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Glucagonoma syndrome.

T Hashizume1, H Kiryu, K Noda

  • 1Department of Dermatology, Saga Prefectural Hospital, Japan.

Journal of the American Academy of Dermatology
|August 1, 1988
PubMed
Summary

Glucagonoma syndrome, a rare condition, was misdiagnosed for a decade in a Japanese man. Surgical removal of pancreatic alpha cell carcinoma resolved characteristic skin eruptions, highlighting the importance of recognizing glucagonoma syndrome symptoms.

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Area of Science:

  • Endocrinology
  • Oncology
  • Dermatology

Background:

  • Glucagonoma syndrome is a rare condition characterized by a glucagon-producing pancreatic tumor, often an alpha cell carcinoma.
  • Clinical presentation can be non-specific, leading to delayed diagnosis and prolonged patient suffering.

Observation:

  • A 53-year-old Japanese male presented with a 10-year history of skin eruptions characteristic of glucagonoma syndrome.
  • Initial plasma glucagon levels were not elevated; however, a later arginine tolerance test revealed elevated glucagon levels.
  • A pancreatic alpha cell carcinoma with lymph node metastasis was identified.

Findings:

  • Surgical resection of the pancreatic tumor (partial pancreatectomy with lymphadenectomy) led to complete resolution of the skin eruption within 10 days.
  • The case underscores the diagnostic challenges and the effectiveness of surgical intervention for glucagonoma syndrome.

Implications:

  • Increased clinical awareness of glucagonoma syndrome is crucial for timely diagnosis and management.
  • Early detection and surgical treatment of pancreatic alpha cell carcinoma can significantly improve patient outcomes and quality of life.

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