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Cytologically , the tumor cells have a low nuclear to cytoplasmic ratio with abundant eosinophilic cytoplasm. Tumors are non-encapsulated, but well circumscribed, when compared to conventional HCC which typically has an invasive border. Diagnosis[ edit ] Due to lack of symptoms, until the tumor is sizable, this form of cancer is often advanced when diagnosed. Symptoms include vague abdominal pain, nausea, abdominal fullness, malaise and weight loss.

They may also include a palpable liver mass. The usual markers for liver disease — aspartate aminotransferase , alanine aminotransferase and alkaline phosphatase — are often normal or only slightly elevated.

It is associated with elevated neurotensin levels. Diagnosis is normally made by imaging ultrasound , CT or MRI and biopsy Treatment[ edit ] In FHCC, plasma neurotensin and serum vitamin B12 binding globulin are commonly increased and are useful in monitoring the disease and detecting recurrence.

Liver resection is the optimal treatment and may need to be performed more than once, since this disease has a very high recurrence rate. As the tumor is quite rare, there is no standard chemotherapy regimen.

Radiotherapy has been used but data is limited concerning its use. The survival rate for fibrolamellar HCC largely depends on whether and to what degree the cancer has metastasized , i. Distant spread metastases , significantly reduces the median survival rate.

Unlike the more common HCC, patients most often do not have coexistent liver disease such as cirrhosis. History[ edit ] This disease was first described by Hugh Edmondson in a year-old female with no underlying liver disease.


Fibrolamellar carcinoma

Mikakasa Radiol Clin North Am ; He had done well since his liver transplantation until Augustwhen a left lung nodule 3 cm in size was noted on a PET CT, in addition to a 2-cm borderline hypermetabolic lesion in the tail of the pancreas. The AFP level was 3. These include the presence of sheets of large polygonal or spindle-shaped tumor cells with abundant granular eosinophilic cytoplasm, prominent nuclei, and paucicellular fibrous stroma arranged in thin parallel lamellae around tumor cells. Due to lack of symptoms, until the tumor is sizable, this form of cancer is often advanced when diagnosed.


Fibrolamellar hepatocellular carcinoma


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