CC BY
25
May 2016, Moscow, Russia
OF PEDIATRIC HEMATOLOGY and ONCOLOGY
LIVER TUMOURS
ABSTRACT NO.: OP-137
Multimodality treatment outcome of hepatoblastoma: our experience in a resource limited country
ATM Atikur Rahman, Mehnaz Akter, Momena Begum, Chowdhury Shamsul Hoque Kibria, Afiqul Islam
Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
Key words: hepatoblastoma, multimodality treatment, outcome
Introduction. Hepatoblastoma is the most common primary hepatic malignancy in childhood, accounting 0.8-2 % of all paediatric cancers. The incidence has been an increasing over the last two decades but the aetiology for it still remain unclear. Predominantly seen in premature and/or very low birth weight children. Significant improvements in multimodality treatments have been achieved over the last two decades. Five-year overall survival (OS) rates now approaching 70-75 %. The purpose of this paper is to report on about the experience in management of hepatoblastoma in a tertiary centre in an developing country like Bangladesh.
Aim. To review the records of management of hepatoblastoma in the Paediatric Haematology and Oncology department of Bangabandhu Sheikh Mujib Medical University (BSMMU) over 8 yrs from January 2004 to December 2012.
Materials and methods. A retrospective analysis has been done in BSMMU with children having hepatoblastoma through January 2004 to December 2012. Patients demographics, mode of presentation, method of diagnosis, extent of tumor at diagnosis,surgical procedures performed, complications of treatment and outcome were compiled by reviewing medical histories, radiology, pathology, and surgery reports. Based on imaging using CT scan or MRI, all patients were assigned a PRETEXT stage and four groups of patients were identified as PRETEXT I-IV.
Patients were treated with neoadjuvant therapy (chemotherapy followed by surgery and then again chemotherapy). Cisplatin and Adriamycin as in the PLADO (PLA, Platinum group; DO, Doxorubicin) regimen was administered to all patients every 21 days interval. Every cycle contains PLA on day-1 at a dose of 80 mg/m2 for continuous 24-hr intravenous infusion 01
and DO at a dose of 30 mg/m2/day over 48-hr continuous intravenous infusion on days 2 and 3.
Patients were routinely reassessed after three cycles for surgical resection. If the tumor was found to be inoperable, one more cycle of chemotherapy has been given to them. Surgical decision had been taken by the pediatric surgical oncology team. Postoperatively, 2 to 3 cycles of chemotherapy were given to a total of 6 cycles. The patients were followed after treatment according to the institution's protocol with serial AFP levels and radio-imaging. Results. In this study population median age of was 12 months (3-60 months) and malefemale was 3.3:1. Nine patients were treated with neoadjuvant chemotherapy incorporating >
cisplatin and adriamycin. Primary surgery was done in four patients. Extent of hepatic resection in the operated patients varied. Mixed type was the predominant histopathological ~~'
diagnosis. Adjuvant chemotherapy was well tolerated with no morbidity or mortality. Five-year event-free survival (EFS) and overall survival (OS) of all the 13 patients is 76.9 %. All the nine patients who could complete multimodality treatment are alive with no evidence of disease or complications with median follow-up of 63 months (46-122 months). On the basis of staging (PRETEXT), the 5-year OS is as follows: PRETEXT-I 100 %, PRETEXT-II 100 %, PRETEXT-III 66.7 % and PRETEXT-IV 0 %. Conclusion. Treatment of hepatoblastomas with multidisciplinary approach were well tolerated. OS and EFS of all patients were comparable with published studies.
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SOCltTt INTERNATIONALE D'ONCOLOGIE PEHATRIQUE
SIOP ASIA CONGRESS
ABSTRACT NO.: P-144
Surgical options in complex treatment of focal liver lesions in pediatric patients
E.F. Kim1, A.V. Film1, A.V. Semenkov1, D.S. Burmistrov1, O.V. Dimova1, T.N. Galjan1, E.U. Krjijanovskaia1, A.V. Metelin1, S.R. Varfolomeeva2, D.Yu. Kachanov2, T.V. Shamanskaya2, R.A. Moiseenko2, A.V. Petrushin2
1Russian Scientific Centre of Surgery named after Academician B.V. Petrovskiy, Moscow, Russia; 2Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia
Key words: focal liver lesions, hepatoblastoma, anatomical resection, liver transplantation, pediatric Introduction. Hepatic tumors account for 1-4 % of the solid tumors in children. Aim. Results assessment of surgery treatment for pediatric liver tumors.
