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Children
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Surgical Management for Children with Primary Liver Tumors
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Surgical Management for Children with Primary Liver Tumors

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Surgery".

Deadline for manuscript submissions: closed (15 February 2022) | Viewed by 12685

Special Issue Editor

Prof. Dr. Piotr Czauderna

E-Mail Website
Guest Editor
Dept Surg Children & Adolescents, Medical University Gdansk, Gdansk, Poland
Interests: pediatric liver tumors; surgical pediatric oncology; pediatric videosurgery; organization of healthcare quality and patient safety

Special Issue Information

Dear Colleagues,

This Special Issue’s primary focus is on surgical management for children with primary pediatric liver tumors, in particular hepatoblastoma, which is the most common malignant neoplasm in children.

They account for up to 1.5–2% of all childhood malignancies and can be considered an example of an ultra-rare disease. Their surgical management requires significant expertise and remains a challenge for every surgeon.

Even experienced pediatric institutions rarely perform more than 5–10 liver resections a year; hence, international cooperation, as well as the exchange of information and knowledge, is of utmost importance. Some principles are based upon rather old data and should be revisited; e.g., the role of atypical liver resections. Even data on the mortality and morbidity of pediatric liver resections are not very recent.

Novel approaches (3D reconstructions and printing, virtual reality, novel imaging techniques) could assist surgeons with better preoperative planning of a complete tumor excision and the avoidance of complications, which are relatively frequent due to common anatomic variations within the liver and hepatobiliary tree. Pediatric experience with other innovative techniques, such as the use of fluorescence-guided surgery, surgery with indocyaning green, or the use of laparoscopy, remains quite limited, too.

In my opinion, it is important to highlight current surgical options and approaches in pediatric liver tumors (hepatoblastoma, HCC, UESL) in order to improve resectability rates and outcomes, especially given that the rarity of these tumors makes adequate expertise infrequent.

Prof. Dr. Piotr Czauderna
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • hepatoblastoma
  • pediatric hepatocellular carcinoma
  • undifferentiated embryonal sarcoma of the liver (UESL)
  • pediatric fibrolamellar carcinoma
  • children
  • pediatric hepatic surgery
  • laparoscopic surgery
  • fluorescence-guided surgery (ICG)
  • preoperative surgical planning
  • complications and outcomes.

Published Papers (6 papers)

