title: Management of a metastasized high grade insulinoma (G3) with refractory hypoglycemia: case report and review of the literature.
abstract: Insulinomas represent the most common functional neuroendocrine tumor of the pancreas. They are usually solitary, benign, well differentiated (G1/G2) and curable by surgery. We describe the case of a 45 year old male Caucasian with a unique malignant, metastasized pancreatic insulinoma (Ki 67 of 70%, G3). To control excessive insulin production emanating in refractory hypoglycemia and growth of the highly proliferating tumor a multimodal therapeutic approach including the consecutive use of tumor debulking surgery, chemotherapy, TACE, SIRT, PRRT as well as a drug therapy with diazoxide, somatostatin analogs and everolimus was employed. Chemotherapy with carboplatin/etoposide plus everolimus provided the longest normoglycemic period. After progress chemotherapy with dacarbazine had the most positive effect, while debulking approaches such as surgery and liver directed therapies, as well as PRRT were less efficient with only transient success.
authors.affiliation: Department of Gastroenterology and Endocrinology, Philipps-University, Marburg, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Scharf
authors.affiliation: Department of Gastroenterology and Endocrinology, Philipps-University, Marburg, Germany.
authors.forename: Daniela
authors.initials: D
authors.lastname: Mueller
authors.affiliation: Department of Gastroenterology and Endocrinology, Philipps-University, Marburg, Germany.
authors.forename: Ute
authors.initials: U
authors.lastname: Koenig
authors.affiliation: Department of Nuclear Medicine, Philipps-University, Marburg, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Pfestroff
authors.affiliation: Department of Pathology, Philipps-University, Marburg, Germany.
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Nimphius
authors.affiliation: Department of Radiology, Philipps-University, Marburg, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Figiel
authors.affiliation: Department of Gastroenterology and Endocrinology, Philipps-University, Marburg, Germany.
authors.forename: Anja
authors.initials: A
authors.lastname: Rinke
authors.affiliation: Department of Gastroenterology and Endocrinology, Philipps-University, Marburg, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Koenig
authors.affiliation: Department of Gastroenterology and Endocrinology, Philipps-University, Marburg, Germany. Electronic address: gress@med.uni-marburg.de.
authors.forename: Thomas
authors.initials: T
authors.lastname: Gress
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D017809:Fatal Outcome; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology / Q000628:therapy*; D007328:Insulin / Q000378:metabolism; D007340:Insulinoma / Q000150:complications / Q000601:surgery / Q000628:therapy*; D019394:Ki-67 Antigen / Q000032:analysis; D008113:Liver Neoplasms / Q000556:secondary / Q000601:surgery; D008297:Male; D008875:Middle Aged; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000150:complications / Q000601:surgery / Q000628:therapy*; D013156:Splenectomy
chemical_list: D007328:Insulin; D019394:Ki-67 Antigen
keywords: G3; Liver directed therapies; Malignant insulinoma; Neuroendocrine pancreatic tumor; PRRT; Refractory hypoglycemia