去分化脂肪肉瘤复发1例报告

去分化脂肪肉瘤是原发性或继发性恶性脂肪细胞性肿瘤,一般为大的无痛性肿物,腹膜后受累最常见,四肢软组织发病较少见[1-2]。我院于2010年7月收治1例发生于肢体脂肪肉瘤患者,经手术切除病理诊断为去分化脂肪肉瘤,现报告如下。1资料与方法1.1临床资料     

眼眶复发性去分化脂肪肉瘤1例

<正>患者男性,52岁,因右眼眼球突出半年余至我院就诊。既往4年前行同位置肿物切除,外院病理诊断为梭形细胞肿瘤,未行辅助治疗。查体：一般情况好,心肺腹未见明显异常。视力：右眼0.6,左眼1.0;眼压：右眼21 mmHg,左眼20mmHg。右眼球向外、下方移位隆起,鼻侧结膜下见暗红色肿物,双角膜清,前房不浅,Tyndall征阴性,虹膜纹清,瞳孔圆,对光反应存,晶体清,视网膜在位。     

去分化脂肪肉瘤的病理学研究进展

脂肪肉瘤是最常见的恶性软组织肿瘤,其形态学范围宽广,可分为多种组织学亚型,其中在形态学和生物学上最令人迷惑的是去分化脂肪肉瘤,它们在形态学上与多种不同的肿瘤极为相似,常造成病理诊断困难。该文对去分化脂肪肉瘤的的概念、发病情况、细胞和分子遗传学特征、病理学特征以及病理诊断与鉴别诊断方面的新进展作了综合介绍,有助于提高对去分化脂肪肉瘤的认识及病理诊断水平。     

去分化软骨肉瘤11例临床病理分析

目的探讨去分化软骨肉瘤的临床、影像学及病理学特点。方法收集11例去分化软骨肉瘤患者的临床及影像资料,对组织进行病理分析。结果患者以男性多见(7/11),平均年龄57.1岁,肿瘤多位于长管状骨及骨盆。影像学显示广泛的骨质破坏,病灶内可有钙化或骨化,病理性骨折3例。眼观:病理软骨成分常位于骨内,去分化肉瘤成分主要位于骨外。镜下包括高分化软骨肉瘤和低分化间叶源性肉瘤两种成分。随访材料中6例死亡,平均存活14个月。结论去分化软骨肉瘤检:呈少见的的软骨肉瘤亚型,去分化类型多样,只有加深对其的认识,才能提高诊断的准确率。     

伴脑膜上皮样旋涡和骨化的去分化脂肪肉瘤

目的：探讨伴脑膜上皮样漩涡和骨化的去分化脂肪肉瘤的形态特点,方法：应用常规病理和免疫组化对1例伴脑膜上皮样旋涡和骨化的去分化脂肪肉瘤进行临床病理分析并复习相关文献。结果：肿块位于后腹膜,直径17cm,包膜不完整,切面灰白色,肿瘤中大量梭形细胞排列成多个脑膜瘤样漩涡,有明显的纤维化,淋巴细胞浸润和出血,邻近漩涡处见成熟的骨小梁形成;肿瘤内见分化良好的硬化性脂肪肉瘤区域,同时伴有认性纤维肉瘤区域,漩涡与硬化性脂肪肉瘤区域,硬化性脂肪肉瘤与黏液性纤维肉瘤区域二者之间界限清楚,呈突然转变之势,结论：去分化脂肪肉瘤伴脑膜上皮样漩涡和骨形成是十分罕见的,脑膜上皮样漩涡 的组织可能来源于类似周细胞或肌纤维母细胞的间叶细胞。     

87例脂肪肉瘤的临床病理学研究：—附17例去分化脂肪肉瘤

87例脂肪肉瘤,40岁以上占70%,发生于深部软组织,以下肢和腹膜后多见。组织学类型分为粘液型、圆细胞型、多形性和分化良好型(包括脂肪瘤样型和硬化型),以分化良好型最多见(54%)。局部切除常有复发,但很少转移。其中17例发生去分化,占总数的20%。向恶性纤维组织细胞瘤分化者最多(47%)。本文还对本病的诊断、鉴别诊断和组织发生进行了探讨。     

