Keywords
Hepatoblastoma - hypertension - paraneoplastic
Introduction
Hepatoblastoma accounts for 1% of pediatric cancers with an incidence peak in the
first 5 years of life and a slight male preponderance.[1]
[2] Clinical presentation may include from rapidly enlarging abdominal mass to abdominal
pain, abdominal pain, failure to thrive in young children, or loss of weight in adolescents.
Paraneoplastic manifestations are commonly described.[3] Extremely rare, a hepatoblastoma, can be the cause of severe high blood pressure
in children, and to the best of our knowledge, this is the fifth report in the literature.[4]
[5]
[6]
[7]
Case Report
An ultrasound performed in a secondary unit for unspecific abdominal chronic abdominal
pain in a 12 years old boy revealed a large hepatic mass involving most of the left
lobe. Computed tomography (CT) scan completed the picture emphasizing satellite distant
liver nodules in segments IV, V, VI, and VIII, left portal vein thrombosis and bilateral
lung metastases. Initial alpha-fetoprotein (AFP) level was 21,290 ng/mL. Seven days
later, the patient manifests severe paroxysmal arterial hypertension, tonic–clonic
seizures and he is referred to our hospital for intensive care.
At admission, the patient presented drowsiness, headaches, blurry vision, and abdominal
distension. The blood pressure was 180/120 mmHg, and the heart rate 120 bpm. His body
mass index related to age was at percentile 98. Blood workup showed a sodium level
of 1292 mmol/L, potassium level of 3,38 mmol/L and neutrophilic leukocytosis. Urine
sample completed the electrolyte imbalance picture with a sodium of 10.12 mmol/L and
potassium of 27.9 mmol/L. AFP level was 39986 ng/mL level. CT scan of the head has
been performed, and no abnormalities were found. Echocardiography has been done revealing
left ventricular hypertrophy.
The seizures were inconstantly controlled using phenytoin and levetiracetam, and the
paroxysms of severe hypertension variably responded to amlodipine, metoprolol, and
enalapril therapy so an open liver biopsy could be performed safely. The histopathologic
and immunohistochemical studies concluded to mixed embryonal/fetal hepatoblastoma,
macrotrabecular pattern, high mitotic rate, and 50% Ki-67 labeling index. The diagnosis
of unresectable, high-risk PRETEXT IV mixed embryonal hepatoblastoma was concluded.
Considering the refractory paroxysms of high blood pressure associating the solid
malignancy, plasma renin activity was measured finding out high levels of 1437 mIU/mL.
The chemotherapy was initiated according to SIOPEL 2001 guidelines for high-risk hepatoblastoma.
The patient responded initially very well to the adjuvant therapy: tumor size regression
and dynamic decreasing of AFP levels. No more seizures and normal blood pressures
were noted. The plasma renin activity after 2 cycles of chemotherapy decreased to
181 mIU/mL. The tumor reduction was insufficient remaining unresectable, the liver
transplant could not have been done in time and chemotherapy had to be interrupted
due to severe thrombocytopenia. The patient died 6 months later due to hepatic failure.
Discussion
Paraneoplastic presentation of hepatoblastoma is common, most often represented by
thrombocytosis, erythrocytosis or hypocalcemia. Isosexual precocious puberty or hypoglycemia
have also been reported.[3]
Symptomatic, severe high blood pressure is rare in children, and most of the times,
it hides an underlying cause, usually a renal disease. Hypertension as paraneoplastic
phenomenon in childhood malignancies has also been in reported in pheochromocytomas
or neuroblastomas– where catecholamine secretion is the main pathogenetic pathway.[8] Hypersecretion of the renin by the tumor cells as the cause leading to high blood
pressure is usually present in benign juxtaglomerular tumors; however, neoplasms Grawitz’s
or Wilms’or Grawitz’s or Wilms’ tumor have also been reported.[4]
[8]
Because of the lack of access to facilities such as immunohistochemical study or polyclonal
antibody for human renin activity, we could not prove the secretion of renin just
by tumor tissue cells, but this is very likely since our patient’s blood pressure
has subsequently decreased as a side response to chemotherapy with no further antihypertensive
or antiseizure treatment required.
A comprehensive research of the literature outlines 4 previous cases of severe hypertension
increased plasma renin activity in hepatoblastoma. Our case presents the eldest patient
of the series so far. Unfortunately, the high-risk features and the contraindication
of surgery endorsed the worst prognosis of the neoplasm.
Hepatoblastoma coexisting with hypertension should be considered and checked as a
possible etiology for the high blood pressure.
Declaration of patient consent
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In the form the patient(s) has/have given his/her/their consent for his/her/their
images and other clinical information to be reported in the journal. The patients
understand that their names and initials will not be published and due efforts will
be made to conceal their identity, but anonymity cannot be guaranteed.
