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Malignant transformation of a solitary fibrous tumor of the liver and intractable hypoglycemia
Author(s) -
Chan Gabriel,
Horton Peter J.,
Thyssen Sandra,
Lamarche Marie,
Nahal Ayoub,
Hill David J.,
Marliss Errol B.,
Metrakos Peter
Publication year - 2007
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/s00534-007-1210-0
Subject(s) - fibrosarcoma , hypoglycemia , solitary fibrous tumor , malignant transformation , medicine , malignancy , pathology , liver tumor , endocrinology , gastroenterology , insulin , biology , hepatocellular carcinoma , stem cell , cd34 , genetics
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.