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:

Hemangioma

Lymphangioma

Sacrococcygeal Teratoma

BENIGN TUMORS

Most common Benign Tumor of Childhood

1. Capillary

2. Cavernous

? Malformations.. ? Hamartoma

Majority are superficial lesions

Malignant Transformation is very Rare

Hemangioma

Site - Skin , Subcutaneous tissue , Mucosa of oral

cavity / lips

Morphology:: Bright red - Blue , Few mm - few cms.

Strawberry Hemangioma Present since Birth.

Capillary Hemangioma

Capillary Hemangioma

Site - Skin , Mucosa , Viscera (Liver)

Morphology Red-Blue , compressible , Spongy , well

defined lesions , 2 - 3 cms

Cavernous Lymphangioma - Cystic Hygroma

Cavernous Hemangioma

Cavernous Hemangioma: spleen

Cavernous Hemangioma

Lymphangioma

Lymphangioma

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:

Teratoma

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

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

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

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

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

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

Mc Malignant tumor of eye in childhood

Multifocal or Bilateral

Spontaneous regression is noted, secondary primary

tumors possible

Familial & Sporadic

Rb gene mutation

RETINOBLASTOMA

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)

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

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

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

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

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

2 to 5 yrs common

Palpable abdominal mass

Hematuria,

Pain,

Intestinal obstruction,

Hypertension

Pulmonary metastases

Wilms tumour – Clinical Course

PROGNOSIS

Very Good,

Nephrectomy + chemotherapy,

2 yrs survival – 90%

Prone for second primary tumours.

Wilms tumour – Clinical Course