A brief discussion about cancers in childhood population.

1. CHILDHOOD
CANCER
MARIA, MEDICAL STUDENT, TSMU

2. INTRODUCTION
 Childhood cancers are rare.
 Hematopoietic tumors (leukemia, lymphoma) are the most
common childhood cancers, followed by brain/central nervous
system (CNS) tumors and sarcomas of soft tissue and bone
 There is wide variability in the age-specific incidence of
childhood cancers.
 Embryonal tumors, such as neuroblastoma and retinoblastoma,
peak during the first 2 years of life
 Acute lymphoblastic leukemia peaks during early childhood
(ages 2 to 5 years)
 Osteosarcoma peaks during adolescence
 Hodgkin disease peaks during late adolescence

3. CNS TUMORS

4. INTRODUCTION
 Second most cause of childhood cancer
 In children and adolescents – primary tumors
 In adults – secondary tumors which metastasized from other carcinomas
 First incidence peak is before the age of 10
 Second peak is after the age of 70
 All types of CNS tumors doesn’t have any gender preferences except
medulloblastoma which is more common in male than in females

5. ETIOLOGY
 Not clearly known
 Usually multifactorial
 In most cases, children who previously treated with radiation therapy
are diagnosed.
 Children who are with inherited syndromes are of high risk
 Neurofibromatous type 1 and 2
 Li – Fraumeni syndrome
 Tuberous fibrosis
 Turcot syndrome
 von Hippel Lindau syndrome

6. CLASSIFICATION

7. CLINICAL MANIFESTATIONS
 Lethargy
 Headache
 Vomiting, especially in morning
on awakening
 Irritation
 Anorexia
 Poor school performance
 Loss of developmental
milestones
 Loss of visual acuity
 Inability to abduct the eye
 Focal weakness or sensory
changes
 Seizures
 Neuroendocrine defects
 Galactorrhea with
prolactinoma
 Excessive growth with GH
secretory tumors
 Precocious puberty
 Ataxia
 Diminished co-ordination

8. DIFFERENTIAL DIAGNOSIS
 Malignant tumor
 Benign tumor
 Arteriovenous malformations
 Aneurysm
 Brain abscess
 Cysticerosis
 Granulomatous disease
 Tuberculosis
 Sarcoid
 Intracranial hemorrhage
 Pseudotumor cerebri
 Vasculitis
 Metastastic tumor (rarely)

9. DIAGNOSIS
 MRI
 Intracanial lesion
 Of whole spine to assess neuraxial dissemination
 CSF test
 To assess the presence of metastatic diseases
 Angiography
 Biopsy

10. MANAGEMENT
 High dose of Dexamethasone – to reduce the tumor associated edema
 Surgical excision of pathological tissues
 Combination of radiation and chemotherapy – in children
 Chemotherapy – in infants

11. COMPLICATIONS
 Short term adverse effect of
therapy
 Nausea, vomiting
 Anorexia
 Fatigue
 Immunosuppression
 Cushingoid symptoms
 Long term adverse effect
 Neurocognitive defects
 Decreased bone growth
 Ototoxicity
 Renal insufficiency
 Cataracts
 Infertility
 Cerebellar mutism syndrome
 Somnolence syndrome
 Posterior fossa syndrome

12. PROGNOSIS
 5 year survival rate in 50-60 % cases
 Cerebeller astrocytoma
 Medulloblastoma
 Extremely poor prognosis
 Intrinsic brain stem gliomas
 Glioblastoma multiforme

13. NEUROBLASTOMA

14. INTRODUCTION
 Neuroblastoma is the most common extracranial solid tumor of
childhood
 The most common malignancy in infancy.
 The median age at diagnosis is 20 months
 Is derived from neural crest cells that form the adrenal medulla and
the sympathetic nervous system.
 In some cases, mutations are seen in ALK and PHOX2B genes.
 Sporadic cases also reported.

