原发性软组织巨细胞瘤良恶性质的重新评价

１９７２年Salm和Sissons报道了１０例原发于软组织的肿瘤,它们的组织学形态与骨巨细胞瘤相同,即由单核细胞和破骨巨细胞样巨细胞均匀混合组成,故称为软组织巨细胞瘤（giant cell tumor of soft tissue,GCT-ST)。他们认为虽然２例有复发,但临床过程仍为良性无转移。同年Ｇuccion和Ｅnzinger报告了３２例富于破骨巨细胞的原发性办组织肿瘤,他们称之为软组织恶性巨细胞瘤。其后Ｓoule和Ｅnriguez研究了一系列良性和恶性组织细胞肿瘤,发现有５例与Ｇuccion和Ｅnzinger所报道病例组织形态相同,认为软组织恶性巨细胞在组织发生上与恶性纤维组织细胞瘤（ＭＦＨ）有关,以后就将其划归在ＭＦＨ项下,并认为是ＭＦＨ的巨细胞亚型,属高恶性,５０％发生转移。Ｓalm和Ｓissons最初提出的软组织可发生与骨巨细胞瘤相同的,而且多为良性的观点遭到长达２０多年的忽视。     

骨肉瘤骨连结蛋白免疫组化研究

应用多克隆抗体bONⅡ检测30例骨肉瘤中骨连结蛋白的表达,旨在探讨它在骨肉瘤诊断中的意义。结果显示:所有骨肉瘤均呈阳性反应,骨样组织中的基质亦为阳性。对照组中软骨肉瘤,尤文肉瘤和骨恶性淋巴瘤为阴性,骨恶性纤维组织细胞瘤和巨细胞瘤中的多核瘤巨细胞显弱阳性。结果表明骨连结蛋白免疫组化染色对骨肉瘤的诊断,特别是骨样基质缺乏或不易确定的骨肉瘤诊断,以及对小细胞型骨肉瘤和其它小圆细胞骨肿瘤的鉴别诊断具有重要的实用价值。     

原发性软组织巨细胞瘤22例

原发性软组织巨细胞肿瘤 (ＧＣＴ ＳＴ)最早由Ｓａｌｍ和Ｓｉｓｓｏｎｓ于 1972年描述 ,其特点是发生于软组织但含有破骨细胞样巨细胞。之后有关该瘤的报道时有所见 ,并将之归为恶性纤维组织细胞瘤或肉瘤等。作者对 2 2例ＧＣＴ ＳＴ进行了临床病理学及免疫组化研究。2 2例ＧＣＴ ＳＴ ,男性 12例 ,女性 10例 ,年龄 5～ 80岁 ,中位年龄 43岁 ;就诊前症状持续时间 2～ 12月 ,中位 4 5月 ,多以无痛性渐大的肿块为特征 ;好发于下肢 (5 0 % ) ,其次是躯干 (31 8% )和上肢 (13 6 % )。眼观 :瘤体直径在 1～ 10ｃｍ之间 ,中位 3ｃｍ ;发生于浅表者…     

血管瘤样纤维组织细胞瘤1例报道并文献复习

血管瘤样恶性纤维组织细胞瘤(angiomatoid malignant fibroos histocytona,AMFH)是一种少见的软组织肿瘤,也有文献称之为血管瘤样纤维组织细胞瘤.WHO(1994)将AMFH确认为中间型(低度恶性)纤维组织细胞肿瘤,其临床及病理学特点不同于普通类型恶性纤维组织细胞瘤,易误诊.笔者报道1例AMFH的光镜及免疫组化观察结果,并结合文献讨论其临床病理特征及诊断与鉴别诊断.     

