title: Metastatic glucagonoma: treatment with liver transplantation.
abstract: Glucagonoma is a rare pancreatic endocrine tumor that is often both well developed and malignant at detection. In the case of metastatic spread the patient has a poor long-term prognosis. We hope to familiarize dermatologists and other specialists with this rare and potentially fatal disorder because early recognition of necrolytic migratory erythema, a clinical feature that may appear in patients with glucagonoma, can lead to possible cure, whereas delayed identification of the disease is associated with metastatic disease and a poor prognosis. We report the case of a 57-year-old patient with a metastatic glucagon-producing tumor; necrolytic migratory erythema was diagnosed and was successfully treated by a multimodal intervention including liver transplantation. Currently, 72 months after transplantation, our patient is in complete remission, which has been verified by somatostatin receptor scintigraphy monitoring, computed tomographic scanning and glucagon serum control. Increased awareness of the clinical symptoms and visible polymorphic mucocutaneous and nonspecific histopathologic features of glucagonoma syndrome is needed to avoid unnecessary delay in the diagnosis of this syndrome.
authors.affiliation: Department of Dermatology, University of Tuebingen, Tuebingen, Germany. calvin32@gmx.de
authors.forename: Peter
authors.initials: P
authors.lastname: Radny
authors.forename: Thomas Kurt
authors.initials: TK
authors.lastname: Eigentler
authors.forename: Karsten
authors.initials: K
authors.lastname: Soennichsen
authors.forename: Dietrich
authors.initials: D
authors.lastname: Overkamp
authors.forename: Hans-Rudolf
authors.initials: HR
authors.lastname: Raab
authors.forename: Richard
authors.initials: R
authors.lastname: Viebahn
authors.forename: Christian
authors.initials: C
authors.lastname: Mueller-Horvart
authors.forename: Karl
authors.initials: K
authors.lastname: Sotlar
authors.forename: Gernot
authors.initials: G
authors.lastname: Rassner
mesh_terms: D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D005934:Glucagon / Q000097:blood; D005935:Glucagonoma / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology / Q000556:secondary* / Q000601:surgery*; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology / Q000556:secondary* / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D015282:Octreotide / Q000008:administration & dosage; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000473:pathology* / Q000601:surgery; D011877:Radionuclide Imaging; D017481:Receptors, Somatostatin / Q000378:metabolism; D013156:Splenectomy
chemical_list: D018931:Antineoplastic Agents, Hormonal; D017481:Receptors, Somatostatin; D005934:Glucagon; D015282:Octreotide