阴囊高分化脂肪肉瘤误诊为黏液性脂肪肉瘤1例

患者男性,63岁,因右下腹痛2天余,以急性阑尾炎收治入院。阑尾炎术后发现患者左侧腹股沟区有一肿块,患者自述肿块存在2年余。查体：站立位,左侧腹股沟区可见一局限性隆起型肿块,进入阴囊,12 cm ×9 cm大小,质地中等偏硬,无压痛,平卧后肿块不消失,不能回纳,透光试验阴性。遂行左侧腹股沟斜疝无张力修补术+左侧阴囊内肿块切除术,术中见阴囊内肿块呈实性,质偏硬,从左侧阴囊底部向内上方将肿块推出左侧腹股沟区切口,完整切除肿块。术后行核磁共振检察,腹膜后未见占位性改变。     

去分化脂肪肉瘤潜在核心基因的生物信息学预测及分析

目的 探讨去分化脂肪肉瘤的潜在核心基因在其恶性生物学行为中的作用.方法 获取基因表达数据库(gene expres-sion omnibus,GEO)数据库中GSE21122和GSE52390的芯片数据,通过GEO2R筛选差异表达基因,对差异表达基因进行GO功能、KEGG通路富集分析和蛋白互作分析,并用Cytoscap...     

腹膜后去分化脂肪肉瘤1例

腹膜后去分化脂肪肉瘤１例１汪露祥２王保太患者女，５２岁。因右季肋部疼痛，Ｂ超示“右上腹腹膜后实质性包块”，而入院手术探查，术中见瘤体位于后腹膜，完整切除瘤体。病理检查结节状标本一件，１４ｃｍ×１４ｃｍ×１４ｃｍ大小。包膜完整，切面呈分叶状，色泽灰黄，...     

38例脂肪肉瘤病理分析

本文报告资料完整的脂肪肉瘤38例,男性23倒(60%),女性15例(40%),男女之比为1.5:1。年龄1～73岁,就诊时的平均年龄为43.6岁,较文献报告偏低;41～60岁者多见。下肢和腹膜后占60%。根据Enzinger的标准进行组织学分型,其中分化良好型14例(36.8%)、粘液样型13例(34.2%),圆形细胞型4例(10.4%),多形细胞型7例(18.6%)。讨论了分型、部位、年龄与预后之间的关系以及鉴别诊断问题。     

Dedifferentiated liposarcoma.

Dedifferentiated liposarcoma is a high-grade nonlipogenic sarcoma that arises in a background of a preexisting well-differentiated liposarcoma. The phenomenon of dedifferentiation is time dependent, and primary or de novo tumors exceed secondary neoplasms in a ratio of 9:1. The tumor occurs most frequently in adults beyond the 6th decade of life, slightly predominates in men, and involves the abdominal cavity most often. Pleomorphic malignant fibrous histiocytoma-like histologic features are the most commonly observed phenotype, although other sarcomatous phenotypes have been described less frequently. Surgical treatment is the main form of therapy for dedifferentiated liposarcoma, which is associated with a reported local recurrence rate of 41% to 52%, 15% distant metastatic rate, and 30% disease-related mortality rate. On a chromosomal level, dedifferentiated liposarcoma frequently displays the same chromosomal abnormality associated with well-differentiated liposarcomas--ie, the presence of a supernumerary ring or giant chromosome derived from the 12q(13-15) region.     

Dedifferentiated liposarcoma of the cheek

Liposarcomas of the head and neck region are rare; only a few cases have been reported to arise in the cheek or buccal mucosa. Dedifferentiated liposarcoma has rarely been reported in the head and neck region and, to the best of our knowledge, this is the first reported case of dedifferentiated liposarcoma of the cheek. Dedifferentiated liposarcoma is a mixed histologic subtype defined by the association of an atypical lipomatous tumor or well-differentiated liposarcoma and a nonlipogenic sarcoma. The patient was a 61-year-old man who presented with a soft-tissue mass of the left cheek and a presumptive diagnosis of salivary neoplasm based on a fine needle aspiration. The tumor was excised and consisted histologically of an atypical lipomatous tumor/well-differentiated liposarcoma composed a well-differentiated lipomatous neoplasm with atypical cells and rare lipoblasts. The tumor recurred in the same location 5 months after surgery. The recurrent tumor was primarily composed of a nonlipogenic spindle sarcoma with focal rhabdomyoblastic differentiation associated with areas of an atypical lipomatous tumor/well-differentiated liposarcoma.     

