[Glucagonoma: a recent series of 7 cases].
We report a series of 7 glucagonoma patients seen between 1994 and 2001, 5 males and 2 females, aged 32-69 years, with: necrolytic migratory erythema (NME) (n = 2), liver metastases (n = 3), jaundice (n = 1) and 1 case of familial history of multiple endocrine neoplasia. The diagnosis combined histology and hyperglucagonemia; 6 patients developed metastasis (5 initially); during the follow-up 3 developed a necrolytic erythema migraticum (NEM) worsening the general status. Somatostatin receptor scanning was highly positive in all. Four patients were operated, 5 received chemotherapy (2 OR and 2 SD), 3 had chemoembolization (1 transient improvement). Somatostatin was efficient on general status or skin lesions in all patients. Two died and 5 are alive with a follow up ranging from 12 to 60 months. We want to emphasize on the higher frequency than expected of this disease, the frequency of NEM, the efficacy of SMS on NEM and general status and on the fairly good prognosis. The high uptake of SMS by tumors on scanning could rise hopes about radioconjugate therapy.
Vauleon
Elodie
E
Département d'oncologie médicale, Centre Eugène Marquis, 35042 Rennes, FRANCE.
Egreteau
Joëlle
J
Boucher
Eveline
E
Desfourneaux
Véronique
V
Bretagne
Jean-François
JF
Raoul
Jean-Luc
JL
D000328:Adult; D000368:Aged; D004890:Erythema / Q000188:drug therapy / Q000209:etiology*; D005260:Female; D005935:Glucagonoma / Q000150:complications* / Q000175:diagnosis / Q000556:secondary; D006728:Hormones / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis; D013004:Somatostatin / Q000627:therapeutic use
D006728:Hormones; D013004:Somatostatin
Le glucagonome: à propos d'une série récente de 7 cas.