Malignant transformation of a solitary fibrous tumor of the liver and intractable hypoglycemia
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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Available from: Aikaterini Berdiaki
- "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 . In addition, elevated levels of IGF2 mRNA were found in both the solitary fibrous tumors that underwent malignant transformation to fibrosarcomas and fibrosarcomas . "
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ABSTRACT: The specific organization of the tumor extracellular matrix (ECM) is an intrinsic and basic step in the convoluted pathways of tumorigenesis. Fibrosarcoma is a rare, lethal, malignant tumor originating from fibroblasts, characterised by the formation of an abundant ECM. Fibroblastoid cells undergoing malignant transformation specifically alter composition and organization of their ECM to facilitate growth, survival and invasion. Fibrosarcoma cells were shown to have a high content and turnover of ECM components including hyaluronan, proteoglycans, collagens, fibronectin and laminin. Cell signaling by endogenous growth factors, such as TGFβ, EGF, FGF2, VEGF and IFG-I, is directly correlated to ECM remodeling, stroma formation and fibrosarcoma progression. In this regard, growth factors affect the expression of matrix macromolecules, such as secreted and cell-associated proteoglycans, hyaluronan and it receptors CD44 and RHAMM, as well as the expression and activity of matrix- degrading metalloproteinases, which are of critical importance in tissue remodeling and fibrosarcoma progression. Therefore, therapeutic approaches considering growth factors and their receptors as well as downstream signaling in human cancers may well be pharmacological targets being currently explored. In this article, we focus on growth factor signaling regulating fibrosarcoma cell ECM organization at the level of deposition and degradation of ECM macromolecules, the relation of ECM remodeling with fibrosarcoma cell malignant behaviour as well as the putative strategies for its therapeutic intervention.
Available from: Quanyan Liu
- "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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ABSTRACT: Solitary fibrous tumors (SFTs) of the liver are rarely described; only 38 cases have been reported in literature, most of which have shown benign clinical characteristics, and only 3 of these cases exhibited malignant variants. In this study, we present a 24-year-old woman with a 1-month history of a rapidly enlarging abdominal mass and a CT showing an exophytic heterogeneous liver mass with a firm parietal bone mass. The patient underwent a transcatheter arterial chemoembolization (TACE) before operation, and an extended right hepatectomy and craniectomy with a negative margin was performed under general anesthesia. The masses showed histological features of oval spindle cells haphazardly arranged in the classic short-storiform or so-called patternless pattern of solitary fibrous tumors. The tumor cells showed positive immunohistochemical reactions to CD34 and bcl-2. The tumor recurred in the residual liver 2 months after operation, metastatic osteoblastic lesions in the thoracic and lumbar vertebrae were identified 3 months after the operation, and lumbar vertebrae metastasis 7 months after operation paralyzed the patient. The patient underwent percutaneous ethanol injection therapy (PEI) and chemotherapy, but the patient died because of the uncontrolled tumor 16 months after the initial operation. To our knowledge, this is the first case of malignant solitary fibrous liver tumors with skeletal metastasis.
Available from: koreamed.org
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ABSTRACT: A 46-year-old woman was found to have a huge liver mass that was detected by abdominal ultrasonography. Abdominal CT and MRI showed a 10 cm-sized, encapsulated mass occupying the anterior segment of the right hepatic lobe. Extended right hemihepatectomy was performed and pathological examination revealed fibroblast-like spindle cells within dense deposits of collagen. On immunohistochemical staining, these spindle tumor cells showed an intense CD34 immunoreactivity. The patient is alive without evidence of tumor recurrence 7 months after the resection. Solitary fibrous tumor is a very rare neoplasm found in the liver parenchyma, and it has been reported in less than 30 patients in the English literature. We present here the first such case in Korea.
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