去分化软骨肉瘤的临床病理诊断

目的 探讨去分化软骨肉瘤的临床、影像和病理学特征及鉴别诊断.方法 收集并分析去分化软骨肉瘤14例的临床及影像学资料,通过光镜观察及免疫组织化学EnVision法(抗体包括波形蛋白、S-100蛋白、平滑肌肌动蛋白、KP-1、PGM-1)分析其病理学特征、免疫表型及鉴别诊断.结果 14例去分化软骨肉瘤平均年龄52岁,男女比9:5.好发部位与普通软骨肉瘤一致,即盆三角、肩三角、肋骨和膝关节上下.影像学表现为双重形态特征的恶性肿瘤.大体中央型多于周围型,组织学由分界清楚的高分化软骨性肿瘤和高级别间变性肉瘤构成.术前单点穿刺活检漏诊、误诊率高.肿瘤内间变性肉瘤成分按发生率高低依次为骨肉瘤、恶性纤维组织细胞瘤和纤维肉瘤.结论 去分化软骨肉瘤为软骨肉瘤的少见特殊类型,具有与普通软骨肉瘤和软骨母细胞型骨肉瘤不同的临床、影像学和病理学特征,预后很差.     

去分化软骨肉瘤40例临床病理特征与预后分析

目的探讨去分化软骨肉瘤(dedifferentiated chondrosarcoma,DDCS)的临床、影像、病理学特征及诊断、治疗和预后等。方法收集2005年1月~2017年8月上海交通大学附属第六人民医院经病理确诊的40例DDCS,包括临床、影像、病理和随访资料,采用HE、免疫组化对其进行分析并复习相关文献。结果 40例DDCS中男性21例,女性19例,平均年龄51岁。发病部位分别为髋部、股骨、肱骨、胸骨、胫骨、肩部、手指、胸椎。临床主要表现为疼痛、肿胀、活动受限等。典型的影像学表现为双相征,即呈现出软骨肉瘤的点状、环状钙化和具有侵袭性特征的软组织肿块两种表现。镜下可见界限清楚的高分化软骨肉瘤成分和高度恶性成分(包括骨肉瘤、恶性纤维组织细胞瘤、纤维肉瘤、不能明确分类的梭形细胞肉瘤),或去分化为低级别肿瘤(包括骨巨细胞瘤或低级别骨肉瘤),两种成分比例不定。40例中有34例术前行穿刺活检,仅9例正确诊断为DDCS,穿刺诊断正确率为26.5%。在初诊时已发生转移患者的平均生存时间明显短于未发生转移患者。结论 DDCS的诊断需临床、影像和病理三结合。其组织学形态表现复杂多样、术前穿刺活检存在局限性,其诊断比较困难。初次诊断时伴有转移的患者预后更差。     

恶性骨肿瘤患者巨噬细胞功能、NK细胞活性和T细胞亚群的变化

我们检测了３４例恶性骨肿瘤患者的巨噬细胞功能、ＮＫ细胞活性和Ｔ细胞亚群的变化,可为临床上恶性骨肿瘤的免疫治疗及病情判定,提供一定的实验依据。１材料和方法１．１研究对象患恶性骨肿瘤患者３４例,均为经ＥＣＴ检查而确诊的住本院患者,包括：骨肉瘤２０例、滑膜肉瘤１例、软骨肉瘤２例、恶性纤维组织细胞瘤１例、转移癌３例及骨巨细胞瘤７例。其中男２３例,女１１例,年龄１０～８５岁平均３０．０岁。此外,另选择本单位健康人３２例均经查体证实,作为对照组,平均年龄３５．５岁。１．２方法①Ｔ细胞亚群的检测〔１〕：取患者…     

腹膜后恶性纤维组织细胞瘤的 MSCT 表现及诊断价值简

目的分析腹膜后恶性纤维组织细胞瘤（MFH）的多层螺旋CT（MSCT）表现,探讨MSCT对该病的诊断价值。方法经手术病理证实的15例腹膜后MFH患者,其中男性11例,女性4例：年龄21～79岁,平均年龄46.5岁。回顾性分析其临床及MSCT表现.并与病理组织学改变相对照。结果15例腹膜后MFH主要临床表现为腹部肿块和疼痛。15例MSCT平扫表现为腹膜后软组织肿块.肿瘤呈圆形或类圆形10例。不规则形5例,肿瘤平均直径为12.5cm,瘤内坏死9例,出血7例,钙化6例。增强扫描肿瘤不均匀强化,13例见分隔状强化。组织学上呈明显多形性,其中车辐状一多形型MFH 11例,巨细胞型MFH 3例,炎症型MFH 1例。结论腹膜后MFH的MSCT表现具有一定特征,对其诊断及鉴别诊断有重要价值。     

