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SOFT TISSUE TUMORS - Coggle Diagram

- - - - DD with other soft tissue tumors
    - - DD with other GIT tumors
  - - - no prognostic meaning
  - - - important for therapy
      - not needed for diagnosis
  - - - germline mutations in c-KIT and PDGFR alpha
    - - somatic mutations of the same genes
- - - - macroscopy: well defined or infiltrative
        
        easily distinguishable from the surrounding tissue
      - microscopy: different cell types and architectural patterns
        
        cell types
        (1st level):
        
        spindle cells
        
        round cells
        
        epithelioid cells
        
        pleomorphic cells
        
        architectural patterns
        (2nd level):
        
        alveolar
        
        glandular
        
        storiform
        
        palisading
        
        LIPOBLAST is specific of liposarcoma!
        
        cells with large nucleus and multiple vacuoles in the cytoplasm
        
        well-differentiated liposarcoma
        (aka atypical lipoma)
        
        malignant tumor, locally aggressive
        
        round cell liposarcoma
        
        myxoid liposarcoma
        
        most common histotype
        
        found in the retroperitoneum
        
        pleomorphic liposarcoma
        
        totally undifferentiated
        
        dedifferentiated liposarcoma
        
        transition from one type of differentiation to another one, e.g. from a myxoid liposarcoma to a leiomyosarcoma
    - - macroscopy: well defined borders / encapsulation
        
        often difficult to distinguish from the surrounding tissue
      - microscopy: easily attributable to a mature connective tissue
  - - - S100+ is frequent, but not specific
      - ALWAYS CONSIDER OTHER TISSUE-SPECIFIC ANTIGENS
        
        podoplanin: lymphatic vessels
        
        CD31 or VEGFR3: blood vessels
        
        smooth muscle actin: smooth muscle
        
        sarcometic actin: skeletal muscle (rhabdomyosarcoma)
        
        MyoD1: TF specific for cells of skeletal muscle
        
        cytokeratins: peripheral nerve sheath cells
        
        TF markers are nuclar, proteins are cytoplasmic
  - - - chronic sporadic
        
        lymphadenopathic
        
        associated to organ transplantation
        
        HIV-associated
        
        look for the presence of HHV8 and vascular markers
        
        CD31, VEGFR3
  - - - pros
        
        comprehensive information in a single test
        
        screening for unknown primary and secondary alterations
        
        sensitive and specific
        
        applicable also to cytological material
      - cons
        
        need for fresh wall preserved samples
        
        long timing for cell culture
        
        small alterations cannot be detected
      - array-CGH
    - - FISH
        
        pros
        
        applicable to fixed tissue material
        
        spatial resolution
        
        detection of small anomalies
        
        rapid execution
        
        cons
        
        "target" approach
        
        need for fluorescence microscope
        
        possibilities
        
        BREAK APART PROBES:
        
        it is known where the fusion is occurring
        
        the probe is built just before and after the point of break
        
        every kind of fusion is seen
        
        more commonly used
        
        BREAK THROUGH PROBES:
        
        it is necessary to have one probe on one gene and the other on the other gene
        
        in the presence of the fusion, the two signals will go together
        
        more expensive
      - amplifications
        
        MDM2 is specific for well differentiated liposarcoma and de-differentiated liposarcoma
        
        leyomiosarcoma is negative for this mutation
      - fusions
        
        synovial sarcoma
        
        young adults
        
        ubiquitous, limbs/joints
        
        monophasic or biphasic
        
        biphasic = spindle cells with storiform growth and epithelioid clls gorming glands
        
        it is more commonly monophasic
        (spindle cell appearahce)
        
        95% --> SS18 fusion
        
        not found in the case of:
        
        technical artifacts
        
        misdiagnosis
        
        undifferentiated small round cell sarcomas of bone and soft tissue
        
        Ewing sarcoma
        
        round cell sarcoma with EWSR1-non-ETS fusions
        
        CIC-rearranged sarcoma
        
        sarcoma with BCOR genetic alterations
        
        in the case of Ewing sarcoma --> look for ESWR1- FLI1 fusion
      - NGS
        
        fast, but very expensive technique
- - - - Li-Fraumeni synfrome (tp53)
        
        osteosarcoma, rhabtomyosarcoma
      - APC gene mutation
        
        polyposis-related cancer in colon and desmoid disease
      - FH gene mutation
        
        renal cell carcinoma
      - vascular involvement
        
        hemangioblastoma
- - - - adipocytic tumors
        
        fibroblastic tumors
        
        fibrohistiocytic tumors
        
        vascular tumors
        
        pericytic tumors
        
        skeletal/smooth muscle tumors
        
        GUST
        
        peripheral nerve sheath tumors
        
        tumors of uncertain differentiation
- - - - they are two different entities: they cannot transform one into the other
    - - generally, hematogenous
        
        lungs
        
        liver
        
        bone
        
        brain