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Case Conference
Maria Victoria Pertubal , MDPGY-2
St Barnabas Hospital - Pediatrics
TS 23 month old girl • --In Israel--
• March 2012
• Noted with decreased activity and seemed less happy, refused to walk
• ER: + anemia, US: + liver mass
• Transferred to Children’s Hospital: + high AFP (~ 600,000)
• CT scan : + tumor 2 lobes of liver, + pulmonary nodules
• April 2012
• Liver biopsy : + consistent with small cell hepatoblastoma
• SIOPEL 4 Cycle 1: Cisplatin + Doxorubicin
• ---flew to NYC---
July 2012Cycle 3 (SIOPEL4) Cisplatin + Doxorubicin
• Case reviewed at Tumor Board : ResectableAFP 189.4
Pathology : 95% tumor necrosisAFP 55.5
August 2012Cycle 4 (SIOPEL 4) Cisplatin
Admitted for nadir sepsis
• In NYC
• May 2012
• Cycle 2 delayed due to nadir sepsis
• MSKCC, confirmed the diagnosis of hepatoblastoma, epithelial type with predominant embryonal component.
• AFP 39,709.9 Cycle 2 (SIOPEL4) Cisplatin + Doxorubicin
• Admitted for nadir sepsis
• June 2012
• CT scan : regression of large pulmonary nodule
• MRI of liver : decreased size of liver tumors
• Surgical eval: unresectable needs liver transplant
• AFP 783.5 Cycle 3 (SIOPEL 4) Cisplatin + Doxorubicin
Hepatoblastoma
Epidemiology
Primary malignant tumors of the liver in pediatric population are _____ in the pediatric age group
Median age of diagnosis is_____
Males to female preponderance is ______
associated with Extremely LBW
Tumor biology
Hepatoblastoma has strong associations with which syndromes? (____ _____)
APC gene mutation is associated with _________
______syndrome associated with loss of heterozygosity IFG-2 gene at chromososme 11 p 15
Pathology
Hepatoblastoma represents _____ % of childhood liver cancers
the remaining ____% is __________
Other Primary malignant tumors of the liver are :
Benign tumors of the liver are:
Commonly arises from _____lobe of liver
Primary liver cancers:
Hepatoblastoma
Hepatocellular carcinoma
extrahepatic biliary tree sarcoma• (angiosarcoma, ERMS)
Primary benign liver tumors:
vascular tumors:• hemangioma• hemangioepithelioma
• hepatic ademona• focal nodular hyperplasia
Histopathology
• Epithelial type• Fetal• Embryonal• Variants : macrotrabecular,
• small cell ( anaplastic type )
• Mixed epithelial + mesenchymal type
Prognosis • Significance by histology is still
unresolved• Complete resection of tumor
( purely fetal type ) + low mitotic activity = Excelent prognosis
• Small cell- anaplastic type, poor prognosis
• Often misdiagnosed due to low AFP levels
Clinical S/sx
• Systemic symptoms• Physical exam:• Abdomen__________• skin __________• Signs of precocious puberty
(3%)
Sites of metastasis
• Most common site __________• other less common_______&____
Imaging and Laboratory
• First line modality for any child presenting with abdominal mass___
• assess the extent of involvement and resectability of tumor ________
• to define vascular involvement_____
Investigation of metastasis
• Chest ct• Bone scans only if bone mets
are suspected
Blood tests• CBC • LFT• AFP - often increased in 80- 90%,
except for the _______ type• - used to monitor residual
disease or recurrence• * AFP levels are eleveated in
infancy, and will start to decline after 1 yr of age.
