Pediatric Liver Tumor | Dr. Dong Rui (Pediatric Surgery) | CMCS Shanghai

About Dr. Dong Rui

Dr. Dong Rui is Director of Pediatric Surgery at Children's Hospital of Fudan University — one of China's foremost paediatric medical centres and a national reference institution for complex paediatric liver tumours, biliary atresia, and congenital gastrointestinal malformations. She is one of China's most respected paediatric surgeons with a strong international reputation, recognised for her expertise in hepatoblastoma resection, precision liver surgery in children, and the integration of 3D preoperative planning, intraoperative ultrasound, and ICG fluorescence navigation into complex paediatric hepatic procedures. Dr. Dong's practice is defined by the philosophy that the goal of paediatric oncological surgery is not merely survival — it is the preservation of normal growth, development, and quality of life for a child who has decades ahead. Every surgical decision must be evaluated not only against its immediate oncological outcome but against its long-term impact on hepatic reserve, vascular integrity, and the child's capacity to grow and thrive. Her department at Children's Hospital of Fudan University has established one of China's most comprehensive paediatric liver tumour programmes, integrating multidisciplinary oncology, precision imaging, neoadjuvant chemotherapy, and structured long-term developmental surveillance into a unified care pathway.

Case Overview

Master Liam O'Brien, a 3-year-old Irish boy based in Shanghai, presented with a one-month history of progressive abdominal distension and a palpable right upper quadrant mass. Serum alpha-fetoprotein (AFP) was markedly elevated at greater than 12,100 ng/mL. Contrast-enhanced CT demonstrated a massive 10 cm x 9 cm x 8 cm hepatic tumour involving the right lobe and caudate lobe, with the critical finding that the tumour was intimately adherent to and partially encasing the retrohepatic inferior vena cava (IVC) and the right hepatic vein (RHV) orifice. Biopsy confirmed mixed fetal/embryonal hepatoblastoma. PRETEXT staging was III (V+, P+) — high-risk disease with vascular involvement.

The multidisciplinary team led by Dr. Dong Rui recommended neoadjuvant PLADO chemotherapy (cisplatin plus doxorubicin) for four cycles before surgery — to reduce tumour volume, create a dissection plane between the tumour and the IVC, and allow compensatory hypertrophy of the future liver remnant. After two cycles, AFP fell from greater than 12,100 to 800 ng/mL and tumour volume reduced by approximately 40%, with an oedematous separation plane appearing between the tumour and the IVC wall. After four cycles, the future liver remnant (left lateral segment) had hypertrophied to 45% of total liver volume — sufficient for safe resection. Dr. Dong performed laparoscopy-assisted right hemihepatectomy with caudate lobe resection and IVC tumour peeling using CUSA ultrasonic dissection, intraoperative ultrasound, and ICG fluorescence guidance. The right hepatic vein was ligated at its IVC confluence. Total operative time was 280 minutes; blood loss 150 mL with 100 mL autologous cell salvage; no allogeneic transfusion. Final pathology confirmed R0 resection with a clear IVC wall margin greater than 2 mm, 60% tumour necrosis, and negative hilar lymph nodes. AFP normalised to below 10 ng/mL at three months with no radiological evidence of recurrence. The child's growth and development remained on track at follow-up.

Patient Background

• Name / Nationality: Master Liam O'Brien (pseudonym) — Irish; 3 years 2 months old; based in Shanghai with his family
• Age / Sex: 3-year-old male
• Chief Complaint: Abdominal mass discovered incidentally by parents one month prior; progressive abdominal distension; reduced appetite; intermittent abdominal pain
• Symptoms: No fever, no jaundice, no haematemesis or melaena; no weight loss beyond mild appetite reduction
• Examination: Abdominal distension; firm, well-demarcated mass approximately 10 cm x 8 cm palpable in the right upper quadrant; non-tender; no hepatic stigmata (no palmar erythema, no spider naevi)
• Developmental parameters: Weight 14 kg (mildly below age-adjusted standard); height 96 cm; developmental milestones appropriate for age

