Dermatologic Manifestations of Glucagonoma Syndrome Treatment & Management
- Author: Nathalie C Zeitouni, MD, CM, FRCPC; Chief Editor: William D James, MD more...
Medical Care
Most traditional chemotherapies are not effective on glucagonoma, probably due to the tumor's indolent nature. No cure by medical means has been reported, but some drug and hormone regimens may induce tumor regression or symptomatic remission of NME.
Streptozocin, a drug that causes selective islet cell damage, shows promise in combination with 5-fluorouracil. The combination can achieve a 60-70% response rate. Dacarbazine induced remission from NME by reducing tumor burden and glucagon levels in at least one instance.[6]
Transcatheter hepatic artery embolization (TAE) may induce selective necrosis of hepatic metastases by providing locally high levels of antineoplastic agents, thus sparing systemic adverse effects. TAE in association with cisplatin-releasing gelatin microspheres has been reported in one study for metastatic liver tumors.[7]
The somatostatin agonists octreotide and lanreotide are the drugs of choice in the treatment of patients with glucagonoma. These somatostatin analogs can be coupled to radioisotopes for both imaging and treatment applications.[8]
Sunitinib, a small molecule inhibitor of multiple receptor tyrosine kinases critical for tumor growth and angiogenesis, including PDGF receptors, has been shown to be effective in treating numerous pancreatic neuroendocrine tumors but may be particularly efficacious in the treatment of glucagonomas.[9]
In pseudoglucagonoma syndrome, treating the underlying disease often resolves NME.
Surgical Care
Surgical resection of the glucagonoma is the only treatment demonstrating a cure. This surgical procedure is possible when the tumor is still localized. Because the tumor is slow growing and tends to be encapsulated, the resection can be performed without adjunct therapy. The use of laparoscopy has been reported in a few cases.
When the tumor is disseminated, surgical management is used to palliate symptoms. Since NME is the major source of morbidity in glucagonoma syndrome, palliative therapies are aimed at reducing the cutaneous eruption. Tumor debulking has been shown to decrease the intensity of the cutaneous symptoms by reducing the level of serum glucagon.
Multimodality therapy for liver metastases combining surgery, transarterial chemoembolization, percutaneous radiofrequency thermal ablation, and long-acting octreotide has been proven to be effective.[10]
Diet
Cases of NME secondary to celiac sprue can resolve with implementation of a gluten-free diet. Deficiencies in amino acids, essential fatty acids, and zinc have been linked to NME by observation that symptoms resolve with supplementation of the deficient nutrient.[11]
Nakashima H, Komine M, Sasaki K, Mitsui H, Fujimoto M, Ihn H, et al. Necrolytic migratory erythema without glucagonoma in a patient with short bowel syndrome. J Dermatol. Aug 2006;33(8):557-62. [Medline].
Doyle JA, Schroeter AL, Rogers RS 3rd. Hyperglucagonaemia and necrolytic migratory erythema in cirrhosis--possible pseudoglucagonoma syndrome. Br J Dermatol. Nov 1979;101(5):581-7. [Medline].
Echenique-Elizondo M, Tuneu Valls A, Elorza Orúe JL, Martinez de Lizarduy I, Ibáñez Aguirre J. Glucagonoma and pseudoglucagonoma syndrome. JOP. Jul 2004;5(4):179-85. [Medline].
Chao SC, Lee JY. Brittle nails and dyspareunia as first clues to recurrences of malignant glucagonoma. Br J Dermatol. Jun 2002;146(6):1071-4. [Medline].
Lewis RB, Lattin GE Jr, Paal E. Pancreatic endocrine tumors: radiologic-clinicopathologic correlation. Radiographics. Oct 2010;30(6):1445-64. [Medline].
van der Loos TL, Lambrecht ER, Lambers JC. Successful treatment of glucagonoma-related necrolytic migratory erythema with dacarbazine. J Am Acad Dermatol. Feb 1987;16(2 Pt 2):468-72. [Medline].
Nitta N, Ohta S, Tanaka T, Takazakura R, Toyama T, Sonoda A, et al. An initial clinical study on the efficacy of cisplatin-releasing gelatin microspheres for metastatic liver tumors. Eur J Radiol. Sep 2009;71(3):519-26. [Medline].
Melen-Mucha G, Lawnicka H, Kierszniewska-Stepien D, Komorowski J, Stepien H. The place of somatostatin analogs in the diagnosis and treatment of the neuoroendocrine glands tumors. Recent Pat Anticancer Drug Discov. Jun 2006;1(2):237-54. [Medline].
Mir O, Coriat R, Goldwasser F. Advances in pancreatic neuroendocrine tumor treatment. N Engl J Med. May 12 2011;364(19):1871; author reply 1873-4. [Medline].
Poggi G, Villani L, Bernardo G. Multimodality treatment of unresectable hepatic metastases from pancreatic glucagonoma. Rare Tumors. Jul 22 2009;1(1):e6. [Medline]. [Full Text].
Blackford S, Wright S, Roberts DL. Necrolytic migratory erythema without glucagonoma: the role of dietary essential fatty acids. Br J Dermatol. Nov 1991;125(5):460-2. [Medline].
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