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

Department of Surgery, MUHC-Royal Victoria Hospital, Montreal, Quebec, H3A 1A1, Canada.
Journal of Hepato-Biliary-Pancreatic Surgery (Impact Factor: 1.6). 02/2007; 14(6):595-9. DOI: 10.1007/s00534-007-1210-0
Source: PubMed


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.

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    • "In some fibrosarcoma patients with nonislet-cell tumor hypoglycemia (NICTH), a high molecular weight form of IGF-II (big IGF-II) derived from tumors is present in the circulation and might be associated with recurrent hypoglycaemia [176]. In addition, elevated levels of IGF2 mRNA were found in both the solitary fibrous tumors that underwent malignant transformation to fibrosarcomas and fibrosarcomas [177]. "
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    Current Medicinal Chemistry 04/2013; 20(23). DOI:10.2174/0929867311320230003 · 3.85 Impact Factor
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    • "The patient may be asymptomatic or have non-specific clinical manifestations, including increased abdominal volume and circumference and abdominal pain, distention, and discomfort, as seen in our patient, or other symptoms, such as anorexia, nausea, vomiting and weight loss. Fever, hypoglycemia, abnormal liver tests and biliary-duct compression that leads to cholestasis and progressive jaundice are less commonly seen [12,13,16,19]. Laboratory results are usually normal; however, a few patients have had deranged liver function tests or elevated serum levels of a-fetoprotein [15,19,20]. "
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