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ONCOCYTOMA & AML

ONCOCYTOMA & AML. ONCOCYTOMA. Recent diagnosis… since 1975 Previously called glandular cell RCC Occurring in Salivary glands,Thyroid glands,Parathyroid glands,Adrenal and Kidney ONKOUSTHAI. Forms 3-5 % of solid renal tumors M:F 2:1 Average age 5-6 decade Two thirds detected incidentally

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ONCOCYTOMA & AML

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  1. ONCOCYTOMA & AML

  2. ONCOCYTOMA • Recent diagnosis… since 1975 • Previously called glandular cell RCC • Occurring in Salivary glands,Thyroid glands,Parathyroid glands,Adrenal and Kidney • ONKOUSTHAI

  3. Forms 3-5 % of solid renal tumors • M:F 2:1 • Average age 5-6 decade • Two thirds detected incidentally • 4% B/L and 90% confined to capsule • ONCOCYTOMATOSIS

  4. Morphology • Tan – mhorgagney brown..lipochrome • Well circumscribed • Encapsulated….not truly… • Homogenous tumor • Central scar • If cysts are present….central near scar • No necrosis or hemorrhage

  5. Oncocyte • Oncocyte……origin from itercalated cells of CD • Large polygonal epithelial cell • Eosinophillic/acidophillic • Abundant mitochondria expanding the cytoplasm( swollen & densely packed mito) • Organoid/tubulocystic/solid/mixed pattern • Less microvillus & absent brush border • Low nuclear grade I-II • Archipelagic architexture

  6. Atypical features • 10-20% can have perinephric fat extension • 20-30% can have hemorrhage • 7-32% can have co-exsistant RCC • 4-13% can be B/L or multiple.

  7. Genetic defects • CH….I • CH….11 • CH…..14 • CH….Y • No changes in CH….3

  8. Investigations • ANGIO : spoke wheel pattern,orderly vascular arrangement,absent AV shunting, no vascular puddling, peritumoral halo (lucent-rim sign). • CT : homogenous mass,stellate scar, • MRI : well defined loww intensity mass on T2 image • SESTAMIBI Tec 99 • Stellate scar : 1/3 by CT and ¼ by USG

  9. Eosinophillic…chromophobe RCC CH ..3 defects Stains for colloidal iron Chromophobe type vesicles. Chromophobe RCC demonstrates vimentin positivity, Oncocytoma CH..1/11/14/Y defects Abundant mito No atypia and nuclear grade I-II oncocytoma shows cathepsin H positivity Diff.diagnosis

  10. FNAC NO to biopsy • Little material to differentiate RCC and oncocytoma • 7-32 % both RCC and oncocytoma co-exist • Within RCC some areas may resemble oncocytoma

  11. Treatment • <3 cm size …follow up • > 3 cm size…nephron sparing surgery

  12. AML • AML lesion described in 1950s • Mature adipose tissue,smooth muscle cells,thick walled vessels • Origin : perivascular epithelial cells • Two forms sporadic & TSC

  13. TSC GROUP 20% of cases of AML are part of TSC ( and 50% of TSC have AML) F:M 2:1 30 yrs More often symptomatic Accelerated growth often seen More commonly B/L and multicentric EWALT: serial screening in 60 children….. SPORADIC GROUP 80% of AML come under this group More common in females* 5-6 decade Rapid growth, B/L, multiple,symptomatic lesions less common

  14. Wunderlich syndrome….10% cases • Retroperitoneal hemorrhage/flank pain/hematuria/tender mass/shock • Next to RCC….commonest cause of retro peritoneal bleed • Thick blood vessel..no inner intimal layer and elastic layer replaced by fibrous tissue.