Diagnostic Workup

Tumour Markers

• Alpha-fetoprotein (AFP): Greater than 12,100 ng/mL — markedly elevated (normal less than 10 ng/mL); AFP is the most sensitive and specific tumour marker for hepatoblastoma; levels above 1,000 ng/mL at diagnosis are characteristic of hepatoblastoma and distinguish it from other paediatric liver tumours
• CEA and CA19-9: Within normal limits — no evidence of alternative primary tumour

Contrast-Enhanced CT (Abdomen)

• Tumour: Massive irregular hypodense mass in the right lobe and caudate lobe; dimensions 10 cm x 9 cm x 8 cm; heterogeneous enhancement with central necrotic areas
• Critical vascular involvement: Tumour intimately adherent to and partially encasing the retrohepatic IVC; right hepatic vein orifice at the IVC confluence involved by tumour; right portal vein branch displaced and compressed
• Future liver remnant (FLR): Left lateral segment showing compensatory hypertrophy; estimated FLR approximately 35% of total liver volume at diagnosis — borderline for safe resection; requires further hypertrophy before surgery
• No extrahepatic disease: No pulmonary nodules, no peritoneal deposits, no lymphadenopathy beyond the hepatic hilum

MRI and MRCP

• Precise delineation of tumour relationship to hepatic veins, IVC, and portal vein branches — confirming CT findings and excluding biliary system invasion
• No intrahepatic bile duct dilatation; no tumour thrombus within the portal vein or IVC lumen

Biopsy

• Technique: Ultrasound-guided percutaneous core needle biopsy
• Pathology: Mixed fetal/embryonal hepatoblastoma — the most common histological subtype; fetal pattern associated with better chemotherapy response than pure embryonal or small cell undifferentiated subtypes
• PRETEXT staging: III (V+, P+) — tumour involving three liver sections with involvement of the hepatic veins (V+) and portal vein (P+); high-risk group per SIOPEL criteria

Dr. Dong's pre-operative assessment: The CT tells us the most important thing immediately: the tumour is wrapped around the inferior vena cava. That is the central surgical challenge. If we operate now, we either leave tumour on the IVC wall and accept an R1 resection, or we attempt to resect the IVC and reconstruct it — which in a 3-year-old with a 14 kg body weight carries a mortality risk we cannot accept. The answer is chemotherapy first. PLADO will shrink the tumour. More importantly, it will create an oedematous plane between the tumour capsule and the IVC wall — a plane that does not exist now but will exist after two cycles. That plane is what makes the peeling technique possible. We wait for the chemotherapy to create the conditions for the surgery.

Multidisciplinary Team Discussion and Neoadjuvant Strategy

The MDT convened by Dr. Dong Rui included paediatric surgery, paediatric oncology, radiology, pathology, and paediatric intensive care. The consensus recommendation was neoadjuvant PLADO chemotherapy for four cycles before surgical resection — the standard approach for PRETEXT III/IV hepatoblastoma per SIOPEL and COG guidelines.

PLADO regimen: Cisplatin (80 mg/m² per cycle) alternating with doxorubicin (60 mg/m² per cycle) — the international standard neoadjuvant regimen for high-risk hepatoblastoma. Cisplatin is the most active single agent in hepatoblastoma; doxorubicin provides complementary cytotoxicity through a different mechanism. The alternating schedule reduces cumulative organ toxicity while maintaining antitumour efficacy.

Rationale for neoadjuvant approach: Direct surgery for PRETEXT III hepatoblastoma with IVC involvement carries an R0 resection rate below 50% at most centres and a perioperative mortality risk that is unacceptable in a 3-year-old child. Neoadjuvant chemotherapy achieves three goals simultaneously: tumour volume reduction to facilitate resection; creation of a chemotherapy-induced oedematous plane between the tumour and the IVC wall that enables the peeling technique; and induction of compensatory hypertrophy of the future liver remnant to ensure adequate post-resection hepatic reserve.