15. ETIOLOGY
 Unknown
 Results from prenatal and perinatal events
 Rarely it is Heriditary (1-2%)

16. CLINICAL MANIFESTATIONS
 Asymptomatic – in children with localized disease
 Symptomatic – when metastasis begin
 Systemic complaints – fever, weight loss, pain, abdominal mass
 Horner syndrome
 Compression of spinal cord – in paraspinal tumors
 Paraneoplastic syndrome
1. Secretory diarrhoea
2. Profuse sweating
3. Opsomyoclonus

17. …cond
 Metastasize to other organs like liver, bone, skin, bone marrow and
lyphmnodes.
 So when orbital bone is involved it show symptom like periorbital
ecchymosis

18. DIFFERENTIAL DIAGNOSIS
 Wilm’s tumor (if it presents as an abdominal flank mass)
 Child abuse (due to periorbital ecchymoses)
 Anemia
 Thrombocytopenia
 Neutropenia
 Leukemia

19. DIAGNOSIS
 CBC
 Plain X-rays – to assess abdominal calcification
 Urianry catecholamines
 Presence of vanilylmandelic acid and homovanillic acid
 CT Scan of abdomen, pelvis, chest
 Bone scan or meta-iodobenzyl guanidine scan
 Bilateral bone marrow aspiration and biopsies

20. MANAGEMENT
 Complete surgical resection – localised neuroblastoma
 Surgery combined with chemotherapy in advanced cases
 Chemotherapic drugs are
 Vincristine
 Cyclophosphamide
 Doxorubicin
 Cisplatin
 Etoposide
 Radiation therapy – primary tumor bed and areaes of metastatic
disease

21. …cond
 In high risk neuroblastoma - Induction chemotherapy, surgery,
myeloablative chemotherapy with autologous hematopoietic stem cell
transplant, and radiation therapy
 High dose of chemotherapy with autologous stem cell rescue has
improved the outcome.
 Addition of cis-Retinoic acid and anti GD2 monoclonal antibody
therapy in combination with granulocyte-macrophage colony-
stimulating factor (GM-CSF) is given to maintain the pediatric
treatment.

22. COMPLICATIONS
 Developmental delay or mental retardation – in children with
opsomyoclonus syndrome
 Irreversible neurological delay – in cases when Spinal cord are
compressed
 Aggressive chemotherapy and radiation therapy
 Ototoxicity
 Nephrotoxicity
 Growth problems
 Second malignancies

23. PROGNOSIS
1. Low risk – if patient is under age of 1
2. High risk – if
1. Tumor is lack of differentiation
2. Lack of hyperdiploidy
3. Mutation in chromosome 1p , 11q or 6p22 .
4. Older patients with 4S stage
3. High risk patients are more prone to relapses due to drug resistant
residual disease.

24. LYMPHOMA

25. INTRODUCTION
 Lymphomas or malignancies of lymphoid tissues
 Third most common malignancy in childhood

26. CLASSIFICATION
 Two major types of lymphomas
1. Hodgkin lymphoma
2. Non-hodgkin lymphoma – diffuse, highly malignant and show
little differentiation.
I. Burkitt lymphoma – small noncleaved cell or B cell
i. Sporadic form
ii. Endemic form
II. Lymphoblastic cell lymphoma – T cell lymphoma
III. Large cell (B or T cell )

27. ETIOLOGY
 Unknown
 EBV infection plays an important role.
 Mutations – chromosomal translocation
 Endemic form have strong association with EBV

28. EPIDEMIOLOGY
 Peak incidences are
 Adolescent or young adults
 After the age of 50
 Boys are more affected than girls
 In NHL
 White peoples
 Associated with congential or acquired immunodeficiency state

29. CLINICAL MANIFESTATION
Hodgkin lymphoma
 Painless,firm lymphadenopathy
(usually supraclavicular and
cervical nodes )
 Cough and dyspnea – in
mediastinal lympadenopathy
 B symptoms – prognostic
significance
1) Fever (>38degree C) – 3
consecutive days
2) Profuse night sweat
3) Unintentional weight loss
Non-hodgkin lymphoma
 B symptoms
 Sporadic form - abdominal pain
 Endemic form
 Airway or SVC obstruction
 Pleural effusion

