Successfully reported this slideshow.

1. pediatric tumors dr. sinhasan, mdzah

21

Share

Upcoming SlideShare
Sims genetics
Sims genetics
Loading in …3
×
1 of 33
1 of 33

More Related Content

Related Books

Free with a 14 day trial from Scribd

See all

1. pediatric tumors dr. sinhasan, mdzah

  1. 1.  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:
  2. 2.  Hemangioma  Lymphangioma  Sacrococcygeal Teratoma BENIGN TUMORS
  3. 3.  Most common Benign Tumor of Childhood 1. Capillary 2. Cavernous  ? Malformations.. ? Hamartoma  Majority are superficial lesions  Malignant Transformation is very Rare Hemangioma
  4. 4.  Site - Skin , Subcutaneous tissue , Mucosa of oral cavity / lips  Morphology:: Bright red - Blue , Few mm - few cms.  Strawberry Hemangioma Present since Birth. Capillary Hemangioma
  5. 5. Capillary Hemangioma
  6. 6.  Site - Skin , Mucosa , Viscera (Liver)  Morphology  Red-Blue , compressible , Spongy , well defined lesions , 2 - 3 cms  Cavernous Lymphangioma - Cystic Hygroma Cavernous Hemangioma
  7. 7. Cavernous Hemangioma: spleen
  8. 8. Cavernous Hemangioma
  9. 9. Lymphangioma
  10. 10. Lymphangioma
  11. 11.  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:
  12. 12. Teratoma
  13. 13. 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
  14. 14.  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
  15. 15.  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
  16. 16.  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
  17. 17.  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
  18. 18.  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
  19. 19.  Mc Malignant tumor of eye in childhood  Multifocal or Bilateral  Spontaneous regression is noted, secondary primary tumors possible  Familial & Sporadic  Rb gene mutation RETINOBLASTOMA
  20. 20. 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)
  21. 21.  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
  22. 22.  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
  23. 23.  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
  24. 24. 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
  25. 25.  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
  26. 26.  2 to 5 yrs common  Palpable abdominal mass  Hematuria,  Pain,  Intestinal obstruction,  Hypertension  Pulmonary metastases Wilms tumour – Clinical Course
  27. 27. PROGNOSIS  Very Good,  Nephrectomy + chemotherapy,  2 yrs survival – 90%  Prone for second primary tumours. Wilms tumour – Clinical Course

×