1. Tumors and tumor like lesions of infancy and childhood

2. Benign tumors and tumor like lesions
Benign tumors more common than malignant tumors.
Three benign tumors more common in pediatric age group
Hemangiomas
Lymphangiomas
Teratoma
Sacrococcygeal teratoma

3. Hemangiomas BT of blood vessels. Locations: Most are cutaneous
Most common tumors in infancy.
Locations: Most are cutaneous
Face and scalp
Significance:
Cosmetic
Associated with hereditary disorders
Von Hippel Lindau syndrome( kidney tumors, brain tumors[hemangioblastoma)
Sturge Weber syndrome (port wine stain, seizures, MR)
May grow with the child
Most hemangiomas regress spontaneously .

4. Hemangiomas Gross: Microscopic types:
Flat to elevated, irregular red blue masses.
Large flat lesions are called port wine stain.
Microscopic types:
Capillary hemangiomas:
Strawberry ( juvenile capillary) hemangiomas
Cavernous hemangiomas:

5. Capillary hemangioma
After 2 years

6. Strawberry ( juvenile capillary) hemangiomas:
a variant of capillary hemangioma
commonly seen on the face in new born
slightly raised, bright red, lobulated vascular tumors
grow rapidly for a few months and begin regressing at age 1-3 years.
80% totally gone by 8 years
No treatment necessary

7. Strawberry hemangioma

8. Lymphangioma Cystic spaces lined by endothelial cells Locations:
Spaces contain pale fluid.
Locations:
Skin
Deeper regions : neck / axilla / mediastinum/ retroperitoneum.
Significance:
Increase in size – may encroach on vital structures
Cystic hygroma:
A type of lymphangioma
Occurs in the neck or axilla in children
Composed of dilated lymphatic channels containing lymphatic fluid
Associated with Turner syndrome (45,X karyotype)

9. Cystic hygroma

10. Teratoma Germ cell tumor Sacrococcygeal teratoma:
Contain structures derived from ectoderm, mesoderm and endoderm.
Sacrococcygeal teratoma:
Most common germ cell tumor of childhood.
Arise in the sacrococcegeal region.
10% associated with congenital anomalies:
Defects of hindgut, cloacal region and midline defects (meningocele and spina bifida).
Can be:
Mature (benign) (majority)
Younger infants (<4 months)
Immature (malignant)
Slightly older children.

11. Sacrococcygeal teratoma

12. Malignant tumors

13. Common malignant Tumors of infancy and childhood
0-4 years
5-9 years
10-14 years
Leukemia
Soft tissue sarcoma
Neuroblastoma
Osteogenic sarcoma
Wilms tumor
Ewing Sarcoma/PNET
Thyroid carcinoma
Brain tumors
Hepatocellular carcinoma
Hepatoblastoma
Hodgkin's lymphoma
Retinoblastoma
Lymphoma
Rhabdomyosarcoma

14. Malignant tumors Most common malignant tumors per system of childhood:
Hematopoietic system
Leukemia some lymphomas
Central nervous system
Astrocytoma, medulloblastoma, ependymoma
Adrenal medulla:
Neuroblastoma
Retina
Retinoblastoma
Soft tissue:
Rhabdomyosarcoma
Bone:
Ewing sarcoma, osteogenic sarcoma
Kidney:
Wilms tumor
Leukemia accounts for more deaths in children.

15. Fun facts Most common organ systems involved by MT in adults:
Most common organ systems involved by malignant in infancy and childhood:
Hematopoietic system
Neural tissue
Soft tissue
Most common organ systems involved by MT in adults:
Lung, prostate, colon

16. Small round blue cell tumors
Group of malignant tumors
Composed predominantly of
Sheets of small cells with small round blue nuclei.
Examples:
Neuroblastoma
Lymphoma
Rhabdomyosarcoma
Ewing sarcoma
Wilms tumor
Retinoblastoma

17. Neuroblastoma Malignant tumor of neural crest origin
MC site of origin :adrenal medulla (abdominal cavity)
Posterior mediastinum
Primarily seen in children <5 years of age.
Median age: 2 years.
3rd or 4th most common malignancy in children
Associations:
Beckwith- Wiedemann syndrome & Neurfibromatosis.
Genetics:
deletion or rearrangement of short arm of chr 1 leading to amplification of n-myc oncogene.

18. More fun facts Location:
Most located in adrenal medulla (abdominal cavity)
Also occur in posterior mediastinum and neck.
May develop anywhere along the sympathetic nervous system chain.

19. FISH in diagnosis
Fluorescence in situ hybridization (FISH) of a probe for N-MYC
Nuclei shows abundant signal (red) in a tumor cell (arrow), indicating N-MYC amplification..

20. Fluorescence in situ hybridization
Don’t worry about this
N-MYC amplification

21. Neuroblastoma Infant with neuroblastoma - Clinical presentation
This infant has a large adrenal neuroblastoma, creating a greatly distended abdomen.
There are numerous bluish subcutaneous nodules of tumor. "blueberry muffin baby."
Congenital neuroblastoma is the most common solid congenital malignancy.
Tumors in infants may pursue a less aggressive clinical course than tumors in older children.