Chemotherapy Response Assessment

• After cycle 2: AFP reduced from greater than 12,100 to 800 ng/mL — 93% reduction; tumour volume reduced by approximately 40% on CT; oedematous separation plane visible between tumour capsule and IVC wall on MRI — the critical finding confirming that the peeling technique will be feasible
• After cycle 4: AFP further reduced to approximately 200 ng/mL; tumour volume reduced by approximately 55%; FLR hypertrophied from 35% to 45% of total liver volume — exceeding the 40% threshold required for safe resection in a chemotherapy-conditioned liver
• Chemotherapy tolerability: Managed with standard antiemetic and haematopoietic support; no dose reductions required; renal function and cardiac function (echocardiography) monitored throughout and remained within acceptable limits

Dr. Dong's restaging assessment: The MRI after cycle 4 shows us exactly what we hoped to see. The plane between the tumour and the IVC is now visible — a thin layer of oedematous tissue separating the tumour capsule from the vein wall. That plane is our surgical corridor. The left liver has grown from 35% to 45% — we have enough reserve. The AFP has fallen by more than 98% from the peak. This tumour is responding. Now we operate.

Operative Procedure

Anaesthesia, Positioning, and Preparation

Anaesthesia: General anaesthesia with endotracheal intubation; arterial line and central venous catheter placed for continuous haemodynamic monitoring and rapid volume replacement if required. Intraoperative cell salvage system activated.

Positioning: Supine; slight left lateral tilt to improve access to the retrohepatic IVC.

3D preoperative planning: CT volumetric data processed to generate a three-dimensional reconstruction of the hepatic vascular anatomy — precisely mapping the relationship between the tumour, the hepatic veins, the IVC, and the portal vein branches. The 3D model was used for surgical rehearsal and to plan the parenchymal transection plane that would achieve R0 margins while preserving the maximum left lateral segment volume.

Phase 1 — Laparoscopic Exploration and Hepatic Mobilisation

Laparoscopic survey: Four-port laparoscopic exploration confirmed no peritoneal deposits, no ascites, no unexpected extrahepatic disease. The tumour was visualised occupying the right lobe and caudate lobe with the anticipated relationship to the IVC.

Conversion to laparoscopy-assisted approach: A right subcostal incision was added to allow direct manual access to the retrohepatic IVC — the critical structure requiring tactile feedback during the peeling dissection that laparoscopic instruments alone cannot safely provide in a 3-year-old.

Hepatic mobilisation: Right triangular and coronary ligaments divided to fully mobilise the right lobe and expose the retrohepatic IVC from the right renal vein to the hepatic vein confluence. The IVC was encircled with a vessel loop above and below the tumour — providing vascular control in the event of IVC wall injury during the peeling dissection.

Phase 2 — Hepatic Inflow Control and Pringle Manoeuvre

Hepatoduodenal ligament dissection: The hepatic artery and portal vein were dissected and individually controlled at the hepatic hilum. The right hepatic artery and right portal vein were ligated and divided — devascularising the right lobe and creating the ischaemic demarcation line that defines the parenchymal transection plane.

Pringle manoeuvre: A hepatoduodenal ligament occlusion tape was placed for intermittent inflow occlusion during parenchymal transection — reducing blood loss during the most haemorrhagic phase of the operation. Total cumulative Pringle time was maintained below 20 minutes — the safe threshold for hepatic ischaemia in a child with a chemotherapy-conditioned liver.

Dr. Dong's operative note: The Pringle time limit in a child is not the same as in an adult. A healthy adult liver tolerates 60 minutes of warm ischaemia. A chemotherapy-conditioned liver in a 3-year-old tolerates much less. We set our limit at 20 minutes cumulative and we enforce it absolutely. If we reach 20 minutes and the transection is not complete, we release the Pringle, reperfuse the liver for 5 minutes, and then reapply. The extra time costs us nothing. Exceeding the ischaemia limit costs us the liver.

Phase 3 — ICG Fluorescence Guidance and Parenchymal Transection

ICG fluorescence imaging: Indocyanine green was administered intravenously before right hepatic artery ligation. Under near-infrared fluorescence imaging, the devascularised right lobe and caudate lobe — containing the tumour — appeared dark (negative staining), while the perfused left lateral segment fluoresced brightly. This negative staining technique provided a real-time, patient-specific map of the ischaemic demarcation line — the precise boundary between the tissue to be resected and the tissue to be preserved.

