases in liver | Synchronous | UC500 germline panel negative. | No, hypoglycemia 1.7 years after diagnosis. 36/18.1/320 | Octreotide Capecitabine/ temozolomide PRRT SBRT Pembrolizumab FOLFOX Carboplatin/etoposide | Octreotide at 2 months after diagnosis. Capecitabine/Temozolomide at 6 months. PRRT x 4 cycles at 1 year. SBRT to acetabulum and L4 met at 1.4 years. Pembrolizumab one dose + Y90 radioembolization at 1.6 years clinical trial.; FOLFOX at 1.8 years; carboplatin/etoposide 3 cycles at 2 years. Hypoglycemia controlled after treatment with FOLFOX | 2.5 died |
14 | M | 30 | 1, Ki67 < 1% | 5 cm tumor pancreatic head. Liver metastases first noted 3.4 years later | Metachronous | Germline testing UC500 germline panel MSH2 pL556S (c. 1667T > C, pLeu556Ser)—missense variant in the MSG2 DNA mismatch (MMR) protein—likely pathogenic for hereditary colorectal cancer | No, hypoglycemia 3.5 years after diagnosis. 41/20/229.7 | Surgeries x 2 Lanreotide Sunitinib Pembrolizumab Diazoxide Capecitabine/ temozolomide | Whipple procedure at diagnosis; Lanreotide at 1.8 years; sunitinib at 6 years Liver resection and microwave ablation at 7.4 years. Pembrolizumab at 8. 3 years (for high tumor mutation burden). Diazoxide at 9 years. Capecitabine/temozolomide 9.1 years Hypoglycemia controlled with food and diazoxide | 9.1 alive |
Abbreviations: PRRT, peptide receptor radionucleotide therapy with 177-Lutetium labelled somatostatin analogs; SBRT, stereotactic body radiation therapy; SIR, selective internal radiation therapy; TACE, transarterial chemoembolization; TAE, transarterial embolization.
Subject . | Gender . | Age of diagnosis . | WHO grading/Ki67 index . | Primary tumor and metastases at diagnosis. Subsequent progression to other sites . | Synchronous or metachronous liver metastases . | Germline testing . | Hypoglycemia at presentation? yes or no. If no, when did hypoglycemia occur? Glucose mg/dL/insulin mU/L/proinsulin pmol/L levels at diagnosis. . | Treatments provided during follow-up . | Order and timing of treatment in relation to original diagnosis of insulinoma. Treatments that led to control of hypoglycemia. . | Follow up duration . |
---|---|---|---|---|---|---|---|---|---|---|
1 | M | 54 | 2; Ki67 11.6% | CT abdomen 3.1 × 1.9 × 2.1 cm lesion in pancreas. > 20 liver metastases. No skeletal metastases | Synchronous | Invitae cancer genetics panel (130 genes tested, 2018). Pathogenic variant in NBN: c.657_661delACAAA (p.Lys219Asnfs*16). | Yes, at diagnosis. 45/51.2/700. | Surgeries X 2 TACE x 3 Everolimus | Distal pancreatectomy; enucleation large liver lesion at 2 months. TACE at 3 months, 7 months; 12 months. Everolimus at 8 months for a total of 7 months. Hepatectomy at 34 months. Hypoglycemia resolved after 4 months following surgery and TACE x 2. | 4.9; alive |
2 | M | 65 | 2; Ki67 15%; | CT abdomen: 2 cm lesion in pancreas. Liver metastases noted 9 years later. No skeletal metastases | Metachronous | NA | Yes, at diagnosis. 47/34.2/93 | Surgery TAE Lanreotide | Surgery at diagnosis. TAE 9 years later. Hypoglycemia controlled by lanreotide. | 10.9; alive |
