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Glucagonomas/diabetico‐dermatogenic syndrome (DDS): A statistical evaluation of 407 reported cases
Author(s) -
Soga Jun,
Yakuwa Yasuko
Publication year - 1998
Publication title -
journal of hepato‐biliary‐pancreatic surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.63
H-Index - 60
eISSN - 1868-6982
pISSN - 0944-1166
DOI - 10.1007/s005340050052
Subject(s) - medicine , glucagonoma , hyperglucagonemia , pancreas , diabetes mellitus , gastroenterology , anemia , pancreatic cancer , pathology , cancer , endocrinology , insulin , glucagon , insulinoma
Of the rare pancreatic endocrinomas, glu‐cagonomas, either with or without diabetico‐dermatogenic syndrome (DDS), are probably third in frequency after insulinomas and gastrinomas. This study was carried out to evaluate the present status of glucagonoma/DDS in a statistically reliable number of cases and to provide precise information to investigators actively working in this particular field of research. A total of 407 cases of glucagonoma were collected from the international literature and evaluated according to characteristic clinicopathologic features. Findings were: (1) The incidence of DDS was 57.2% (233/407). (2) The tail of the pancreas was predominantly involved, in 53.7% (213/397). (3) One‐third of the tumors (80 of 276 for whom size was recorded; 29.0%) measured 20 mm or less. (4) Metastases occurred in 51.4% (209/407) and malignant tumors in 60.7% (247/407). (5) Multiplicity occurred in 11.8% (48/407), and associated multiple endocrine neoplasia type 1 in 13.0% (53/407). (6) In the patients with DDS, the rates of hyperglucagonemia, necrolytic migratory erythema, diabetes mellitus, loss of weight, hypo‐aminoacidemia, or anemia, as representative constituents of DDS, were all higher than rates in the overall series ( P < 0.01). (7) The 10‐year survival rate in the 233 patients with DDS was 51.6% in those with metastases and 64.3% in those without metastases ( P < 0.001).