pediatric tumors

2.  Important cause of death in young children
 Malignancies Need to distinguish from benign lesions
 Heterotopia / Choristoma
 Hamartoma
 Early diagnosis is the aim
 Treatment is better tolerated
NEOPLASTIC LESIONS:

3.  Hemangioma
 Lymphangioma
 Sacrococcygeal Teratoma
BENIGN TUMORS

4.  Most common Benign Tumor of Childhood
1. Capillary
2. Cavernous
 ? Malformations.. ? Hamartoma
 Majority are superficial lesions
 Malignant Transformation is very Rare
Hemangioma

5.  Site - Skin , Subcutaneous tissue , Mucosa of oral
cavity / lips
 Morphology:: Bright red - Blue , Few mm - few cms.
 Strawberry Hemangioma Present since Birth.
Capillary Hemangioma

7. Capillary Hemangioma

8.  Site - Skin , Mucosa , Viscera (Liver)
 Morphology  Red-Blue , compressible , Spongy , well
defined lesions , 2 - 3 cms
 Cavernous Lymphangioma - Cystic Hygroma
Cavernous Hemangioma

9. Cavernous Hemangioma: spleen

10. Cavernous Hemangioma

11. Lymphangioma

12. Lymphangioma

13.  Contains the tissues derived from all ectoderm,
endoderm and mesoderm.
 Skin is most commonly seen: hence the name dermoid
cyst.
 Any tissues can be seen: cartilage, bone, tooth, hair,
sebaceous glands, brain tissues…..etc.
 Most commonly seen in the midline structures.
Congenital Teratoma:

15. Teratoma

16. MALIGNANT TUMORS
0 TO 4 YRS 5 TO 9 YRS 10 TO 14 YRS
Leukemia Leukemia HCC
Retinoblastoma Retinoblastoma Soft tissue
sarcoma
Neuroblastoma Neuroblastoma Osteogenic
sarcoma
Wilm’s tumor HCC Thyroid carcinoma
Hepatoblastoma Soft tissue
sarcoma
HD
CNS tumors CNS, ES, NHL

17.  Histologically malignant pediatric tumors are alike
 Primitive embryonal morphology
 Sheets of small cells, scant cytoplasm, dense round nucleus
 SBRCT
 Neuroblastoma, Lymphoma, RMS, ES, Wilm’s tumor

19.  Most common solid tumor of childhood next to CNS
tumors
 Most occur below 5 yrs of age
 Tendency to regress spontaneously
 Head to toe  Sympathetic chain
 75% arise within abdomen Adrenals
 Can arise in brain, Head & neck

20.  Range in size
 Advanced tumors may invade the renal vein
 Grey-White, Soft, Friable, Large areas of hemorrhage,
Cystic, Calcification
 Tendency to metastasize early and invade adjacent
structures
MORPHOLOGY- NEUROBLASTOMA

21.  SBRCT
 Some areas of differentiation seen
 Homer-Wright pseudorosettes
 Some ganglion like cells  Maturation
 Ganglioneuroblastoma
 Ganglioneuroma  Better differentiated
 Presence of Schwann cell stroma
MICROSCOPY- NEUROBLASTOMA

22.  Protuberant abdomen, fever, weight loss
 Hepatomegaly, metastasis, bone pain
 Elevated blood levels of catecholamines: VMA
 Staging of the tumors & age of patient decides the prognosis
 Del (1p36) & amplification of N-myc oncogene  Bad
prognosis
CLINICAL FEATURES

23.  Mc Malignant tumor of eye in childhood
 Multifocal or Bilateral
 Spontaneous regression is noted, secondary primary
tumors possible
 Familial & Sporadic
 Rb gene mutation
RETINOBLASTOMA

24. RETINOBLASTOMA
 1 in 20000 infants and children
 60% Familial; 40% Sporadic
 Autosomal dominant trait
 Gene is present on chromosome 13q14,
 Loss of both alleles leads to Retinoblastoma
(deletions)

25.  Arise from neuroepithelial cell
 SBRCT
 Some differentiated structures may be present
 Flexner- Wintersteiner rosettes
 Unlike pseudorosettes
 Dissemination by Optic nerve  CNS. Skull bones, LN.
MORPHOLOGY

26.  Median age at presentation is 2 yrs
 Poor vision, strabismus, Leukocoria (Cat’s eye reflex)
 Rx: Enucleation
 Spontaneous regression, with or without Rx is also noted
 Secondary tumors like OS & Soft tissue sarcomas.
CLINICAL FEATURES

28.  Most common renal tumour in children
 10 / million, under 15 yrs,
 Common age – 2 to 5 yrs,
 5 to 10% bilateral – synchronous or metachronous
Wilms tumour

29. PATHOGENESIS AND GENETICS:
1. WAGR syndrome: Aniridia, Genital anomalies, Mental
retardation .
2. Denys- Drash syndrome: Gonadal dysgenesis (male
Psuedohermaproditism)
3. Beckwith-Wiedmann syndrome: Organomegaly,
Macroglossia, Hemihypertrophy, Omphalocele, Adrenal
cytomegaly.
Wilms tumour

30.  Tissue features showing an attempt to recapitulate
nephrogenesis
 Triphasic Tumor:: Blastemal, Stromal, Epithelial
components +
 Sheets of small blue cells without differentiation-
Blastemal
 Fibromyxoid stromal component, Smooth muscle, cartilage
 Abortive tubules & Glomeruli
MICROSCOPY

31.  2 to 5 yrs common
 Palpable abdominal mass
 Hematuria,
 Pain,
 Intestinal obstruction,
 Hypertension
 Pulmonary metastases
Wilms tumour – Clinical Course

32. PROGNOSIS
 Very Good,
 Nephrectomy + chemotherapy,
 2 yrs survival – 90%
 Prone for second primary tumours.
Wilms tumour – Clinical Course