Pediatric malignant solid tumors christosova,brankov

Multiprofile Hospital TOKUDA Sofia
Multiprofile Hospital TOKUDA SofiaConsultant at Multiprofile Hospital "Tokuda" Sofia um Multiprofile Hospital TOKUDA Sofia
PEDIATRIC MALIGNANT SOLID
TUMORS
Iskra Christosova,
Ognyan Brankov
Pediatric Oncohaematology
Pediatric Surgery
Sofia BULGARIA
Pediatric Cancer
 2 nd leading cause of death in children
 1/350 children diagnosed annually
 or the incidence per year would be 15-16
cases / 100 000 children per year/
 11 000 new cases in children under 20 years
of age each year in the whole world.
 Considered in the past as hopeless diseases
now 70% of children with cancer can be
cured definitively
Cancerogenesis
I. Exogenous Factors
 Radiation Exposure.
 Other Factors of the Surrounding. Enviroment -
Chemical Cancerogens.
 Oncogenic Viruses.
II. Endogenous Factors
 Familial and Genetic Factor
 Cancer Malformation Syndromes
 Multiple Primary Tumors
 Second Malignant Neoplasms
Charactreristic Features of
Childhood Cancer
 Child’s Organism is with forming Immune
System and with Rapid Growth.
 Different Histological Types from those of
Adults.
 Different Localizations from those of Adults.
 Higher Sensitiveness to Chemotherapy than
Adults.
Clasification of Pediatric Malignancies
Systemic Neoplasms (Leukaemias and Lymphomas) : Solid Tumors = 1:1.
Systemic Neoplasms - 50%
 Leukaemias - 1/3 of Pediatric neoplasms - 35%
 Lymphomas (NHL 55% and Hodgkin’s Disease
45%) - 15%
Malignant Solid Tumors - 50%
 Embrional Tumors - 17% (Neurollastoma - 8%,
Nephroblastoma - 7%, Retinollastoma - 1.5%,
Hepatoblastoma - 0.5%)
 Brain Tumors - 17%
(Astrocytoma,Medulloblastoma....)
 Soft Tissue Sarcomas 8%
 (Rhabdomyosarcoma - 6%...)
 Bone Tumors - 4% (Sarcoma,
 Osteogenes, Sarcoma Ewing...)
 Germ Cell Tumors - 2%
 Epithelial Tumors - Carcinomas and
 other very Rare Tumors - 2%
Malignant Solid Tumors - 50%
Wilms” Tumors
(Nephroblastoma)
 Epidemiology - 7% of Neoplastic Diseases in
Children
 Unfavonrable Histology (Focal or Diffuse
Anaplasia) - 10%
 Favourable Histology (Multicystic and with
Fibroadenomatous Structures) - 90%
 Gene Mutations - 11p 13 for WT1 and 11p 15
for WT2
 Caner Malformation Syndromes
Incidence
0,0
0,5
1,0
1,5
2,0
2,5
<1 1 2 3 4 5 6 7 8 9 10 11 12 13 14
Girls Boys
Incidence rates per 100,000
Age
Wilms Tumor:
Histology: mixture of immature
cells metanephric, stromal,
tubular
Cajaiba MM et al. (2006) Rhabdomyosarcoma, Wilms tumor, and deletion of the patched gene in Gorlin
syndrome Nat Clin Pract Oncol 3: 575–580 doi:10.1038/ncponc0608
Clinical Manifestation
 Good Clinical State
 Haematuria - 25%
 Abdominal pain - 35%
 High blood pressure - 35%
 An abdominal Tumor mass - discovered
accidentally
Clinical stages
 I. Tumor limited to the Kidney, in size - 5 cm. Intact Renal Capsule,
Excised Completly.
 II. Tumor extends outside the Kidney in size - 10 cm, Excised
Completly.
 III. Tumor over 10 cm in size. Infiltrated other Organs in Ablomen,
without Hematogenous Mts, Complete Excision Impossible.
