1 / 48

Case Conference

Case Conference. Maria Victoria Pertubal , MD PGY-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

nishi
Download Presentation

Case Conference

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Case Conference • Maria Victoria Pertubal , MD • PGY-2 • St Barnabas Hospital - Pediatrics

  2. 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---

  3. July 2012 • Cycle 3 (SIOPEL4) Cisplatin + Doxorubicin • Case reviewed at Tumor Board : Resectable • AFP 189.4 • Pathology : 95% tumor necrosis • AFP 55.5 • August 2012 • Cycle 4 (SIOPEL 4) Cisplatin • Admitted for nadir sepsis

  4. 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

  5. Hepatoblastoma

  6. 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

  7. 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

  8. 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

  9. Primary liver cancers: • Hepatoblastoma • Hepatocellular carcinoma • extrahepatic biliary tree sarcoma • (angiosarcoma, ERMS)

  10. Primary benign liver tumors: • vascular tumors: • hemangioma • hemangioepithelioma • hepatic ademona • focal nodular hyperplasia

  11. Histopathology • Epithelial type • Fetal • Embryonal • Variants : macrotrabecular, • small cell ( anaplastic type ) • Mixed epithelial + mesenchymal type

  12. 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

  13. Clinical S/sx • Systemic symptoms • Physical exam: • Abdomen__________ • skin __________ • Signs of precocious puberty (3%)

  14. Sites of metastasis • Most common site __________ • other less common_______&____

  15. 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_____

  16. Investigation of metastasis • Chest ct • Bone scans only if bone mets are suspected

  17. 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.

  18. 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)

  19. Staging • based on post-surgical findings

  20. PreText Staging

  21. Chemotherapy • Cisplatin, 5- FU, vincristine • Doxorubicin – reserved for unresponsive and recurrent tumors • Cyclophosphamide • irinotecan

  22. Treatment • Complete resection – 40 – 60% long term cure • Pre-op chemo – for large unresectable tumors resectable • Orthotopic liver transplant – for unresectable tumors

  23. Hepatomegaly

  24. True or false:A palpable liver is always hepatomegaly.____

  25. How to assess Liver size: • Liver span: • percussion (upper edge) • palpation (lower edge) • Newborns: 3.5 cm • children : 2cm • auscultation- scratch test

  26. Normal liver span • 1 week new born: 4.5 - 5 cm • 12 year old: 7-8 cm (boys) • 6 to 6.5 cm (girls)

  27. 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)

  28. Riedel lobe

  29. Normal liver span • 1 week new born: 4.5 - 5 cm • 12 year old: 7-8 cm (boys) • 6 to 6.5 cm (girls)

  30. Mechanisms for Hepatomegaly: • inflammation • congestion • excessive storage • infiltration • obstruction

  31. 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

  32. Clinical Evaluation • History: • Non-specific symptoms: • fatigue • anorexia • weight loss • bowel movement changes, color changes, blood in stools • fever • jaundice

  33. Clinical Evaluation • History: • Family history • Inherited disease • travel • food intake • exposure to environmental toxins

  34. Clinical Evaluation • Physical exam: • Liver size • nodularity, firmness • auscultation (bruits, increased flow)

  35. Laboratory: • 2 true Liver Function tests: ____, ____ • PT - prolongation with loss of >80% synthetic capacity • Albumin

  36. 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.

  37. 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.

  38. 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.

  39. 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

  40. 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

More Related