Hepatocellular carcinoma and cirrhosis of liver

The occurrence of hepatocellular carcinoma in an eight year old child with cirrhosis of liver, is presented. The association of the two conditions in pediatric age group is very rare.     

Amebic cerebral abscess mimicking bacterial meningitis

We report a case of an amebic brain abscess in a 2-year-old girl, with symptoms mimicking bacterial meningitis with no evidence of disease elsewhere. Histological evaluation of the abscess revealed the organisms, and the abscess regressed in response to specific medical treatment. This article reviews the rarity of these abscesses and difficulty in the diagnosis.     

Intramedullary cervical neurenteric cyst mimicking an abscess

We describe a cervical intramedullary neurenteric cyst in a 12-year-old male patient who presented with gradual onset and progressively worsening neck pain, spastic quadriparesis and impaired sensation in the C(2) dermatome. MR imaging revealed a well-defined peripherally enhancing cystic intramedullary lesion with a posteroinferior enhancing nodule at the C(2)-C(3) level mimicking an abscess. There was no evidence of spinal dysraphism. The lesion was completely resected through a posterior approach and the patient showed radical improvement in his symptomatology. At follow-up after 3 years, he was asymptomatic and the MR imaging showed no evidence of any residual or recurrent cyst. The case presented here is unique, since a spinal neurenteric cyst showing intense peripheral contrast enhancement mimicking an abscess is unusual. The radiological features, pathogenesis and surgical considerations in cervical intramedullary neurenteric cysts are discussed and the relevant literature is briefly reviewed.     

Hepatocellular carcinoma in children and effect of living-donor liver transplantation on outcome

Hepatocellular carcinoma (HCC) is primarily observed in the older children and in most cases it develops in association with liver cirrhosis. Liver transplantation offers a good chance for long-term cure. To evaluate the outcome of children with HCC and the impact of living-donor orthotopic liver transplantation (OLT) on survival a retrospective review of radiographic, laboratory, pathologic, and therapeutic data in 13 children (six female and seven male) with chronic liver disease accompanied with HCC were studied. The patients were divided into two groups according to therapeutic modality: transplanted and non-transplanted patients. Kaplan-Meier survival curves in various therapeutic groups were plotted. The mean age of patients was 6.4 +/- 4.8 yr. Pediatric end-stage liver disease score was adapted to model for end-stage liver disease score for HCC and ranged between 1-44 and 18-44, respectively. The underlying liver diseases were tyrosinemia type 1 (n = 6), chronic hepatitis B infection (n = 6), glycogen storage disease type 1 (n = 1). Alfa-feto protein levels were elevated in all patients except one. Median number of tumor nodules was three (1-10), median maximal diameter of tumor nodules was 3.4 cm (0.5-8). Eleven patients were eligible for OLT whereas two patients were not eligible. Seven of the 11 patients considered for transplantation underwent living-donor OLT. Remaining four patients died while waiting on cadaveric transplant list. Overall 1 and 4-yr survival rates for all patients were 53.3 and 26.6%, respectively, and were found significantly higher in transplanted children than non-transplanted children (72%, 72% vs. 33% and 16.6%). No patient had tumor recurrence at median of 36-month follow-up after OLT. OLT is a life-saving procedure for children with chronic liver disease accompanying with HCC. Living-donor OLT avoids the risk of tumor progression and transplant ineligibility in these children.     

Hepatocellular carcinoma in children

Hepatocellular carcinoma (HCC) occurs more frequently in subsaharan Africa and the Orient than in other geographical regions, but remains an uncommon tumour of childhood. We review six children with HCC (mean age 13 years) treated by the paediatric oncology unit at Tygerberg Hospital in Cape Town over an 8-year period (1983–1990). Patients presented with epigastric and right upper quadrant discomfort and hepatomegaly. The hepatitis B serum antigen (HbsAg) was positive in three patients; serum alpha-fetoprotein (AFP) levels were markedly elevated in three (range 100-453,000 g/1). Age and sex did not differ significantly and all patients initially had irresectable advanced-stage tumours. Morphologically, three were highly malignant adult-type pleomorphic HCCs, two were differentiated tumours, and one a fibrolamellar subtype. The mean 2-year survival was 33% and the 5-year survival 16.6%. The biological behaviour and response to treatment of the tumours varied. Whereas three patients had a poor response to therapy, two with poorly-differentiated tumours, negative HbsAg, and normal serum AFP levels responded to doxorubicin/cisplatinum chemotherapy. This facilitated radical surgical excision. One patient of this group has survived for more than 75 months following surgical resection and remains well. HCC remains an uncommon tumour of childhood with a high mortality. Aggressive chemotherapeutic regimes in combination with surgical resection may lead to improved survival in some cases. Prevention of hepatitis B remains a priority.     