 IV. Tumor with Hematogenous Mts (Lungs - 10% liver - 1% ets.)
 V. Bilateral Renal Tumors
Pediatric malignant solid tumors christosova,brankov
Laboratory and RadiologicalExaminations
 Hb, Plt, WBS,
 LDH,
 UrineanalysisCT and Abdominal UltrasoundChest X-
ray
 Tumor Histology
Pediatric malignant solid tumors christosova,brankov
Risk Factors
 Low Risk - Cases withl Favourable Histology,
in I and II Stages, under 3 Year of Age
 High Risk - Cases with Unfavourable
Histology, in III, IV and V Stages, over 3 Year
of Age
Treatment
 Surgery - Nephrectomy, Lymphadenotomy,
Excision when it is possibly of lung or liver
Mts. In V stade -partial resection.
 Radiotherapy. No RT in I and II stage. In III
and IV stage RT in Tumor Region with Dose
20-30 GY. In Mts regions - 15 GY
Chemotherapy
 L. R. Actinomycin D, Vincristine
 H. R. Act D, Vcr, Farmarubicin, VP16
Prognosis
 Low Risk - 85% survival
 High Risk - 40% survival
Neuroblastoma
 Epidemiology - 8% of Neoplastic Diseases in children
 Unfavourable Histology (Neuroblastoma) - 90%
 Favourable Histology (Ganglioneuroblastoma) - 10%
Neuroblastoma Localization -
 70% Abdomen (1/2 of Cases from suprarenal gl.)
 15% Mediastinum,
 3% Neck,
 8% Paravertebral Region,
 4% Other Rare Regions (Olfactory Region, Multiple
Primary Tumors C.N.S ets)
Pediatric malignant solid tumors christosova,brankov
Neuroblastoma
 “Small blue round cell” tumor
 Immunohistochemical stains:
neurofilament proteins, synaptophysin,
NSE
 Electron microscopy: neurosecretory
granules, microtubules and filaments
 Chromosome 1 deletions or N-myc
oncogene amplification
From, Principles and Practice of Pediatric Oncology, Lippincott Williams & Wilkins,
p 903.
Pediatric malignant solid tumors christosova,brankov
0
2
4
6
8
10
<1 1 2 3 4 5 6 7 8 9 10 11 12 13 14
Girls Boys
Incidence rates per 100,000
Age
Incidence
Clinical Manifestation
 Poor clinical State
 Symptoms of Primary Localization
 Symptoms of Metastatic Localization
 Paraneoplastic Symptoms
There are orbital and skull
vault metastases,
with associated enhancing
soft-tissue masses.
The skull lesions are
extradural masses which
deform the underlying brain.
The right orbital lesion
forms a superior extraconal
mass,
depressing the right globe.
bilateral ecchymosis in a child with metastatic neuroblastoma.
Clinical Stages (Evans et al.)
 I Tumor Limited to the Organ or Structure of Origin. Excised
Comletely.
 II Tumor with Regional Spread, not Crossing the Midline.
 III Tumor Crossing the Midline, Bilateral Lymph Nodes May by
involved. Complete Excision imposible.
 IV Tumor with Distant Mts (Bone - 50% of cases, Lymph
Nodes, Organs, Soft Tissues)
 IV-S Tumor in I and II Cl Stage, with Limited Dissemination to
Liver, Skin and Bone Marrow (without Bones), Infants under 2
Years of Age, especially under 1 Year of Age.
Pediatric malignant solid tumors christosova,brankov
Laboratory and Radiological
Examinations
 Hb, Plt, WBC, LDH,
 Bone Marrow Aspiration
 Urinanalysis
 CT and Abdominal Ultrasound
 Bone Isotope Scanning, Scanning with 131I-MIBG
 Chest X-ray
 Tumor Markers - N-myc, Ferritin, NSE, Cantecholamines’
Metabolites
 Tumor Histology
Risk Factors
 Low Risk - Cases with
Ganglioneuroblastoma, in I, II and IV-S
Stages, under 1 Year of age, with Neck and
Mediastinum Localisation
 High Risk - Cases with Neuroblastoma, in III
and IV Stages (Bone Mts), over 2 Years of
age, with High Levels of LDH, NSE and
Fevritin, with Abdominal and Paravertebral
Localisation, with Amplification of N-myc.
Treatment
 Surgery - Survival is better when Radical
Excision is done.
 Radiotherapy - No RT in I, II and IV-S Stages
 In III and IV Stages RT in Tumor and Mts
Redions with Dose 15-35 GY.
