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Small round cell_tumor_DR NARMADA

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SMALL BLUE ROUND CELL TUMOUR, PEDIATRIC NEOPLASM,

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Small round cell_tumor_DR NARMADA

  1. 1. Moderator:-Dr. Poonam Nanwani Speaker:- Dr. Narmada Prasad Tiwari
  2. 2. Histologically, many of the pediatric neoplasms have more primitive origin characterized by sheets of cells ,with small , round nuclei. Because of their primitive histologic appearance many childhood tumor have been collectively referred to as small round blue cell tumor.
  3. 3. The differential diagnosis of such tumors are:- Neuroblastoma Wilms tumour(Nephroblastoma) Rhabdomyosarcoma Ewing’s sarcoma/PNET Medulloblastoma Retinoblastoma Lymphoma
  4. 4. NEUROBLASTOMA  most common extracranial solid tumor of childhood most frequently diagnosed tumor of infancy. Median age at diagnosis is 21 months. Most occur sporadically. 1 to 2% occur familial- Germ line mutation in the anaplastic lymphoma kinase (ALK) gene
  5. 5. Clinical course- In childhood 40% of neuroblastoma arise in adrenal medulla. Other sites-along sympathetic chain post. Mediastinum neck, brain. • under 2 year - large abdominal mass, fever ,weight loss. About 90% of neuroblastoma regardless of location produce catecholamines. Neuroblastoma – size- minute nodules to large masses
  6. 6. Gross-
  7. 7. Neuorblastoma Morphology Small round blue cell tumor neuorpil formation (fibers, i.e., axons dendrites, mostly unmyelinated) rosette formation immunochemistry – neuron specific enolase EM – secretory granules (catecholamine) Usual features of anaplasia high mitotic rate is unfavorable evidence of Schwann cell or ganglion differentiation favorable
  8. 8. Histologically
  9. 9. Undifferentiated type
  10. 10. Differentiating type Poorly differentiated type
  11. 11. Neuroblastoma may metastasize widely through the hematogenous & lymphatic system, particularly to liver, CNS, bone, lymph nodes and bone marrow.
  12. 12. Prognostic factors in neuroblastoma Variable Favourable Unfavourable (1) Stage 1, 2A,2B,4S 3,4 (2) Age <18 month > 18 month (3)Histology:- (a)Evidence of schwannnian stroma & gangliocytic differentiation. (b) Mitosis-karyorrhexis index Present < 200/5000 cells Absent >200/5000 cells (4) DNA ploidy Hyperdiploidy or near triploidy Near diploid (5) N-Myc Not amplified Amplified (6) Chromosome 17q gain Absent Present (7) Chromosome 1 p loss Absent Present (8) Chromosome 11q loss Absent Present (9) Trk A expression Present Absent (10)TrkB expression Absent Present (11) Telomerase expression Low or Absent Highly expressed.
  13. 13. WILMS’ TUMOR(NEPHROBLASTOMA) Age:- 3 -6 years Sex:- No sex predeliction Clinical features-Large abdominal mass Hematuria Pain in abdomen Hypertension
  14. 14. Molecular Genetic Genetic loci predisposing to wilms’ tumor are WT1 ( located on chromosome 11p 13 ) WT2 ( located on chromosome 11p 15.5 ) - Mutations of B catenin gene-14-20% - Conditions associated with wilms’ tumor are:- WAGR syndrome:- Wilms’ tumor Aniridia Genital anomalies Retardation
  15. 15. Beckwith wiedemann Syndrome:- Omphalocele Macroglossia Hemihypertrophy of organs Denys Drash Syndrome:- Gonadal dysgenesis( male psuedohermaphroditism) Early onset nephropathy
  16. 16. Gross:- solid, well circumscribed. On cut-:-solid & pale gray & often exhibit areas of cystic changes, necrosis & hemorrhage.
  17. 17. Microscopically :- Three major component are identified. I- Undifferentiated blastema II – Mesenchymal ( stromal) tissue III – Epithelial tissue Blastematous - small round to oval cells, scanty cytoplasm The mesenchymal element- spindle cell fibroblast like configuration. Epithelial component- embryonic glomerular and tubular structures.
  18. 18. Additional morphological features- Ciliated,mucinous, squamous or transitional epithelium, neuroepithelium,mature adipose tissue,Cartilage & bone
  19. 19. Anaplastic wilms tumour
  20. 20. Spread and metastasis- Local spread Lymph nodes-15% cases Distant metastasis- lungs, liver and peritoneum.
  21. 21. Rhabdomyosarcoma:- Rhabdomyosrcoma is the most common soft tissue sarcoma of childhood & adolescence, usually appear before age 20 year. Types:- Embryonal (most common) Alveolar Rhabdomyosarcoma Pleomorphic (least common)
  22. 22. Morphology:- Pleomorphic Rhabdomyosarcoma:- It is least common. Site:- Extremities & thigh. Age:- Adult Grossly :- It is confined within fascial compartment & have the shape of muscle from which it arises.
  23. 23. Microscopically:-Pleomorphic type Tumor is pleomorphic with giant cells.
  24. 24. Embryonal rhabdomyosarcoma Clinical Feature:-Arise from unsegmented & undifferentiated mesoderm. Site:- Common in head & neck region Orbit Nasopharynx Bile duct Urogenital tract Age :- 3 -12 years, can occur in adults also. Grossly-poorly circumscribed, white,soft.
