1. د.عبد الرحمن عبد العزيز استشاري الجراحة العامة وجراحة الاطفال
PEDIATRIC ONCOLOGY
د.عبد الرحمن عبد العزيز
استشاري الجراحة العامة وجراحة الاطفال

2. Leukemia is the most common childhood cancer
Brain tumors are second most common
Lymphomas are the third most common
Then solid tumors outside the CNS
Neuroblastoma - neural crest derived
Wilms - renal tumors and syndromes
Bone tumors
Rhabdomyosarcoma - soft tissue sarcomas
Sacrococcygeal teratoma

3. Childhood Cancers Brain Tumors Leukemia Other Lymphoma Retino-
blastoma
Bone tumors
Other
Kidney tumors
Soft tissue sarcomas
Lymphoma
Neuroblastoma

4. Leukemias

5. Definition and General Characteristics
 Uncontrolled proliferation of immature white blood cells with a different immunological
subtype which is lethal within 1–6 months without treatment
 The disorder starts in the bone marrow, where normal blood cells are replaced by
leukemic cells
 Morphological, immunological, cytogenetic, biochemical, and molecular genetic
factors characterize the subtypes with various responses to treatment

6. Leukemia: Signs and Symptoms
Bone marrow infiltration
Anemia
Pallor, lethargy
Dyspnea, murmur
 Platelets
Bleeding, petechiae, purpura
Neutropenia
Fevers and infections
Bone pain
Limp,  walking, irritability

7. Leukemia: Signs and Symptoms
Extramedullary spread
Lymphadenopathy
Hepatosplenomegaly
Orthopnea, cough
mediastinal mass
tracheal compression
Facial nerve palsy
Testicular enlargement
Skin lesions
Gingival hypertrophy
Fever of malignancy

8. CNS Tumors

9. MRI

10. MRI

11. Brain Tumors of Childhood
Histogenesis:
* Cell of origin:
glial, neural, primitive, choroid, mixed
* Location:
 posterior fossa: 70%
 supratentorial: 30%
* Clinical presentation:
location
age
type and grade of the tumor

12. Brain Tumors of Childhood
Infratentorial
70%
esp. < 6 y/o
Supratentorial
30%
esp. > 8 y/o

13. Symptoms may include: Increased intracranial pressure seizures
secondary to obstruction of CSF at aqueduct
hydrocephalus (infants), headache, papilledema, vomiting
seizures
focal neurological deficits
hormonal changes (pituitary adenoma)
visual changes (diplopia, field defects)
pressure on optic chiasm

14. Lymphomas

15. Childhood Lymphomas Hodgkin’s Disease (HD)
Signs and Symptoms depend on:
Lymphoma subtype
Hodgkin’s Disease (HD)
Non Hodgkin’s Lymphoma (NHL)
* Lymphoblastic
* Burkitt’s
* Large Cell lymphoma
Location

16. Presentation of Hodgkin’s Disease
Age: adolescents >> young child
Painless lymphadenopathy
Progresses over weeks  months
Location
Cervical/supraclavicular LNS
unilateral or bilateral
Mediastinal ± hilum
LNs below diaphragm and spleen
Liver, lung, bone marrow
95%

17. Presentation of Hodgkin’s Disease
Systemic symptoms
Fevers
Night sweats
Weight loss
Pruritus
“B” symptoms
25%
Superior Mediastinal Syndrome (SMS)
Orthopnea, SOB, strider, hypoxia
Tracheal
Bronchial
Cardiac
compression
= Oncologic Emergency

18. Presentation of Non Hodgkin's lymphoma
Lymphoblastic lymphoma

19. Burkitt’s Lymphoma = Oncologic Emergency B-cell origin > 5 y/o
Abdominal mass
Large mass + LNs
Terminal ileum, Cecum or appendix
Jaw
Tumor lysis syndrome
Uric acid, phosphorus, creatinine
Treatment can precipitate renal failure
= Oncologic Emergency

20. Other Abdominal Tumors

21. Malignant Abdominal Masses
Most common:
Burkitt’s lymphoma
Neuroblastoma
Wilms Tumor
Other:
Hepatoblastoma
Rhabdomyosarcoma
pelvic
Ovarian germ cell tumors

