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Mehta et al. Hepatoma Res 2018;4:7 I http://dx.doi.org/10.20517/2394-5079.2017.35 Page 7 of 9

Unfortunately at time of consultation in our institution the patient presented here was not eligible for
transplant given the extent of HCC. This prompted multidisciplinary care focused downstaging her cancer.
Radioembolization in the setting of PV thrombosis has been shown to be as effective and better tolerated
than TACE as PV thrombosis increases risk of necrosis [21,22] . Given progression after c-TACE, and lack of
portal supply, radioembolization was favored as a treatment. It is suggested in some cases that a background
[22]
of cirrhosis can protect from such injury . In this case, c-TACE had already been performed at an outside
facility. TACE has been shown to be safe and effective in patients with advanced or BCLC C disease which
includes patients with varying degrees of PV thrombosis [23,24] . However, PV thrombosis does portend a
[24]
poorer prognosis . As anticipated post embolization syndrome is the most common side effect reported
post TACE, while encephalopathy was found in approximately 5% of patients, and elevated liver function
[23]
tests as high as 20% of cases . In a comparison, c-TACE and drug eluting bead TACE had similar safety
profiles and survival rates comparable to treatment with sorafenib .
[23]
The use of AFP as an oncologic marker of response to loco-regional therapy for HCC has been proven to
[25]
be effective . The median time to response has been reported to be between 2 and 4 months therefore it
has been suggested that AFP used to identify patients who do not respond to treatment and prompt earlier
[25]
consideration of implementation of alternative strategies . Cases such as the one discussed here where there
is a dramatic increase in AFP despite imaging response with delayed response in the marker (~7 months)
have not widely been reported. Elevated levels of AFP have been seen in the setting of hepatic necro-
[26]
inflammatory activity, which could lead to over production of AFP . This may explain the incongruent
increase in AFP initially with delayed response in this case.

Despite dramatic imaging response and eventual decline of AFP, the patient developed REILD, which results
from normal hepatic parenchyma exposure to radiation. The clinical course is driven by a form of sinusoidal
obstruction syndrome marked by jaundice, ascites and mild increase in liver function tests. After 3 months
[27]
bilirubin can rise to 3 or higher . The incidence of REILD is reported to be between 0%-4% overall .
[28]
The patient was known to be at higher risk given decompensated liver function in the past therefore lobar
approach was selected. However in patient with cirrhosis REILD has been noted in 0%-33% of patients
[27]
who underwent whole liver treatment and 8%-15% in patients who underwent partial liver treatment .
Management as in this case is supportive.

In this case the patient did derive a survival benefit from radioembolization. Patients with intermediate
[29]
stage HCC are expected to have a median survival of 16 months from time of diagnosis . After
radioembolization the patient survived another 22 months. The biology of her disease suggests that initial
disease was less aggressive given that she survived 8 years beyond diagnosis.

In conclusion, congenital agenesis of the PV is a rare congenital anomaly due to abnormal embryologic
progression. The prognosis of patients affected with CAPV can vary depending on associated heart and
liver anomalies in infancy or the progression of hepatic dysfunction. Those patients are at risk of developing
HCC. Liver transplantation has been effective in patients with hepatic dysfunction. When transplantation
cannot be offered loco-regional therapy can offer palliative disease control and improved overall survival.
However liver directed therapy in this population could be associated with increased risk of liver failure.

DECLARATIONS
Authors’ contributions
Read and wrote the initial report: Mehta A
Corrected and amended areas related to imaging, intervention, and follow up: Venkat SR
Focused on the radiation aspects in terms of response and editing of clinical presentation: Portelance L
Helped review and highlighted oncology considerations: Feun LG