1. The document discusses common benign and malignant pediatric tumors, including hemangiomas, neuroblastoma, Wilms tumor, and teratomas. 2. Neuroblastoma is the most common extracranial solid tumor in childhood and often presents as an abdominal mass, fever, or weight loss in children under 2. 3. Wilms tumor is the most common renal tumor of childhood, occurring mostly in ages 2-5, and may be associated with genetic syndromes like WAGR or Beckwith-Wiedemann syndrome.

1. Dr. ROOPAM JAIN
PROFESSOR & HEAD
INFANCY & CHILDHOOD
DISORDERS -TUMOR

2. BENIGN TUMORS
Hemangioma
Lymphatic Tumors
Fibrous Tumors
Teratomas
MALIGNANT TUMORS
Leukemia
Retinoblastoma
Neuroblastoma
Wilms Tumor
Hepatoblastoma
Hepatocellular Carcinoma
Soft Tissue Sarcoma
Others

3. Common paediatric benign tumours &
tumour-like lesions

4. Common paediatric malignant tumours

5.  In infants and children under 4 years of age:
MC malignant tumours are various types of
blastomas.
 Children between 5 to 9 years of age:
haematopoietic malignancies.
 In the age range of 10-14 years:
soft tissue & bony sarcomas.

6. Common paediatric benign tumours
& tumour-like lesions

7. Hemangioma
 Hemangioma. Hemangiomas are the most
common tumors of infancy
 In children, most are located in the skin, particularly
on the face and scalp, where they produce flat to
elevated, irregular, red-blue masses; some of the
flat, larger lesions are referred to as port-wine
stains
 Hemangiomas may enlarge along with the growth
of the child, but in many instances they
spontaneously regress

8. Lymphatic Tumors
 lymphatic origin
 lymphangiomas—are hamartomatous or
neoplastic, whereas others k/s lymphangiectasis.
 The lymphangiomas are usually characterized by
cystic and cavernous spaces.

9. Teratomas
 Teratomas may occur as benign, well-differentiated cystic
lesions (mature teratomas), immature teratomas, or as
malignant teratomas
 two peaks in incidence: the first at - 2 years of age & the
second in late adolescence or early adulthood
 Sacrococcygeal teratomas are the most common
teratomas of childhood (40%)
 Approximately 75% - mature teratomas
 about 12% - unequivocally malignant and lethal.
 The remainder is immature teratomas;

10. Sacrococcygeal teratoma

11. Common paediatric MALIGNENT
tumours

13. NEUROBLASTOMA
 Neuroblastic tumors include tumors of:
 Sympathetic ganglia
 Adrenal medulla
 These sites have cells derived from
primordial Neural Crest

14. NEUROBLASTOMA
 Among Neuroblastic tumors, Neuroblastoma is
the most common extracranial solid tumor of
childhood
 It is the most frequently diagnosed tumor of
infancy
 Median age of diagnosis is 18 months & 40% are
diagnosed during infancy

15. NEUROBLASTOMA
 Germline mutations in Anaplastic Lymphoma
Kinase (ALK) gene is the major cause of familial
predisposition to Neuroblastoma
 40% of cases arise in Adrenal medulla
 Other sites of involvement are:
 Paravertebral region of Abdomen (25%)
 Posterior Mediastinum (15%)
 Rest of the common sites are Pelvis, Neck & within
the Brain (Cerebral Neuroblastoma)

16. NEUROBLASTOMA
 Morphology (Grossly):
 Size ranges from small nodules (k/s in-situ lesions) to
large masses weighing more than 1 kg.
 In-situ lesions occur more frequently
 majority of which regress leaving only a focus of
fibrosis or calcification in the adult

17. NEUROBLASTOMA
 Morphology (Grossly):
 Some have fibrous pseudocapsules but others are
infiltrative invading surrounding structures
 On cut section:
 Soft
 Composed of Gray-tan tissue
 May show areas of Hemorrhage and Necrosis

19. NEUROBLASTOMA
 Morphology (Microscopically):
 Composed of:
 Sheets of small cells with dark nuclei
 Scanty cytoplasm
 Poorly defined cell borders
 Pleomorphism
 Mitotic activity
 Homer-Wright rosettes may be seen

21. NEUROBLASTOMA
 Clinical progress:
 In children under 2 years of age, Neuroblastoma
presents with large abdominal mass, fever and
weight loss
 In older children, they may not come into attention
until metastasis causing manifestations related to
affected organs such as:
 Bone Pain
 Respiratory Symptoms
 Gastrointestinal Symptoms

23. WILMS TUMOR
 Also known as Nephroblastoma
 In U.S.A., it is most common primary Renal tumor of
childhood
 Peak incidence is seen in the age group of 2-5 years
 95% cases occur before the age of 10 years
 5-10% cases involve both the kidneys
 Synchronous: If both kidneys affected at same time
 Metachronous: If affected one after another

24. WILMS TUMOR
 The exact cause of Wilms tumor is not clear but in rare cases,
heredity plays a role
 Risk factors:
 African-American race
 Family history of Wilms tumor
 Some cases can occur as a part of three syndromes:
 WAGR Syndrome
 Denys-Drash Syndrome
 Beckwith-Wiedemann Syndrome

25. WILMS TUMOR
 WAGR Syndrome:
 It includes Wilms tumor, Aniridia, Genitourinary system
abnormalities and Mental Retardation
 These cases carry germline deletion of chromosome 11p13
 This chromosome carry the first Wilms Tumor associated
gene WT1
 The lifetime chances of occurrence of Wilms tumor is 33%

26. WILMS TUMOR
 Denys-Drash Syndrome:
 It includes gonadal dysgenesis and early onset nephropathy
 These cases also carry germline abnormalities in WT1 which
affects DNA binding properties
 These cases have higher risk of Wilms tumor (~90%)

27. WILMS TUMOR
 Beckwith-Wiedemann Syndrome:
 This is clinically different from both WAGR & Denys-Drash
syndromes but this also has high risk of developing Wilms
Tumor
 This syndrome is characterized by enlargement of body
organs, macroglossia, ear abnormalities & abnormal large
cells in Adrenal Cortex
 The chromosome region implicated has been localized to
band 11p15.5 and termed as WT2

28. WILMS TUMOR
 Morphology:
 Grossly:
 Tumor is large, solitary and well circumscribed
 10% cases are bilateral or multicentric
 On cut section,
• Soft
• Homogenous
• Tan to gray in color
• Occasionally foci of hemorrhage and necrosis
seen

29. Wilms tumor in the lower pole of the
kidney with the characteristic tan-to-gray
color & well-circumscribed margins

30. WILMS TUMOR
 Morphology:
 microscopically:
 Tumor is composed of sheets of small blue cells
 5% cases show features of anaplasia i.e. Presence of
cells with nuclei features:
• Large
• Hyperchromatic
• Pleomorphic
• With mitotic figures

31. Focal anaplasia was present in this Wilms tumor
in other areas, characterized by cells with
hyperchromatic, pleomorphic nuclei, and
abnormal mitoses.

32. WILMS TUMOR
 Clinical features:
 Large abdominal mass which may extend to pelvis
 Hematuria
 Abdominal pain
 Intestinal obstruction
 In some cases, pulmonary metastasis is present at
the time of primary diagnosis