A 9-year-old male came to clinic with fever and stomachache. His rapid strep was positive and he was treated with penicillin.
Three weeks later he complained of abdominal pain again, accompanied by a low-grade fever, some nausea, early satiety and mild fatigue.
He had no sore throat, diarrhea, changes in bowel or urinary patterns, upper respiratory symptoms, or rashes.
Some of his friends had colds, and he had no pet exposure or travel history.
The past medical history showed a healthy male.
The review of systems revealed no night sweats, weight loss, pruritus, jaundice, bleeding, bruising, or other pain.
The pertinent physical exam showed a slightly tired male in no distress. His temperature was 38.2° Celsius, he had a 1 pound weight loss from the previous visit, but the rest of the vital signs were normal.
HEENT showed no icterus and some anterior cervical nodes that were <0.5 cm. He had no other lymphadenopathy.
His abdominal examination showed fullness of the right side of the abdomen and a liver edge that was 2-3 cm above the umbilicus and crossing the midline. He had no splenomegaly, other palpable masses or ascites.
He had no skin changes including prominent vessels.
The radiologic evaluation of right upper quadrant ultrasound found a large heterogeneous mass in the right lobe of the liver.
The patient was transferred to a regional children’s hospital for further evaluation where a CT scan confirmed the diagnosis.
He was taken to the operating room where a resection of the right lobe of the liver and mass was completed.
Anatomic pathology reported a diagnosis of embryonal cell sarcoma with tumor free surgical margins and negative abdominal lymph node biopsies.
The laboratory evaluation included liver function tests, hepatitis panel, alpha-fetoprotein levels, human chorionic gonadotropin levels, carcinoembryonic antigen, and routine cancer markers. All were negative.
The patient’s clinical course post-operatively was uneventful and he was begun on chemotherapy.
Figure 67 – Axial image from an ultrasound exam of the
liver shows a large inhomogenous mass in the right lobe of the liver.
The mass is primarily hyperechoic in appearance, with multiple
hypoechoic regions within it.
Figure 68 – A coronal two dimensional reconstruction
delayed phase image of the liver from a computed tomography exam of
the abdomen performed with oral and intravenous contrast. A large
lesion, measuring 12 cm in diameter, is seen in the right lobe of the
liver. There is peripheral vascular enhancement of the mass, which
has a primarily necrotic center. Differential diagnosis included
hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma.
Liver masses in children include primary liver cancer, metastatic cancer from other sites, cysts and inflammatory masses.
Primary liver cancer only constitutes 1-2% of all pediatric malignancies.
Primary liver malignancies in infants and young children usually are caused by hepatoblastoma. In older children hepatocellular carcinoma is the most common.
For intermediate ages both are common along with transitional cell tumors and primary sarcomas. Other malignant liver tumors include angiosarcoma, biliary rhabdomyosarcoma, undifferentiated sarcomas and rhabdoid tumor.
Benign liver tumors include adenoma, focal nodular hyperplasia, infantile hemangioma, mesenchymal hamartoma and teratoma.
Primary hepatic sarcoma is rare with the outcome depending on tumor resection, sensitivity to chemotherapy and/or radiotherapy and tumor histology.
In a study of 30 patients with primary liver sarcomas, 11 had resection plus chemotherapy. The overall actuarial 5 year survival was 70%. Those with embryonal sarcoma had an actuarial 5 year survival of 80%.
On physical examination, normal liver size is < 3.5 cm below the right costal margin for newborns and < 2.0 cm for older children.
A normal liver span for a newborn is ~4.5-5 cm and at 12 years of age is 6-6.5 cm for females and 7-8 cm for males. In between these times there should be appropriate linear growth with the child.
Hepatomegaly is caused by inflammation, infiltration, obstruction, storage abnormalities and vascular congestion.
The differential diagnosis of hepatomegaly includes:
- Autoimmune disease
- Bile acid enzyme defects
- Canalicular bile acid transport defects
- Infections – virus, abscess
- Idiopathic neonatal hepatitis
- Kupffer cell hyperplasia – granulomatous hepatitis, sepsis, vitamin A toxicity
- Cysts – choledochal, parasitic, polycystic liver disease
- Extramedullary hematopoiesis – erythroblastosis fetalis
- Hemophagocytic syndromes
- Primary liver tumors – malignant and benign, as above
- Metastatic liver tumors – histiocytosis, leukemia, lymphoma, neuroblastoma, Wilm’s
- Biliary atresia
- Choledochal cyst
- Liver tumors
- Storage abnormalities
- Fat – cystic fibrosis, diabetes, fatty acid oxidation defect, malnutrition, mucopolysaccharidoses, parenteral nutrition, Reye syndrome
- Glycogen – diabetes, glycogen storage disease, parenteral nutrition
- Lipid – Gaucher disease, Neimann-Pick disease, Wolman disease
- Metal – hemochromtosis (iron), porphyria (heme), Wilson disease (copper)
- Protein – alpha-1-antitrypsin deficiency, amyloidosis, carbohydrate-glycoprotein deficiency
- Vascular congestion
- Budd-Chiari syndrome (hepatic vein thrombosis)
- Congestive heart failure
- Pericardial disease (restrictive)
- Suprahepatic web of the IVC.
- Veno-occlusive disease
Questions for Further Discussion
1. What other organs can cause factitious enlargement of the liver?
2. What laboratory testing should be considered for initial evaluation of hepatomegaly?
3. What are the indications for liver transplantation?
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Information prescriptions for patients can be found at MedlinePlus for this topic: Liver Cancer
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
Wolf AD, Lavine JE.
Hepatomegaly in neonates and children.
Pediatr Rev. 2000 Sep;21(9):303-10.
Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:1478.
Tumors of the liver in children.
Surg Oncol. 2007 Nov;16(3):195-203.
Weitz J, Klimstra DS, Cymes K, Jarnagin WR, D’Angelica M, La Quaglia MP, Fong Y, Brennan MF, Blumgart LH, Dematteo RP.
Management of primary liver sarcomas.
Cancer. 2007 Apr 1;109(7):1391-6.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital
October 13, 2008