RESUMEN
BACKGROUND AND AIM: We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. METHODS: Patients enrolled on the protocol from 1996 to 2014 were included. PRETEXT staging, SIOPEL, and CHIC risk groups were retrospectively assigned. RESULTS: Sixty patients were enrolled with median age at diagnosis of 1.1 years and median follow-up time of 6.8 years. Alpha-fetoprotein (AFP) was raised (>100 ng/mL) in 58 (97%) patients. Five (8%) had metastases at presentation and 7 (12%) experienced tumor rupture prior to or during treatment. Twenty-nine patients (48%) received a first-line cisplatin, 5-fluorouracil, and vincristine regimen only while 23 (38%) also had alternative chemotherapeutic agents. Hepatic resection could be performed in 48 (80%) patients. Three (5%) patients underwent upfront liver transplantation. Five-year event-free survival and overall survival rates were 69.2% ± 6.1% and 77.6% ± 5.5% respectively. Among the 16 patients with relapse/progression, 9 had intrahepatic failure only, 5 had distant failure only, and 2 had combined local and distant failure. Predictors of inferior outcome included advanced Evans staging, disease involving both lobes, rupture, low AFP, and suboptimal response to first-line chemotherapy. Assigned in 44 patients, PRETEXT staging, SIOPEL, and CHIC risk groups significantly predicted EFS and OS. CONCLUSIONS: Although the consensus HB/HCC 1996 protocol led to cure in three-quarters of pediatric HB patients, an upfront risk stratification system is required to identify and improve the outcome of high-risk patients.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Hepatoblastoma/mortalidad , Neoplasias Hepáticas/mortalidad , Trasplante de Hígado/mortalidad , Recurrencia Local de Neoplasia/mortalidad , Niño , Preescolar , Terapia Combinada , Consenso , Femenino , Estudios de Seguimiento , Hepatoblastoma/patología , Hepatoblastoma/terapia , Hong Kong , Humanos , Lactante , Neoplasias Hepáticas/patología , Neoplasias Hepáticas/terapia , Estudios Longitudinales , Masculino , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/terapia , Estudios Prospectivos , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
Infants with hypertrophic pyloric stenosis typically present at 2 to 4 weeks of age with nonbilious projectile vomiting. Hypertrophic pyloric stenosis is exceedingly rare in newborn infants and is scarcely reported in literature. Also, the diagnostic criteria for ultrasonographic measurements in newborn infants have yet to be determined. This report is of a newborn infant with hypertrophic pyloric stenosis. The patient presented with high-volume non-bile-stained output from a nasogastric tube and a dilated gastric bubble on abdominal radiograph. Contrast study ruled out intestinal malrotation. Two ultrasound tests showed that the pyloric muscle thickness and pyloric canal length were within normal limits. Subsequent laparotomy showed a thickened pylorus and pyloromyotomy was performed. The patient showed marked improvement in feeding postoperatively. A high index of suspicion is required for newborn infants presenting with gastric outlet obstruction. Ultrasound and contrast studies provide additional information, but definitive diagnosis may only be available intra-operatively.
Asunto(s)
Laparotomía/métodos , Estenosis Hipertrófica del Piloro/diagnóstico , Vómitos/etiología , Humanos , Recién Nacido , Intubación Gastrointestinal , Masculino , Estenosis Hipertrófica del Piloro/fisiopatología , Estenosis Hipertrófica del Piloro/cirugía , Píloro/patología , Píloro/cirugíaRESUMEN
BACKGROUND: Hepatocellular carcinoma (HCC) is a rare hepatic malignancy in children. Hepatitis B virus (HBV) infection is a key predisposing factor in endemic regions but its impact on outcome has not been studied. We aim to evaluate the prognostic implication of HBV seropositivity and role of cancer surveillance in children with HCC from East Asian populations with national HBV vaccination. METHODS: Review of population-based databases for patients (< 18 years old) diagnosed with HCC from 1993 to 2017 in two Southeast Asian regions with universal HBV vaccination (instituted since 1988 and 1987 in Hong Kong and Singapore, respectively). RESULTS: Thirty-nine patients were identified (Hong Kong, 28; Singapore, 11). Thirty were male; median age at diagnosis was 10.8 years (range, 0.98-16.6). Abdominal pain was the commonest presentation while five patients were diagnosed through surveillance for underlying condition. Alpha-fetoprotein was raised in 36 patients (mean, 500,598 ng/ml). Nineteen had bilobar involvement, among the patients in whom pretreatment extent of disease (PRETEXT) staging could retrospectively be assigned, 3 had stage I, 13 had stage II, 4 had stage III, and 11 had stage IV disease. Seventeen had distant metastasis. HBsAg was positive in 19 of 38 patients. Two patients had fibrolamellar HCC. Upfront management involved tumor resection in 16 (liver transplantation, 2), systemic chemotherapy in 21, interventional procedures in 6 [transarterial chemoembolization (TACE), 5, radiofrequency ablation (RFA), 1], and radiotherapy in 4 (selective internal radiation, 3, external beam radiation, 1). Five-year event-free survival (EFS) and overall survival (OS) were 15.4 ± 6.0 and 26.1 ± 7.2%, respectively. Patient's HBsAg positivity, metastatic disease and inability to undergo definitive resection represent poor prognostic factors in univariate and multivariable analyses. Patients diagnosed by surveillance had significantly better outcome. CONCLUSION: Pediatric HCC has poor outcome. HBV status remains relevant in the era of universal HBV vaccination. HBV carrier has inferior outcome and use of surveillance may mitigate disease course.