and Daniela Cristina Stefan2
(1)
Université Mohammed VI des Sciences de la Santé Cheikh Khalifa Hospital, Casablanca, Morocco
(2)
South African Medical Research Council, Cape Town, South Africa
Keywords
Liver tumorAbdominal massYoung childrenAbdominal ultrasoundSurgeryAlpha fetoproteinChemotherapyCase Presentation
Radouane, a 13-month-old boy, who was previously healthy, was brought to the hospital by his parents with a 2-week history of abdominal pain . The mother also noted an abdominal mass when she was giving him a bath. There was no vomiting or any stool changes.
Findings on examination:
Radouane did not look acutely or chronically ill.
Weight = 13 kg, height = 81 cm.
Observations: normal.
No pallor, lymphadenopathy, jaundice, clubbing, or edema was present.
The cardiovascular, respiratory, and neurological examinations were normal.
On examination of the abdomen, the following were found: distension and a large painless mass occupying the right hypochondrium and epigastrium (10 × 15 cm). The mass could not be distinguished from the liver and was not ballotable. No other masses or organomegaly were noted.
What Is the Differential Diagnosis?
Abdominal masses : nephroblastoma and other renal tumors, neuroblastoma, hepatoblastoma, lymphoma, benign renal mass.
What Investigations Would You Like to Request?
CBC, renal function, bilirubin, liver enzymes.
Alpha fetoprotein (AFP) .
Abdominal ultrasound .
Chest X-ray (PA and lateral).
CT or MRI scan of the abdomen.
Herein listed are available results for your case scenario:
Wcc | Hb | MCV | Pl | Neutro | Lymph | Na | K | Urea | Creat |
|---|---|---|---|---|---|---|---|---|---|
9.8 | 11.5 | 75 | 385 | 2.8 | 5.5 | 138 | 4.2 | 3 | 35 |
Bili | AST | ALT | LDH | AFP |
|---|---|---|---|---|
7 | 22 | 24 | 157 | 800,564 ng/mL |
The ultrasound showed a massively enlarged liver with heterogenous areas (Figs. 9.1, 9.2, and 9.3).
Fig. 9.1
Abdominal ultrasound
Fig. 9.2
Chest X-ray (normal)
Fig. 9.3
MRI abdomen
A large multilobulated heterogenously enhancing mass (135 × 98 × 132 mm) was seen in the right liver lobe. Multiple areas of necrosis present. The majority of the liver is involved; only segment 6 is spared. Hemorrhage within the mass is noted. No vascular involvement seen. Radiological features are in keeping with hepatoblastoma (Fig. 9.4).
Fig. 9.4
Fine needle aspiration of liver mass
Liver tumors are rare in children. Two major types occur, namely hepatoblastoma and hepatocellular carcinoma, of which hepatoblastoma is far more common.
Epidemiology
Hepatoblastoma (HB) is a rare tumor, representing only about 0.5–2 % of all childhood cancers . It has a male predominance. The incidence of HB has increased significantly in the United States and it is thought that the ever increasing survival of very low birth weight babies has contributed to this, since there is a strong association between a low birth weight and HB. A Japanese study reported the risk of HB to be 15 times higher in babies with a birth weight below 1000 g. Most cases of HB occur in children younger than 3 years of age; about 90 % of all liver tumors in children under 4 years are hepatoblastomas.
In addition to a low birth weight, the following are also associated with an increased incidence of HB (Table 9.1): Aicardi syndrome, hemi-hypertrophy, Beckwith–Wiedemann syndrome, familial adenomatous polyposis (FAP), glycogen storage disease, trisomy 18 (and other trisomies).
Table 9.1
Risk factors/associated conditions associated with hepatoblastoma and hepatocellular carcinoma
Malignancy | Risk factors/associated conditions |
|---|---|
Hepatoblastoma | Aicardi syndrome, hemihypertrophy, Beckwith–Wiedemann, familial adenomatous polyposis, glycogen storage disease, trisomy 18 (and other trisomies), low birth weight |
Hepatocellular carcinoma | Alagille syndrome, glycogen storage disease, hepatitis B & C, progressive familial intrahepatic cholestasis, tyrosinemia |
Histology
Hepatoblastoma is derived from hepatocyte precursors. There are two main pathological subtypes: epithelial (56 %) and mixed epithelial/mesenchymal (44 %). The epithelial group includes the pure fetal type (31 %), as well as the embryonal (19 %), macrotrabecular (3 %), and small cell undifferentiated (3 %) types. The presence of mesenchymal elements such as osteoid and cartilage is associated with a better outcome in advanced disease. The small cell undifferentiated type predicts a poor outcome, while the presence of pure fetal histology in a completely resected tumor is associated with an improved outcome.
Clinical Presentation
The usual clinical presentation is that of an asymptomatic, enlarging abdominal mass situated mainly in the right hypochondrium, but could also extend into the epigastrium or other areas depending on the size of the tumor. The right liver lobe is involved more often than the left. Less frequently, abdominal pain, weight loss, anorexia, and nausea and vomiting may be present. Rarely, jaundice related to bile duct compression may be observed or even signs of an acute abdomen due to tumor rupture. The tumor metastasizes to the lungs and sometimes the bladder or diaphragm may be involved contiguously.
Diagnostic Work-Up
Laboratory Tests
Hematological
A baseline hematological evaluation should be performed: complete blood count and clotting profile. Thrombocytosis is observed in 20 % of cases due to production of thrombopoietin by the tumor.
Biochemistry
Renal function studies (electrolytes, urea, and creatinine) should be performed, as well as liver function studies (bilirubin, albumin, AST, ALT, LDH, GGT). The liver function is most often preserved, even in large tumors. Alpha-fetoprotein (AFP) is the most important laboratory test and is increased in 90 % of cases. This finding is not pathognomonic however. An increased AFP level is also found in two-thirds of hepatocellular carcinoma cases, as well as in patients with infantile hemangio-endothelioma and mesenchymal hamartoma. A low AFP level is associated with a very poor outcome. AFP, physiologically produced by the fetal liver, is increased at birth, after which it decreases gradually to reach normal values at 1 year (Table 9.2). The half-life is 5–7 days and it is extremely useful and reliable in the monitoring and follow-up of patients. Following treatment, the level normalizes within a few days or weeks. Rarely, beta-HCG may also be elevated.
Table 9.2
Normal values of AFP birth to 8 months
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