3 | F | 55 | 2; Ki67 5% | MR abdomen up to 10 cm in pancreas, liver metastases. Octreoscan noted lesions in liver, pancreas, porta hepatis and spleen near hilum; lung nodules; L2 vertebral body. Subsequently development of more metastatic disease in skeleton & lungs | Synchronous | (Athena diagnostics MEN 1 mutation analysis, 2013). Negative MEN1 screen | Yes, at diagnosis. 40/35/297 . | Octreotide Everolimus PRRT x3 Lanreotide Capecitabine/ temozolomide cabozantinib Nivolumab/ ipilimumab SIRT SBRT | No surgery. Octreotide at diagnosis. Everolimus at 2 months after diagnosis. PRRT 7 months,10 months 12 months after diagnosis. Switched to lanreotide at 1.6 years. SIRT and SBRT (liver) at 2 yrs. Capecitabine/temozolomide at 2.6 years. Cabozantinib at 3.8 years. Briefly participated in a nivolumab/ipilimumab clinical trial before death (at 4.5 years after diagnosis). Hypoglycemia resolved after PRRT treatment. | 4.7; died |
4 | F | 65 | 2; Ki-67 7.3% | CT abdomen: pancreatic mass 3.6 cm; 4 liver metastases. 8 years later developed periportal and retroperitoneal nodes; cervical spine body lesion | Synchronous | Invitae cancer genetics panel 71 genes tested, 2016. VUS FLCN gene(c.748C > A, p.Leu250Met) | Yes, at diagnosis. 47/181/657 | octreotide TAE x 4 Surgery TACE x 1 microwave ablation SBRT FOLFOX | First treated with octreotide. TAE x 2 at 5 yrs. Distal pancreatectomy and left hepatectomy at 5 yrs. TAE 5 yrs. TACE 5.5 yrs. TAE, 5.6 yrs., Microwave ablation 5.7 yrs. Briefly given sunitinib but stopped because of intratumoral bleed. SBRT to hepatic mets at 6.9 yrs. FOLFOX at 7 yrs. Octreotide controlled hypoglycemia. TAE temporarily reduced need for octreotide to control hypoglycemia. | 8.2; died |
5 | M | 48 | NA | CT abdomen and PET/CT scan: pancreatic and subcentimeter liver lesions. 4.2 years later, mass right iliac bone multiple foci retroperitoneal nodes and widespread metastases in skeleton | Synchronous | NA | Yes, at diagnosis. 36/37/160 | Diazoxide Octreotide TACE Capecitabine/ temozolomide Everolimus Sunitinib SBRT | Initially treated with diazoxide but had persistent hypoglycemia. Unclear if patient took drug consistently. Octreotide 6 months after diagnosis. TACE at 3 yrs. - octreotide held for one year before restarting. Capecitabine/temozolomide at 4.5 yrs. for progression. SBRT to right iliac bone at 4.6 yrs. Everolimus at 4.9 yrs. Hypoglycemia better after TACE. Then had mild hypoglycemia which resolved with octreotide therapy. | 5.4; died |
6 | F | 58 | 1; Ki 67 < 2% (1.3%) | DOTATE PET CT scan—pancreatic tail lesion and liver metastases.. One year later widespread skeletal metastases.. | Synchronous | (Ambry genetics 49 panel 2015). Negative MEN1 screen | Yes, at diagnosis. 38/79.6/700 | Surgery TACE x 2 Octreotide Everolimus Carboplatin/ etoposide PRRT x 4 SBRT x3 | Diazoxide and octreotide at diagnosis. TACE at 2 weeks and 2 months. Hypoglycemia resolved and diazoxide discontinued. Distal pancreatectomy, hepatectomy at 1.9 yrs. External radiation spine for metastatic disease at 2.3 yrs. Everolimus initiated. External radiation left hip and femur 3. 8 years. Everolimus discontinued. PRRT 4 cycles at 4 years. External radiation the sacrum, spine, rib. Restart everolimus 4.5 years. Recurrent hypoglycemia and started on carboplatin/etoposide at 4.8 years. Died 5 yrs. after diagnosis. Hypoglycemia controlled after TACE, octreotide and surgery. Recurrent hypoglycemia with progression and controlled with carboplatin/etoposide. | 5.1; died |