Chemotherapy
 L. R. - Vcr, Endoxan, Farmarubicin
 H. R. - Vcr, Endoxan, VP16, Cisplatin,
Carboplatin, Holoxan, Farmarubicin
Prognosis
 Low Risk - 80% survival
 High Risk - 35% survival
Rhabdomyosarcoma
 Epidemiology - 5% of NeoplasticDiseases in
Children
 Histology - Embrional and Botroid - 75%;
Alveolar + Pleomorphic - 20%;
Undifferentiated - 5%.
 Mts - Lungs, Bones, Lymph Nodes, Brain.
Incidence
0,0
0,3
0,6
0,9
1,2
1,5
<1 1 2 3 4 5 6 7 8 9 10 11 12 13 14
Girls Boys
Incidence rates per 100,000
Age
Rhabdomyosarcoma Localization
 Head and Neck - 40%;
 Pelvis + Urinary Tract - 25%;
 Limbs - 20%;
 Other Rare Localizations - 15%; (Diapharagm
Thorax and Abdominal walls, Viscera and every
Region originated from Mesenchyme arising in
Striated Muscle.)
Rhabdomyosarcoma
 Clinical Manifestation depends from Primary
Localisation.
Nasopharyngeal Rhabdomyosarcoma
Pediatric malignant solid tumors christosova,brankov
Rhabdomyosarcoma
 Alveolar
– 20% of pediatric cases
– Chromosomal translocation:
t(2;13) or t(1;13)
– Gene amplification
– Tetraploid DNA
From, Surgical Pathology of the Head and Neck, Lippincott Williams & Wilkins,
p 157.
Rhabdomyosarcoma
 Botryoid
– 5-10% of pediatric cases
– Grape-like tumor masses
 Pleomorphic
– Rare in children
From, Diagnostic Surgical Pathology of the Head and Neck,
W.B.Saunders, p 554.
Clinical Stage
 I Limited Tumor Excised Comletely.
 II Grossly Removed Tumor with microscopic
residual disease.
 III Incomplete Removal or only Biopsy with
Gross Residual Tumor.
 IV Metastatic Disease at Diagnosis.
Laboratory and Radiological
Examinations
 Hb, Plt, WBC, LDH
 CT and MRI of Primary Tumor
 Chest X-ray and CT
 Bone Scan
 Tumor Histology
Risk Factors
 Low Risk - I and II stages - 70% survival
 High Risk - III and IV stages, Parameningial
Localization, Alveolar Histology - 30%
survival
Treatment
 Surgery
 Radiotherapy
 Chemotherapy - Vcr, Holoxan
 Farmarubicin, VP16, Endoxan
 Carloplatin, Actinomycin D.
Pediatric malignant solid tumors christosova,brankov
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Pediatric malignant solid tumors christosova,brankov

  • 1. PEDIATRIC MALIGNANT SOLID TUMORS Iskra Christosova, Ognyan Brankov Pediatric Oncohaematology Pediatric Surgery Sofia BULGARIA
  • 2. Pediatric Cancer  2 nd leading cause of death in children  1/350 children diagnosed annually  or the incidence per year would be 15-16 cases / 100 000 children per year/  11 000 new cases in children under 20 years of age each year in the whole world.  Considered in the past as hopeless diseases now 70% of children with cancer can be cured definitively
  • 3. Cancerogenesis I. Exogenous Factors  Radiation Exposure.  Other Factors of the Surrounding. Enviroment - Chemical Cancerogens.  Oncogenic Viruses.
  • 4. II. Endogenous Factors  Familial and Genetic Factor  Cancer Malformation Syndromes  Multiple Primary Tumors  Second Malignant Neoplasms
  • 5. Charactreristic Features of Childhood Cancer  Child’s Organism is with forming Immune System and with Rapid Growth.  Different Histological Types from those of Adults.  Different Localizations from those of Adults.  Higher Sensitiveness to Chemotherapy than Adults.
  • 6. Clasification of Pediatric Malignancies Systemic Neoplasms (Leukaemias and Lymphomas) : Solid Tumors = 1:1.
  • 7. Systemic Neoplasms - 50%  Leukaemias - 1/3 of Pediatric neoplasms - 35%  Lymphomas (NHL 55% and Hodgkin’s Disease 45%) - 15%
  • 8. Malignant Solid Tumors - 50%  Embrional Tumors - 17% (Neurollastoma - 8%, Nephroblastoma - 7%, Retinollastoma - 1.5%, Hepatoblastoma - 0.5%)  Brain Tumors - 17% (Astrocytoma,Medulloblastoma....)
