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TUMORS of the KIDNEY - Coggle Diagram

- - - - oncocytoma
        
        papillary adenoma
    - - clear cell
        
        papillary (types I and II)
        
        chromophobe
        
        collective duct
        
        others (uncommon, unclassified)
  - - - blocks must come from the whole tumor
  - - - CLEAR CELL
        MORPHOLOGY
        
        the presence of glycogen makes these cells clear in 99% of cases
        
        PAS-d staining is very useful when making DD with metastatic tumors in the kidney (which frequently produce mucin)
      - EOSINOPHILIC CELL
        MORPHOLOGY
        
        much less common
        
        cells can be lightly or
        densely eosinophilic
        
        densely eosinophilic
        = dark pink =
        ONCOCYTIC FEATURES
      - mixed forms
  - - - indolent by definition
        
        very small (<5 mm)
        
        grey-to-yellow
      - micro
        
        cellular features similar to RCC, papillary type
        
        from morphological and molecular POV, papillary adenomas are exactly the same as papillary carcinomas
        
        evolutive risk is not well understood
    - - most frequent benign
        tumor of the kidney
        
        main issue: DD!
      - specific characteristics
        
        macro
        
        ochraceous with a central scar
        
        architecture
        
        nesting
        
        cytological atypia
        
        mild, but with prominent nucleoli
        
        cytoplasmic features
        
        deeply eosinophilic
        
        invasiveness
        
        absent
        
        high grade features
        
        absent
        
        there may be ischemic-type necrosis
        (due to ALTERED METABOLISM)
        
        chromosomal alterations
        
        TOTAL LOSS OF CHR 1
        
        negative CK7 staining, negative HALE staining
  - - - 90% of kidney tumors
        
        adults
        
        M:F = 3:1
      - risk factors: smoking, hypertension, genetic predisposition (VHL and others)
      - specific characteristics
        
        macroscopy
        
        yellow, not homogeneously
        (there is always hemorrhage)
        
        architecture
        
        alveolar, less diffuse if
        compared to oncocytoma
        
        cytological atypia
        
        dependent on the grade
        
        grade 1: mild;
        grade 3: severe
        
        cytoplasmic features
        
        clear, with some granular appearance
        
        invasiveness
        
        present (at least of the capsule)!
        
        high grade features
        
        not frequent
        
        chromosomal alterations
        
        LOSS OF CHR 3p
    - - benign
        
        from the cytological POV, it is the same as CCC (clear cells and loss of chr 3p)
        
        architecture is different: cystic rearrangements
    - - second most frequent (10%)
        
        M:F = 3:1
        
        architecture is mainly papillary, but not exclusively
        
        25% are metastatic at diagnosis
      - types
        
        type I RCC
        
        type II RCC
      - specific characteristics
        
        macroscopy
        
        yellow with hemorrhage, cystic formation and degenerative changes (i.e. fibrotic changes)
        
        architecture
        
        papillary or solid
        
        more evident in type II
        
        cytological atypia
        
        different between the two types
        
        type I
        
        very mild, better prognosis
        
        more papillary and with mild atypia, also with histiocytes in the vascular stalk of papillae
        
        type II
        
        more present, worse prognosis
        
        no papillae, but rather trabecular growths, larger cells with more pronounced cytological atypia
        
        cytoplasmic features
        
        variable
        
        scant, eosinophilic, sometimes
        clear, latge or small
        
        HETEROGENEITY
        
        invasiveness
        
        present
        
        capsule, vessels, renal sinuses
        
        high grade features
        
        occasional
        
        chromosomal alterations
        
        GAINS OF CHR 7 and 17
        
        IHC
        
        RACEMASE+
    - - third most common
        
        M = F
        
        malignant by definition
        (low clinical malignancy)
        