Dedifferentiated liposarcoma with rhabdomyoblastic differentiation

Dedifferentiated areas of dedifferentiated liposarcoma (DDL) usually show malignant fibrous histiocytoma (MFH)- or fibrosarcoma-like features and lack any histologic signs of specific differentiation. However, some reports have demonstrated specific differentiation in these areas, with histologic features resembling those of rhabdomyosarcoma, leiomyosarcoma, and osteosarcoma. We report here a pathologic and genetic analysis of three cases of DDLs with rhabdomyosarcomatous areas. MFH- or fibrosarcoma-like areas of one primary DDL and two recurrent DDLs contained various amounts of rhabdomyoblasts, which were immunoreactive for desmin, myoglobin, muscle actin (HHF-35), and myogenin. An ultrastructural examination demonstrated rhabdomyoblasts with abundant cytoplasm containing thin and thick filaments and Z-bands. By real-time PCR, amplification of mdm2 and cdk4 was confirmed in both well-differentiated and dedifferentiated areas with rhabdomyoblasts of all cases. To our knowledge, only seven cases of DDLs with rhabdomyosarcomatous components have been reported, and furthermore, the genetic profiles of the rhabdomyosarcomatous components in DDLs have not been investigated. This study demonstrates that DDLs with rhabdomyosarcomatous areas have genetic alterations that are common to well-differentiated/dedifferentiated liposarcomas.     

伴脑膜上皮样旋涡的去分化脂肪肉瘤一例

患者男,56岁。因阵发性左下腹疼痛半个月,于2004年6月11日入院。体检：腹部稍丰满,可扪及包块位于左下及右上腹部,质硬,轻压痛,不活动,表面尚光滑。CT示：腹部巨大包块(畸胎瘤可能性大)。发现包块3d后进行手术,术中见包块位于小肠系膜,与周围无粘连。     

Dedifferentiated liposarcoma with extensive lymphoid component

An unusual variant of dedifferentiated liposarcoma with extensive lymphocytic component is described. A 71-year-old patient suffered from a relapse of an atypical lipomatous tumor/well-differentiated liposarcoma with early micronodular (low-grade) dedifferentiation, which had been resected 4 years before. The relapse revealed features of a dedifferentiated liposarcoma with spindle-cell, partly pleomorphic dedifferentiation and osseous metaplasia. Clearly separated from the spindle-cell areas, an extensive homogeneously dense lymphoid (lymphocytic) tumor-component was evident, with relative abrupt transition to the well-differentiated liposarcoma component. Using immunohistochemistry and PCR, the lymphoid ("lymphoma-like") infiltrate proved to be a polyclonal lymphocytic proliferation. Fluorescence in situ hybridization (FISII) analysis revealed no signs of MDM2- and CDK4-gene amplification in the lymphoid areas, although within this mononuclear lymphoid population, large polymorphic nuclei displayed an amplified number of MDM2/CDK4 gene copies, indicating the presence of truly dedifferentiated tumor cells within the lymphoid component. The results favor a reactive lymphocytic infiltration versus a neoplastic one, which might be caused for example by chemoattractive agents. An extensive lymphoid "overgrowth" must be considered within the spectrum of unusual variants and in the differential diagnosis of dedifferentiated liposarcoma.     

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.     

Dedifferentiated liposarcoma of the oral cavity with angiosarcomatous dedifferentiation

We report a unique case of a 42-year-old woman with a dedifferentiated liposarcoma of the soft tissue of the oral cavity with angiosarcomatous dedifferentiation. Liposarcomas compromising the head and neck region are very unusual, and most of the cases in oral cavity show a well-differentiated pattern. Dedifferentiation in liposarcomas occurs in about 10% of the cases and, when it occurs, the dedifferentiated areas usually resemble high-grade fibrosarcoma or pleomorphic sarcoma. Divergent differentiation might also occur. To the best of our knowledge, there are only nine cases of dedifferentiated liposarcoma of the oral cavity, none of which show an angiosarcomatous dedifferentiation.     

