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ADRENAL PATHOLOGY - Coggle Diagram

- - - - high mitotic index
      - frequent necrosis
    - - <5 years old
    - - regression to a benign lesion, e.g. ganglioneuroma
      - sometimes
        therapy-related
    - - whitish
      - soft
      - extra-adrenal extension
        
        retroperitoneum
        
        anterior mediastinum
    - - small cells
        
        lymphocyte-like
        
        only nucleus, no cytoplasm
        
        "blue small cell tumor of infancy" family
        
        NEUROBLASTS
        
        precursors of neurons
        
        +++ neurofilaments
        
        ++ synaptophysin and chromogranin A
        
        sometimes, ++ catecholamines
        
        when the tumor arises from the adrenal medulla
      - diffuse growth
      - rosettes formation
    - - lymphomas
        
        due to lymphocytes, which are small by definition
      - blastomas
        
        embryonal-like tumors, mainly formed of small blue cells
      - small cell lung cancer
        
        adults
    - - age <1 year
      - low stage
      - microscopic gangliar differentiation
      - low mitotic index
      - presence of calcifications
      - absence of MYC amplification
      - histological type of tissue
        
        100% undifferentiated tissue is an index of negative prognosis
  - - - malignant
        
        bilateral
        
        pediatric
        
        extra-adrenal
        (paraganglioma)
        
        hereditary
        
        paraganglioma
        
        pheochromocytoma involving paraganglia
        
        neural crest derivation
        
        secretion of catecholamines + enkephalins
        
        type of stimulation
        
        sympathetic
        
        para-/pre-vertebral sympathic ganglia
        
        neural structures of pelvic/retroperitoneal organs
        
        neuronal stimuli
        
        parasympathetic
        
        chemical stimuli
        
        1 more item...
        
        cervical and thoracic location (vagus/glossopharingeal nerves)
        
        hormonal product
        
        sympathetic
        
        epinephrine (and a little norepinephrine)
        
        parasymopathetic
        
        norepinephrine and dopamine
        
        type of neoplasm
        
        sympathetic
        
        paragangliomas
        
        neuroblastomas
        
        ganglioneuromas
        
        ganglioneuroblastomas
        
        frequent catecholamine-related symptoms
        
        parasympathetic
        
        paragangliomas
        
        rare catecholamine-related symptoms
        
        macroscopic pathology
        
        same as pheochromocytoma
        
        reddish
        
        microscopic pathology
        
        smaller clusters
        
        small cells
        
        same architecture of pheochromocytoma
        
        alveolar structure surrounded by S100++ cells
        
        main DD: ACC
        
        paraganglioma
        
        S100 and chromogranin A +++
        
        ACC
        
        melanin A and calretinin +++
        
        synaptophysin +++
        &
        cytokeratin ---
    - - clinical features can be decided already
        
        clinically benign
        
        well-encapsulated
        
        reddish
        
        exactly like the medulla itself
        
        homogeneous
        
        clinically malignant
        
        unencapsulated
        
        invasion of the capsule/extra-adrenal tissue
        
        hemorrhagic/necrotic foci
    - - very large and eosinophilic/basophilic cells
      - high levels of atypia
      - alveolar architecture
    - - CgA
        
        typical neuroendocrine marker
      - S100
        
        from sustentacular cells, with a probable osteocytic origin
    - - PASS score is not used anymore
      - GAPP score
        
        cellularity
        
        vascular/capsular invasion
        
        Ki-67 index
        
        architecture
        
        clinical information
        
        about architecture
        
        Zellballen
        
        alveolar architecture + sustentacular cells
        
        well-differentiated
        
        large and irregular nest
        
        pseudorosette formations
        
        loss of differentiation
        
        about clinical information
        
        catecholamines production
        
        type of hormones
        
        rarely applicable
  - - - MEN2
      - VHL
      - NF1
    - - "dominant"
        
        alone or with other lesions
      - germline mutations
        
        SDH
        
        pheochromocytoma
        &
        GI stromal tumor
        
        MAX
        
        pheochromocytoma only
- - - - glomerulosa
        
        small cells
        
        aldosterone
      - fasciculata
        
        large cells
        
        cortisol
      - reticularis
        
        intermediate cells
        
        sexual steroids and cortisol
- - - - ONCOCYTIC VARIANT
        
        oncocytic means diffusely growing with eosinophilic cells and atypia
        
        by applying the Weiss system, everything that is oncocytic will be identified as cancer
        
        for this reason, major parameters are:
        
        vascular invasion
        
        mitotic index >5 in 50 HPF
        
        only one is enough for cancer definition
        
        also consider minor parameters:
        
        necrosis
        
        capsular invasion
        
        sinusoidal invasion
        
        size and weight
        
        atypical mitoses
      - MYXOID VARIANT
        
        some WEISS system criteria cannot be evaluated in this case
        
        growth tyoe
        
        synusoidal invasion
        
        small tumors, with no atypia
        
        risk of underestimation
  - - - cutoff >8
- - - - main markers to consider:
        
        SF1
        
        only found in cells producing steroid hormones
        
        Leyding cells in the testis
        
        stromal cells in the ovary
        
        diffuse in ACC
        
        in the case of metastases,
        +++ in steroidogenic tumors ONLY
        
        --- in all the other types of metastases
        
        melan A
        
        +++ focal/diffuse in ACC
        
        +++ in melanoma and other rare neoplasms
        
        quite sentitive, not specific
        
        chromogranin A
        
        --- in ACC
        
        +++ in pheochromocytoma (adrenal medulla lesion)
        
        cytokeratins
        
        --- in ACC
        
        +++ in carcinomas and metastases
        
        after confirmation of ACC diagnosis, perform grading
        
        mind Ki-67,
        the proliferation index
        
        if Ki-67>10%
        
        adjuvant MITOTANE
        
        different from paraganglioma/pheochromocytoma, where it only indicates the risk (no adjuvant treatment even if Ki-67 >50%)
        
        consider
        mitotic figures
        
        5 MFs in 10 mm2
        
        CARCINOMA
        
        20 MFs
        
        HIGH-GRADE
    - - predisposition for adrenocortical lesions in general
        
        MEN1
        
        FAP
        
        Beckwith-Wiedemann
      - predisposition for ACC only
        
        Li-Fraumeni
        
        Lynch
      - high frequency of TP53 polymorphism in southern Brazil