7 | F | 67 | 2; Ki 67 16%; | CT abdomen 2.8 cm pancreatic body lesion & hepatic masses. 10 years later lung nodules measuring up to 2.6 cm. | synchronous | NA | No, hypoglycemia occurred 8 months after diagnosis. 42/104/260 | Octreotide TACE x 1 PRRT x 5 carboplatin/ etoposide capecitabine/ temozolomide SBRT | Hypoglycemia 8 months after diagnosis. PRRT two cycles eight months later. Also, TACE. Hypoglycemia controlled on octreotide. PRRT two cycles at 3.5 yrs. Carboplatin etoposide at 4 years. Switched to capecitabine/temozolomide for 1.8 yrs. and discontinued for progression. Remained on octreotide. External radiation to liver mets at 8.6 yrs. One more dose PRRT at 9.5 yrs. Died 10.4 yrs. after diagnosis. Hypoglycemia controlled after PRRT and octreotide. | 10.4; died |
8 | M, | 73 | 1; Ki67 1.3% | CT abdomen showed 6.4 cm pancreatic lesion and lymph node involvement. 2.5 years, later liver metastases. Subsequently developed retroperitoneal nodes; osseous metastatic disease; small degree of lung disease | Metachronous | Cancer susceptibility multigene panel, 2015. Ambry Cancer Next Panel 2017. Negative for MEN1 mutations. VUS MRE11A gene | Yes, at diagnosis. 39/NA/NA | Surgery x 3 TAE x4 Octreotide Diazoxide Everolimus Capecitabine/ temozolomide PRRT x 4 | Distal pancreatectomy at diagnosis with resolution of hypoglycemia. Liver metastases 2.5 years after diagnosis. Started Octreotide at 2.6 years. TAE 2.6 years; 3.3 years. Diazoxide at 3.5 years with mild improvement hypoglycemia. Resection liver metastasis with LN dissection at 4 yrs. - significant improvement in hypoglycemia. Everolimus at 4.8 yrs. with immediate resolution of hypoglycemia. TAE at 5.3 yrs.; 5.5 yrs. Partial hepatectomy & LN dissection 5.8 yrs. Everolimus & octreotide discontinued. Interval progression of metastatic disease. Restarted octreotide at 6.3 yrs. PRRT 6.4, 6.5. 6.8. 7 yrs. Capecitabine/temozolomide at 11 yrs. for progression of disease. Also, on octreotide. Hypoglycemia controlled with surgeries and everolimus. | 12.3; alive |
9 | F | 62 | 2; Ki 67 3.5 % | Initially only pancreas. Portocaval nodes; liver nodules noted 3.75 yrs. Later | Metachronous | Invitae Genetics 79 gene panel 2016. Negative MEN1 screen | Yes, at diagnosis. 56/105.8/294 | Surgery Octreotide SBRT | Pancreatectomy at diagnosis. Recurrent hypoglycemia 3 years later. Liver metastases noted at 3.75 yrs. Octreotide therapy at 4 yrs. SBRT at 5 yrs. Died from cirrhosis related to NASH. Hypoglycemia controlled once patient went on octreotide. | 5.8;died |
10 | F | 20 | 2, Ki 67 20% | CT 3.9 cm lesion splenic hilum; hepatic lesions; breast, L1 lesion; osseous mets. Rt internal mammary node | Synchronous | UC500 germline panel negative | No, hypoglycemia documented 1.5 years after diagnosis, 48/45.1/195. | Capecitabine/ temozolomide Pembrolizumab PRRT Lanreotide | capecitabine/Temozolomide at diagnosis for 1 year. TACE at 1.4 years; than pembrolizumab + PRRT clinical trial (2 cycles) at 1.6 years. Also started lanreotide at 1.6 years Hypoglycemia controlled with combination of pembrolizumab, PRRT and lanreotide | 1.9 alive |
