Treatment
Surgical resection
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When feasible, surgery is the cornerstone therapeutic modality for patients with FLC as it represents the only potentially curative option.
Complete surgical resection of the FLC tumor with negative margins along with an adequate lymph node dissection is the ideal treatment. In a systematic review by Mavros et al, the authors analyzed 575 patients with FLC.64
The authors noted that patients who underwent resection of FLC had a 5-year survival of 70% compared with 0% among those patients who did not undergo surgical resection.64
The average size of the FLC tumor resected was between 9 cm and 13 cm.6,50,65 In a separate study, Stipa et al reported on 28 resected FLC patients and noted that 75% of the patients who underwent surgery for FLC required either a hemi-hepatectomy or an extended hepatectomy.6
As the surgeries are often complex, a complete (R0) resection is not always possible, but it is important for survival. In a study by Darcy et al, which looked at 21 patients who underwent resection for FLC at a highly specialized cancer center, a complete (R0) resection was achieved in 17 (80.9%) patients, an R1 in two patients (9.5%), and an R2 in two patients (9.5%).65
The overall 5-year survival in this cohort was 42.6% (95% confidence interval, 20–65.2), while the 5-year overall survival of those who underwent complete resection was 51.6%. Improved long-term overall survival was associated with R0 resection (P=0.003).65
| Table I – Prognostic factors in fibrolamellar carcinoma |
| Positive prognostic factors | ||
| Complete resection | ||
| Earlier tumor stage at diagnosis | ||
| Absence of large vessel invasion | ||
| Female sex | ||
| Negative prognostic factors | ||
| Positive lymph node status | ||
| Multiple tumors | ||
| Metastatic disease | ||
| Older age at diagnosis |
In addition to R0 resection, regional lymph node dissection is warranted due to the high incidence of lymph node metastasis and regional recurrence in patients with nodal disease.6,66,67
Several factors are associated with a better prognosis following surgery including younger age at diagnosis, earlier tumor stage at diagnosis, as well as absence of large vessel invasion or thrombosis.5,12
Factors associated with a particularly poor prognosis include lymph node metastasis, multiple tumors, metastatic disease at presentation, and vascular invasion.6,13,65,68 There have been conflicting data regarding sex as a prognostic factor, as studies have variably reported female sex to be both a favorable and adverse factor associated with long-term survival (Table 1).5,68,69
Prognosis following resection of FLC has also been suggested to be better than typical HCC (Figure 3).3,5–7,58,70 There are several factors that may contribute to the better prognosis of FLC patients, including that FLC patients are typically younger and healthier.
In addition, FLC patients have normal underlying liver parenchyma, which may allow for more aggressive resections and decrease the risk of de novo future disease. As noted, the ability to perform complete resection has been reported to be one of the most important and well described prognostic factors for FLC.5,6,65,68,71
Despite a generally good long-term prognosis, recurrence following resection of FLC is relatively common with rates ranging from 33% to 100% and a median recurrence-free survival of 20–48 months.6,72
For example, in a small series of 28 patients who underwent resection of FLC, Stipa et al reported a 5-year recurrence-free survival of only 18% with an overall recurrence rate of 60%.6 The most common sites of recurrence include lymph nodes, liver, peritoneum, lungs, and bone.73 Due to the high recurrence associated with FLC, diligent postoperative surveillance is indicated.
