Pediatric Renal Tumors
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Pediatric Renal Tumor , WILMS TUMOUR , NEPHROBLASTOMA , MESENCHYMAL TUMOUR , CLEAR CELL SARCOMA , RHABDOID TUMOUR , PATHOLOGY , HISTOPATHOLOGY , CANCER ,WHO CLASSIFICATION #PediatricRenalTumor #Renal #Tumor #WilmsTumor #Pathology #Pediatrics
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Pediatric Renal Tumors
- 1. Pediatric Renal Tumors Presenter – Dr Utsab Das Dept of Pathology ,Calcutta National Medical College Moderator – Dr Piyali Ghosh Demonstrator ,Dept of Pathology ,Calcutta National Medical College
- 3. Peditaric Renal Tumors-- • Broadly classified into 2 categories (WHO) --- • (A) Nephroblastic and cystic tumours • (B) Mesenchymal tumours
- 4. (A) Nephroblastic and cystic tumours occurring mainly in children • 1) Nephrogenic Rest • 2) Nephroblastoma / Wilm’s Tumour • 3) Cystic partially differentiated nephroblastoma • 4) Paediatric cystic nephroma
- 5. 1) Nephrogenic Rest • Defn – Persistent foci of embryonal cells (> 36 wk POG) • Epidemiology— 40% cases of Wilms Tumor • Histopathology- Perilobar and Intralobar type- • Each type subclassified into- • Dormant/incipient • Regressing /sclerosing • Hyperplastic
- 9. 2) Nephroblastoma / Wilm’s Tumour • Defn- Malignant embryonal neoplasm derived from nephrogenic blastemal cells,mimics developing kidney &shows divergent patterns of differentiation • Epidemiology- 1 in 8000 • 90% cases <6yrs age • Signs and symptoms- • Abdominal mass • Pain ,haematuria, proteinuria • Hypertension • Secondary to traumatic rupture
- 10. • Conditions associated with highest risk of Wilms tumor- • WAGR syndrome • Beckwith–Wiedemann syndrome • Hemihypertrophy • Denys–Drash syndrome • Familial nephroblastoma • Other conditions - • Cutaneous nevi and angiomas • Trisomy 18 • Klippel–Trénaunay syndrome • Neurofibromatosis • Bloom syndrome • Frasier syndrome • Perlman syndrome • Cerebral gigantism (Sotos syndrome).
- 11. - • Macroscopy- • Gross- Solitary, well circumscribed, soft • cut section -Pale gray ,cystic change, necrosis, hemorrhage .
- 12. 1 Variegated appearance. 2 More homogeneous and nodular. 3 Extensive areas of infarct-like necrosis.
- 13. • Histopathology- • Triphasic pattern- • 1) Undifferentiated blastema • 2) Mesenchymal (stromal) tissue • 3) Epithelial tissue. • Blastemal- Highly cellular, overlapping nuclei . • Diffuse, nodular ,serpentine, basaloid growth • Mesenchymal- Spindle cells with smooth muscle features • Epithelial- Small round tubular structures resembling rosettes
- 14. 1 combination of blastema, stroma,epithelial tubular formations,immature glomeruli 2 blastema, stroma, and immature tubular formations.
- 17. Molecular Genetics • WT1 -- 11p13 • WT2 -- 11p15.5 • WTX gene Inactivation (6-30%) • Activation of β-catenin CTNNB1 (14–20%) • Mutation/overexpression of TP53 (5%) • MYCN amplification • SIX/2 assoc with Blastemal WT • Abnormalities in chromosomes—1p, 7q,8,12,16q
- 18. IHC Markers- Epithelial – KERATIN ,EMA Mesenchymal – Desmin ,Myogenin Neural elements – NSE , GFAP , S-100 Additional Markers – Nuclear WT1 (80%) Nuclear PAX8 CD 56 (96%) TTF-1 (17%)
- 19. Metastasis - • 1) Local spread – Perirenal soft tissues • 2) Regional LN Spread (15%) • 3) Distant Mets – Lungs >> Liver, Peritoneum Treatment Regimes – *SIOP – pre-operative chemo followed by surgery *COG - Initial resection with subsequent therapy post tumor histology and stage
- 20. Prognosis - • 1. Age • 2. Stage • 3. Anaplasia*** (4% of the cases ) ***criteria for anaplasia- (A)marked enlargement of nuclei (B) obvious hyperchromasia (C) multipolar mitotic figures
- 21. • Prognosis – (contd..) • 4. Size • 5. Extensive tubular differentiation*** • 6. Skeletal muscle differentiation*** • 7. Post-chemotherapy morphology • 8. TP53 mutation • 9. Loss Of Heterozygosity at 1p and 16q
- 22. Anaplastic (“Unfavorable Histology”) Wilms Tumor. Marked pleomorphism with giant hyperchromatic nuclei and atypical mitoses.
- 23. Anaplastic (“Unfavorable Histology”) Wilms Tumor. Strong nuclear immunoreactivity for p53.
- 26. 3)Cystic Partially Differentiated Nephroblastoma • Multilocular, exclusively cystic neoplasm containing nephroblastomatous tissue • Large size ,mean diameter 10 cm • Well circumscribed and consist cysts of variable size. Thin septa.
