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- 女30Y，大腿肿物5Y，直径3cm
图片不清楚，至少要考虑和排查脂肪肉瘤。
Pleomorphic liposarcoma
Definition
● Widespread high grade, pleomorphic tumor cells with some lipoblasts but no evidence of well differentiated liposarcoma
Epidemiology
● 5% of all liposarcomas
● No gender preference
● Median age 54-70 years
● Rare in childhood
● Associated with radiation therapy or neurofibromatosis
Sites
● Thigh (29%), internal trunk (21%), limb girdles (18%), upper extremity (16%), thoracoabdominal wall (10%)
● Often deep seated or extracompartmental
Treatment and prognosis
● Wide local excision or amputation with postoperative radiotherapy (AJSP 2004;28:1257), also chemotherapy
● Aggressive; often recurs locally (40%), metastasizes (40%), causes death (35%)
● 5 year overall survival 60%
● Poor prognostic factors: age 60+, truncal (central) location, deep, size >
Gross description
● Often large (median 8
● Usually deep (subfascial), often myxoid or necrotic areas
Micro description
● Well circumscribed but non-encapsulated with infiltrative borders
● At least focal typical liposarcomatous areas
● Pleomorphic cells cover > 65% of cut surface with MFH-like, round cell liposarcoma-like (without vascular network), spindle cell liposarcoma-like or epithelioid cells (Mod Path 1999;12:722)
● Usually high grade with enlarged round to bizarre nuclei; tumor necrosis common; median 25 mitotic figures/10 HPF
● May have neutrophils within giant cells, hemangiopericytic foci, extra- and intracellular hyaline droplets
Positive stains
● Vimentin, S100 (lipogenic areas in 33%), smooth muscle actin (45%), CD34, keratin (21%), desmin (13%)
Electron microscopy
● Abundant coalescing lipid droplets, numerous cytoplasmic organelles
Differential Diagnoses
● Dedifferentiated liposarcoma - has well differentiated component
● MFH-pleomorphic - no definite fat vacuoles
● Pleomorphic rhabdomyosarcoma - skeletal muscle differentiation
● Metastatic carcinoma - keratin+ or other evidence of epithelial differentiation, no liposarcomatous differentiation
此外还可以IHC鉴别上皮样肉瘤。
本例可能是具有非典型性组织学特征的腺泡状软组织肉瘤
Enzinger and Weiss：The individual cells are large, rounded, or more often polygonal and display little variation in size and shape. They have distinct cell borders and one or more vesicular nuclei with small nucleoli and abundant granular, eosinophilic, and sometimes vacuolated cytoplasm. Mitotic figures are scarce. Rare pleomorphic tumors have been reported in the literature ——Evans HL: Alveolar soft-part sarcoma. A study of 13 typical examples and one with a histologically atypical component. Cancer 1985; 55:912.
腺泡状软组织肉瘤的边缘常看见扩张的静脉，并常见脉管浸润，很类似于副节瘤或血管周细胞瘤
回复96298，免疫组化不支持，期待你们做进一步的工作，看看最后诊断？学习、学习。
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