  • 9.  Soft Tissue Sarcomas 8%  (Rhabdomyosarcoma - 6%...)  Bone Tumors - 4% (Sarcoma,  Osteogenes, Sarcoma Ewing...)  Germ Cell Tumors - 2%  Epithelial Tumors - Carcinomas and  other very Rare Tumors - 2% Malignant Solid Tumors - 50%
  • 10. Wilms” Tumors (Nephroblastoma)  Epidemiology - 7% of Neoplastic Diseases in Children  Unfavonrable Histology (Focal or Diffuse Anaplasia) - 10%  Favourable Histology (Multicystic and with Fibroadenomatous Structures) - 90%  Gene Mutations - 11p 13 for WT1 and 11p 15 for WT2  Caner Malformation Syndromes
  • 11. Incidence 0,0 0,5 1,0 1,5 2,0 2,5 <1 1 2 3 4 5 6 7 8 9 10 11 12 13 14 Girls Boys Incidence rates per 100,000 Age
  • 12. Wilms Tumor: Histology: mixture of immature cells metanephric, stromal, tubular Cajaiba MM et al. (2006) Rhabdomyosarcoma, Wilms tumor, and deletion of the patched gene in Gorlin syndrome Nat Clin Pract Oncol 3: 575–580 doi:10.1038/ncponc0608
  • 13. Clinical Manifestation  Good Clinical State  Haematuria - 25%  Abdominal pain - 35%  High blood pressure - 35%  An abdominal Tumor mass - discovered accidentally
  • 14. Clinical stages  I. Tumor limited to the Kidney, in size - 5 cm. Intact Renal Capsule, Excised Completly.  II. Tumor extends outside the Kidney in size - 10 cm, Excised Completly.  III. Tumor over 10 cm in size. Infiltrated other Organs in Ablomen, without Hematogenous Mts, Complete Excision Impossible.  IV. Tumor with Hematogenous Mts (Lungs - 10% liver - 1% ets.)  V. Bilateral Renal Tumors
  • 16. Laboratory and RadiologicalExaminations  Hb, Plt, WBS,  LDH,  UrineanalysisCT and Abdominal UltrasoundChest X- ray  Tumor Histology
  • 18. Risk Factors  Low Risk - Cases withl Favourable Histology, in I and II Stages, under 3 Year of Age  High Risk - Cases with Unfavourable Histology, in III, IV and V Stages, over 3 Year of Age
  • 19. Treatment  Surgery - Nephrectomy, Lymphadenotomy, Excision when it is possibly of lung or liver Mts. In V stade -partial resection.  Radiotherapy. No RT in I and II stage. In III and IV stage RT in Tumor Region with Dose 20-30 GY. In Mts regions - 15 GY
  • 20. Chemotherapy  L. R. Actinomycin D, Vincristine  H. R. Act D, Vcr, Farmarubicin, VP16
  • 21. Prognosis  Low Risk - 85% survival  High Risk - 40% survival
  • 22. Neuroblastoma  Epidemiology - 8% of Neoplastic Diseases in children  Unfavourable Histology (Neuroblastoma) - 90%  Favourable Histology (Ganglioneuroblastoma) - 10%
  • 23. Neuroblastoma Localization -  70% Abdomen (1/2 of Cases from suprarenal gl.)  15% Mediastinum,  3% Neck,  8% Paravertebral Region,  4% Other Rare Regions (Olfactory Region, Multiple Primary Tumors C.N.S ets)
  • 25. Neuroblastoma  “Small blue round cell” tumor  Immunohistochemical stains: neurofilament proteins, synaptophysin, NSE  Electron microscopy: neurosecretory granules, microtubules and filaments  Chromosome 1 deletions or N-myc oncogene amplification From, Principles and Practice of Pediatric Oncology, Lippincott Williams & Wilkins, p 903.
  • 27. 0 2 4 6 8 10 <1 1 2 3 4 5 6 7 8 9 10 11 12 13 14 Girls Boys Incidence rates per 100,000 Age Incidence
  • 28. Clinical Manifestation  Poor clinical State  Symptoms of Primary Localization  Symptoms of Metastatic Localization  Paraneoplastic Symptoms
  • 29. There are orbital and skull vault metastases, with associated enhancing soft-tissue masses. The skull lesions are extradural masses which deform the underlying brain. The right orbital lesion forms a superior extraconal mass, depressing the right globe.
