肾脂肪肉瘤(附三例报告及文献复习)

本文报道三例肾脂肪肉瘤,并结合文献复习,讨论了其病理,临床表现、影像学特征、诊断和治疗。     

53例脂肪肉瘤形态学观察与预后探讨

报道53例脂肪肉瘤(高分化型7例、粘液型34例、圆细胞型3例及多形性型9例)。男性34例,女性19例。高发年龄41～60岁。主要症状是无痛性(51/53)或痛性肿块。肿瘤直径为3～24cm,多数有包膜(37/53)。8例作了超微结构观察,分别对四型脂肪肉瘤的超微结构特征进行了详细描述。随访结果显示圆细胞型预后差,高分化型预后好;肿瘤体积大,核分裂象多者预后差;肿瘤体积小,核分裂象少者预后好。     

Inflammatory malignant fibrous histiocytomas and dedifferentiated liposarcomas: histological review, genomic profile, and MDM2 and CDK4 status favour a single entity

Inflammatory malignant fibrous histiocytoma (inflammatory MFH) is a very rare tumour that occurs most often in the retroperitoneum. So far, it has been considered to be a special subtype of MFH. As it is now widely accepted that most retroperitoneal pleomorphic MFHs are dedifferentiated liposarcomas, the present study compared histological features, genomic profile (CGH analysis), and MDM2 and CDK4 status (immunohistochemistry, FISH, and quantitative PCR) in inflammatory MFHs from 12 patients and dedifferentiated liposarcomas that had an inflammatory MFH component from eight patients. Metaphase cytogenetic and FISH analyses were also performed on one inflammatory MFH. Histological review showed areas of well-differentiated liposarcoma in nine inflammatory MFHs. CGH analysis showed 12q13-15 amplification or gain in six of seven inflammatory MFHs and in seven of seven dedifferentiated liposarcomas. Immunohistochemistry showed positivity of tumour cells for MDM2 in every tumour in both groups and for CDK4 in ten and seven inflammatory MFHs and dedifferentiated liposarcomas, respectively. Metaphase cytogenetic and FISH analysis performed on one inflammatory MFH showed the presence of a supernumerary large marker chromosome and ring chromosome with high-level amplification of both MDM2 and CDK4 genes. FISH analysis on paraffin wax-embedded sections showed amplifications of MDM2 and CDK4 in seven of seven inflammatory MFHs and in seven of seven dedifferentiated liposarcomas. Quantitative PCR showed amplification of MDM2 in six and of CDK4 in seven of nine inflammatory MFHs. In conclusion, this study strongly suggests that most so-called inflammatory MFHs are dedifferentiated liposarcomas.     

Dedifferentiated liposarcoma with chondroblastic osteosarcomatous dedifferentiation

We describe a rare case of dedifferentiated liposarcoma with features resembling chondroblastic osteosarcoma in the dedifferentiated component. The tumor was removed from the left thigh in a 78-year-old male. It consisted of a well-differentiated liposarcoma and an anaplastic component that contained numerous osteoid and cartilaginous tissues surrounded by high-grade spindle cell sarcoma. To our knowledge, only two cases similar to the divergent chondroblastic osteosarcomatous dedifferentiation of this disease have been reported in the literature.     

Intramuscular dendritic fibromyxolipoma: Myxoid variant of spindle cell lipoma?

Dendritic fibromyxolipoma (DFML) is an uncommon, recently described, benign soft tissue lesion that shares many clinical and pathological features with myxoid variants of spindle cell lipoma (SCL). As described, DFML is distinguished from SCL by the presence of dendritic cytoplasmic processes, abundant keloidal collagen and a prominent, often plexiform vascular pattern. We describe the first known reported case of an intramuscular DFML that occurred in the right shoulder region of a 73-year-old man. The tumor displayed the typical histopathological features of DFML but also included foci of chondroid metaplasia, a previously unreported finding. This report also discusses the differential diagnosis, particularly distinguishing DFML from SCL and myxoid liposarcoma. In view of the similarities in many clinical and pathological features between SCL and DFML, we speculate that DFML probably represents an unusual variant of myxoid SCL.     

十二指肠原发性去分化脂肪肉瘤1例

患者男,79岁,外院腹部CT发现十二指肠病变1周;既往有胃溃疡病史。查体未见明显异常。腹部CT:十二指肠降段管壁增厚、管腔狭窄,增强后局部明显不均匀强化,内见条状稍高密度影,部分层面紧邻胰头(图1A、1B)。MRI:十二指肠降段管壁明显不均匀增厚达2.3 cm,肠腔狭窄,T1WI呈稍低信号,T2WI为稍高信号,扩散加权成像(b=800 s/mm 2)呈高信号;增强后管壁不均匀明显强化,周围脂肪间隙尚清晰,部分层面与邻近胰头分界欠清(图1C)。     

38例腹膜后脂肪肉瘤的临床分析

目的 分析腹膜后脂肪肉瘤的治疗结果。方法 1958～1998年我院收治38例腹膜后脂肪肉瘤。21例为首程治疗,17例局部复发后再程治疗。21例初治患者均接受手术治疗,手术完整切除率为86％。3例接受术后放射治疗。17例外院术后复发再程治疗患者有13例在我院接受手术,4例接受单纯放疗。再程手术完整切除率为54％,1例接受术后放放射治疗。放疗剂量DT30Gy～70Gy,中位剂量50Gy。结果 全组患者5年总生存率为50％,5年远转率为7.3％,5年局部复发率为65％,初程治疗和再程治疗组的5年总生存率为53％和48％,无显著性差异(P=0.293,5年局部复发率为59％和74％,无显著差别(P=0.615)。全组手术完整切除组5年生存率为58％,部分切除或未切除者5年生存率为27％,有显著性差异(P=0.006)。34例有肿瘤大小记录的患者,其中4例肿瘤<10cm,均存活;22例肿瘤10～30cm,有13例死亡;8例肿瘤≥30cm,有6例死亡。结论 腹膜后脂肪肉瘤的治疗以手术为主,手术完整切除可显著提高生存率。经积极治疗,复发患者仍能取得很好的效果。