Metastatic glucagonoma: treatment with liver transplantation.
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.
Radny
Peter
P
Department of Dermatology, University of Tuebingen, Tuebingen, Germany. calvin32@gmx.de
Eigentler
Thomas Kurt
TK
Soennichsen
Karsten
K
Overkamp
Dietrich
D
Raab
Hans-Rudolf
HR
Viebahn
Richard
R
Mueller-Horvart
Christian
C
Sotlar
Karl
K
Rassner
Gernot
G
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
D018931:Antineoplastic Agents, Hormonal; D017481:Receptors, Somatostatin; D005934:Glucagon; D015282:Octreotide