Intrahepatic cholangiocarcinoma (ICC) is usually diagnosed in advanced stages of disease, more than half of the patients being unresectable at the time of their diagnosis.
Moreover, the disease is very aggressive, almost half of untreated patients dying during the first 4 month after diagnosis. Even in patients who undergo curative-intent treatment, the prognosis is still modest, with median survival less than 30 months in most series.
Although this dismal prognosis is typical for ICC, some patients experience unusual long-term survival, even when poor clinico-pathologic prognostic factors are present.
A 59 year-old female presented on CT scan and MRI an equivocal lesion, refusing biopsy for two years. During this period the tumor doubled its diameter and the patient eventually accepted to undergo percutaneous CT-guided biopsy. Pathologic evaluation of the specimen and immunohistochemistry assay suggested an ICC.
Subsequently, the patient underwent curative-intent surgery, being alive, without evidence of disease at 10 years after operation.
A discussion about diagnosis and prognostic factors related to overall survival and recurrence after curative-intent surgery is carried out, emphasizing the emerging role of molecular biomarkers.
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