30. DIFFERENTIAL DIAGNOSIS
Non-infectious
 Leukemia
 Rhabdomyosarcoma
 Nasopharyngeal carcinoma
 Germ cell tumors
 Thymomas
Infectious
 Infectious mononucleosis
(EBV)
 Branchial cleft and
thyroglossal duct cyst
 Car scratch disease
 Mycobacterial infection
 Toxoplasmosis
 Tinea capitis
 appenditis

31. DIAGNOSIS
 CBC
 ESR
 Serum electrolytes, calcium, phosphorus
 Lactate dehydrogenase
 Uric acid
 Chest X-ray
 Tissue or fluid sampling
 BM aspiration
 Reed-Sternberg cells – Hodgkin disease (hallmark marker)

32. TREATMENT
 Radiation for localised disease
 Widespread disease
 Combined chemotherapy
 Followed by low dose involved
field radiation
 Radiation and chemotherapy
Hodgkin Non-Hodgkin

33. COMPLICATIONS
 Immunocompromised
 infertility
 Renal amyloidosis
 Ischemic heart disease
 Hypothyroidism
 Sub-clinical pulmonary
dysfunction
 Intoxication
 Stroke
 Immunocompromised
 Second malignancies
 Infertility
 Sub clinical pulmonary
dysfunction
 Dental caries
Hodgkin lymphoma Non-Hodgkin lymphoma

34. PROGNOSIS
 Non-Hodgkin lymphoma
 3 year survival rate – 70-90 %
 Hodgkin lymphoma
 5 year survival rate
1. 100% in low risk patients
2. 70-90 % in high risk patients

35. WILM’S TUMOR

36. INTRODUCTION
 Intra-renal
 Arise from primitive, metanephric blastema
 Wilm’s tumor bearing kidney has 20-40% of nephrogenic rests
which are the foci of embryonal cells.

37. ETIOLOGY
 Unknown
 Genetic predisposition
 Risk peoples
 Children with congenital anomalies or genetic conditions
 Children with WAGR syndrome
 Patient with Beck with Wiedemann syndrome
 Patient with over growth syndrome
 Heriditary form – bilateral presentation and occur at young age

38. CLINICAL MANIFESTATIONS
 Abdominal mass, associated with
 Pain
 Fever
 Hypotension
 Hematuria

39. DIFFERENTIAL DIAGNOSIS
 Hydronephrosis
 Polycystic disease of kidney
 Benign tumors
 Mesoblastic nephroma
 Hamartoma
 Malignant tumors
 HCC
 Neuroblastoma
 Lymphoma
 Retro-peritoneal rhabdomyosarcoma

40. DIAGNOSIS
 Abdominal ultrasound
 Abdominal CT Scan
 Evaluation of IVC
 CBC
 Urinalysis
 Liver and renal function tests
 Chest X-ray
 Biopsy of tumor tissue

41. MANAGEMENT

42. COMPLICATIONS
 Late effects
 Scoliosis
 Cardiomyopathy
 Hypertension
 Pre-hypertension
 Renal and bladder insufficiency
 Pulmonary dysfunction
 Hepatic dysfunction
 Infertility
 Second malignancies
 In female survivors – complicated pregnancy and deliveries

43. PROGNOSIS
 Worse prognosis
 in cases of anaplasia
 Loss of heterozygosity
 Good prognosis – classic Wilm’s tumor
 Localised Wilm’s tumor - >85 % cure rate
 With pulmonary metastases – 70-80% survival rate

44. REFERENCES
 https://emedicine.medscape.com/article/987101-overview
 https://emedicine.medscape.com/article/987540-overview
 https://emedicine.medscape.com/article/989398-overview
 https://emedicine.medscape.com/article/988284-overview