  25. 25. Embryonal rhabdomyosarcoma composed predominantly of round cells. There is perivascular pseudorosette around blood vessels.
  26. 26. Microscopically(Embryonal type) Tumor cells are small & spindle shaped. Oval eccentric nuclei acidophilic cytoplasm.
  27. 27. Botryoid type When beneath a mucosal membrane , such as vagina, urinary bladder or nasal cavity it frequently form large polypoid mass resembling a bunch of grapes- Hence name “Sarcoma Botryoides” Dense zone of undifferentiated tumor cells immediately beneath the epithelium , aformation of known as Nicholson’s Cambium Layer.
  28. 28. Alveolar rhabdomyosarcoma Common Site:- Forearm Arm Perirectal & perianal region Head and neck region. Age- 10-25 yrs.
  29. 29. Alveolar type
  30. 30. Microscopically( alveolar type) Tumor cells are small,round are sepearted in nest by connective tissue septa
  31. 31. Special techniques- Special Stains:- PTAH Masson’s trichome Silver impregnation technique Immunohistochemically:- Markers are Myogenin Desmin Sarcomeric actin Myosin Myoglobin
  32. 32. Tropomyosin a actinin,titin, Z protein Vimentin Enzymes( creatine kinase) Neurofilament & S-100 protein CARP- cardiac ankyrin related protein
  33. 33. EWINGS SARCOMA Ewing’s sarcoma limited neural differentiation. PNET show more neural features. Age:- 5 to 20 years (commonly) Infancy or adulthood rarely Sex:- Male predilection. It generally arise in medullary cavity of shaft from which it permeate the cortex & invade the soft tissue.
  34. 34. EWINGS SARCOMACommon site- Long bones( femur,tibia, humerus,fibula). Rare site- Bone of pelvis, rib , vertebra, mandible, clavicle. Clinical features: Pain Fever Leuckocytosis
  35. 35. Genetic Predisposition:- Over 95% show reciprocal translocation of chromosome 11 : 22 (q24 : q 12). This leads to fusion of EWS gene with FLI-1.
  36. 36. This tranlocation can be detected by RT-PCR. This can be used for the detection of primary and metastatic or residual disease in tissue & body fluids including blood. The EWS rearrangement has also been detected by FISH technique.
  37. 37. Radiograph:- Ewing’s sarcoma of fibula. Onion skin appearance
  38. 38. Gross-
  39. 39. Microscopically:-
  40. 40. Histochemically:-
  41. 41. Immunohistochemically:- Positive for Vimentin. Neuron specific enolase Neurofilament Leu 7 CD -99
  42. 42. Medulloblastoma  5-10 yrs. Site:- Commonly arise from Cerebellum. Rapid growth may occlude the flow of CSF leading to hydrocephalous.
  43. 43. The tumor - circumscribed, gray & friable.  microscopic - extremely cellular.  small cells with scanty cytoplasm & hyperchromatic nuclei that frequently crescent shaped. Abundant mitosis.
  44. 44. Variants of medulloblastoma:- - Classical Medulloblastoma - Desmoplastic Medulloblastoma - Neuroblastic medulloblastoma - Anaplastic Medulloblastoma
  45. 45. Medulloblastoma
  46. 46. Desmoplastic medulloblastoma :-Micronodular zone of reduced cellularity( “ pale island”)
  47. 47. “Neuroblastic “ medulloblastoma. This variant of medulloblastoma is typified by the linear streaming of rounded, ‘neurocytic’ tumor cell nuclei within amassed cytoplasmic processes
  48. 48. Large cell/anaplastic medulloblastoma. Showing prominent nucleoli & pronunced mitotic & apoptotic activity .
  49. 49. LYMPHOMA(Chronic lymphocytic leukemia/small lymphocytic lymphoma Age:- median age is 60 years. Sex ratio:- 2:1 male to female Clinical feature:- Mostly asymptomatic
  50. 50. Morphology:- SLL/CLL:- Low power view show diffuse effacement of nodal architecture.
  51. 51. -1.with absolute lymphocytosis. 2.associated with monoclonal gammopathy 3. hypogammaglobulinemia 10-15% cases – autoimmune hemolytic anemia. May transform into diffuse large B cell lymphoma- richter transformation. IHC- CD20,CD23,CD5, .
  52. 52. RETINOBLASTOMA Retinoblastoma is the most common intraocular neoplasm of children- 16 mths- 2 yrs. It characteristically present as a LEUKOCORIA / strabisumus . Bilateral in 30% > 90% familial cases. Trilateral retinoblastoma
  53. 53. Genetic:- congenital. Sporadiac – 60% Familial – 40% Autosomal dominant Gene located on Chromosome – 13q14( retinoblastoma Rb gene)
  54. 54. Knudsons 2 hit hypothesis- Genetic mutation in both allele are necessary to produce retinoblastoma. Hereditary retinoblastoma – somatic Mutation in second allele. Sporadic retinoblastoma – both mutations are somatic.
  55. 55. GROSS:-flat or elevated Endophytic type:- This is protrude into vitrous. Exophytic type:-They may grow between retina & pigmented epithelium.
  56. 56. Microscopic:-
  57. 57. Retinoblastoma with typical “ Flexner – wintersteiner rosettes”.
  58. 58. Prognosis- Invasion of optic nerve. Invasion of uveal tract. Invasion of meninges. IHC- NSE,GFAP,S-100 protein retinal binding protein, retinal S antigen. Long term survivors- osteosarcoma, rhabdomyosarcoma.
  59. 59. THANK YOU

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