11 | M | 71 | 3, Ki 67 80% (high-grade neuroendocrine neoplasm) | CT abdomen 4.9 cm mass pancreatic head; liver metastases; skeleton | Synchronous | Invitae 62 gene germline panel negative | Yes, at diagnosis, 60/49/664.2 | Surgery Octreotide, Lanreotide, pembrolizumab PRRT | Octreotide soon after diagnosis; Whipple procedure + RFA 2 months later. carboplatin + etoposide 6 cycles started 4 months later. pembrolizumab + PRRT clinical trial (2 cycles) 10 months later. Lanreotide 10 months later Hypoglycemia controlled after PRRT and lanreotide | 1.2 alive |
12 | M | 50 | 2, Ki67 8% | MR abdomen 4 × 2.5 cm mass distal body/tail of pancreas; multiple metastases in liver | Synchronous | ---- | No, hypoglycemia 5 years after diagnosis 53/34/96.5 | Microwave ablation liver metastases. Surgery octreotide PRRT | Microwave ablation liver metastases 2 months after diagnosis.; Y90 SIRT at 5 months; Resection pancreatic tumor and lymph node dissection at 9 months. Octreotide at 1.4 years. PRRT x 2 cycles at 7 years. Hypoglycemia controlled after PRRT and octreotide | 7.3 alive |
13 | F | 64 | 2, Ki67 17.3% | CT abdomen 4.5 × 3.5 × 3.3 cm mass pancreatic tail and multiple metastases in liver | Synchronous | UC500 germline panel negative. | No, hypoglycemia 1.7 years after diagnosis. 36/18.1/320 | Octreotide Capecitabine/ temozolomide PRRT SBRT Pembrolizumab FOLFOX Carboplatin/etoposide | Octreotide at 2 months after diagnosis. Capecitabine/Temozolomide at 6 months. PRRT x 4 cycles at 1 year. SBRT to acetabulum and L4 met at 1.4 years. Pembrolizumab one dose + Y90 radioembolization at 1.6 years clinical trial.; FOLFOX at 1.8 years; carboplatin/etoposide 3 cycles at 2 years. Hypoglycemia controlled after treatment with FOLFOX | 2.5 died |
14 | M | 30 | 1, Ki67 < 1% | 5 cm tumor pancreatic head. Liver metastases first noted 3.4 years later | Metachronous | Germline testing UC500 germline panel MSH2 pL556S (c. 1667T > C, pLeu556Ser)—missense variant in the MSG2 DNA mismatch (MMR) protein—likely pathogenic for hereditary colorectal cancer | No, hypoglycemia 3.5 years after diagnosis. 41/20/229.7 | Surgeries x 2 Lanreotide Sunitinib Pembrolizumab Diazoxide Capecitabine/ temozolomide | Whipple procedure at diagnosis; Lanreotide at 1.8 years; sunitinib at 6 years Liver resection and microwave ablation at 7.4 years. Pembrolizumab at 8. 3 years (for high tumor mutation burden). Diazoxide at 9 years. Capecitabine/temozolomide 9.1 years Hypoglycemia controlled with food and diazoxide | 9.1 alive |
Subject . | Gender . | Age of diagnosis . | WHO grading/Ki67 index . | Primary tumor and metastases at diagnosis. Subsequent progression to other sites . | Synchronous or metachronous liver metastases . | Germline testing . | Hypoglycemia at presentation? yes or no. If no, when did hypoglycemia occur? Glucose mg/dL/insulin mU/L/proinsulin pmol/L levels at diagnosis. . | Treatments provided during follow-up . | Order and timing of treatment in relation to original diagnosis of insulinoma. Treatments that led to control of hypoglycemia. . | Follow up duration . |
---|---|---|---|---|---|---|---|---|---|---|
1 | M | 54 | 2; Ki67 11.6% | CT abdomen 3.1 × 1.9 × 2.1 cm lesion in pancreas. > 20 liver metastases. No skeletal metastases | Synchronous | Invitae cancer genetics panel (130 genes tested, 2018). Pathogenic variant in NBN: c.657_661delACAAA (p.Lys219Asnfs*16). | Yes, at diagnosis. 45/51.2/700. | Surgeries X 2 TACE x 3 Everolimus | Distal pancreatectomy; enucleation large liver lesion at 2 months. TACE at 3 months, 7 months; 12 months. Everolimus at 8 months for a total of 7 months. Hepatectomy at 34 months. Hypoglycemia resolved after 4 months following surgery and TACE x 2. | 4.9; alive |