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Research

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11 pages, 584 KiB  
Article
Surgical Outcome of Children with a Malignant Liver Tumour in The Netherlands: A Retrospective Consecutive Cohort Study
by Merel B. Klunder, Janneke L. M. Bruggink, Leon D. H. Huynh, Frank A. J. A. Bodewes, Alida F. W. van der Steeg, Kathelijne C. J. M. Kraal, C. P. (Kees) van de Ven, Martine van Grotel, József Zsiros, Marc H. W. A. Wijnen, I. Q. (Quintus) Molenaar, Robert J. Porte, Vincent E. de Meijer and Ruben H. de Kleine
Children 2022, 9(4), 525; https://0-doi-org.brum.beds.ac.uk/10.3390/children9040525 - 07 Apr 2022
Cited by 1 | Viewed by 1521
Abstract
Introduction: Six to eight children are diagnosed with a malignant liver tumour yearly in the Netherlands. The majority of these tumours are hepatoblastoma (HB) and hepatocellular carcinoma (HCC), for which radical resection, often in combination with chemotherapy, is the only curative treatment option. [...] Read more.
Introduction: Six to eight children are diagnosed with a malignant liver tumour yearly in the Netherlands. The majority of these tumours are hepatoblastoma (HB) and hepatocellular carcinoma (HCC), for which radical resection, often in combination with chemotherapy, is the only curative treatment option. We investigated the surgical outcome of children with a malignant liver tumour in a consecutive cohort in the Netherlands. Methods: In this nationwide, retrospective observational study, all patients (age < 18 years) diagnosed with a malignant liver tumour, who underwent partial liver resection or orthotopic liver transplantation (OLT) between January 2014 and April 2021, were included. Children with a malignant liver tumour who were not eligible for surgery were excluded from the analysis. Data regarding tumour characteristics, diagnostics, treatment, complications and survival were collected. Outcomes included major complications (Clavien–Dindo ≥ 3a) within 90 days and disease-free survival. The results of the HB group were compared to those of a historical HB cohort. Results: Twenty-six children were analysed, of whom fourteen (54%) with HB (median age 21.5 months), ten (38%) with HCC (median age 140 months) and one with sarcoma and a CNSET. Thirteen children with HB (93%) and three children with HCC (30%) received neoadjuvant chemotherapy. Partial hepatic resection was possible in 19 patients (12 HB, 6 HCC, and 1 sarcoma), whilst 7 children required OLT (2 HB, 4 HCC, and 1 CNSET). Radical resection (R0, margin ≥ 1.0 mm) was obtained in 24 out of 26 patients, with recurrence only in the patient with CNSET. The mean follow-up was 39.7 months (HB 40 months, HCC 40 months). Major complications occurred in 9 out of 26 patients (35% in all, 4 of 14, 29% for HB). There was no 30- or 90-day mortality, with disease-free survival after surgery of 100% for HB and 80% for HCC, respectively. Results showed a tendency towards a better outcome compared to the historic cohort, but numbers were too small to reach significance. Conclusion: Survival after surgical treatment for malignant liver tumours in the Netherlands is excellent. Severe surgical complications arise in one-third of patients, but most resolve without long-term sequelae and have no impact on long-term survival. Full article
(This article belongs to the Special Issue Surgical Management for Children with Primary Liver Tumors)
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10 pages, 874 KiB  
Article
Feasibility of Real-Time Central Surgical Review for Patients with Advanced-Stage Hepatoblastoma in the JPLT3 Trial
by Tomoro Hishiki, Shohei Honda, Yuichi Takama, Yukihiro Inomata, Hideaki Okajima, Ken Hoshino, Tatsuya Suzuki, Ryota Souzaki, Motoshi Wada, Mureo Kasahara, Koichi Mizuta, Takaharu Oue, Akiko Yokoi, Takuro Kazama, Shugo Komatsu, Isamu Saeki, Osamu Miyazaki, Tetsuya Takimoto, Kohmei Ida, Kenichiro Watanabe and Eiso Hiyamaadd Show full author list remove Hide full author list
Children 2022, 9(2), 234; https://0-doi-org.brum.beds.ac.uk/10.3390/children9020234 - 10 Feb 2022
Cited by 1 | Viewed by 1510
Abstract
In the JPLT3 study, a real-time central surgical reviewing (CSR) system was employed aimed at facilitating early referral of candidates for liver transplantation (LTx) to centers with pediatric LTx services. The expected consequence was surgery, including LTx, conducted at the appropriate time in [...] Read more.
In the JPLT3 study, a real-time central surgical reviewing (CSR) system was employed aimed at facilitating early referral of candidates for liver transplantation (LTx) to centers with pediatric LTx services. The expected consequence was surgery, including LTx, conducted at the appropriate time in all cases. This study aimed to review the effect of CSR on institutional surgical decisions in cases enrolled in the JPLT3 study. Real-time CSR was performed in cases in which complex surgeries were expected, using images obtained after two courses of preoperative chemotherapy. Using the cloud-based remote image viewing system, an expert panel consisting of pediatric and transplant surgeons reviewed the images and commented on the expected surgical strategy or the necessity of transferring the patient to a transplant unit. The results were summarized and reported to the treating institutions. A total of 41 reviews were conducted for 35 patients, and 16 cases were evaluated as possible candidates for LTx, with the treating institutions being advised to consult a transplant center. Most of the reviewed cases promptly underwent definitive liver surgeries, including LTx per protocol. Full article
(This article belongs to the Special Issue Surgical Management for Children with Primary Liver Tumors)
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9 pages, 625 KiB  
Article
Effect of Centralization on Surgical Outcome of Children Operated for Liver Tumors in Switzerland: A Retrospective Comparative Study
by Jasmine Leoni, Anne-Laure Rougemont, Ana M. Calinescu, Marc Ansari, Philippe Compagnon, Jim C. H. Wilde and Barbara E. Wildhaber
Children 2022, 9(2), 217; https://0-doi-org.brum.beds.ac.uk/10.3390/children9020217 - 06 Feb 2022
Cited by 1 | Viewed by 1353
Abstract
Background: Pediatric liver surgery is complex, and complications are not uncommon. Centralization of highly specialized surgery has been shown to improve quality of care. In 2012, pediatric liver surgery was centralized in Switzerland in one national center. This study analyses results before and [...] Read more.
Background: Pediatric liver surgery is complex, and complications are not uncommon. Centralization of highly specialized surgery has been shown to improve quality of care. In 2012, pediatric liver surgery was centralized in Switzerland in one national center. This study analyses results before and after centralization. Methods: Retrospective monocentric comparative study. Analysis of medical records of children (0–16 years) operated for any liver tumor between 1 January 2001 and 31 December 2020. Forty-one patients were included: 14 before centralization (before 1 January 2012) and 27 after centralization (after 1 January 2012). Epidemiological, pre-, intra-, and post-operative data were collected. Fischer’s exact and t-test were used to compare groups. Results: The two cohorts were homogeneous. Operating time was reduced, although not significantly, from 366 to 277 min. Length of postoperative stay and mortality were not statistically different between groups. Yet, after centralization, overall postoperative complication rate decreased significantly from 57% to 15% (p = 0.01), Clavien > III complications decreased from 50% to 7% (p < 0.01), and hepatic recurrences were also significantly reduced (40% to 5%, p = 0.03). Conclusion: Centralization of the surgical management of liver tumors in Switzerland has improved quality of care in our center by significantly reducing postoperative complications and hepatic recurrence. Full article
(This article belongs to the Special Issue Surgical Management for Children with Primary Liver Tumors)
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Review

