Hepatoblastoma In Infants Research Articles

Update on the Pathology of Pediatric Liver Tumors: A Pictorial Review

Liver tumors in children are uncommon and show remarkable morphologic heterogeneity. Pediatric tumors may arise from either the epithelial or mesenchymal component of the liver and rarely may also show both lines of differentiation. Both benign and malignant liver tumors have been reported in children. The most common pediatric liver tumors by age are benign hepatic infantile hemangiomas in neonates and infants, malignant hepatoblastoma in infants and toddlers, and malignant hepatocellular carcinoma in teenagers. Here, we provide an up-to-date review of pediatric liver tumors. We discuss the clinical presentation, imaging findings, pathology, and relevant molecular features that can help in the correct identification of these tumors, which is important in managing these children.

Two-stage laparoscopic resection of giant hepatoblastoma in infants combined with liver partial partition and artery ligation

PurposeLaparoscopic resection of giant hepatoblastoma (HB) in children has long been a subject of controversy. Here, a new procedure of two-stage laparoscopic resection of giant HB in infants was firstly reported and the feasibility was discussed.MethodsThe clinical data of three infants with HB were retrospectively reviewed, all of which received 3–5 cycles of neoadjuvant chemotherapy. Stage 1 laparoscopic selective hepatic artery ligation and liver partial partition were performed. Stage 2 laparoscopic hepatectomy was performed 2 weeks later.ResultsThe results demonstrated that (1) the tumors shrank considerably in size and had relatively clear boundaries after neoadjuvant chemotherapy; (2) after stage 1 surgery, the tumor volume further reduced, while the intratumoral necrosis expanded; (3) 2 weeks later, stage 2 laparoscopic hepatectomy was performed successfully; (4) none of the cases had intraoperative complications such as tumor rupture, air embolism, hemorrhage, biliary fistula, or liver failure, and there was no recurrence or metastasis during follow-up.ConclusionsTwo-stage laparoscopic hepatectomy associating selective hepatic artery ligation and liver partial partition for HB in infants has the benefits of small invasiveness, fast recovery, improved safety, and high feasibility. However, more cases and longer follow-up are needed to assess its long-term efficacy.

Microwave ablation combined with transcatheter arterial chemoembolization is effective for treating unresectable hepatoblastoma in infants and children

The present study is to evaluate the feasibility and efficacy of microwave ablation (MWA) combined with transcatheter arterial chemoembolization (TACE) in the treatment for unresectable hepatoblastoma in infants and children. A total of 17 patients with PRETEXT stage III and IV hepatoblastoma that was unresectable by conventional resection were included in the present study. The patients were treated with TACE, MWA, and chemotherapy. All cases were diagnosed by computed tomography (CT) and liver tumor biopsy before TACE procedure. All patients received 2 courses of TACE and 1 to 2 times of MWA. Finally, several cycles of chemotherapy were arranged. Among the 17 patients, 14 were alive and had normal alpha-fetoprotein (AFP) levels. The other 3 patients died from tumor progression. The follow-up periods ranged from 10 to 68 months. Complete ablation was achieved in the 14 patients (14/17, 82.35%). Most patients were well tolerated during the whole course except for 1 patient with pneumonedema after TACE and another 1 with biloma after MWA. No marked chemotherapeutic agent-induced toxicity occurred. After chemotherapy or TACE, transient blood indicators and symptoms were observed as follows: myelosuppression, abnormal liver function, gross hematuria, fever, and abdominal pain. Transient symptoms after MWA were fever, abdominal pain, and massive gross hematuria. The present study demonstrates that MWA combined with TACE is a safe and effective method for treating unresectable hepatoblastoma in infants and children with controllable side effects.

Preoperative Transcatheter Arterial Chemoembolization of Hepatoblastoma in Infants

