Journal of Hepato-Biliary-Pancreatic Surgery

, Volume 14, Issue 6, pp 595–599 | Cite as

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

  • Gabriel Chan
  • Peter J. Horton
  • Sandra Thyssen
  • Marie Lamarche
  • Ayoub Nahal
  • David J. Hill
  • Errol B. Marliss
  • Peter Metrakos
Case reports of interest

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 II Hypoglycemia Fibrosarcoma Liver neoplasm 

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Copyright information

© Springer-Verlag Tokyo 2007

Authors and Affiliations

  • Gabriel Chan
    • 1
  • Peter J. Horton
    • 1
  • Sandra Thyssen
    • 2
  • Marie Lamarche
    • 3
  • Ayoub Nahal
    • 4
  • David J. Hill
    • 2
  • Errol B. Marliss
    • 3
  • Peter Metrakos
    • 1
  1. 1.Department of SurgeryMUHC-Royal Victoria HospitalQuebecCanada
  2. 2.Lawson Health Research InstituteSt. Joseph's Health CareLondonCanada
  3. 3.McGill Nutrition and Food Science CentreMUHC-Royal Victoria HospitalQuebecCanada
  4. 4.Department of PathologyMUHC-Montreal General HospitalQuebecCanada

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