tumors_eb2574d3f86e6feac9a712e24407cf32.pdf

Pediatrics

1. Tumors
1

2. Case
◉A 4 years old boy developmentally normal, well
thriving child presented in pediatric OPD with
complaints of a lump in his tummy which has been
felt by his mother yesterday while bathing him. The
child is otherwise very happy.
◉On examination: a round lump palpable, approx 5cm
diameter, over left lumbar area; smooth surface,
regular border. BP-130/90mm Hg
2
What is/are the clinical differential(s)?
What specific emphasize should you make while examining
the kid?

3. Yuvraj…
◉CBC, Biochemistry: WNL
◉USG abdomen: a large mass in left renal fossa;
impaired color flow in left renal vein
Needle biospy
3
Now what to do?
Next what? CECT abdomen What next?
PTaPTTPTIINR
1350981.1
Acquired
vWD
Histopathology
Triphasictumor with epithelial/ blastemal
& stromalcomponents

4. Yuvraj……..
◉Staging?
4
Final diagnosis
Stage 4 TriphasicWT of left kidney
with tumor thrombus in left renal
vein with acquired vWD

5. Wilm’stumor

6. Wilm’stumor
◉Most common pediatric renal tumor & 2ndMC
extra-cranial solid malignancy
◉Typical age: 2-4 years (Median: 44 mo)
◉No gender predisposition
◉Associated syndrome (8-10% of cases): DDS/
BWS/ WAGR/ Fraser/ Perlman syndromes
6

7. Wilm’stumor
◉Clinical features: Incidental mass, hematuria, HTN
◉Lump description: firm, smooth surface, well defined
margin, doesn’t cross midline, bimanually palpable
◉H/P/E: triphasic-Blastemal/ epithelial/ stromal
components
◉Staging: surgico-histopathological+ Chest CT
7

8. Treatment
◉Chemotherapy
◉Surgical resection
◉Young age, low stage and low weight are
favorable prognostic factors
8

9. Case
◉Abhinav, a 4 years old boy comes with complaints of
sudden onset inability to walk, severe B/L lower limb
pain & continuous urinary dribbling since last 1 week.
◉On examination: B/L lower limbs with hypotonia,
power 2/5, DTR: hyporeactive. While examining
abdomen; you felt a left lumbar mass, which is non
ballottable.
9
What is/are the clinical differential(s)?
Which immediate intervention should one do?

10. Spinal cord compression in pediatric oncology
◉Neuroblastoma
◉Para spinal Ewing’s sarcoma
◉NHL
10

11. Abhinav……
◉What next?
◉What next:
◉staging??
1.MIBG scintigraphy
2.Bone marrow aspiration
11

12. Neuroblastoma

13. Neuroblastoma
◉Malignant embryonaltumor of sympathetic chain
◉Age: most common intra-abdominal and extra-
cranial tumor
◉Median age: 22 months; 50% before 2 years of
age & 80% below 5 years
◉Location: 50% adrenal, rest extra adrenal
(abdomen> chest)
13

14. Neuroblastoma
◉Clinical features
14
Loco-regional
Abdomen: abdominal distension
Pelvic: features of bladder-bowel
compression
Chest: features of airway/vascular
compression
Neck: aero-digestive compression,
Horner syndrome
Spinal cord/ nerve root compression:
paraparesis
Systemic-Metastatic
Bone marrow> Bone> CNS, orbit
4s: Liver/ skin
BM: cytopenias
Bone: bone pain, limping
CNS: focal deficit/ raised ICP
Orbit: proptosis, peri-orbital
infiltrates, Raccon’ssye

15. Neuroblastoma
◉Para neoplasticmanifestations
1.Opsoclonus myoclonus ataxia syndrome
2.Kerner-Morrison syndrome: VIP
15

16. NBL: Diagnosis/staging/prognosis
◉Diagnosis:
1.Unequivocal histology on tissue specimen
2.Elevated urine VMA with BM infiltration
◉Staging:
MIBG/ DOTATATE PET, B/L bone marrow
aspiration & biopsy
◉Prognostic variables:
Age/ stage/ degree of histological differentiation/
n MYC status
16

17. NBL vsWT
17
Characters NBL WT
Age Very young,infants Typical age: pre-school
age
General well being Sick looking, unhappy,
irritable
Happy,well
Systemic features Fever, bone pain, skin bleeds,
pallor, OMAS, Raccoon eye
None
MassHard, lumpy-bumpy,
irregularborder, never
ballottable
Firm, well defined
regular border, smooth
surface, ballottable,
bimanually palpable
Crossesmidline? YesNo
Hypertensive crisisNo Yes

18. Case
◉An 1 yr 2 month old child went to her uncle’s
house & aunty noticed abnormal white reflex
over the black hole of left eye.
18
What is/are the clinical differential(s)?
Which investigation should one do next ?