22. Neuroblastoma Cervical, Thoracic, Pelvic Age
90% < 5 y/o; 50% < 2 y/o
Occasional ultrasonography detection in utero
Location: any neural crest tissue
Adrenal
Paraspinal sympathetic tissue
Cervical, Thoracic, Pelvic
Often metastatic at diagnosis
Bone and/or bone marrow

23. Neuroblastoma: Signs and Symptoms
Abdominal mass
Often crosses midline
Lower extremity weakness
Spinal cord compression
Thoracic
abdominal
Cervical, high thoracic mass
Horner’s syndrome
Miosis, ptosis, anhydrosis

24. Neuroblastoma: Signs and Symptoms
Signs of metastatic disease
Irritability
Bone pain
Fever
Proptosis
Bone lesions
Periorbital
ecchymoses

25. Periorbital Ecchymoses of Neuroblastoma
1 month into
therapy
13 months old
at diagnosis

26. Neuroblastoma: Signs and Symptoms
Paraneoplastic syndromes
Watery diarrhea – Vasoactive Intestinal Peptide
 Urinary catecholamines
VMA – 85%
 BP – 25%
Renal compression
Catecholamine secretion

27. Wilms tumor: Signs and Symptoms
Abdominal mass
Often asymptomatic
Healthy appearing
Encapsulated
mass
2 days
before dx

28. Wilms tumor: Signs and Symptoms
­ BP – 25%
Mass enlarges toward pelvis

29. Signs and Symptoms of Wilms tumor
Associated anomalies, syndromes – 15%
WAGR syndrome
Wilms, aniridia, ambiguous genitalia, mental retardation
due to 11p13 deletion (WT-1)
Aniridia

30. Bone tumors

31. Bone Tumors in Childhood
Age – Adolescents > younger children
Signs and symptoms
Bone pain,  palpable mass,   motion
Often hx of sports injury (coincidental)
Ewing Sarcoma
All bones:
Long: diaphyses
Flat
Pelvis
Skull
Ribs
Osteogenic Sarcoma
Metaphyses of long bones:
Distal femur
Proximal tibia
Proximal humerus
Pelvis

32. Presentation of Bone Tumors
Plain X-Rays are usually abnormal
Classic X-ray of Ewing:
Moth-eaten
lytic lesion
Classic X-ray of O.S.:
“Sunburst pattern”
Periosteal reaction
Soft tissue mass + calcium

33. Presentation of Bone Tumors
Further radiographic evaluation may help with differential diagnosis of bone pain
Bone scan
MRI
Chest CT scan
Metastases 20%

34. Soft tissue sarcomas

35. Presentation of Soft Tissue Sarcomas
Rhabdomyosarcoma – most common
Age
Birth to > 20 y/o
70% < 10 y/o
Sites
Head and neck – 40%
Genitourinary – 20%
Extremities – 20%
Trunk – 10%
Retroperitoneal – 10%
Signs and symptoms
depend on
age and site

36. Head and neck Rhabdomyosarcomas: Signs and Symptoms Orbit
Proptosis
Periorbital swelling
Parameningeal
Cranial nerve palsies
Hearing loss
Chronic aural or sinus drainage

37. Rhabdomyosarcomas: Signs and Symptoms
Botryoid:
grape-like
Genitourinary
Bladder and prostate
Hematuria
Urinary obstruction
Paratesticular
Painless mass -  testicle
Vagina and uterus
Abdominal mass
Vaginal mass
Vaginal bleeding or discharge

38. Rhabdomyosarcoma – other sites
Can grow up at any site and any age
6 week old
Newborn

39. Sacrococcygeal teratoma
Its a rare tumor, occurring in approximately 1 in 40,000 live births. They arise from the caudal end of the spine, usually protruding
from the inferior end of the infant’s spinal column and displacing the anus forwards. They are much more common in girls, with the female to male ratio being at least 3:1. It is generally agreed that sacrococcygeal teratoma is the result of continued multiplication of totipotent cells from Hensen’s node, which fail to involute at the end of embryonic life.