2 | M | 65 | 2; Ki67 15%; | CT abdomen: 2 cm lesion in pancreas. Liver metastases noted 9 years later. No skeletal metastases | Metachronous | NA | Yes, at diagnosis. 47/34.2/93 | Surgery TAE Lanreotide | Surgery at diagnosis. TAE 9 years later. Hypoglycemia controlled by lanreotide. | 10.9; alive |
3 | F | 55 | 2; Ki67 5% | MR abdomen up to 10 cm in pancreas, liver metastases. Octreoscan noted lesions in liver, pancreas, porta hepatis and spleen near hilum; lung nodules; L2 vertebral body. Subsequently development of more metastatic disease in skeleton & lungs | Synchronous | (Athena diagnostics MEN 1 mutation analysis, 2013). Negative MEN1 screen | Yes, at diagnosis. 40/35/297 . | Octreotide Everolimus PRRT x3 Lanreotide Capecitabine/ temozolomide cabozantinib Nivolumab/ ipilimumab SIRT SBRT | No surgery. Octreotide at diagnosis. Everolimus at 2 months after diagnosis. PRRT 7 months,10 months 12 months after diagnosis. Switched to lanreotide at 1.6 years. SIRT and SBRT (liver) at 2 yrs. Capecitabine/temozolomide at 2.6 years. Cabozantinib at 3.8 years. Briefly participated in a nivolumab/ipilimumab clinical trial before death (at 4.5 years after diagnosis). Hypoglycemia resolved after PRRT treatment. | 4.7; died |
4 | F | 65 | 2; Ki-67 7.3% | CT abdomen: pancreatic mass 3.6 cm; 4 liver metastases. 8 years later developed periportal and retroperitoneal nodes; cervical spine body lesion | Synchronous | Invitae cancer genetics panel 71 genes tested, 2016. VUS FLCN gene(c.748C > A, p.Leu250Met) | Yes, at diagnosis. 47/181/657 | octreotide TAE x 4 Surgery TACE x 1 microwave ablation SBRT FOLFOX | First treated with octreotide. TAE x 2 at 5 yrs. Distal pancreatectomy and left hepatectomy at 5 yrs. TAE 5 yrs. TACE 5.5 yrs. TAE, 5.6 yrs., Microwave ablation 5.7 yrs. Briefly given sunitinib but stopped because of intratumoral bleed. SBRT to hepatic mets at 6.9 yrs. FOLFOX at 7 yrs. Octreotide controlled hypoglycemia. TAE temporarily reduced need for octreotide to control hypoglycemia. | 8.2; died |
5 | M | 48 | NA | CT abdomen and PET/CT scan: pancreatic and subcentimeter liver lesions. 4.2 years later, mass right iliac bone multiple foci retroperitoneal nodes and widespread metastases in skeleton | Synchronous | NA | Yes, at diagnosis. 36/37/160 | Diazoxide Octreotide TACE Capecitabine/ temozolomide Everolimus Sunitinib SBRT | Initially treated with diazoxide but had persistent hypoglycemia. Unclear if patient took drug consistently. Octreotide 6 months after diagnosis. TACE at 3 yrs. - octreotide held for one year before restarting. Capecitabine/temozolomide at 4.5 yrs. for progression. SBRT to right iliac bone at 4.6 yrs. Everolimus at 4.9 yrs. Hypoglycemia better after TACE. Then had mild hypoglycemia which resolved with octreotide therapy. | 5.4; died |