Dedifferentiated liposarcoma of retroperitoneum and mesentery: varied growth patterns and histological grades--a clinicopathologic study of 32 cases

The purpose of this study was to obtain further information regarding cellular differentiation and proliferative characteristics of dedifferentiated liposarcoma (DDL) arising in the retroperitoneum and mesentery for accurate diagnosis and prognostic criteria. The patients included 20 men and 12 women, mean age, 60 years (range, 33 to 80 years). Twenty-seven tumors were located in the retroperitoneum and 5 in the mesentery. Tumor size ranged from 9 to 51 cm (mean, 24 cm). Follow-up was available on all patients and ranged from 4 to 243 months (mean, 64 months). Twenty-four (75%) patients developed local recurrences, 3 (9%) had distant metastasis, and 16 (50%) died of the disease. The predominant histology of dedifferentiation (DD) included fibrosarcoma or malignant fibrous histiocytoma (MFH) in 15 (47%), myxofibrosarcoma (myxoid MFH) in 5 (16%), mixed type in 10 (31%), and a whorling pattern in 2 (6%). Divergent differentiation, such as osseous, osteosarcomatous, chondrosarcomatous, and leiomyosarcomatous, was observed in 9 (28%). Immunoreactivity for vimentin, desmin, CD34, neurofilament, alpha-smooth muscle actin, p53, and MDM2 was observed in 32 (100%), 14 (44%), 8 (25%), 13 (41%), 14 (44%), 19 (59%), and 18 (56%) of DD areas, respectively. On the basis of a histological grading using MIB-1 (MIB-1 index range, 3% to 80%; mean, 27%) to replace mitosis counts (1 to 35/10 high-power fields [HPF]; mean, 13/10 HPF), 16 tumors each were classified as low-grade (grade 2) and high-grade (grade 3). The mixed type with poorly differentiated areas including scattered lipoblasts could be mistaken for myxoid liposarcoma but lacked the C/EBP-homologous protein-translocated in liposarcomat (CHOP-TLS) fusion genes specific for myxoid liposarcoma. Period to the first recurrence and histological grade using the MIB-1 index were associated with overall survival. Identification of DD areas, especially a poorly recognized variant of the mixed type, careful follow-up to detect early recurrence, and histological malignancy grading combined with proliferation indices are important in providing an accurate prognosis for all patients with retroperitoneal and mesenteric liposarcoma.     

Dedifferentiated myxoid liposarcoma: a clinicopathological study suggesting a closer relationship between myxoid and well-differentiated liposarcoma

Myxoid/round cell liposarcoma is arguably the commonest type of liposarcoma occurring in the extremities and may show gradual progression from low-grade, pure myxoid liposarcoma to high-grade round cell liposarcoma. Rarely myxoid/round cell liposarcoma is associated with areas of well-differentiated or pleomorphic liposarcoma (mixed liposarcoma). We describe the clinicopathological features of three unusual myxoid/round cell liposarcomas which showed morphological features of de novo dedifferentiation. All patients were male and were aged 66, 70 and 76 years, respectively. One lesion each arose in the retroperitoneum, inguinal region and peritoneal cavity. Histologically, in one case the myxoid/round cell component was juxtaposed to a high-grade non-lipogenic component resembling non-pleomorphic storiform 'malignant fibrous histiocytoma' ('MFH'), one case showed a combination of myxoid liposarcoma and a high-grade myxofibrosarcoma-like component (so-called myxoid 'MFH'), and in the third case, a well-differentiated myxoid liposarcoma with a discontinuous micronodular pattern of dedifferentiation was seen. Follow-up information of 30, 28 and 26 months revealed two recurrences each in two patients. These patients died of postoperative pulmonary embolism and abdominal haemorrhage, respectively; systemic metastases were not noted. These cases demonstrate that myxoid/round cell liposarcoma can show, albeit very rarely, histological features of dedifferentiation. Cases like these, combined with the occurrence of mixed-type liposarcoma (well-differentiated/myxoid liposarcoma) and the vicinity of chromosomal regions involved by specific karyotypic aberrations in these tumours, suggest that myxoid/round cell liposarcoma and well-differentiated liposarcoma (including its dedifferentiated variant) are more closely related in biological terms than is generally believed.     

Dedifferentiated liposarcoma presenting as jejunal polyp. Case report

We report a case of primary jejunal dedifferentiated liposarcoma presenting as a submucosal polyp mimicking a benign neoplasm. This is an extremely rare presentation. The histological feature of interest was the spindle cell lipoma-like appearance of the well-differentiated component. The use of MDM2 immunostaining in differentiating benign lipomatous tumours from well-differentiated liposarcomas is mentioned, which is of value especially in lipomatous tumours of the gut where ulcerated benign tumours can show varying degrees of atypia.