Paediatric liver tumours

Mohamed Kamel
Clinical fellow, paediatric surgery
Surg Clin North Am. 2010 August ; 90(4): 643–653. doi:10.1016/j.suc.2010.04.017.
Birmingham Children’s hospital 2021
Paediatric liver tumours

Left
2 3 4 vein
Hepatic

1IVC 5
PV
Paediatric liver tumours

2 4

Surg Clin North Am. 2010 August ; 90(4): 643–653. doi:10.1016/j.suc.2010.04.017.

 Paediatric Liver Tumours
Benign Malignant

• Hemangioma
• Hamartoma
• FNH
• Adenoma
 Paediatric Liver Tumours
Benign Malignant

• Hemangioma Hepatocellular Non hepatocellular

• Hamartoma
• FNH • Undifferentiated embryonal sarcoma of the liver
• Infantile choriocarcinoma of the liver
• Adenoma • Biliary rhabdomyosarcoma

Hepatoblastoma Hepatocellular Carcinoma Hepatocellular malignant tumour NOS

Fibrolamellar HCC

Wholly Epithelial Mixed Epithelial and Mesenchymal

• Fetal
Low mitotic activity • MEM with teratoid features
Mitotically active • MEM without teratoid
Case courtesy features
of Dr Hazem M Almasarei, Radiopaedia.org, rID: 51815
• Embryonal Case courtesy of Dr Oscar Osorio, Radiopaedia.org, rID: 57459
• Epithelial mixed
• Small cell undifferentiated
• Cholangioblastic
• • Grow in sheets, organized tubular or acinar
Cells measuring 20-30 microns in diameter
• Grow as one to two-cell thick cords formations around central lumen
• • Rounded or often angulated nuclei
Centrally placed round small nuclei
• • Scant cytoplasm
Small nucleoli
• • Nuclear cytoplasmic ratio markedly higher than
Clear cytoplasm
normal hepatocytes and fetal hepatoblastoma
• In addition to other epithelial components, 20–
30 % of HBLs also contain stromal derivatives
including spindle fibroblastic cells, osteoid,
skeletal muscle, and cartilage
• Teratoid means presence of other heterologous
component, such as stratified squamous
epithelium, epithelium containing mucus-
producing cells, neuroglial tissue, melanin-
containing cells
Some points to clarify the significance of Histopathology classification

• Pure fetal HBL with minimal mitotic activity (<2 per HPF) is surgically curable
tumour

• Recent COG study revealed that chemotherapy after complete resection is un-
necessary.

• Fetal HBL which is mitotically active (>2HPF) chemotherapy is needed even if the
tumour is completely resected.

• SCUD HBL may present with morphological and biological features consistent
with malignant rhabdoid tumours which requires different therapy.
USS

CT:
• Using the lowest radiation exposure possible (ALARA principle)
• CT slice acquisition thickness should be 1.5 mm or less.
• Dual phase (arterial and portal venous) abdominal CT is strongly recommended.
• IV enhanced portal venous phase should be performed from just above the diaphragm to the symphysis pubis.

MRI:
• Axial images and coronal images should be acquired with at least two pulse sequences
• Gadolinium should be given if appropriate and if there is normal renal function.
• After contrast injection, images are obtained through the liver during
o The arterial phase (20 to 30 seconds post injection)
o Portal venous phase (60 to 80 seconds after injection)
o At equilibrium (3 to 5 minutes after injection).
o Delayed images can be obtained if needed for further lesion characterization.
Metastatic Site Imaging:
• Chest CT
• Bone scan
Staging
Staging
Treated according to the standard arm of SIOPEL 6 (cisplatin only)

Treated with the dose intensive ‘super PLADO’ from SIOPEL 3

Sorafenib and cisplatin/doxorubicin (PLADO)

Treated according to SIOPEL 4

 Surgical Resection at Diagnosis
• HB, GROUP A; HCC, GROUP E
• segmentectomy or hemi-
hepatectomy
• PRETEXT I or PRETEXT II with >1 cm
radiographic margin on the middle
hepatic vein, the retrohepatic IVC,
or portal bifurcation (negative V,
negative P).
Tumor biopsy only at diagnosis Surgical Resection After Biopsy
• PRETEXT II with less than 1 cm • HB, GROUP B and C
radiographic margin Following 2 cycles of chemo
• PRETEXT III, PRETEXT IV • HB, GROUP D
Following induction chemo
• Any V+/P+, or metastatic disease
• HCC, GROUP F
Following induction chemo
• UESL
• Rhabdoid Tumour
• Angiosarcoma
• Parenchymal RMS
• Hepatic Arterial Chemoembolization (HACE) Trans Arterial Chemoembolization (TACE)

• Pre-Operative Portal Venous Embolization

• SURGICAL MANAGEMENT OF PULMONARY METASTASIS

• LIVER TRANSPLANT AND EXTREME RESECTION GUIDELINES

• SURGEON RESPONSIBILITIES