  • 30. bilateral ecchymosis in a child with metastatic neuroblastoma.
  • 31. Clinical Stages (Evans et al.)  I Tumor Limited to the Organ or Structure of Origin. Excised Comletely.  II Tumor with Regional Spread, not Crossing the Midline.  III Tumor Crossing the Midline, Bilateral Lymph Nodes May by involved. Complete Excision imposible.  IV Tumor with Distant Mts (Bone - 50% of cases, Lymph Nodes, Organs, Soft Tissues)  IV-S Tumor in I and II Cl Stage, with Limited Dissemination to Liver, Skin and Bone Marrow (without Bones), Infants under 2 Years of Age, especially under 1 Year of Age.
  • 33. Laboratory and Radiological Examinations  Hb, Plt, WBC, LDH,  Bone Marrow Aspiration  Urinanalysis  CT and Abdominal Ultrasound  Bone Isotope Scanning, Scanning with 131I-MIBG  Chest X-ray  Tumor Markers - N-myc, Ferritin, NSE, Cantecholamines’ Metabolites  Tumor Histology
  • 34. Risk Factors  Low Risk - Cases with Ganglioneuroblastoma, in I, II and IV-S Stages, under 1 Year of age, with Neck and Mediastinum Localisation  High Risk - Cases with Neuroblastoma, in III and IV Stages (Bone Mts), over 2 Years of age, with High Levels of LDH, NSE and Fevritin, with Abdominal and Paravertebral Localisation, with Amplification of N-myc.
  • 35. Treatment  Surgery - Survival is better when Radical Excision is done.  Radiotherapy - No RT in I, II and IV-S Stages  In III and IV Stages RT in Tumor and Mts Redions with Dose 15-35 GY.
  • 36. Chemotherapy  L. R. - Vcr, Endoxan, Farmarubicin  H. R. - Vcr, Endoxan, VP16, Cisplatin, Carboplatin, Holoxan, Farmarubicin
  • 37. Prognosis  Low Risk - 80% survival  High Risk - 35% survival
  • 38. Rhabdomyosarcoma  Epidemiology - 5% of NeoplasticDiseases in Children  Histology - Embrional and Botroid - 75%; Alveolar + Pleomorphic - 20%; Undifferentiated - 5%.  Mts - Lungs, Bones, Lymph Nodes, Brain.
  • 39. Incidence 0,0 0,3 0,6 0,9 1,2 1,5 <1 1 2 3 4 5 6 7 8 9 10 11 12 13 14 Girls Boys Incidence rates per 100,000 Age
  • 40. Rhabdomyosarcoma Localization  Head and Neck - 40%;  Pelvis + Urinary Tract - 25%;  Limbs - 20%;  Other Rare Localizations - 15%; (Diapharagm Thorax and Abdominal walls, Viscera and every Region originated from Mesenchyme arising in Striated Muscle.)
  • 41. Rhabdomyosarcoma  Clinical Manifestation depends from Primary Localisation.
  • 44. Rhabdomyosarcoma  Alveolar – 20% of pediatric cases – Chromosomal translocation: t(2;13) or t(1;13) – Gene amplification – Tetraploid DNA From, Surgical Pathology of the Head and Neck, Lippincott Williams & Wilkins, p 157.
  • 45. Rhabdomyosarcoma  Botryoid – 5-10% of pediatric cases – Grape-like tumor masses  Pleomorphic – Rare in children From, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 554.
  • 46. Clinical Stage  I Limited Tumor Excised Comletely.  II Grossly Removed Tumor with microscopic residual disease.  III Incomplete Removal or only Biopsy with Gross Residual Tumor.  IV Metastatic Disease at Diagnosis.
  • 47. Laboratory and Radiological Examinations  Hb, Plt, WBC, LDH  CT and MRI of Primary Tumor  Chest X-ray and CT  Bone Scan  Tumor Histology
  • 48. Risk Factors  Low Risk - I and II stages - 70% survival  High Risk - III and IV stages, Parameningial Localization, Alveolar Histology - 30% survival
  • 49. Treatment  Surgery  Radiotherapy  Chemotherapy - Vcr, Holoxan  Farmarubicin, VP16, Endoxan  Carloplatin, Actinomycin D.