Fibrolamellar hepatocellular carcinoma: current clinical perspectives

the Cancer Therapy Advisor take:

Fibrolamellar carcinoma (FLC) is a rare form of hepatocellular carcinoma (HCC) that accounts for about 1% to 9% of all HCCs.

Although FLC is a type of HCC, it typically affects young adults age 10 to 35 years with no underlying liver disease.

FLC typically presents with vague abdominal pain, nausea, abdominal fullness, malaise, and weight loss. Although physical exam may be within normal limits, patients often present with a palpable abdominal mass or enlarged liver with or without pain in the right upper quadrant of the abdomen.

In regard to diagnosis, magnetic resonance imaging (MRI) is the preferred imaging modality because it is helpful in distinguishing FLC from other liver lesions.¹⁸

F-FDG positron emission tomography–computed tomography (PET/CT) may be useful for diagnosis as well, but further studies of the effectiveness of PET/CT in FLC are warranted.

The mainstay of treatment of FLC is surgical resection as it represents the only potentially curative approach. The ideal treatment is complete surgical resection of the tumor with negative margins in addition to adequate lymph node dissection.

Patients who undergo surgical resection have a 5-year survival rate of 51.6% to 70% compared with 0% among those who do not undergo resection.

On the other hand, FLC is not typically responsive to systemic chemotherapy, but some regimens like GEMOX and FOLFOX have been used with some success.

Fluorouracil combined with recombinant interferon alpha-2b may also be beneficial. Locoregional therapies such as radiation therapy have been considered for FLC as well.

For example, Maniaci et al have proposed an intensive surveillance protocol following surgery that includes CT and serum vitamin B12-binding protein levels every 3–6 months for the first 2–3 years postoperatively.⁵⁴ In cases where serum vitamin B12-binding protein is elevated and CT scan is negative, the authors recommend PET/CT.⁵⁴

While this or other protocols have not been vigorously studied – and therefore cannot be endorsed – the data collectively suggest that close surveillance is warranted. If recurrence is detected, depending on the site and number of recurrent lesions, repeat surgical resection should be considered, as other treatment options are not effective.

For example, Maniaci et al reported on ten patients with FLC treated with resection followed by close surveillance and re-excision, systemic chemotherapy, as well as radiotherapy for any relapses. This study showed a median overall survival of 9.3 years (95% confidence interval, 3.0–18.5) with two patients showing at least partial response to cisplatin and fluorouracil.⁵⁴

For patients with FLC who present with unresectable disease, liver transplantation should be considered as 3-year survival following 75%–80% transplantation approaches.⁷⁴ While transplantation may be used in cases of FLR, it is much more commonly indicated for HCC than FLC.⁷⁰

This is likely due to the fact HCC is more common than FLC as well as the fact that regional lymph node metastasis (a relative contraindication to transplant) is more common in FLC (42.2%) compared with HCC (22.2%).⁷⁰

Topics:

Gastrointestinal Cancer

Fibrolamellar hepatocellular carcinoma: current clinical perspectives

the Cancer Therapy Advisor take:

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