Hepatoblastoma in pediatrics
Description
● Introduction:
Hepatoblastoma is an uncommon malignancy in children. Every year, about 100 instances of hepatoblastoma are documented. An globally accepted criterion was created to categorise patients as standard or high-risk due to the disorder's low occurrence. It has been shown that studies including liver transplants, surgery, and chemotherapeutic medicines increase the disease-free survival rate. Chemotherapeutic drugs and surgery together showed that these regimens might reduce the initial diagnostic staging of tumours and turn previously incurable tumours into ones that could be removed.
● Case Example:
kid, age 4, who was born preterm (25 weeks, 750 g).
first showed signs of a URI, then vomiting, fever, and hepatomegaly.
●Investigations:
➣CT: ascites, effusions, and a massive, irresectable liver tumour (179 mm).
➣Biopsy: embryonal hepatoblastoma.
➣>600,000 ng/mL for AFP.
➣Staging: PRETEXT IV with high-risk lung metastases.
● Treatment:
•Cisplatin + Doxorubicin chemotherapy, but tumor progressed.
•Referred for experimental chemotherapy since they are not eligible for a transplant.
•died from progression after around 11 months.
● Epidemiology:
• children's most prevalent primary malignant liver tumour.
• is responsible for 1-2% of all children's malignancies and 79% of paediatric liver tumours.
• Peak age: 0–5 years old (rarely beyond 5 years old).
• Incidence per year: around 1.5 cases per million children; greater in babies under one year.
• more prevalent among white youngsters and boys.
● Risk Elements and Correlations
➣Genetic/Syndromic conditions:
•Beckwith–Wiedemann syndrome
•FAP, or familial adenomatous polyposis
•Hemihypertrophy
•Atresia biliary
➣Other associations:
• Prematurity, low birth weight
• Hepatitis B infection in its early stages
● Clinical Characteristics
➣Most common: hepatomegaly or an enlarged abdomen;
➣less common: anorexia, vomiting, weight loss, abdominal discomfort, and early puberty.
➣10–20% metastases at diagnosis, mostly to the lungs.
➣Lab results:
• Alpha-fetoprotein (AFP) is nearly always high.
• Liver enzymes and bilirubin are often normal.
• Platelet abnormalities: anaemia or thrombocytopenia are occasionally seen, along with thrombocytosis, which is prevalent.
● PRETEXT System staging:
➣PRETEXT I–IV: number of liver sections ➣ PRETEXT I–IV: the quantity of liver sections that are involvedFactors for annotation:
•V: vena cava/hepatic veins
•P: portal veins
•E: extrahepatic extension
•F: multifocality
•R: rupture
•M: metastasis
Pathological/Surgical Stages: I (full resection) → IVb (metastatic, partial resection).
The prognosis is worse at higher stages.
Protocol
● Management
➣Chemotherapy:
•Cisplatin is the most effective medication.
•Cisplatin alone or in combination (Cisplatin + Doxorubicin / 5-FU / Vincristine) are common regimens.
•Neoadjuvant chemotherapy reduces tumour size and renders incurable patients treatable.
• Adverse effects include marrow suppression, ototoxicity, and nephrotoxicity.
➣Surgery:
• If resectable, lobectomy or partial hepatectomy is the preferred course of therapy.
• Because of regeneration, it is safe to remove up to 85% of the liver.
• Complete tumour removal is the aim.
•High chance of recurrence if resection is not completed.
➣ Liver transplant:
• For tumours that cannot be removed without spreading outside the liver.
• Better results if AFP drops by more than 99% with chemotherapy prior to transplant.
•Chemotherapy after transplantation increases survival.
➣Radiotherapy:
•not commonly utilised, however it may be used for lung metastases that are resistant to chemotherapy or for microscopic illness that still exists.
● Health Care Administration
➣ Medicine
•Difficulties: Patients frequently arrive with advanced staging or unresectable tumours, which precludes immediate surgical excision.
•Management: To reduce the tumor's size, lower PRETEXT staging, and increase surgical resectability, combination chemotherapy regimens containing doxorubicin and cisplatin are used.
➣Surgery
•Problems Faced:The tumour may be initially unresectable due to its large size or an unfavourable placement.
•Management: Surgical resection, such as a lobectomy, is used to remove the tumour following a successful chemotherapy-induced response. Rarely, if the tumour cannot be removed with the most aggressive treatment, liver transplantation may be considered.
➣ Radiotherapy
• Issues: Remaining tumour or metastasis that is not responding to chemotherapy and surgery, particularly lung involvement.
•Management: In high-risk situations or when the illness has spread, radiotherapy may be administered for palliation or local control.
● Nursing Interventions
→ Side Effects Associated with Chemotherapy
➣ Issue: Chemotherapeutic drugs (doxorubicin, cisplatin) can induce myelosuppression, nephrotoxicity, mucositis, nausea, and vomiting.
➣ Intervention:
• Antiemetic Administration: Prevent nausea and vomiting by proactively administering drugs such ondansetron.
• Oral Care Protocols: To avoid mucositis, apply calming gels, rinse your mouth often, and do routine mouth examinations.
• Hydration and Renal Monitoring: To prevent nephrotoxicity, make sure you are getting enough fluids by IV or PO, and regularly check your kidneys' BUN and creatinine levels.
• Infection Control: Prophylactic antibiotics, stringent hand hygiene, and neutropenic measures are used to avoid infection during myelosuppressive episodes.
→ Postoperative/Surgical Complications :
➣Issues: include pain, bleeding risk, liver impairment, and delayed wound healing following a transplant or lobectomy.
➣Intervention: • Pain Management: Utilise non-pharmacological comfort methods, age-appropriate pain scales, and analgesics as directed.
• Bleeding Assessment: Keep an eye out for haemorrhage symptoms.
• Liver Function Monitoring: Monitor liver enzyme, bilirubin, and INR levels; check for encephalopathy or jaundice.
Wound Care: Change sterile dressings and check for infection or dehiscence at the sites of incisions.
→ Nutritional Deficits :
➣ Issue: Poor oral intake and malnutrition due to anorexia, vomiting, and liver dysfunction.
➣ Intervention:
• Dietary Support: Work with nutritionists to offer meals that are high in calories and protein and customised to each person's tolerance.
• Enteral/Parenteral Nutrition: If oral intake is insufficient, start tube feedings or TPN.
• Track Labs and Weight: Evaluate prealbumin, weight, and other nutritional indicators on a regular basis.
→psychosocial issues.
➣Problem: Emotional anguish associated with diagnosis, procedures, changes in body image, and family coping issues
➣Intervention:
• Psychological Support: Provide opportunities for play therapy or counselling together with age-appropriate explanations.
•Family Education and Support: Help parents connect with support groups and educate them about the causes, symptoms, and at-home care of illnesses.
• School Reintegration: Plan for educational requirements when students resume their regular activities by coordinating with instructors and social workers.
→ Metastatic or Advanced Disease:
➣ Issue: Lung metastases can cause worry, respiratory distress, and the requirement for close observation.
➣ Intervention:
• breathing Support: Keep an eye on pulse oximetry and breathing rate, and give more oxygen as required.
• Chest Imaging and Assessment: Continue regular radiographic surveillance and clinical assessment to detect any changes or resolutions.
Notes
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