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9 pages, 2196 KiB  
Review
Fluorescence Image-Guided Navigation Surgery Using Indocyanine Green for Hepatoblastoma
by Eiso Hiyama
Children 2021, 8(11), 1015; https://0-doi-org.brum.beds.ac.uk/10.3390/children8111015 - 05 Nov 2021
Cited by 6 | Viewed by 1720
Abstract
In the past decade, navigation surgery using fluorescent indocyanine green (ICG) dye for hepatoblastoma (HB) has been developed for the resection of primary or metastatic tumors. Since HB cells can take up ICG but cannot excrete it to the bile duct, ICG remains [...] Read more.
In the past decade, navigation surgery using fluorescent indocyanine green (ICG) dye for hepatoblastoma (HB) has been developed for the resection of primary or metastatic tumors. Since HB cells can take up ICG but cannot excrete it to the bile duct, ICG remains in the HB cells, which can be used for navigation by fluorescent activation. The complete resection of the primary tumor as well as metastatic tumors, along with appropriate neoadjuvant and adjuvant chemotherapy, is essential for cure. ICG fluorescence can detect microscopic residual lesions in the primary lesion and identify micro-metastases in the lung or other lesions; consequently, ICG navigation surgery may improve outcomes for patients with HB. The basic technique and recent advances in ICG navigation for HB surgery are reviewed. Full article
(This article belongs to the Special Issue Surgical Management for Children with Primary Liver Tumors)
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Other

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5 pages, 801 KiB  
Case Report
Liver Resection Using Saline-Linked Radiofrequency Technology in an Infant with Congenital Hepatoblastoma
by Giovanni Torino, Michele Ilari, Edoardo Bindi, Francesca Mariscoli and Giovanni Cobellis
Children 2022, 9(3), 418; https://0-doi-org.brum.beds.ac.uk/10.3390/children9030418 - 15 Mar 2022
Viewed by 1480
Abstract
We herein report a case of giant congenital hepatoblastoma in a 3-month-old male treated with neoadjuvant chemotherapy and hepatic resection. After considerable reduction of the tumor with chemotherapy, a right bloodless hemihepatectomy using saline-linked radiofrequency technology (SLRT) and without clamping of the hepatic [...] Read more.
We herein report a case of giant congenital hepatoblastoma in a 3-month-old male treated with neoadjuvant chemotherapy and hepatic resection. After considerable reduction of the tumor with chemotherapy, a right bloodless hemihepatectomy using saline-linked radiofrequency technology (SLRT) and without clamping of the hepatic pedicle was performed. Intraoperative blood loss was minimal, and consequently, no blood transfusions were required. The surgery lasted 140 min, and SLRT was used for a total of 60 min. No complications were observed during or after the surgery. In conclusion, congenital hepatoblastoma is a very rare cancer for which surgery is an essential therapeutic step, and in our presented case, we showed that SLRT allowed for a safe and effective bloodless liver resection. Full article
(This article belongs to the Special Issue Surgical Management for Children with Primary Liver Tumors)
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8 pages, 2775 KiB  
Case Report
Combined Use of Three-Dimensional Construction and Indocyanine Green-Fluorescent Imaging for Resection of Multiple Lung Metastases in Hepatoblastoma
by Shugo Komatsu, Keita Terui, Mitsuyuki Nakata, Ryohei Shibata, Satoru Oita, Yunosuke Kawaguchi, Hiroko Yoshizawa, Tomoya Hirokawa, Erika Nakatani and Tomoro Hishiki
Children 2022, 9(3), 376; https://0-doi-org.brum.beds.ac.uk/10.3390/children9030376 - 08 Mar 2022
Cited by 4 | Viewed by 3369
Abstract
It is essential to accurately and safely resect all tumors during surgery for multiple lung metastases. Here, we report a case of hepatoblastoma (HB) with multiple pulmonary nodules that ultimately underwent complete resection using combined three-dimensional image reconstruction and indocyanine green (ICG) fluorescence [...] Read more.
It is essential to accurately and safely resect all tumors during surgery for multiple lung metastases. Here, we report a case of hepatoblastoma (HB) with multiple pulmonary nodules that ultimately underwent complete resection using combined three-dimensional image reconstruction and indocyanine green (ICG) fluorescence guidance. A 1-year-old boy was diagnosed with HB and multiple lung metastases. After intensive chemotherapy, complete resection with subsegmentectomy (S5 + 6) and partial resection (S3, S8) were performed. More than 100 pulmonary nodules, which remained visible on computed tomography (CT) despite additional postoperative chemotherapy, were subjected to pulmonary resection. We used the SYNAPSE VINCENT software (Fujifilm Medical, Tokyo, Japan) to obtain three-dimensional images of the nodules. We numbered each nodule, and 33 lesions of the right lung were resected by multiple wedge resections through a right thoracotomy, with the aid of palpation and ICG fluorescence guidance. One month after the right metastasectomy, resection of 64 lesions in the left lung was performed via left thoracotomy. Postoperative CT showed complete clearance of the lung lesions, and the patient remained disease-free for 15 months after the treatment. This case study confirms that the combination of three-dimensional localization and ICG fluorescence guidance allows for accurate and safe resection of nearly 100 lung metastases. Full article
(This article belongs to the Special Issue Surgical Management for Children with Primary Liver Tumors)
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