Primary spinal epidural extraosseous Ewing's sarcoma mimicking a spinal abscess

Ewing's sarcoma is one of the most common malignant tumors of the skeletal system in children and young adults. It most frequently stems from the long bones of the extremities. However, though uncommon, extraosseous localization can be seen. Epidural extraosseous presentations are extremely rare. In this case, the authors report on the long-term follow-up of a patient with this well-demonstrated, but uncommon localization, and the initial unique presentation of the patient, which clinically and radiologically mimicked a cervical abscess.     

Hepatocellular carcinoma in thalassemia major

The occurrence of hepatocellular carcinoma in a 22-year-old man with thalassemia major is reported. As a result of transfusional hemochromatosis, this patient had already developed diabetes, hypogonadism, heart failure, and the sicca syndrome; he was serum and tissue HBsAg negative. Liver iron concentration measured postmortem was found to be 50 times normal. Multiply transfused patients are at risk of developing hepatocellular carcinoma. Serial measurements of serum alpha-fetoprotein should permit early detection of the tumor and reduce mortality. Preventive measures include early immunisation against hepatitis B virus and prevention of iron accumulation by intensive use of desferrioxamine. Treatment of hemochromatosis-associated hypogonadism with androgens should be considered with caution.     

Bacterial liver abscess in children

From November 1987, 136 children with bacterial liver abscess were encountered. There were 97 males and 39 females and the age ranged from 1-15 years (mean = 8.42 years). The clinical signs and symptoms of liver abscess were confirmed by radiography, ultrasonography, percutaneous transhepatic drainage (PTHD) and radioisotope scanning. 103 children had solitary abscess and the remaining 33 children, multiple abscesses. Of the children with multiple abscesses, 23 had abscesses confined to one lobe of the liver and 10 had involvement of both lobes of the liver. 86 children had culture of liver abscesses done and only 63 (73.2%) yielded positive culture. Staphylococcus aureus and Escherichia coli were the commonest organisms cultured from liver abscesses. 72 cases had drainage of liver abscesses, one of them ended up with hepatic artery ligation. A further 15 cases treated by PTHD survived. Of the remaining 49 cases who had antibiotic therapy, 2 died of septicemia, giving a mortality rate of 1.47%. 36.3% of children with liver abscesses responded to antibiotic therapy. The indication and method for surgical management are discussed. Percutaneous transhepatic drainage (PTHD) of liver abscesses, under the guidance of ultrasonography is found to be safe and effective.     

Hepatocellular carcinoma in Nigerian children

Childhood primary hepatocellular carcinoma is rare and accounts for less than 1% of all abdominal malignancies in children < or = 14 years of age. A review of the records of the Cancer Registry, Ibadan, Nigeria covering the period 1960-1995 was scrutinised and 19 cases of hepatocellular carcinoma (HCC) were registered, accounting for 0.49% of all abdominal malignancies over the period of review. The mean (SD) age at presentation was 10.4 (3.0) years and the duration of illness before presentation was short. All the children presented late with abdominal distension and hepatomegaly as the major clinical features. Weight loss was evident in 80% of cases, splenomegaly occurred in 50% and jaundice was present in a third of them. The prognosis was poor; all the cases died within 2 weeks of presentation in hospital. There was evidence to suggest an association between hepatitis B virus infection and HCC in all the liver tissue stained by Shikata-Orcein. This review shows that HCC, though uncommon, is important enough to be considered a possible cause of unexplained hepatomegaly in Nigerian children and that hepatitis B virus is an important aetiological factor. Though the number of cases under review is small, universal early vaccination against hepatitis B virus is necessary in Nigerian children in order to reduce the burden of chronic hepatitis B disease and hepatocellular carcinoma.     

Amebic liver abscess in an infant

Summary An infant with amebic liver abscess in the left lobe of the liver is reported. The diagnosis was based on finding E.H. cysts in the stools. Treatment was successful with chloroquine. From the Departments of Medicine and Pediatrics, V.S.S. Medical College, Burla, District Sambalpur, Orissa.     

Mesenchymal hamartoma of the liver mimicking hepatoblastoma

Mesenchymal hamartoma of the liver is a cystic benign liver mass occurring in children. Diagnostic confusion with hepatoblastoma may arise when alpha-feto-protein (AFP) level is elevated. We report an extremely rare case of mesenchymal hamartoma in an 11-month-old boy. Serum AFP was elevated and fine-needle aspiration biopsy suggested the lesion as hepatoblastoma, so he received preoperative chemotherapy. At the end of the preoperative chemotherapy, the tumor size and AFP level decreased. A right hepatectomy was performed. The pathologic examination of the specimen revealed mesenchymal hamartoma. Mesenchymal hamartoma of the liver with increased serum AFP levels may mimic hepatoblastoma if a cytological examination samples only the hepatocellular component of mesenchymal hamartoma. According to our knowledge, this is the first case of the mesenchymal hamartoma of the liver, which showed reduction in serum levels of AFP and involution of the tumor size by preoperative chemotherapy.