  15. PRESENTATION • TRIAD…pain/tender mass/gross hematuria • Other symptoms : anemia/HT/renal failure/nausea/vomiting/loin pain/renal failure • 50% asymptomatic

  16. HISTOLOGY • FAT: immature – mature fat cells • Vessels : thick walled easily friable • Smooth muscles: spindle/round epitheloid cells around vessels

  17. CT PICTURE • -30 to –40 classical fat hu • Fat and calcification----RCC • Homogenous mass with homogenous enhancment • 14% fat not seen… • Fat seen in few cases of RCC • Fat also seen in WILMS,SARCOMA,ONCOCYTOMA

  18. USG PICTURE • Hyperechogenic mass with shadowing ( due to high fat content/multiple tissue interfaces/vascularity) • Few are hypoechogenic

  19. ANGIO….. • Multisacculated pseudoaneurysmal dilatation • Whorl vessel pattern • Absent AV shunting • Sunburst capillary nephrogram • Onion peel peripheral vessels in venous phase

  20. AML is seen as high intensity signel lesion in T1 and low intensity in T2 images RCC is seen as low intensity lesion in T1 and high intensity lesion in T2 images MRI

  21. Extra renal….hilar LN,retroperitonium,liver • ER/PR present • HMB-45 Ab….melanoma associated antigen( also seen in melanoma, cardiac rhabdomyoma,cerebral tubers) • EPITHELOID AML…malignant variant can have local invasion-distant mets.this has abundant epitheliod polygonal muscle cells.

  22. AML size >4cm……80% symptomatic • Sporadic AML …growth rate 5% • TSC/MULTICENTRIC……growth rate 20% • Biopsy ……not advised Bleeding/seedling/negative biopsy dilemma

  23. TREATMENT • Angio embolisation( gelatin/absolute alcohol/iodised oil/polyvinyl alcohol/coils)…5% abscess,3% pleural effusion Post embolization syndrome • Nephron sparing surgery

  24. Nephrectomy • Whole kidney replaced by tumor • Solitay large tumor at hilum • Suspicion of malignancy • Large retro peritoneal bleed

  25. AML with renal vein tumor thrombus • AML with RCC…. 1-3% of TSC….young women • Recurrent AML • AML and pregnency ? Spontanous rupture common ? prophylactical removal >4 cm size

  26. TSC • Bourneville classical description • VOGT triad..MR,seizures,adenoma sebaceum • FORME FROSTE….TCS without MR • Gene……TSC 1 & TSC 2 • TSC 2 more severe disease more renal involvement

  27. TSC gene1 Protein HAMARTIN for cell to ceel adhersion Viz actin and plasma membrane binding protien TSC gene 2 Encodes for TUBERIN which regulates cytoplasmic vesicle transport…defect leads to accumulation of microvesicles in smooth muscle…( GTPase based) TYPES

  28. Neurological manifestations • Cortical tubers • Subependymal nodules • Subependymal giant cell astrocytoma • Seizure disorders • Cognative and behavioral disturbances ………………………vigabratin

  29. Dermatological • Ashleaf spot ( hypomelanotic macule on trunck/buttock) ..UV light/woods lamp • Adenoma sebaceum facial angiofibroma • Shagreen patches lumbosacral angiofibroma • Café au lait spot • Koenen tumors…..periungual fibroma

  30. Renal • AML • RENAL CYST • RCC • ONCOCYTOMA • PREIRENAL CYST • PCK • Renal problems are emerging as primary with good control of seizures • Commonest manifestation is AML

  31. AML IN TSC • Younger age….30 yrs • B/L…84% • Multiple ….91% • 20-45% have also renal cysts • 2/3 show gradual growth • Hmb-45 positive • 4 cm cut off(Oesterling) & 3.5 cm cut off(Van Baal)

  32. Renal cyst in TSC • Second most common renal finding • 18-50% cases • Common in females • 40% cases progress to ESRD • Commonly present as CRF/HT • Cysts have hyperplastic epithelium & abundant eosinophillic cytoplasm

  33. RCC in TSC • Seen in 2-4 % • Age average is 28 yrs • 80% in females • 40% B/L • Usually low grade/low metastatic potentialcommonly clear cell type

  34. CVS & TSC : cardiac rhabdomyoma,peripheral arterial aneurysm • RS & TSC : lymphangioleiomyomatosis • OCULAR : retinal hamartoma • AML IN LIVER

  35. EVALUATION OF TSC • CT brain • CT/MRI abdomen • CXR • Echo • Woods lamp for skin macules

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