Dataset Information

Hepatocellular Carcinoma in Children and Adolescents: Clinical Characteristics and Treatment.

ABSTRACT:

Background

Hepatocellular carcinoma (HCC) occurs rarely in children and adolescents (C&A), and its clinical characteristics, prognostic factors, and treatment were rarely explored.

Methods

This retrospective study focused on 65 HCC patients aged ≤20 years from August 1994 to August 2012. Cox regression models and Kaplan-Meier curves were used to investigate prognostic factors and compare overall survival (OS), respectively.

Results

We found 61.5% of patients to have multiple tumors, 30.8% to have portal vein tumor thrombus, and 16.9% to have distant metastasis. Diameter of tumors was 10.2 ± 4.1 cm. OS at 5 years was 15.8%. Multivariate analyses showed initial treatment (P < 0.001) to be a predictor for OS. For moderate-stage HCC, the median OS of patients who underwent resection was longer than that of patients who underwent transarterial chemoembolization (TACE) or supportive treatment (ST) (P < 0.001). For advanced-stage HCC, the median OS of patients who underwent TACE was longer than that of patients who underwent ST (P = 0.045).

Conclusions

HCC in C&A tends to be more advanced than that in adults, and resection remains the mainstay of treatment for those patients. Moreover, compared with ST, TACE may benefit C&A with moderate- and advanced-stage HCC, which needs further study.

SUBMITTER: Wang J

PROVIDER: S-EPMC5486687 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature

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Publications

Hepatocellular Carcinoma in Children and Adolescents: Clinical Characteristics and Treatment.

Wang Juncheng J Mao Yize Y Liu Yongcheng Y Chen Zhenxin Z Chen Minshan M Lao Xiangming X Li Shengping S

Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract 20170410 7

<h4>Background</h4>Hepatocellular carcinoma (HCC) occurs rarely in children and adolescents (C&A), and its clinical characteristics, prognostic factors, and treatment were rarely explored.<h4>Methods</h4>This retrospective study focused on 65 HCC patients aged ≤20 years from August 1994 to August 2012. Cox regression models and Kaplan-Meier curves were used to investigate prognostic factors and compare overall survival (OS), respectively.<h4>Results</h4>We found 61.5% of patients to have multipl ...[more]

PMID: 28397025

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Project description:PurposeFibrolamellar hepatocellular carcinoma (FLHCC) is a rare primary liver malignancy often diagnosed at advanced stages. While there are limited data on the efficacy of specific agents, we aim to report outcomes of patients treated with systemic therapies and explore prognostic factors.Patients and methodsMedical records of patients treated between 2010 and 2022 were reviewed. Treatments were defined after multidisciplinary assessment. Descriptive statistics were used for baseline demographics. Time-to-event outcomes were estimated using the Kaplan-Meier method, compared by log-rank and adjusted by a regression model. Radiomic features (including size, shape, and texture) of the primary lesion were extracted and dimensionality reduced. An unsupervised Gaussian Mixture Model (GMM) clustering was performed, and survival was compared between clusters.ResultsWe identified 23 patients: 12 males, with a median age of 23.6 years. At diagnosis, 82.6% had metastases, most frequently to the lungs (39.1%), lymph nodes (39.1%), and peritoneum (21.7%). Patients received a median of three lines (1-8) of treatment, including different regimens. Sorafenib (39.1%), capecitabine (30.4%), and capecitabine/interferon (13%) were the most used first-line regimens. The median time-to-failure was 3.8 months (95% CI: 3.2-8.7). Capecitabine + interferon (42.1%) and platinum combinations (39.1%) were the most used second-line regimens, with a time-to-failure of 3.5 months (95% CI: 1.5-11.6). Median overall survival was 26.7 months (95% CI: 15.1-40.4). A high baseline neutrophil-to-lymphocyte ratio (NLR) was associated with worse survival (p=0.02). Radiomic features identified three clusters, with one cluster (n=6) having better survival (40.4 vs 22.6 months, p=0.039). Tumor sphericity in the arterial phase was the most relevant characteristic associated with a better prognosis (accuracy=0.93).ConclusionFLHCC has unique features compared to conventional HCC, including young onset, gender balance, and absence of hepatopathy. Systemic therapies can provide encouraging survival, but lack of uniformity precludes defining a preferable regimen. Radiomics and NLR were suggested to correlate with prognosis and warrant further validation.

Project description:BackgroundHepatocellular carcinoma (HCC) is a highly heterogeneous tumor with several rare pathological subtypes and which is still poorly understood. This study aimed to describe the epidemiological and clinical spectrum of five rare HCC subtypes and develop a competing risk nomogram for cancer-specific survival prediction.MethodsThe study cohort was recruited from the Surveillance, Epidemiology, and End Results database. The clinicopathological data of 50,218 patients histologically diagnosed with classic HCC and five rare subtypes (ICD-O-3 Histology Code = 8170/3-8175/3) between 2004 and 2018 were reviewed. The annual percent change (APC) was calculated utilizing Joinpoint regression. The nomogram was developed based on multivariable competing risk survival analyses. Akaike information criterion, Bayesian information criterion, C-index, calibration curve, and area under the receiver operating characteristic curve were obtained to evaluate the prognostic performance. A decision curve analysis was introduced to examine the clinical value of the models.ResultsDespite scirrhous carcinoma, which showed a decreasing trend (APC = -6.8%, P = 0.025), the morbidity of other rare subtypes remained stable from 2004 to 2018. The incidence-based mortality was plateau in all subtypes during the period. Clear cell carcinoma is the most common subtype (n = 551, 1.1%), followed by subtypes of fibrolamellar (n = 241, 0.5%), scirrhous (n = 82, 0.2%), spindle cell (n = 61, 0.1%), and pleomorphic (n = 17, ~0%). The patients with fibrolamellar carcinoma were younger and more likely to have a non-cirrhotic liver and better prognoses. Scirrhous carcinoma shared almost the same macro-clinical characteristics and outcomes as the classic HCC. Clear cell carcinoma tended to occur in the Asia-Pacific elderly male population, and more than half of them were large HCC (Size>5cm). Sarcomatoid (including spindle cell and pleomorphic) carcinoma was associated with a larger tumor size, poorer differentiation, and more dismal prognoses. The pathological subtype, T stage, M stage, surgery, alpha-fetoprotein, and cancer history were confirmed as the independent predictors in patients with rare subtypes. The nomogram showed good calibration, discrimination, and net benefits in clinical practice.ConclusionThe rare subtypes had unique clinicopathological features and biological behaviors compared with the classic HCC. Our findings could provide a valuable reference for clinicians. The constructed nomogram could predict the prognoses with good performance, which is meaningful to individualized management.

Dataset Information

Hepatocellular Carcinoma in Children and Adolescents: Clinical Characteristics and Treatment.

Background

Methods

Results

Conclusions

Publications

Hepatocellular Carcinoma in Children and Adolescents: Clinical Characteristics and Treatment.

Similar Datasets

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