PurposeTo evaluate the effect of preoperative transcatheter arterial chemoembolization of hepatoblastoma in infants. Materials and MethodsClinical data of 21 infants with hepatoblastoma treated between July 2008 and July 2012 in a single hospital were retrospectively analyzed. After preliminary diagnosis, surgical resection was performed in 9 infants (group I), and transcatheter arterial chemoembolization was performed in 12 infants (group II) before conventional resection. Surgical resection was performed when the tumor bulk appeared sufficiently reduced after transcatheter arterial chemoembolization alone or transcatheter arterial chemoembolization following chemotherapy in cases of pulmonary metastases. ResultsTumor shrinkage ranged from 25%–91% with a mean reduction of 69% (t = 3.816, P = .003) in group II. α-Fetoprotein levels were markedly decreased from 49%–99% with a mean level of 95% (t = 4.871, P = .000) in group II. Specimens in group II showed massive necrosis with a mean percentage of 72% with no significant treatment-related toxicity. In group II, the surgical time was significantly shorter (t = 3.438, P = .003), intraoperative blood loss was considerably less (t = 3.459, P = .003), and the weight of the resected liver was significantly less (t = 3.785, P = .001). Of 21 patients, 16 survived for 50 months without recurrence. ConclusionsTranscatheter arterial chemoembolization effectively reduced tumor volume, decreased α-fetoprotein, and reduced intraoperative hemorrhage. It represents a safe and effective adjuvant bridge to successful surgery for hepatoblastoma in infants.

An effective approach for treating unresectable hepatoblastoma in infants and children: Pre-operative transcatheter arterial chemoembolization

The objective of the present study was to investigate the feasibility and efficacy of pre-operative transcatheter arterial chemoembolization (TACE) for unresectable hepatoblastoma in infants and children. A total of 24 patients (14 males and 10 females) with unresectable hepatoblastoma, aged between 26 days and 41 months, were treated with pre-operative TACE between March 2007 and March 2011. All cases were confirmed by computed tomography (CT) and liver tumor biopsy prior to TACE. Arteriography was performed and the chemoembolization mixture (pirarubicin and cisplatin emulsified in lipiodol) was injected, followed by polyvinyl alcohol (PVA). The procedure was performed one to four times depending on the patient’s response. There was a significant reduction in tumor volume associated with decreased α-fetoprotein (AFP) levels following TACE. Tumor volumes decreased by between 46.1 and 90.2%, with a mean value of 72%. The AFP levels fell by between 63.8 and 99.9%, with a mean value of 95.7%. A total of 22 cases underwent subsequent safe complete surgical resection and the remaining two patients accepted a partial resection. To evaluate the toxicity of TACE, the alanine aminotransferase (ALT), serum creatinine (Cr) and creatine kinase (CK) levels of the patients were measured to assess liver, renal and cardiac function, respectively. The results showed that no marked chemotherapeutic agent-induced toxicity occurred during TACE. It may be concluded that TACE is an effective and feasible pre-operative therapeutic approach for treating unresectable hepatoblastoma and that it may improve the resectability of bulky liver tumors.

Preoperative Transcatheter Selective Arterial Chemoembolization in Treatment of Unresectable Hepatoblastoma in Infants and Children

The purpose of this study was to evaluate the clinical feasibility and efficacy of transcatheter selective arterial chemoembolization (TACE) for unresectable hepatoblastoma in infants and children. The study was performed with the approval of our institutional review board. Sixteen patients (13 boys, 3 girls) with unresectable hepatoblastoma were treated one to three times with preoperative TACE in an effort to improve the surgical and clinical outcome. Their ages ranged from 50 days to 60 months, with a mean age of 20.4 months. All cases were pathologically proved hepatoblastoma by fine-needle biopsy. After an intra-arterial catheter was selectively inserted into the main feeding artery of the tumor, cycles of cisplatin (40 to 50 mg/m(2)) and adriamycin (20 to 30 mg/m(2)) mixed with lipiodol were given, followed by gelatin foam particles or stainless-steel coils. Tumor response was evaluated according to tumor shrinkage, alpha-fetoprotein (AFP) levels, and pathological findings. TACE procedure was performed one to three times, depending on the patient's response. Surgical resection was carried out when the tumor volume appeared sufficiently reduced to allow safe resection by either lobectomy or extended lobectomy. A marked reduction in tumor size associated with decreased AFP level occurred after treatment. According to paired-samples test, tumor shrinkage ranged from 19.0% to 82.0%, with a mean value of 59.2%. AFP levels decreased 99.0% to 29.0% from initial levels, with a mean decrease of 60.0%. TACE allowed subsequent complete surgical resection in 13 cases and the other 3 cases underwent partial resection. One patient underwent successful orthotopic liver transplantation after receiving TACE therapy. Pathological examination showed that the mean percentage of necrotic area in the surgical specimens was 87%. Overall survival rate at 1, 3, and 5 years was 87.5%, 68.7%, and 50%, respectively. Correspondingly, event-free survival rate was 75%, 62.5%, and 43.7%, respectively. In addition, there was no marked chemotherapeutic agent-induced toxicity noted during the observation period. We conclude that TACE is feasible, well tolerated, and effective in inducing surgical resectability of hepatoblastoma in pediatric patients, which has become an independent palliative or curative therapeutic option, especially for patients without distant metastasis.