Tumour margin delineation: ICG fluorescence also delineated the tumour boundary within the liver parenchyma — hepatoblastoma cells accumulate ICG due to impaired biliary excretion, creating a positive tumour signal that defines the resection margin under fluorescence imaging. The combination of negative staining (ischaemic demarcation) and positive tumour staining provided a dual fluorescence map guiding the entire parenchymal transection.

Parenchymal transection: CUSA (cavitron ultrasonic surgical aspirator) used for parenchymal dissection — selectively destroying hepatocyte parenchyma while preserving vascular and biliary structures for individual ligation. Vessels and bile ducts greater than 3 mm were controlled with Hem-o-lok clips before division. Bipolar electrocautery used for haemostasis of small vessels and bile duct radicles.

Intraoperative ultrasound (IOUS): Real-time IOUS used throughout parenchymal transection to confirm the position of the left portal vein, middle hepatic vein, and left hepatic vein — ensuring the transection plane maintained greater than 1 cm clearance from these critical structures at all times.

Phase 4 — IVC Tumour Peeling — The Critical Step

Exposure: With the right lobe parenchyma transected and the specimen attached only by the retrohepatic IVC adhesion and the right hepatic vein, the IVC was fully exposed from the right renal vein to the left hepatic vein confluence. The vessel loops above and below the tumour attachment zone were tightened to provide immediate vascular control.

Right hepatic vein management: The right hepatic vein — its orifice involved by tumour — was ligated flush with the IVC using a vascular stapler. The IVC wall at the RHV orifice was inspected: no transmural tumour invasion confirmed; the IVC wall was intact.

IVC peeling technique: Using CUSA at its lowest power setting and fine-tipped dissecting forceps, Dr. Dong dissected the tumour capsule away from the IVC adventitia in the chemotherapy-induced oedematous plane — the plane that had been created by the neoadjuvant therapy and confirmed on pre-operative MRI. The dissection proceeded circumferentially around the IVC, maintaining the plane between the tumour capsule and the outer adventitial layer of the vein wall. No entry into the IVC lumen occurred. The IVC wall was inspected after tumour removal: intact, no perforation, no residual tumour on the adventitial surface.

Dr. Dong's operative note: The peeling plane is everything. When the chemotherapy has worked correctly, the plane is there — you can feel it with the CUSA tip, a slightly different resistance between the tumour capsule and the vein wall. You follow that resistance. You do not force. You do not cut. You dissect. The CUSA removes the tumour tissue cell by cell at the interface, and the vein wall stays intact. If you lose the plane — if you feel the resistance change and the tissue becomes uniform — you stop, you reorient with the intraoperative ultrasound, and you find the plane again. In this case, the plane was present throughout the entire circumference of the IVC contact zone. The chemotherapy had done its job perfectly.

Specimen extraction: The fully mobilised specimen — right lobe, caudate lobe, and tumour — was placed in a retrieval bag and extracted through a protected lower midline incision, avoiding tumour rupture and peritoneal contamination.

Operative data: Total operative time 280 minutes; total blood loss approximately 150 mL; autologous cell salvage returned 100 mL; no allogeneic red cell transfusion; no IVC reconstruction required.

Post-operative Course and ERAS Outcomes

ERAS Milestones

• Post-operative day 1: Nasogastric tube removed; oral fluids commenced; assisted mobilisation to sitting position
• Post-operative day 3: Oral diet resumed; abdominal drain output clear yellow, less than 30 mL per day; drain removed; no bile leak
• Post-operative day 7: Liver function tests showing mild ALT/AST elevation (expected post-hepatectomy transaminase rise); bilirubin normal; AFP reduced to 120 ng/mL
• Post-operative day 10: Discharged home; community paediatric follow-up arranged

Post-operative Complications

• Mild ascites: Small-volume ascites developed on post-operative day 3 — expected after major hepatectomy in a child with a chemotherapy-conditioned liver; managed with albumin infusion and diuretic therapy; resolved by day 7
• No bile leak, no haemorrhage, no hepatic failure, no Budd-Chiari syndrome
• No IVC thrombosis: Doppler ultrasound on post-operative day 3 confirmed normal IVC flow velocity and no thrombus formation at the peeling site