成人原发性肾肉瘤的临床诊治

目的 探讨成人原发性肾肉瘤的诊断、治疗和预后。方法 收集1985~2009年收治的成人肾脏肿瘤1654例,对其中17例原发性肾肉瘤进行回顾性分析。结果 17例患者中10例以腹部肿块为首发症状。患者均行手术治疗,术后病理：平滑肌肉瘤7例,横纹肌肉瘤2例、恶性纤维组织细胞瘤2例,低分化肉瘤2例,脂肪肉瘤、纤维肉瘤、胚胎性横纹肌肉瘤及平滑肌肉瘤合并嫌色细胞瘤各1例。术中死亡1例,15例患者规律随访5~60月,目前1例无瘤存活9个月,死亡14例,14例患者术后平均生存期18（5~60）个月;平滑肌肉瘤组28（11~60）个月,2例恶性纤维组织细胞瘤分别存活4和8个月。结论 原发性肾肉瘤临床症状与晚期肾细胞癌类似,总体预后较差,肿瘤分期、病理类型及术后有无残余瘤是决定预后的重要因素。     

腹膜后恶性纤维组织细胞瘤的MSCT表现及诊断价值

目的分析腹膜后恶性纤维组织细胞瘤(MFH)的多层螺旋CT(MSCT)表现,探讨MSCT对该病的诊断价值。方法经手术病理证实的15例腹膜后MFH患者,其中男性11例,女性4例;年龄21~79岁,平均年龄46.5岁。回顾性分析其临床及MSCT表现,并与病理组织学改变相对照。结果 15例腹膜后MFH主要临床表现为腹部肿块和疼痛。15例MSCT平扫表现为腹膜后软组织肿块,肿瘤呈圆形或类圆形10例,不规则形5例,肿瘤平均直径为12.5 cm,瘤内坏死9例,出血7例,钙化6例。增强扫描肿瘤不均匀强化,13例见分隔状强化。组织学上呈明显多形性,其中车辐状-多形型MFH 11例,巨细胞型MFH 3例,炎症型MFH 1例。结论腹膜后MFH的MSCT表现具有一定特征,对其诊断及鉴别诊断有重要价值。     

Soft tissue giant cell tumor of low malignant potential: a proposal for the reclassification of malignant giant cell tumor of soft parts.

Although "giant cell tumor of soft parts" has traditionally been considered a single entity as reflected in the original term "malignant giant cell tumor of soft parts (MGCT)" and later by the term "malignant fibrous histiocytoma, giant cell type" the degree of atypia and mitotic activity varies in this group, suggesting biologic heterogeneity. The clinicopathologic features of 31 tumors meeting the traditional criteria of MGCT but having only mild to moderate nuclear atypia are presented. Patients with these tumors (19 females; 12 males) ranged in age from 14 to 84 years (mean, 40 years) and presented with masses of involving either superficial (n = 16) or deep (n = 13) soft tissue. Most occurred on the arm or hand (n = 16) and ranged in size from 0.7 to 6.5 cm (mean, 2.1 cm). The tumors consisted of sheets and nodules of rounded mononuclear cells that blended with spindled cells and benign osteoclastic giant cells. Pleomorphic giant cells were absent. Osteoid was noted in 10 cases, but features typically associated with tenosynovial giant cell tumors (such as dense stromal hyaline, siderophages, and xanthoma cells) were nearly always absent. Mitotic figures ranged from 1-10/10 HPF (mean, 2-3/10 high-powered field), and angiolymphatic invasion was present in 10 cases. Necrosis was absent, however. The mononuclear cells expressed CD68, tartrate-resistant acid phosphatase, and smooth muscle actin, but lacked CD45, S100 protein, desmin, and lysozyme, an immunophenotypic profile identical to that of giant cell tumor of bone. Follow-up information in 19 patients (mean, 3 yrs; median, 1-7 yrs) indicated recurrences in four patients, but none developed metastasis. This behavior contrasts significantly with the high-grade behavior traditionally associated with MGCT of soft parts. These giant cell tumors can be consistently recognized by the lack of cytologic atypia even in the face of mitotic activity and vascular invasion. Although their long term metastatic risk is not fully defined, we propose they be termed "giant cell tumors of low malignant potential" and regarded as the soft tissue analogue of giant cell tumor of bone. The term "malignant giant cell tumor of soft parts" or giant cell malignant fibrous histiocytoma should be restricted to histologically high-grade lesions.     