Midterm results with hepatectomy after preoperative chemotherapy in hepatoblastoma

We evaluated the results of surgical treatment for hepatoblastoma in infants and children after intensive preoperative chemotherapy, with special reference to histology and extent of liver involvement. The clinical features of 10 children with hepatoblastoma were reviewed regarding response to neoadjuvant chemotherapy, histological subtypes, extent of hepatectomy, operative complications, and prognosis. Response to chemotherapy was measured by volumetric assessment of tumour size by computed tomography scan. Cisplatin and Adriamycin (PLADO regime) up to three cycles markedly reduced the tumour volume on computed tomography (mean regression rate 65.9%); alpha-foetoprotein (AFP) levels also decreased from an initial mean of 16,116.4 ng/ml to 2,050.9 ng/ml. Five patients underwent right hepatectomy, two had right trisegmentectomy, two had left hepatectomy, and one had left trisegmentectomy. Histopathology of resected specimens revealed foetal histology in four patients, poorly differentiated (anaplastic) subtype in three, and mixed histology with mesenchymal components and osteoid formation in three. There was 100% resectability including six unresectable tumours (prechemotherapy). Moreover, hepatic resection tended to be less invasive in patients whose tumours had been much reduced after preoperative chemotherapy. Preoperative administration of cisplatin and Adriamycin reduces the tumour size significantly so that a safe radical hepatectomy can be performed. It also allows early administration of postoperative chemotherapy. Although overall good results were obtained with the current protocol, we also document our experience of unfavourable outcomes in patients with bilobar tumours (despite trisegmentectomy), patients with tumours showing poor response to neoadjuvant chemotherapy, and patients with anaplastic histology. Overall, at a 60-month follow-up we report an 80% survival rate by a combined approach.

Application o preoperative transcatheter arterial chemoembolization in treatment of hepatoblastoma

目的 探讨肝母细胞瘤术前介入性肝动脉化疗栓塞(transarterial chemoembolization,TACE)的临床可行性、疗效及适应证及在综合治疗中的地位.方法对常规估计不能切除的13例肝母细胞瘤先行经动脉化疗栓塞,再行Ⅱ期外科手术切除,观察分析患儿临床症状的变化反应、术中情况和远期疗效.结果 TACE术后数天至1个月复查,肿瘤体积明显缩小7.5%～66.3%(平均38.0%),AFP水平显著降低11%～61.2%(平均52.3%),无明显的化疗毒性反应,TACE后其中11例患儿都安全地施行外科手术,手术切除标本的病理改变肿瘤明显坏死,平均坏死区域面积达70.9%.介入后2年死于肺转移1例,介入后3年死于肺、脑转移的1例,肝移植后死亡1例,3个月后肝衰竭者1例,3例复发,无瘤存活6例,其平均生存期22.3个月,平均随访时间为30个月.结论 TACE能有效杀死肿瘤细胞,控制肿瘤生长,使部分不能Ⅰ期手术的患儿重新获得手术机会,改善预后,无严重并发症,是一种安全、有效、实用的肝母细胞瘤的辅助治疗方法,而且对防止术中肿瘤细胞的扩散也有一定的作用。

Successful resection for advanced hepatoblastoma, combined with perioperative chemotherapy.

The aim of this study was to evaluate our results of surgical treatment with intensive perioperative chemotherapy for hepatoblastoma in infants and children. Seven patients (mean age, 30 months; range 1 month to 6 years) with hepatoblastoma who were followed-up for more than 3 years were reviewed. All patients underwent hepatectomy, performed using a microwave tissue coagulator, after they had received neoadjuvant chemotherapy comprising up to four cycles of cisplatinum and doxorubicin. The main outcome criteria were the clinical response rates to neoadjuvant chemotherapy and the overall survival. Neoadjuvant chemotherapy markedly reduced the tumor volume on computed tomography (mean regression rate, 73%). Alpha-fetoprotein (AFP) levels also decreased, from a mean value of 138 x 104 to 990 ng/ml (excluding values for one patient with tumor thrombus in the portal vein). The surgical procedures included extended right lobectomy in one patient, extended left lobectomy in two patients, hepatic left trisegmentectomy in one patient, and hepatic subsegmentectomy in three patients. The postoperative clinical courses in all seven patients were good, and no serious complications were observed. No relationship was observed between the DNA ploidy pattern and the histopathological findings of the resected specimens regarding survival. Six patients (excluding the patient with a tumor thrombus in the portal vein) who underwent complete resections survived without any signs of recurrence during a follow-up period ranging from 47 to 150 months. In conclusion, the perioperative chemotherapy greatly improved both the resection rate and overall survival in patients with hepatoblastoma. DNA ploidy pattern analysis may be useful when predicting the prognosis of patients with hepatoblastoma. The use of the microwave coagulator was safe for performing hepatectomy, even in infants.