Final Pathology

• Tumour: Hepatoblastoma, predominantly epithelial type (mixed fetal/embryonal); post-chemotherapy tumour size 6 cm x 5 cm x 4 cm — significant reduction from the pre-treatment 10 cm x 9 cm x 8 cm mass
• Tumour necrosis: Approximately 60% — indicating good chemotherapy response; necrosis rate above 50% is associated with favourable prognosis in hepatoblastoma
• Surgical margins: All parenchymal margins negative (R0 resection); IVC wall margin greater than 2 mm — no residual tumour on the IVC adventitia
• Lymph nodes: 0/3 hilar lymph nodes positive — no nodal metastasis
• Vascular invasion: No lymphovascular invasion identified in the resected specimen

Adjuvant Treatment and Long-term Follow-up

Two further cycles of PLADO chemotherapy were commenced two weeks post-operatively to complete the planned six-cycle total course — the standard adjuvant approach for high-risk hepatoblastoma regardless of pathological response. The chemotherapy intensity was adjusted based on the 60% necrosis rate (indicating good but not complete response) and the child's post-operative hepatic reserve.

At three months post-operatively, MRI demonstrated no residual or recurrent disease and AFP had normalised to below 10 ng/mL — the most important prognostic milestone in hepatoblastoma follow-up. AFP normalisation after treatment completion is associated with a five-year event-free survival rate above 80% in PRETEXT III disease. The child's weight and height were tracking appropriately for age at three-month follow-up, with no evidence of growth impairment attributable to the chemotherapy or surgery.

Expert Commentary — Dr. Dong Rui

1. Neoadjuvant Chemotherapy: Creating the Conditions for Surgery

For PRETEXT III and IV hepatoblastoma with vascular involvement, neoadjuvant chemotherapy is not a preliminary step before the real treatment — it is the treatment that makes the real treatment possible. Direct surgery for a tumour encasing the retrohepatic IVC in a 3-year-old child carries an R0 resection rate below 50% and a perioperative mortality risk that no family should be asked to accept when an effective alternative exists. PLADO chemotherapy achieves tumour volume reduction in more than 80% of hepatoblastoma cases — but the volume reduction is not the most important effect. The most important effect is the creation of the oedematous separation plane between the tumour capsule and the IVC adventitia. This plane does not exist in the untreated tumour — the tumour is adherent to the vein wall without a dissectable interface. After two cycles of PLADO, the plane appears: a thin layer of chemotherapy-induced oedema and fibrosis that separates the tumour from the vessel. That plane is the surgical corridor that makes IVC preservation possible. Without it, we are choosing between an R1 resection and an IVC reconstruction. With it, we can peel the tumour off the vein and achieve R0 without touching the IVC lumen.

2. The IVC Peeling Technique: Vascular Preservation Over Reconstruction

The conventional approach to a tumour encasing a major vein is resection and reconstruction — excise the involved vein segment and replace it with a graft. In adults with hepatocellular carcinoma or cholangiocarcinoma involving the IVC, this approach is well established. In a 3-year-old child, it is a different calculation entirely. A synthetic graft in a 14 kg child will not grow with the child — it will become relatively stenotic as the child grows, requiring revision surgery. An autologous vein graft requires harvesting from another site, adding operative time and donor site morbidity. And the IVC reconstruction itself — clamping the IVC in a small child, managing the haemodynamic consequences of caval occlusion, and constructing a watertight anastomosis in a vessel of 1–1.5 cm diameter — carries risks that are disproportionate to the benefit when an alternative exists. The peeling technique, when the chemotherapy has created the correct plane, achieves R0 resection without entering the IVC lumen, without reconstruction, and without the long-term complications of a prosthetic or autologous graft. It requires patience, tactile sensitivity, and the discipline to follow the plane rather than cut through it. But when it works — and in this case it worked perfectly — the child wakes up with an intact IVC, normal venous return, and no risk of Budd-Chiari syndrome.