19. Retinoblastoma
◉Most common primary intraocular tumor
◉Mostly occurs in infancy and childhood.
◉Genetics: RB1 gene on chromosome 13 is
responsible.
19

20. Clinical features
◉Pupillary white reflex (Leucokoria)
◉Strabismus, poor visual tracking and glaucoma
are also seen
◉Orbital inflammation, hyphemaand irregular
pupil, phthisis buIbiand a fungating ocular mass
suggest advanced disease.
◉Staging:
○intraocular
○Extraocular-CNS, lymph nodes, bone marrow
20

21. Daignosis
◉History
◉Clinical examination
◉Imaging-Imaging studies such as ultrasound, CT
/MRI (preferred) scans are used to assess the
orbital, optic nerve and for intracranial extension.
◉Trilateral retinoblastoma
21

22. Treatment
◉Aim: survival with eradication of disease;
maintenance of vision
◉Retinoblastoma is curable when the disease is
intraocular.
1.Laser
2.Cryotherapy
3.Chemotherapy-vincristine, carboplatin and
etoposide
4.Enucleation-uniocular disease
5.radiotherapy
22

23. Prognosis
◉Intraocular lesions are mostly cured.
◉Extraocular involvement has bad prognosis
◉All first degree relatives should be followed till 7
years of age.
23

24. Overview of Pediatric
Brain Tumors
◉Abnormal growths or masses that occur in the
brain tissue
◉Most common solid tumor in children
○Under the age of 15
○Represent about 20% childhood cancers
◉Treatment and chance of recovery (i.e.,
prognosis) depends on the tumor:
●Type
●Location within the brain
●Whether it has infiltrated other brain tissue
●Child’s age and health

25. Symptomology
◉Increased head size in infants
◉Seizures
◉Morning headache or headache that goes away
after vomiting
◉Unusual sleepiness
◉Alterations in vision, hearing and speech
◉Frequent nausea
◉Personality changes

26. Types of Pediatric Tumors
The most common types of brain tumors in children are gliomasand medulloblastomas

27. Brain Tumor Genetic Predisposition Disorders
SyndromeGene AbnormalityCharacteristic CNS LesionOther Findings
Nuerofibromatosis Type-1NF1
Low-grade glioma (optic
pathway and brainstem)
Café-au-lait spots, lisch nodules, axillary
freckling
Neurofibromatosis Type-2NF2
Bilateral acoustic
schwannomas, meningiomas
and ependymomasIncreased risk of cataracts and seizures
Tuberous SclerosisTSC1 and TSC2
Subependymal giant cell
astrocytoma (SEGA)Increased risk of skin and renal growths
Li FraumeniTP53
Malignant glioma, choroid
plexus carcinoma
Numerous cancers at younger ages
(breast, sarcoma, adrenal cortical
carcinoma)
Gorlin's (Nevoid Basal Cell
Carcinoma Syndrome)PTCHMedulloblastmaBasal cell carcinoma
Familial Adenomatous
Polyposis (Gardner’s,
Turcot’s)APC
Medulloblastoma and malignant
glioma
Multiple colon polyps and increased risk
of colon cancer
Rhabdoid Tumor
Predisposition Syndrome
SMARCB1 and SMARCB4
(INI-1)
Atypical Teratoid Rhabdoid
Tumor (AT/RT)
Rhabdoid tumors in kidney,
schwannomatosis; usually < 1 y/o at
diagnosis
Retinoblastoma (germline)RB1
Trilateral retinoblastoma
(unilateral or bilateral
retinoblastoma +
pineoblastoma)
The pineoblastomain these cases is
usually diagnosed after the
retinoblastoma but often before the age
of 5 y/o

28. CNS Tumors:
Localizing Symptoms Based on Anatomy
Anatomic LocationCommon Signs and Symptoms
Frontal lobePersonality changes, decreased motor speech(Broca’s),
seizures
Temporal lobeSeizures, poor memory, language comprehension
(Wernicke’s)
Parietal lobeDecreasedsense of touch/pain, poor spatial and visual
perception, poor interpretation of language
Occipital lobePoor/Loss of vision
CerebellumAtaxia, muscle movement/coordination, posture
BrainstemWeakness, cranial neuropathies (III-XII), autonomic
dysfunction
ThalamusWeakness/motor control, consciousness, sleep/wake cycle
HypothalamusAutonomic dysfunction (temperature regulation, thirst, hunger,
etc.) endocrinopathies
Table 1

29. CNS Tumors:
Symptoms
Generalized "Non-Localizing"
Symptoms
Increased Intracranial
Pressure/Obstructive
Hydrocephalus"Localizing Symptoms"Endocrine Symptoms
Developmental DelayHeadache
Seizures (temporal or
frontal lobe tumor)Diabetes Insipidus
Behavioral ChangesEmesis
Vision Changes (optic
pathway
or occipital lobe tumor)Hypothyroidism
Decline in School PerformanceSleepiness/Lethargy
Motor Weakness (tumor in
motor strip of cerebrum)Weight Gain or Loss
Tiredness/Sleepiness
Papilledema (Vision
Changes)
Cranial Neuropathies
(brainstem tumor)Panhypopituitarism
Full/Bulging FontanellePrecocious Puberty
Table 2

30. Craniopharyngioma
◉Rare epithelial tumor of thought to be due to maldevelopmental origin.
◉Represents about 6-9% of all pediatric brain tumors.
◉Often presents with headache, emesis, visual field cut and endocrinopathies.○80-90% will have endocrinopathies at presentation if tested
◉Most common location is suprasellar/pituitary.
◉Imaging usually shows a mixed solid and cystic mass within the suprasellar region.
◉Treatment is maximum surgical resection.
◉Although progression-free and overall survivals are good, many patients have numerous morbidities affecting quality of life (endocrinopathies, behavioral issues, visual dysfunction and seizures).

31. Treatment
◉Multidisciplinary approach
◉Surgery
◉Chemotherapy and radiation
31