Transcatheter arterial chemoembolization in the treatment of hepatoblastoma

Background: The prognosis of hepatoblastoma is poor unless the tumor is completely resected. Various types of chemotherapy have been developed to increase its resectability. Recently, transcatheter arterial chemoembolization (TACE) has been developed for the treatment of unresectable adult hepatoma with favorable results. The authors applied this procedure to hepatoblastoma in infants and children. Methods: TACE was performed in eight hepatoblastoma cases. After an intraarterial catheter was inserted into the main feeding artery of the tumor, injection of adriamycin or THP-adriamycin (20 to 30 mg/m 2) dispersed in lipiodol and cisplatin (40 to 60 mg/m 2) followed by embolization using Gelfoam pieces was performed. Effects of TACE were evaluated according to shrinkage of tumor mass on imaging examinations, alpha-fetoprotein (AFP) levels, and pathological findings of the surgical specimens 4 weeks after TACE. Results: A marked reduction in tumor size associated with a decrease in AFP level occurred 1 month after the treatment. Tumor shirinkage ranged from 0.9% to 45.0% with a mean value of 25.8%. AFP levels decreased by 0.2% to 11.9% with a mean level of 4.6% from initial levels. In addition, there was no marked chemotherapeutic agent-induced toxicity noted during the observation period. Resection of the tumors was performed safely after TACE in all cases. Pathological examination showed massive necrosis in the surgical specimens, and the mean percentage of necrotic area within the tumor was 71.1%. Two patients died of extensive lung metastasis 2 months and 3 years after the operation, respectively. The remaining six were doing well and free of disease at a mean follow-up period of 50 months. Conclusion: TACE is an effective, safe, and useful method for the initial treatment of hepatoblastoma.

Acarboxy Prothrombin (PIVKA‐II) as a Marker of Hepatoblastoma in Infants

SummaryWe evaluated plasma PIVKA‐II (protein induced by vitamin K absence or antagonist‐II, acarboxy prothrombin) levels in three infants with hepatoblastoma as a tumor marker. PIVKA‐II levels were highly elevated in all three patients. Vitamin K administration, performed in two patients, resulted in only moderate reduction of PIVKA‐II levels. Chemotherapy against tumor cells reduced the PIVKA‐II levels without exception. Immunohistochemical study of the liver tissue indicated the presence of PIVKA‐II in the hepatoblastoma cell. These findings suggest that elevated PIVKA‐II in these patients was not due to nutritional vitamin K deficiency, but to excess production of tumor cells. A measurement of plasma PIVKA‐II may be useful as a new marker of hepatoblastoma.

Acarboxy Prothrombin (PIVKA-II) as a Marker of Hepatoblastoma in Infants

We evaluated plasma PIVKA-II (protein induced by vitamin K absence or antagonist-II, acarboxy prothrombin) levels in three infants with hepatoblastoma as a tumor marker. PIVKA-II levels were highly elevated in all three patients. Vitamin K administration, performed in two patients, resulted in only moderate reduction of PIVKA-II levels. Chemotherapy against tumor cells reduced the PIVKA-II levels without exception. Immunohistochemical study of the liver tissue indicated the presence of PIVKA-II in the hepatoblastoma cell. These findings suggest that elevated PIVKA-II in these patients was not due to nutritional vitamin K deficiency, but to excess production of tumor cells. A measurement of plasma PIVKA-II may be useful as a new marker of hepatoblastoma.

Hepatic lobectomy for hepatoblastoma in infants and children : L. W. Martin and K. S. Woodman. Arch. Surg. 98:1–8 (January), 1969

Hepatic lobectomy for hepatoblastoma in infants and children : L. W. Martin and K. S. Woodman. Arch. Surg. 98:1–8 (January), 1969