Author/Editor		Mušič, Mark; Pleskovič, Alojz; Ferlan-Marolt, Vera; Luzar, Boštjan
Title		Glukagonom
Translated title		Glucagonom
Type		članek
Source		Med Razgl
Vol. and No.		Letnik 42, št. 4
Publication year		2003
Volume		str. 419-25
Language		slo
Abstract		Glucagonoma is a rare neuroendocrine tumor with pancreatic alpha cell differentiation. This tumor is characterized by hypersecretion of a peptide hormone glucagon, which can be detected in this type of neoplasia. The majority of tumors are malignant and most patients already have a metastasis. The average patient age at the time of tumor discovery is 52.5 years. A characteristic, although not a pathognomonic sign of the tumor is a skin rash in the form of necrolytic migratory erythema. Along with diabetes or glucose intolerance, weight loss or cahexia, thromboembolic problems, anemia and diarrhea are the main signs of glucagonoma syndrome. In our case. A 53-year-old female patient had all of the major characteristic signs of glucagonoma syndrome, the diagnosis of which was delayed for three years. She was successfully operated on. No signs of abdominal metastases were found during surgery. Shortly after surgery, she was free of all symptoms. The histopathological examination revealed a benign, neuroendocrine tumor with a positive reaction to glucagon. Conclusion. One should be suspicious of this tumour in all patients with chronic erythema, especially in combination with other symptoms of glucagonoma syndrome. Early diagnosis is very important for improving the patient's survival and quality of life. The only successful treatment is complete surgical excision of the tumour. Ten year survival rates for a small number of patients with a limited disease have been calculated to be 64% and for those with metastases about 50%.
Summary		Uvod. Glukagonom je redek, nevroendokrini tumor, ki se razvije iz celic alfa trebušne slinavke. Značilnost tumorja je povečana tvorba glukagona, čeprav lahko proizvaja tudi druge hormone. V večini primerov je glukagonom maligen, ob odkritju ima več kot polovica bolnikov že zasevke. Najpogosteje zbolijo bolniki med 50. in 70. letom starosti. V klinični sliki glukagonoma opažamo značilen nekrolitični migrirajoči eritem, moteno toleranco za glukozo ali sladkorno bolezen, hujšanje, normocitno anemijo, trombembolizme in motnje črevesne gibljivosti. Vsi ti znaki so značilnost tako imenovanega sindroma glukagonoma. Prikaz primera. Proučili smo 53-letno bolnico, pri kateri se je sindrom glukagonoma pojavil z vsemi značilnimi znaki. Čas do diagnoze je bil tri leta. Med operacijo v trebušni votlini razsoja bolezni nismo našli. Dva centimetra velik tumor se je nahajal v repu trebušne slinavke. Tumor je bil izrezan z varnostnim robom. Bolnica je uspešno okrevala. S histopatološkim pregledom tkivnih vzorcev trebušne slinavke in tumorja smo ugotovili benigen nevroendokrini tumor, vrste glukagonoma. Zaključek. Na glukagonom moramo pomisliti pri bolnikih, ki imajo nepojasnjen kronični migrirajoči eritem, posebno v povezavi s spremembami v ustni sluznici, z moteno toleranco za glukozo ali sladkorno boleznijo, normocitno anemijo ter s hujšanjem. Edino uspešno zdravljenje je popolna kirurška odstranitev tumorja pri zamejeni bolezni. Desetletno preživetje bolnikov z zamejeno boleznijo je okoli 64%, pri bolnikih z zasevki pa okoli 50%. Ker tovrstni tumor ni pogost, so bila preživetja izračunana le pri majhnih skupinah bolnikov.
Descriptors		GLUCAGONOMA PANCREATIC NEOPLASMS MIDDLE AGE SKIN DISEASES ERYTHEMA CHRONICUM MIGRANS DIABETES MELLITUS CACHEXIA ANEMIA THROMBOEMBOLISM