- 27. Histopathology- • Cysts separated by septa • Flattened, cuboidal or hobnail epithelium • Primitive nephroblastic elements focally within septa
- 28. 4) Paediatric cystic nephroma • Multilocular, cystic • Septa - fibrous tissue & differentiated tubules • Histopathology- Cysts separated by septa • D/D- Cystic partially differentiated nephroblastoma • Genetic profile- DICER 1 mutations.
- 29. Gross appearance of pediatric cystic nephroma involving most of the kidney
- 30. • . Low-power microscopic appearance - Multiple cysts lined by flattened epithelium separated by cellular spindle cell stroma
- 31. The epithelial lining of the cyst has a hobnail quality stroma is loose and hypocellular
- 32. Pediatric Cystic Nephroma-- stroma shows subtle cellularity adjacent to the epithelium, which is more atrophic.
- 33. Type- B) Mesenchymal tumours occurring mainly in children 1) Clear Cell Sarcoma 2) Rhabdoid Tumor 3) Congenital mesoblastic nephroma 4) Ossifying renal tumour of infancy
- 34. 1) Clear Cell Sarcoma Synonym – Bone-metastasizing renal tumour Features- • 4% of malignant renal tumours in childhood • Male:female 2: 1 • Genetics- • Somatic internal tandem duplication in BCOR sequence (85%) • YWHAE-NUTM2 fusion (10%).
- 35. Gross Appearance – Well circumscribed and whitish, bulges on the cut surface
- 36. • Histology- • Nests or cords of cells separated by regularly spaced, arborizing fibrovascular septa Histologic Patterns- 1)Classical 2)Trabecular 3)Myxoid 4)Sclerosing 5)Acinar 6)Palisading 7)Cellular 8)Storiform 9)Anaplastic
- 37. Clear cell sarcoma of kidney, prototypical appearance-branching capillary Vasculature and open chromatin of epithelioid cord cells
- 38. Clear cell sarcoma of kidney, trabecular pattern of growth
- 39. Myxoid pattern
- 40. Sclerosing pattern -- resembling osteoid
- 41. - • Acinar pattern Resembling nephroblastoma Palisading pattern Resembling schwannoma
- 43. • Metastasis- Skeletal metastases,particularly skull Regional lymph nodes, brain, lung, liver *Prognosis- • Treatment with doxorubicin • Stage • Age at diagnosis • Tumor necrosis -- all are independent prognostic factors
- 44. 2) Rhabdoid Tumor • Highly Aggressive • Part of SMACRB1-associated neoplasms • Epidemiology- 2% of renal neoplasms in children • Clinical features- • Haematuria , abdominal mass • CNS counterpart : Atypical Teratoid/ Rhabdoid tumour(posterior fossa) (15%)
- 45. Gross- • Solid ,soft ,well circumscribed • Microscopy- • Large eosinophilic hyaline globule displace nucleus laterally (Plasmacytoid)
- 46. - • Cytological triad of – • Vesicular chromatin • Prominent cherry-red nucleoli • Hyaline pink cytoplasmic inclusion
- 47. Rhabdoid Tumor of Kidney. The nuclear grade is high. An eosinophilic amorphous (“hyaline”) material fills the scanty cytoplasm and pushes the nucleus aside
- 48. - Diffuse growth of neoplastic cells Sheet like Diffuse pattern Of monomorphic Neoplastic cells Overrunning a Native glomeruli
- 49. Syncytial sheets of highly atypical cells, with admixed inflammation.
- 50. • Molecular Genetics- • hSNF5/INI-1 (chr22q11.2) deletion/mutation • SMARCB1 retaining – SMARCA4/BRG1 • Markers- • Vimentin or cytokeratin • Loss of SMARCB1 (hSNF5) • Prognosis- * High tumor stage and male sex -unfavorable
- 51. 3)Congenital mesoblastic nephroma *Low-grade fibroblastic neoplasm *Most common renal tumor of newborns(2-4%) • Clinical features * 90% <1 yr age • Abdominal mass
- 52. - • Macroscopy- • Solid • Yellow-gray • Well-circumscribed and its fibrous cut surface well illustrated
- 53. Classic CMN- monotonous proliferation of spindle cells with bland nuclei, resembling “Infantile Fibromatosis”
- 54. Cellular CMN- Accompanied by Mitotic Activity. Resembles “Infantile Fibrosarcoma”
- 55. • Genetic profile • Polysomy of chromosomes 8, 11, 17,20 *Cellular CMN – t(12;15)(p13;q25) results in ETV6–NTRK3 gene fusion Treatment & Prognosis- *Nephrectomy *7% recurrence with local invasion *Age at diagnosis & adequacy of excision -more important than morphology
- 56. 4)Ossifying renal tumour of infancy • Intracalyceal mass composed of - 1) Osteoid trabeculae 2) Osteoblast-like cells 3) Spindle cell component * Arise from and attached to Medullary Pyramid. • Clinical features- • Male predominance, • Age <2 years • Gross haematuria **
- 57. • Macroscopy- • Well circumscribed , 1 -6 cm diameter • Histopathology • Osteoid core • Osteoblastic cells within and periphery • Bland spindle cells • Genetic profile • Relation with other paediatric renal neoplasms uncertain • A small number of cases shown trisomy 4
- 58. Ossifying renal tumour of infancy
- 59. My references- • 1) WHO Classification of Tumours of Urogenital system -2016 • 2) ROSAI AND ACKERMAN’S Surgical Pathology 11th Ed • 3) Sternberg's Diagnostic Surgical Pathology 6th Ed
- 60. -