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Endocrine Abstracts (2021) 73 EP152 | DOI: 10.1530/endoabs.73.EP152

Donostia Unibertsitate Ospitalea, Donostia, Spain


Introduction

Malignant insulinoma is a rare pancreatic neuroendocrine tumor that accounts for only 10% of all cases of insulinoma. The clinical picture is characterized by the presence of severe hyperinsulinemic hypoglycaemic syndrome in a patient with pancreatic tumor with locoregional and/or distant metastases. Therapeutic management is challenging due to the need to control both hypoglycemic syndrome and tumor growth. Curative surgery is rarely applicable due to widespread metastases. We analyze clinico pathological characteristics, treatments and prognosis of 3 patients diagnosticated from 2009 to 2015.

Surgery was the first choice of treatment in all the cases but it was dismissed from surgeon. Glycaemic control was the therapeutic challenge in these patients. In case 2 the hypoglycemic was controlled with diazoxide and lanreotide LAR. The others needed corticosteroid therapy and high doses of diazoxide to avoid hypoglycemia. After somatostatin analog Everolimus was the first line treatment. Case 1 refused this therapy and case 2 took only for one month because of bad tolerance. Hepatic metastases embolization improve the glycemic control in case 1. Lu- DOTATE was not available in our center.

  CASE 1 CASE 2 CASE 3
SEX Female Female Male
AGE 37 37 77
TUMOR SIZE 4.5 5 5
METASTASIS Hepatic + lymph node Hepatic + lymph node Hepatic + lymph node
GLUCOSE 10 42 38
INSULINE 86 38 40
C PEPTIDE 7.7 8.7 9.5
PROINSULINE  > 100  > 100  > 100
KI67 20%  > 20% 5-10%
HYPOG TREATMENT Diazoxide + Dexameta Diazoxide Diazoxide + Dexameta
SURGERY No No No
S. ANALOGS Lanreotide LAR Lanreotide LAR Lanreotide LAR
2° LINE Hepatic mets embolizat Everolimus Everolimus
3° LINE   Temozola + Capecita Sunitib
SURVIVAL 22 months 26 months 29 months

Conclusion

Surgery is the unique curative treatment but is rarely applicable due to the widespread metastasis at diagnosis. The hypoglycemic syndrome is often refractory and decreased quality of life of these patients, in addition to medical treatment, debulking surgery or locoregional therapies can improve the symptoms control.

Volume 73

European Congress of Endocrinology 2021

Online
22 May 2021 - 26 May 2021

European Society of Endocrinology 

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