6 | F | 58 | 1; Ki 67 < 2% (1.3%) | DOTATE PET CT scan—pancreatic tail lesion and liver metastases.. One year later widespread skeletal metastases.. | Synchronous | (Ambry genetics 49 panel 2015). Negative MEN1 screen | Yes, at diagnosis. 38/79.6/700 | Surgery TACE x 2 Octreotide Everolimus Carboplatin/ etoposide PRRT x 4 SBRT x3 | Diazoxide and octreotide at diagnosis. TACE at 2 weeks and 2 months. Hypoglycemia resolved and diazoxide discontinued. Distal pancreatectomy, hepatectomy at 1.9 yrs. External radiation spine for metastatic disease at 2.3 yrs. Everolimus initiated. External radiation left hip and femur 3. 8 years. Everolimus discontinued. PRRT 4 cycles at 4 years. External radiation the sacrum, spine, rib. Restart everolimus 4.5 years. Recurrent hypoglycemia and started on carboplatin/etoposide at 4.8 years. Died 5 yrs. after diagnosis. Hypoglycemia controlled after TACE, octreotide and surgery. Recurrent hypoglycemia with progression and controlled with carboplatin/etoposide. | 5.1; died |
7 | F | 67 | 2; Ki 67 16%; | CT abdomen 2.8 cm pancreatic body lesion & hepatic masses. 10 years later lung nodules measuring up to 2.6 cm. | synchronous | NA | No, hypoglycemia occurred 8 months after diagnosis. 42/104/260 | Octreotide TACE x 1 PRRT x 5 carboplatin/ etoposide capecitabine/ temozolomide SBRT | Hypoglycemia 8 months after diagnosis. PRRT two cycles eight months later. Also, TACE. Hypoglycemia controlled on octreotide. PRRT two cycles at 3.5 yrs. Carboplatin etoposide at 4 years. Switched to capecitabine/temozolomide for 1.8 yrs. and discontinued for progression. Remained on octreotide. External radiation to liver mets at 8.6 yrs. One more dose PRRT at 9.5 yrs. Died 10.4 yrs. after diagnosis. Hypoglycemia controlled after PRRT and octreotide. | 10.4; died |
8 | M, | 73 | 1; Ki67 1.3% | CT abdomen showed 6.4 cm pancreatic lesion and lymph node involvement. 2.5 years, later liver metastases. Subsequently developed retroperitoneal nodes; osseous metastatic disease; small degree of lung disease | Metachronous | Cancer susceptibility multigene panel, 2015. Ambry Cancer Next Panel 2017. Negative for MEN1 mutations. VUS MRE11A gene | Yes, at diagnosis. 39/NA/NA | Surgery x 3 TAE x4 Octreotide Diazoxide Everolimus Capecitabine/ temozolomide PRRT x 4 | Distal pancreatectomy at diagnosis with resolution of hypoglycemia. Liver metastases 2.5 years after diagnosis. Started Octreotide at 2.6 years. TAE 2.6 years; 3.3 years. Diazoxide at 3.5 years with mild improvement hypoglycemia. Resection liver metastasis with LN dissection at 4 yrs. - significant improvement in hypoglycemia. Everolimus at 4.8 yrs. with immediate resolution of hypoglycemia. TAE at 5.3 yrs.; 5.5 yrs. Partial hepatectomy & LN dissection 5.8 yrs. Everolimus & octreotide discontinued. Interval progression of metastatic disease. Restarted octreotide at 6.3 yrs. PRRT 6.4, 6.5. 6.8. 7 yrs. Capecitabine/temozolomide at 11 yrs. for progression of disease. Also, on octreotide. Hypoglycemia controlled with surgeries and everolimus. | 12.3; alive |
9 | F | 62 | 2; Ki 67 3.5 % | Initially only pancreas. Portocaval nodes; liver nodules noted 3.75 yrs. Later | Metachronous | Invitae Genetics 79 gene panel 2016. Negative MEN1 screen | Yes, at diagnosis. 56/105.8/294 | Surgery Octreotide SBRT | Pancreatectomy at diagnosis. Recurrent hypoglycemia 3 years later. Liver metastases noted at 3.75 yrs. Octreotide therapy at 4 yrs. SBRT at 5 yrs. Died from cirrhosis related to NASH. Hypoglycemia controlled once patient went on octreotide. | 5.8;died |