Materials and methods. From April 2008 to December 2015, 81 pediatric patients (3 months - 17 years old) with a variety of focal liver lesions underwent surgery at Petrovskiy National Research Centre for Surgery. Malignancies (75.3 %) were presented by hepatoblastoma (66.7 %), undifferentiated (embryonal) sarcoma (3.7 %), hepatocellular carcinoma (2.5 %), malignant rhabdoid tumor (1.2 %) and rhabdomyosarcoma (1.2 %). Benign lesions or neoplasms (22.2 %) were presented by focal nodular hyperplasia (7.4 %), mesenhymal hamartoma (4.9 %), hepatocellular adenoma (2.5 %), infantile hemangioendothelioma (2.5 %), benign teratoma (2.5 %), cavernous hemangioma (1.2 %), inflammatory pseudotumor (1.2 %). Two patients (2.5 %) had a parasitic liver disease: cystic and alveolar echinococcosis.
Results. Surgical treatment consisted of anatomical resection (lobe- and extended lobectomies - 82.5 %, segmentectomies - 4.9 %, and living-donor liver transplantations - 12.4 %. In one case of cystic echinococcossis was made total pericystectomy. In groups of benign and parasitic diseases there were no deaths. The treatment's results for patients with the hepatoblastoma are presented below. The age of patients in this group ranged from 3 months to 17 years (median - 19.13; 25th and 75th quartiles - 10.53- 34.1 months). In 87 % of cases, the age did not exceed 4 years old. Patients classified by system PRETEXT: I - 2 % (n = 1), II - 39 % (n = 21), III - 31 % (n = 17), IV - 28 % (n = 15). In 13 children (24 %) detected lung metastases. In 2 cases hepatoblastoma developed on the background of Beckwith-Wiedemann syndrome. A biopsy of the tumor before treatment was performed in 31 (57.4 %) patients. Neoadjuvant chemotherapy performed in 54 cases (96 %). Tumor regression with reduction of the tumor stage were achieved in 7 patients (13.5 %). Progression of tumor and stage of the disease were found in 4 cases (7.7 %). Lung surgery due to the remote metastases required in 6 cases (11.4 %). In 89 % (n = 48) of patients performed anatomical resection of the liver and in 11 % (n = 6) - living related liver transplantation. Extended hemihepatectomies and bilateral liver resections were performed in 65 % (n = 35). All resection performed without Pringle maneuver. The volume of blood loss in patients with resection was 17.02 ± 14.67 ml/kg. Hospital mortality rate was 1.85 % (n = 1). Three patients (5.6 %) died for the first year after surgery. The causes of death were complications of adjuvant chemotherapy (n = 2) and pulmonary embolism during surgery for tumor recurrence (n = 1). One patient died after 15 months due to the progression of distant metastases in the lungs. The actuarial survival rate (overall/disease-free) for patients with hepatoblastoma was, respectively: 1 year 0.94 ± 0.03/0.84 ± 0.05; 3 years 0.89 ± 0.04/0.84 ± 0.05; 5 years 0.89 ± 0.04/0.84 ± 0.05; 7 years 0.89 ± 0.04/0.84 ± 0.05. In patients after resection the same results were higher: 1 year 0.96 ± 0.03/0.88 ± 0.05; 3 years 0.90 ± 0.05/0.86 ± 0.05; 5 years 0.90 ± 0.05/0.86 ± 0.05; 7 years 0.90 ± 0.05/0.86 ± 0.05. Conclusion. Surgery is the preferred treatment of benign and malignant liver lesions in pediatric patients. Long-term results with high survival rates for combined treatment of hepatoblastoma demonstrate broad opportunities both resection and liver transplantation in cases of selection of optimal surgery plan and chemotherapy protocols.
ABSTRACT NO.: OP-212
Clinical experience and outcome of hepatoblastoma in children: a 9-year retrospective study
Sanjeev Khera, Amita Trehan, KLN Rao, Deepak Bansal, Nandita Kakkar, Radhika Srinivasan
Postgraduate Institute of Medical Education & Research, Chandigarh, India
Key words: hepatoblastoma, outcome, high risk
Introduction. Hepatoblastoma (HBL) is the most common primary liver tumor in children. Metastatic disease at presentation, alpha-fetoprotein (AFP) less than 100 ng/ml, PRETEXT IV, undifferentiated histology are usual poor prognostic markers. Neoadjuvant chemotherapy with surgical resection has led to increased survival of these patients. Aim. This single centre study assesses the outcome and the factors affecting prognosis of children treated as per guidelines of SIOPEL 3 in a low middle income country (LMIC). Materials and methods. Forty children with HBL treated as per protocol from Jan 2007 to Dec 2015 were analyzed. The diagnosis was established by imaging, AFP and histology / cytology.
