Journal of Hepato-Biliary-Pancreatic Surgery

, Volume 14, Issue 6, pp 595–599

Malignant transformation of a solitary fibrous tumor of the liver and intractable hypoglycemia

Authors

  • Gabriel Chan
    • Department of SurgeryMUHC-Royal Victoria Hospital
  • Peter J. Horton
    • Department of SurgeryMUHC-Royal Victoria Hospital
  • Sandra Thyssen
    • Lawson Health Research InstituteSt. Joseph's Health Care
  • Marie Lamarche
    • McGill Nutrition and Food Science CentreMUHC-Royal Victoria Hospital
  • Ayoub Nahal
    • Department of PathologyMUHC-Montreal General Hospital
  • David J. Hill
    • Lawson Health Research InstituteSt. Joseph's Health Care
  • Errol B. Marliss
    • McGill Nutrition and Food Science CentreMUHC-Royal Victoria Hospital
  • Peter Metrakos
    • Department of SurgeryMUHC-Royal Victoria Hospital
Case reports of interest

DOI: 10.1007/s00534-007-1210-0

Cite this article as:
Chan, G., Horton, P., Thyssen, S. et al. J Hepatobiliary Pancreat Surg (2007) 14: 595. doi:10.1007/s00534-007-1210-0

Abstract

Sarcomas of the liver are rare. We report a case of intractable hypoglycemia secondary to a solitary fibrous tumor that underwent malignant transformation into a fibrosarcoma. A 70-year-old man presented with a hepatic mass and tumor-associated hypoglycemia which was resistant to medical management. Blood tests were remarkable only for elevated serum insulin-like growth factor (IGF)-2. The hypoglycemia resolved following resection of a solitary fibrous tumor surrounded by a high-grade fibrosarcoma. Real time reverse transcriptase polymerase chain reaction (RT-PCR) measured elevated levels of IGF2 mRNA in both the solitary fibrous tumor and the fibrosarcoma. Immunoblotting demonstrated a series of bands in the size range of pro-IGF2. Unfortunately, disseminated metastases developed 1 year later, concurrent with a recurrence of hypoglycemia, marked again by elevation of serum IGF2. Solitary fibrous tumors of the liver have a real risk of malignant transformation. The severity of the tumor-associated hypoglycemia may parallel the tumor burden and activity. The syndrome is the systemic effect of IGF2 secreted by the tumor. Surgery can treat the hypoglycemia syndrome and the underlying malignancy.

Key words

Insulin-like growth factor IIHypoglycemiaFibrosarcoma Liver neoplasm

Copyright information

© Springer-Verlag Tokyo 2007