Management• 2 approaches• COG – Children’s Oncology
Group• SIOPEL - Société Internationale
d’Oncologie Pédiatrique – Epithelial Liver Tumor Study Group.• International Society Of
Pediatric Oncology Group - (European based grp)
Staging
• • based on post-surgical findings
PreText Staging
Chemotherapy
• Cisplatin, 5- FU, vincristine• Doxorubicin – reserved for
unresponsive and recurrent tumors
• Cyclophosphamide• irinotecan
Treatment
• Complete resection – 40 – 60% long term cure
• Pre-op chemo – for large unresectable tumors resectable
• Orthotopic liver transplant – for unresectable tumors
Hepatomegaly
True or false:A palpable liver is always hepatomegaly.____
How to assess Liver size:
Liver span: • percussion (upper edge)• palpation (lower edge)
• Newborns: 3.5 cm• children : 2cm
• auscultation- scratch test
Normal liver span
1 week new born: 4.5 - 5 cm
12 year old: 7-8 cm (boys) • 6 to 6.5 cm (girls)
A palpable liver is NOT always hepatomegaly
Conditions that can displace the liver inferiorly:• fluid or air in the thorax• retroperitoneal mass
(choledochal cyst, abscess)• narow chest walls - pectus
excavatum• normal variant of R lobe of
liver (Riedel lobe)
Riedel lobe
Normal liver span
1 week new born: 4.5 - 5 cm
12 year old: 7-8 cm (boys) • 6 to 6.5 cm (girls)
Mechanisms for Hepatomegaly:
• inflammation • congestion• excessive storage• infiltration• obstruction
Clinical Evaluation
• History: • Birth
• perinatal infections
• maternal infections, h/o IV drug abuse
• Rh/ABO incompatibility
• Newborn
• hyperbilirubinema, NBS
• umbilical catherterization (risk of hepatic abscesses
Clinical Evaluation
• History: • Non-specific symptoms:
• fatigue
• anorexia
• weight loss
• bowel movement changes, color changes, blood in stools
• fever
• jaundice
Clinical Evaluation
• History: • Family history
• Inherited disease
• travel
• food intake
• exposure to environmental toxins
Clinical Evaluation• Physical exam:
• Liver size
• nodularity, firmness
• auscultation (bruits, increased flow)
Laboratory:
• 2 true Liver Function tests: ____, ____• PT - prolongation with loss of >80% synthetic capacity
• Albumin
Question 176
A mother brings in her 5-week-old infant girl because of feeding difficulties. The baby
weighed 3,300 g when born at term, and she has breastfed exclusively. Approximately 2 weeks ago, the parents noted that the baby became increasingly irritable, particularly
during feedings, and she began spitting-up 4 to 6 times per day.
Physical examination demonstrates a well-developed, alert but irritable infant whose
weight is 3.85 kg, heart rate is 180 beats/min, and respiratory rate is 70 breaths/min. Lung
sounds are clear. On physical examination, you note a hyperdynamic precordium and a grade
2/6 holosystolic cardiac murmur. Chest auscultation yields normal results. You palpate a firm liver edge 5.0 cm below the right costal
margin. The spleen is not palpable.
You also note a 2x2-cm hemangioma on the abdominal wall.
Results of laboratory tests include:•Hemoglobin, 9.8 g/dL (9.8 g/L)•White blood cell count, 4.8x103/mcL (4.8x109/L)•Platelet count, 80x103/mcL (80x109/L)•Peripheral blood smear, Burr cells and schistocytes noted•Electrolytes, normal•Bilirubin, 1.6 mg/dL (27.4 mcmol/L)
Chest radiography demonstrates mild cardiomegaly.
Of the following, the study that is MOST likely to demonstrate the cause of this infant’s symptoms is
A.abdominal ultrasonography
B.acid alpha-glucosidase assay
C.bone marrow aspiration
D.Coombs test
E.echocardiography
References:
• Wolf , A, Lavine Hepatomegaly in Neonates and Children
• Pediatrics in review Vol 21 No 9. Sept 2000, pp 303-310
• Abeloff: Abeloff's Clinical Oncology, 4th ed. Chapter 99:Pediatric solid tumors
• PREP 2012