John Libbey Eurotext

Bulletin du Cancer

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Glucagonoma: a recent series of 7 cases Volume 91, issue 7, Juillet - Août 2004

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Authors
Département d’oncologie médicale, Centre Eugène Marquis, 35042 Rennes Cedex, Département de chirurgie viscérale, Service des maladies de l’appareil digestif, CHRU Pontchaillou, 35033 Rennes Cedex, J.-L. Raoul.

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 developped metastasis (5 initially); during the follow-up 3 developped 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.