        HIGHLY RECURRENT
      - specific characteristics
        
        macroscopy
        
        brown with hemorrhage
        
        similar to oncocytoma, but with no central scar
        
        architecture
        
        solid
        
        cytological atypia
        
        mild-to-moderate with some nucleoli
        
        cytoplasmic features
        
        eosinophilic cytoplasm with clear perinuclear halo
        
        invasiveness
        
        +++
        
        high grade features
        
        rare
        
        chromosomal alterations
        
        LOSS OF CHR 1, 6, 10, 17
        
        IHC
        
        parvalbumin
    - - the diagnosis is challenging
        
        clear cells are typically observed
        
        immunophenotype is different from that of CCC:
        THE TUMOR IS POSITIVE FOR CAIX and CK7
        
        loss of chromosome 3p is seen in CCC and CCTPC
    - - between oncocytoma and chromophobe: typical findings of oncocytoma and chromophobe may be seen
      - 10% of tumors of eosinophilic type
    - - part of MiT renal cell carcinomas
        
        histology: large cells with eosinophilic changes and higher degree of atypia
        
        translocation of TFE3
        
        MiT tumors present the expression of:
        
        TFE3 or TEFB
        
        PAX8 and CD10
        
        cathepsin K
        
        AMACR
    - - part of MiT family
        
        translocation of TEFB
        
        morphology: clear cells with a glomerular structure
    - - similar to oncocytomas or chromophobe RCC as eosinophilic cells are observed
        
        anyway, the diagnostic clue is the presence of SDH loss
        
        always look for SDHB loss at IHC
    - - based on the recognition of the presence and the size of nucleoli
      - additional findings useful in assessing the biological aggressiveness
        
        nuclear pleomorphism, giant cells and sarcomatoid/rhabdoid changes
    - - based on the size of the tumor and the depth of invasion
        
        the most important and typical sign is HEMATURIA
      - T1: <7 cm (T1a <4 cm; T1b: 4-7 cm)
        
        T2: >7 cm, limited to the kidney
        
        T3: major veins or perinephritic tissues extension
        
        T4: invasion beyond Gerota's fascia
    - - every patient being diagnosed with RCC can be treated with these drugs, but it is not possible to know before whether the patient will respond to the treatment or not
        
        generally, theraoeutic strategies consist of:
        
        anti-angiogenic drugs
        
        monoclonal antibodies
        
        inhibitors of tyrosine kinase
    - - VHL --> CCC
        
        tuberous sclerosis --> angiomyolipoma
        
        familial P-RCC: familial-only form or familial form + papillary thyroid carcinoma (associated with c-MET mutations)
        
        Birt-Hogg-Dube --> oncocytic, chromophobe and hybrid
        
        hereditary uterine leiomyomatosis and RCC --> FH mutations
        
        Cowden syndrome --> different histotypes
        
        hyperPTH-jaw tumor syndrome --> parafibromin gene mutation
        
        RCC with constitutive chr3 translocation (MiT family)
        
        SDH-deficient tumors
        
        BAP1 hereditary cancer predisposition --> CCC
        
        MITF-associated --> susceptibility to melanoma and RCC syndrome in chX
    - - age <46
        
        multifocal/bilateral tumors
        
        at least one fisrt degree relative with RCC
        
        unequivocal histology
    - - typically seen in children (median age: 40 months); silent nephroblastomas may
        be diagnosed in elderly
        
        blue cell tumor of infancy
        
        M = F
        
        not extremely rare
        
        morphology is enough to make
        the diagnosis (no markers needed)
        
        main prognostic marker: ANAPLASIA
      - morphologically, it is a mix of embryonal, stromal and epithelial structures
        
        look for not well-formed tubuli, which appear to be mimicking glands
        
        mind the density of cells and the presence of immature fibrotic differentiation
- - - - difficult DD with epithelial tumors
        
        remember that in the case of urothelial tumors cytokeratins are negative, while melanocytic markers are positive
        
        overactivation of mTOR pathway is associated with germline mutations involving the TSC1/2 inctivation (typically associated with TS or somatic mutations)
    - - unspecific markers
        
        smooth muscle: Sm actin
        
        blood vessels: CD31 and VEGFR3
      - specific markers
        
        melan A, HBM45 and SOX10