Results. 27 boys & 13 girls; median age 12 months (2-120) with a mean symptom diagnosis interval (SDI) of 5.3 weeks (1-24) were diagnosed. Multifocal involvement was seen in 7/40. Median AFP at diagnosis: 16 500 ng/ml (3-406198). 2 had AFP less than 100 ng/ml. Mean platelet count at diagnosis 701375/cmm (95 % CI 595510-807239). 7 (17.5 %) had metastatic disease (6 lungs; 1 adrenal). PRETEXT: I-IV: 6, 13, 11 & 9. Nineteen: high risk (HR) disease (7 with SDI more than 8 weeks).
13/40 defaulted/refused therapy. All cases received neoadjuvant chemotherapy. 3 deaths occurred prior to surgery. 25 underwent surgery; complete resection (CR): 18/25 (14: well; 3 relapsed, 1 died). 7 cases subtotal resection: 2: well, 4 had persistant disease and 1 death. 2/3 children who received salvage chemotherapy with Docetaxel had no response. HR patients had a poorer outcome (P = 0.012), more multifocal disease (P = 0.0395) and significantly lower CR (P = 0.0016) as compared to standard risk patients. On comparing well Vs relapsed/residual disease cases; there was no difference in AFP levels, volume reduction after neoadjuvant therapy, metastasis, & histopathology (P = ns). Relapsed/residual disease was significantly higher in those with higher mean age (24 mth Vs 12 mth); PRETEXT IV & multifocal disease (P = 0.02 & 0.04). 16 of 27 patients are presently well; median follow up being 48.3 months (7-101).
Conclusion. 59 % (16/27) of cases who were treated are well. Higher age at presentation, multifocality and high PRETEXT was associated with poor outcome. HR patients had a longer SDI. Docetaxel as a salvage therapy was not effective. Liver transplant, not available at our centre, would have helped the children with incomplete resection.Delayed referral and diagnosis remains a problem in LMIC's as evident by greater SDI in those with HR disease.
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May 2016, Moscow, Russia
OF PEDIATRIC HEMATOLOGY and ONCOLOGY
ABSTRACT NO.: P-3 21
Assessment of early tumor response to cisplatin monotherapy in patients with standard risk hepatoblastoma
D.Yu. Kachanov1, E.V. Pheoktistova1, T.V. Shamanskaya1, R.A. Moiseenko1, A.A. Merishavyan1, M.A. Tarasov1, G.V. Tereshchenko1, S.R. Talypov1, E.F. Kim2, D.S. Burmistrov2, A.V. Filin2, S.R. Varfolomeeva1
1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Russian Scientific Centre of Surgery named after Academician B.V. Petrovskiy, Moscow, Russia
Key words: hepatoblastoma, children
Introduction. Hepatoblastoma is the most common primary malignant liver tumor in children. Implementation of protocols for risk-adapted therapy may de-escalate therapy in the group of standard risk patients with preservation of the effectiveness of treatment.
Aim. To assess the dynamics of tumor response to cisplatin monotherapy in the group of standard risk patients with hepatoblastoma treated in the Federal Scientific and Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev.
Materials and methods. The study included 21 patients of standard risk hepatoblastoma treated during the period 01.2012-09.2015 (45 months). The diagnosis was based on histological examination. Staging was according to the PRETEXT system. Patients were treated according to SIOPEL-3 SR protocol, including single-agent cisplatin (4 courses of neoadjuvant therapy and 2 courses of adjuvant therapy). Assessment of the level of alpha-fetoprotein (AFP) and volume of tumor (cm3) was done at diagnosis and after the 2nd course of chemotherapy.
Results. 16 patients were included in the assessment of the dynamics of the response. Male to female ratio was 0.6:1. The median age at diagnosis was 6.3 months (range 0.1-36.9 months). The distribution for PRETEXT stages: stage I - 2 (12.5 %) patient; II - 12 (75.0 %), III - 2 (12.5 %).
The median AFP level at diagnosis (n = 16) was 329.741 (range 847-1.971.991), after 2 courses of cisplatin (n = 16) - 20.229 (range 211-181.400) (P = 0.0006). The decrease in AFP levels of > 1 log was observed in 11 (68.7 %) patients, less than 1 log - in 4 (25.0 %) patients, increase in the level of AFP is detected in 1 (6.3 %) case. Estimation of the volume of the tumor at diagnosis (n = 16): median 332 cm3 (range 180-970), after 2 cycles of chemotherapy (n = 16): median 230 cm3 (range 15-800) (P = 0.001). All patients continued scheduled therapy. The patient with increasing values of the AFP was transferred to the intensified therapy according to the SIOPEL-4 protocol. The median duration of follow-up was 18.7 months (range 5.8-36.2 months). All 16 patients are alive without any events.
Conclusion. The findings suggest that cisplatin as monotherapy is effective in the treatment of standard risk hepatoblastoma. The decrease in the level of AFP is less than 1 log after 2 courses of treatment with cisplatin should not be considered as a criterion of intensification therapy.
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