10 | F | 20 | 2, Ki 67 20% | CT 3.9 cm lesion splenic hilum; hepatic lesions; breast, L1 lesion; osseous mets. Rt internal mammary node | Synchronous | UC500 germline panel negative | No, hypoglycemia documented 1.5 years after diagnosis, 48/45.1/195. | Capecitabine/ temozolomide Pembrolizumab PRRT Lanreotide | capecitabine/Temozolomide at diagnosis for 1 year. TACE at 1.4 years; than pembrolizumab + PRRT clinical trial (2 cycles) at 1.6 years. Also started lanreotide at 1.6 years Hypoglycemia controlled with combination of pembrolizumab, PRRT and lanreotide | 1.9 alive |
11 | M | 71 | 3, Ki 67 80% (high-grade neuroendocrine neoplasm) | CT abdomen 4.9 cm mass pancreatic head; liver metastases; skeleton | Synchronous | Invitae 62 gene germline panel negative | Yes, at diagnosis, 60/49/664.2 | Surgery Octreotide, Lanreotide, pembrolizumab PRRT | Octreotide soon after diagnosis; Whipple procedure + RFA 2 months later. carboplatin + etoposide 6 cycles started 4 months later. pembrolizumab + PRRT clinical trial (2 cycles) 10 months later. Lanreotide 10 months later Hypoglycemia controlled after PRRT and lanreotide | 1.2 alive |
12 | M | 50 | 2, Ki67 8% | MR abdomen 4 × 2.5 cm mass distal body/tail of pancreas; multiple metastases in liver | Synchronous | ---- | No, hypoglycemia 5 years after diagnosis 53/34/96.5 | Microwave ablation liver metastases. Surgery octreotide PRRT | Microwave ablation liver metastases 2 months after diagnosis.; Y90 SIRT at 5 months; Resection pancreatic tumor and lymph node dissection at 9 months. Octreotide at 1.4 years. PRRT x 2 cycles at 7 years. Hypoglycemia controlled after PRRT and octreotide | 7.3 alive |
13 | F | 64 | 2, Ki67 17.3% | CT abdomen 4.5 × 3.5 × 3.3 cm mass pancreatic tail and multiple metastases in liver | Synchronous | UC500 germline panel negative. | No, hypoglycemia 1.7 years after diagnosis. 36/18.1/320 | Octreotide Capecitabine/ temozolomide PRRT SBRT Pembrolizumab FOLFOX Carboplatin/etoposide | Octreotide at 2 months after diagnosis. Capecitabine/Temozolomide at 6 months. PRRT x 4 cycles at 1 year. SBRT to acetabulum and L4 met at 1.4 years. Pembrolizumab one dose + Y90 radioembolization at 1.6 years clinical trial.; FOLFOX at 1.8 years; carboplatin/etoposide 3 cycles at 2 years. Hypoglycemia controlled after treatment with FOLFOX | 2.5 died |
14 | M | 30 | 1, Ki67 < 1% | 5 cm tumor pancreatic head. Liver metastases first noted 3.4 years later | Metachronous | Germline testing UC500 germline panel MSH2 pL556S (c. 1667T > C, pLeu556Ser)—missense variant in the MSG2 DNA mismatch (MMR) protein—likely pathogenic for hereditary colorectal cancer | No, hypoglycemia 3.5 years after diagnosis. 41/20/229.7 | Surgeries x 2 Lanreotide Sunitinib Pembrolizumab Diazoxide Capecitabine/ temozolomide | Whipple procedure at diagnosis; Lanreotide at 1.8 years; sunitinib at 6 years Liver resection and microwave ablation at 7.4 years. Pembrolizumab at 8. 3 years (for high tumor mutation burden). Diazoxide at 9 years. Capecitabine/temozolomide 9.1 years Hypoglycemia controlled with food and diazoxide | 9.1 alive |
Abbreviations: PRRT, peptide receptor radionucleotide therapy with 177-Lutetium labelled somatostatin analogs; SBRT, stereotactic body radiation therapy; SIR, selective internal radiation therapy; TACE, transarterial chemoembolization; TAE, transarterial embolization.
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