3. ICG Fluorescence and 3D Planning: Seeing What the Eye Cannot See

Paediatric liver surgery is technically more demanding than adult liver surgery in one critical respect: the structures are smaller, the margins for error are narrower, and the consequences of inadvertent vascular or biliary injury are proportionally greater. A 3 mm bile duct in a 3-year-old is not a minor structure — it is the entire biliary drainage of a liver segment. ICG fluorescence addresses this challenge by making the invisible visible. The negative staining technique — where the devascularised segment appears dark against the fluorescing perfused parenchyma — provides a real-time, patient-specific map of the ischaemic demarcation line that is more accurate than any anatomical landmark. The positive tumour staining — where hepatoblastoma cells accumulate ICG due to impaired biliary excretion — delineates the tumour boundary within the parenchyma, guiding the resection margin in real time. Together with the 3D preoperative reconstruction that maps every vascular and biliary structure in three dimensions before the first incision, ICG fluorescence transforms paediatric liver surgery from an exercise in anatomical inference to a navigation-guided procedure where the surgeon always knows exactly where the critical structures are and exactly where the tumour ends.

4. The Goal of Paediatric Oncological Surgery: Survival and a Normal Life

The five-year survival rate for hepatoblastoma has improved from below 30% in the 1970s to above 80% in high-risk cases managed at specialised centres today. This improvement is the result of the combination of effective chemotherapy and increasingly precise surgery — but it has created a new responsibility. When a child survives hepatoblastoma, they have sixty or seventy years of life ahead of them. The surgical decisions we make at age 3 will affect the quality of those sixty years. A child who survives with an intact IVC, a functioning liver remnant, and no growth impairment from excessive hepatic resection has a fundamentally different long-term trajectory than a child who survives with a reconstructed IVC, a marginal liver remnant, and the sequelae of repeated surgical interventions. At Children's Hospital of Fudan University, our outcome measure is not five-year survival. It is the child at age 20 — in school, in work, in relationships, living a life that the cancer did not define. That is the standard we hold ourselves to, and it is the standard that drives every decision from the first chemotherapy cycle to the last follow-up visit.

How CMCS Shanghai Coordinated This Case

CMCS Shanghai supported the O'Brien family from initial mass discovery through three-month post-operative follow-up, including: urgent coordination of AFP, CEA, CA19-9, and full blood count with same-day results and bilingual interpretation; specialist referral to Dr. Dong Rui at Children's Hospital of Fudan University's Department of Pediatric Surgery with emergency consultation scheduling; coordination of contrast-enhanced CT, MRI, MRCP, and ultrasound-guided biopsy with bilingual radiology and pathology report translation; bilingual interpretation throughout all MDT discussions, chemotherapy consent, and surgical planning sessions; coordination of PLADO chemotherapy cycles including cisplatin and doxorubicin scheduling, antiemetic support, haematological monitoring, and cardiac and renal function surveillance; real-time updates to the child's parents and their paediatrician in Ireland after each chemotherapy cycle covering AFP trends, tumour response, and FLR volumetry; 3D preoperative planning model coordination and surgical planning discussion with bilingual summary for the family; real-time surgical updates to the parents during the 280-minute procedure, including explanation of the IVC peeling technique and its successful completion; post-operative daily bilingual updates covering liver function trends, AFP trajectory, drain output, ascites management, and discharge planning; adjuvant chemotherapy cycle coordination post-operatively with tolerability monitoring; three-month MRI and AFP follow-up coordination with results communicated to the family and their paediatric oncologist in Dublin; and establishment of a long-term surveillance protocol with six-monthly AFP and annual MRI, with direct liaison between Dr. Dong's team and the child's paediatric oncologist in Ireland.

For international families with children requiring complex paediatric surgery, liver tumour resection, or management of congenital surgical conditions in Shanghai, Dr. Dong Rui's team at Children's Hospital of Fudan University represents paediatric surgical expertise at the international frontier — combining neoadjuvant chemotherapy optimisation, 3D preoperative planning, ICG fluorescence navigation, and precision vascular preservation to achieve oncological cure while protecting the child's long-term growth, development, and quality of life. CMCS ensures that expertise is accessible: in the family's language, with overseas paediatricians and oncologists informed at every step, from the first AFP result through long-term developmental surveillance.

This case report is de-identified and published for educational purposes. All clinical details have been anonymized in accordance with patient privacy standards. CMCS Shanghai is a medical concierge service and does not provide direct medical care.