22. neuroblastoma

23. Neuroblastoma Clinical findings:
Palpable abdominal mass with abdominal distention
Tumor cells secrete catecholamines:
Diastolic hypertension
Metastasis to skin and bone (most common site)

24. Kidney
Neuroblastoma   
Kidney, adrenal/renal tumor - Gross, cut surface  This image shows a large, hemorrhagic mass superior to the upper pole of the kidney. The adrenal gland cannot be seen.  

25. Neuroblastoma micro

26. Neuroblastoma Gross: Micro: Neuroblasts:
Soft in consistency and invades surrounding tissue (kidney,pancreas,liver).
Micro:
Composed of small round blue cells (neuroblasts) and characteristic Homer Wright pseudorosettes.
Neuroblasts:
S100 and Neuron specific enolase (NSE) positive
contain neurosecretory granules.

27. Neuroblastoma Differential diagnosis:
ALL
Wilms tumor, Ewing’s sarcoma .
May spontaneously differentiate into less aggressive tumor.
neuroblastoma  ganglioneuroblastoma  ganglioneuroma.
Prognosis:
Age of the patient is the single most important factor determining prognosis
Cure rate of 85-90% under 1 year age
15-40 cure rate in older children.

28. Neuroblastoma Laboratory Findings:
Increased Urinary Vanillylmandelic acid (VMA)
Neuroblasts are S-100 positive
Homovanillic Acid (HVA)
(Metabolic End Product Of Dopamine)

29. Wilms tumor (Nephroblastoma)
Most common malignant tumor of kidney in children.
Occurs in children 2-5 years of age.
Patients present with
Unilateral palpable mass in the abdomen, abdominal pain, hematuria, hypertension and fever.
Types:
Sporadic (90%)
Associated with congenital malformations

30. Wilms tumor Gross: Microscopically show:
WT are large, soft, well circumscribed gray white tumors with areas of hemorrhage and necrosis
50% show invasion of renal vein.
Microscopically show:
Composed of small round blue cells
Triphasic pattern
Primitive cells (small round blue cells) = blastema
Spindle cell stroma
Immature tubules and glomeruli

31. Wilms tumor Residual kidney
Kidney, Wilms tumor - Gross, cut surface  A large, tan, well-circumscribed mass greatly distorts the upper pole of the kidney.

32. Small round blue cells = blastemal element
Primitive tubules
Small round blue cells = blastemal element
Kidney, Wilms tumor - High power  Epithelial and blastemal elements resemble embryonic kidney.  

33. Wilms tumor Clinical findings: Prognosis:
Unilateral palpable mass and hypertension caused by renin secretion
Prognosis:
Frequently metastasize to lungs, lymph nodes
Good prognosis with appropriate therapy
Surgery + Chemo+ radiation.

34. Retinoblastoma Is the most common malignant eye tumor of childhood.
Unusual tumor:
Occurs as a congenital tumor
Can be multifocal and bilateral
Undergoes spontaneous regression
Associated with second primary tumors.
Occurs in two patterns:
Sporadic:
Familial (AD inherited disease):

35. Retinoblastoma Sporadic: Familial (AD inherited disease):
Inactivation of both Rb suppressor genes on chromosome 13 acquired after birth.
Unilateral and uni-focal
Familial (AD inherited disease):
One Rb gene is already inactivated, the other is inactivated after birth.
Multiple tumors, often bilateral
Increased risk of osteogenic sarcoma

36. Retinoblastoma Clinical features: Treatment:
Median age at presentation is 2 years.
May be present at birth.
Poor vision, pain in the eye.
White eye reflex when a light is shined into the eye (cat’s eye reflex = Leukokoria)
Untreated tumors are fatal
Treatment:
Enucleation, chemo and radiotherapy.
Good prognosis

37. Retinoblastoma

38. Retinoblastoma
Eye, retinoblastoma - Gross, sagittal section  A large tumor fills the globe. Note the whitish calcium flecks. This is the most common malignant eye tumor of childhood. It is familial in up to 40% of cases. Familial cases are often bilateral and occur in patients at a younger age.

39. Rosettes
Eye, retinoblastoma - High power  Numerous true rosettes (tubules with lumens) are present in this tumor, which is composed of primitive round blue cells.

40. Retinoblastoma Gross: Nodular mass in the eye replacing the vitreous.
Micro:
tumor composed of small round blue cells with large nuclei and scant cytoplasm.
Characteristic structures k/a Flexner-Wintersteiner rosettes.
Consist of clusters of cuboidal cells arranged around a central lumen.
Metastasis:
Via optic nerve or subarachnoid space
Sites: CNS, Skull, bones.

41. Rhabdomyosarcoma Most common soft tissue tissue of childhood
Can occur in any anatomic location
Most occur in head and neck, genitourinary tract, and extremities
Three types
Embryonal
Alveolar
Pleomorphic variants

42. Rhabdomyosarcoma Avelolar rhabdomyosarcoma
Associated with 2;13 or 1;13 translocation (PAX3/FKHR) which codes for skeletal muscle differentiation.

43. Sarcoma botyroides

44. Embryonal rhabdo micro
Don’t worry about this

45. My aveolar Rhabdo case
Tumor around the eye

46. Don’t worry about this

47. Don’t worry about this

48. Don’t worry about this

49. Aveolar Rhabdo micro