Notaci´ on

Poorly differentiated conventional fibrosarcoma, sclerosing epithelioid fibrosarcoma, high grade myxoid/round cell liposarcoma, epithelioid liposarcoma, pleomorphic liposarcoma≠_{, some mixed-type liposarcoma (myxoid/round cell and pleomorphic liposar-}

coma), pleomorphic myofibrosarcoma, “true” conventional malignant hemangiopericytoma, poorly differentiated leiomyosarco- ma, pleomorphic leiomyosarcoma, leiomyosarcoma, pleomorphic leiomyosarcoma, leiomyosarcoma with prominent osteoclastic gi- ant cells, epithelioid leiomyosarcoma≠_{, inflammatory leiomyosarcoma, rhabdomyosarcoma}↑↑↑ ≠_{(any type – embryonal, alveolar,}

pleomorphic), angiosarcoma↑↑↑ ≠_{(any type – conventional, epithelioid, poorly differentiated)} 245 246_{, intimal sarcoma, glomangiosarco-}

ma≠ 255 263_{, malignant peripheral nerve sheath tumor (poorly differentiated, epithelioid, pleomorphic)}≠_{, malignant granular cell tumor} 264_{, malignant Triton tumor, conventional epithelioid sarcoma (“distal-type”, classic form}≠ § _{and variants} 26a,67a,67b_{– fibroma-like, ang-}

iectoid), proximal-type epithelioid sarcoma, extra-renal malignant rhabdoid tumor↑↑↑ ≠_{, synovial sarcoma}≠ §_{, mesenchymal}

chondrosarcoma↑↑↑_{, “high-grade” extraskeletal myxoid chondrosarcoma}262_{and its analogous variant with neuroendocrine dif-}

ferentiation, extraskeletal osteosarcoma↑↑↑ ≠_{(any type – conventional and giant-cell rich), alveolar soft part sarcoma}§_{, clear cell sar-}

coma§_{, soft tissue PEComas (monotypic epithelioid angiomyolipomas)}≠_{, Ewing’s sarcoma / primitive neuroectodermal tumor}↑↑↑ ≠_,

desmoplastic small round cell tumor↑↑↑_{, high grade malignant fibrous histiocytoma group (pleomorphic malignant fibrous histiocy-}

toma [undifferentiated pleomorphic sarcoma], osteoclastic giant cell malignant fibrous histiocytoma [undifferentiated pleomorphic sarcoma with osteoclastic giant cells], inflammatory malignant fibrous histiocytoma / undifferentiated pleomorphic sarcoma with prominent inflammation).

≠_{Primary analogue high grade tumors also described in cutaneous site} 2_{, for which the predicted prognosis should not be applied.} ↑↑↑_{Definitionally}

high grade sarcomas. §_{May give rise to metastasis after a long duration follow-up, despite the absence of local recurrence.}

Clinical and Pathological Parameters influencing Prognosis (“Prognostic Predictors”)265-272

Clinicopathologic features235-237 243 243a 271 _{– e.g. anatomical sites (abdominal cavity, retroperitoneum, head-neck worse than}

limbs, and abdominal and chest wall); tumor location (deep soft tissue worse than skin and subcutis); tumor size (the larger the worse); patient’s age (old age generally more adverse than young); clinical setting (secondary sarcomas arising in benign tumors, immunosuppression-associated tumors, virus-induced tumors, …); others …

Grading systems and histological grades265-271_{–e.g. grade I, II, III according to the French/FNCLCC grading system}266-268_{, which}

evaluates 3 independent parameters (tumor differentiation, mitosis count, and necrosis) each scored 1 to 3: grade I → 2-3; grade II → 4-5; grade III → 6-8.

Special histological types269_{– e.g. definitionally low grade tumors, definitionally high grade tumors, non-gradable tumors, tu-}

mors with not-yet-established grading parameters.

Clinical stage272_{– e.g. TNM/UICC staging system: stage I (A: low grade, small, superficial, and deep; B: low grade, large, superfi-}

cial); stage II (A: low grade, large, deep; B: high grade, small, superficial, and deep; C: high grade, large, superficial); stage III (hig grade, large, deep), stage IV (any metastasis).

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