tumors_eb2574d3f86e6feac9a712e24407cf32.pdf
- 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? PT aPTT PTI INR 13 50 98 1.1 Acquired vWD Histopathology Triphasic tumor with epithelial/ blastemal & stromal components
- 4. Yuvraj…….. ◉ Staging? 4 Final diagnosis Stage 4 Triphasic WT of left kidney with tumor thrombus in left renal vein with acquired vWD
- 5. Wilm’s tumor
- 6. Wilm’s tumor ◉ Most common pediatric renal tumor & 2nd MC 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’s tumor ◉ 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 embryonal tumor 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’s sye
- 15. Neuroblastoma ◉ Para neoplastic manifestations 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 vs WT 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 Mass Hard, lumpy-bumpy, irregular border, never ballottable Firm, well defined regular border, smooth surface, ballottable, bimanually palpable Crosses midline? Yes No Hypertensive crisis No 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, hyphema and irregular pupil, phthisis buIbi and 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 gliomas and medulloblastomas
- 27. Brain Tumor Genetic Predisposition Disorders Syndrome Gene Abnormality Characteristic CNS Lesion Other Findings Nuerofibromatosis Type-1 NF1 Low-grade glioma (optic pathway and brainstem) Café-au-lait spots, lisch nodules, axillary freckling Neurofibromatosis Type-2 NF2 Bilateral acoustic schwannomas, meningiomas and ependymomas Increased risk of cataracts and seizures Tuberous Sclerosis TSC1 and TSC2 Subependymal giant cell astrocytoma (SEGA) Increased risk of skin and renal growths Li Fraumeni TP53 Malignant glioma, choroid plexus carcinoma Numerous cancers at younger ages (breast, sarcoma, adrenal cortical carcinoma) Gorlin's (Nevoid Basal Cell Carcinoma Syndrome) PTCH Medulloblastma Basal 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 pineoblastoma in 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 Location Common Signs and Symptoms Frontal lobe Personality changes, decreased motor speech (Broca’s), seizures Temporal lobe Seizures, poor memory, language comprehension (Wernicke’s) Parietal lobe Decreased sense of touch/pain, poor spatial and visual perception, poor interpretation of language Occipital lobe Poor/Loss of vision Cerebellum Ataxia, muscle movement/coordination, posture Brainstem Weakness, cranial neuropathies (III-XII), autonomic dysfunction Thalamus Weakness/motor control, consciousness, sleep/wake cycle Hypothalamus Autonomic 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 Delay Headache Seizures (temporal or frontal lobe tumor) Diabetes Insipidus Behavioral Changes Emesis Vision Changes (optic pathway or occipital lobe tumor) Hypothyroidism Decline in School Performance Sleepiness/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 Fontanelle Precocious 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