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首页> 外文期刊>Modern Pathology >Well-differentiated spindle cell liposarcoma (|[lsquo]|atypical spindle cell lipomatous tumor|[rsquo]|) does not belong to the spectrum of atypical lipomatous tumor but has a close relationship to spindle cell lipoma: clinicopathologic, immunohistochemical, and molecular analysis of six cases
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Well-differentiated spindle cell liposarcoma (|[lsquo]|atypical spindle cell lipomatous tumor|[rsquo]|) does not belong to the spectrum of atypical lipomatous tumor but has a close relationship to spindle cell lipoma: clinicopathologic, immunohistochemical, and molecular analysis of six cases

机译：高分化梭形细胞脂肪肉瘤（|||||非典型梭形细胞脂肪瘤|||）并不属于非典型脂瘤的光谱，但与梭形细胞脂肪瘤有密切关系：临床病理学，免疫组织化学和分子分析六例

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Well-differentiated spindle cell liposarcoma represents a rare atypical/low-grade malignant lipogenic neoplasm that has been regarded as a variant of atypical lipomatous tumor. However, well-differentiated spindle cell liposarcoma tends to occur in subcutaneous tissue of the extremities, the trunk, and the head and neck region, contains slightly atypical spindled tumor cells often staining positively for CD34, and lacks an amplification of MDM2 and/or CDK4 in most of the cases analyzed. We studied a series of well-differentiated spindle cell liposarcomas arising in two female and four male patients (age of the patients ranged from 59 to 85 years). The neoplasms arose on the shoulder, the chest wall, the thigh, the lower leg, the back of the hand, and in paratesticular location. The size of the neoplasms ranged from 1.5 to 10?cm (mean: 6.0?cm). All neoplasms were completely excised. The neoplasms were confined to the subcutis in three cases, and in three cases, an infiltration of skeletal muscle was seen. Histologically, the variably cellular neoplasms were composed of atypical lipogenic cells showing variations in size and shape, and spindled tumor cells with slightly enlarged, often hyperchromatic nuclei. Multivacuolated lipoblasts were present in three neoplasms. Focal myxoid stromal changes were seen in three cases. Immunohistochemically, CD34 was at least focally positive in all cases, whereas scattered tumor cells only showed a nuclear expression of MDM2 in two neoplasms. FISH analysis revealed a deletion of the Rb-1 gene in all six cases, whereas no MDM2/CDK4 amplification was identified in all cases tested. Follow-up information was available in four cases (range from 4 to 24 months), and revealed a local recurrence in one case. Although well-differentiated spindle cell liposarcoma and atypical lipomatous tumor behave clinically similar, it can be speculated on the basis of clinicopathologic and molecular findings that well-differentiated spindle cell liposarcoma may constitute an independent entity rather than a morphologic variant of atypical lipomatous tumor, and may represent the atypical/low-grade counterpart of spindle cell lipoma.
机译：分化良好的梭形细胞脂肪肉瘤代表一种罕见的非典型/低度恶性生脂瘤，已被视为非典型脂瘤性肿瘤的变体。然而，分化良好的梭形细胞脂肪肉瘤往往发生在四肢，躯干和头颈部区域的皮下组织中，含有轻微的非典型梭形肿瘤细胞，通常对CD34呈阳性染色，并且缺乏MDM2和/或CDK4的扩增。在大多数情况下进行分析。我们研究了在两名女性和四名男性患者（患者年龄介于59至85岁之间）中出现的一系列分化良好的纺锤状细胞肉瘤。肿瘤出现在肩膀，胸壁，大腿，小腿，手背以及睾丸旁位置。肿瘤的大小范围为1.5至10？cm（平均：6.0？cm）。所有肿瘤均被完全切除。肿瘤局限于皮下组织三例，三例可见骨骼肌浸润。从组织学上讲，可变细胞肿瘤由大小和形状各异的非典型脂肪细胞和纺锤状肿瘤细胞组成，细胞核稍增厚，常为增色核。在三个肿瘤中存在多真空成脂细胞。在三例中可见局灶性粘液样基质变化。免疫组织化学分析，在所有情况下，CD34至少均呈局灶性阳性，而散布的肿瘤细胞仅在两个肿瘤中显示MDM2的核表达。 FISH分析显示在所有6例病例中Rb-1基因均缺失，而在所有测试病例中均未发现MDM2 / CDK4扩增。有4例（4到24个月不等）的随访信息，其中1例显示局部复发。尽管分化良好的梭形细胞脂肪肉瘤和非典型性脂肪瘤的临床表现相似，但可以根据临床病理和分子学发现推测，分化良好的纺锤形细胞脂肪肉瘤可能构成一个独立的实体，而不是非典型脂瘤性肿瘤的形态变异，并且可能代表纺锤体细胞脂肪瘤的非典型/低等对应物。

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