Histological and clinical characteristics of malignant giant cell tumor of bone

Malignant giant cell tumors of bone (MGCTB) are rare, and the diagnosis can be difficult due to the occurrence of a variety of malignant tumors containing giant cells. To better understand its clinicopathological features, we have reviewed our experience with 17 cases of MGCTB. Five cases were primary malignant giant cell tumor of bone (PMGCTB), and 12 cases were giant cell tumors of bone initially diagnosed as benign but malignant in a recurrent lesion (secondary MGCTB, SMGCTB). The patients included six women and 11 men (age ranged from 17 to 52 years; mean, 30.5 years). The tumor arose in the femur (six cases), the tibia (seven cases), the humerus (three cases), and the fibula (one case). Microscopically, PMGCTB showed both conventional giant cell tumor and malignant sarcoma features. SMGCTB were initially diagnosed as conventional giant cell tumor of bone, the recurrent lesion showing malignant features. Histologically, the malignant components included osteosarcoma (11 cases), undifferentiated high-grade pleomorphic sarcoma (two cases), and fibrosarcoma (four cases). SMGCTB cases showed strong expression of p53. Follow-up information revealed that four patients died of lung metastasis, two patients are alive with lung metastases, and 11 patients are alive without tumor. MGCTB should be considered as a high-grade sarcoma. It must be distinguished from GCTB and other malignant tumors containing giant cells. p53 might play a role in the malignant transformation of GCTB.     

颗粒细胞瘤15例临床病理分析

目的探讨颗粒细胞瘤的组织起源及临床病理学特征。方法回顾分析15例颗粒细胞瘤的临床资料及组织学形态特征,并采用免疫组化(SP法)观察其免疫表型。结果良性颗粒细胞瘤13例,恶性颗粒细胞瘤2例。男性5例,女性10例,年龄19~69岁,平均年龄41·6岁,2例恶性颗粒细胞瘤年龄分别为67岁和69岁。良性颗粒细胞瘤直径0·4~5·3cm,平均2·3cm,2例恶性颗粒细胞瘤直径分别为6和14cm。均为单发病例,临床上主要表现为真皮、皮下或黏膜下孤立性无痛性结节,分别位于腰部3例、腋下、胸壁各2例,乳腺、上臂、子宫、肛周、声带、食管、结肠、舌部各1例。病理组织学上,良性颗粒细胞瘤的肿瘤细胞通常有比较丰富的嗜酸性颗粒状胞质和小而深染的胞核,而恶性颗粒细胞瘤的肿瘤细胞中可见带有明显核仁的空泡状核,细胞核明显异型及部分细胞呈梭形。免疫组化示神经标记物NSE、S-100蛋白强阳性,表达溶菌酶的标记物CD68也强阳性,而表达平滑肌和横纹肌的标记物SMA、MG均阴性。结论颗粒细胞瘤为来源于雪旺细胞的肿瘤,恶性者少见,大多发生在年龄大的患者,且肿瘤体积比较大。     

Orbital solitary fibrous tumor: encompassing terminology for hemangiopericytoma, giant cell angiofibroma, and fibrous histiocytoma of the orbit: reappraisal of 41 cases

Hemangiopericytomas and solitary fibrous tumors are uncommon neoplasms found in many locations, including the orbit. Both mesenchymal neoplasms share several clinicopathologic features, thus prompting intense debate as to whether they are variants of the same entity or merit separate designations in the orbit. These 2 entities, with the addition of giant cell angiofibroma of orbit, are of benign- to uncertain-behavior, CD34-positive, collagen-rich, specialized fibroblastic tumors, which may have overlapping or histologically identical features. In addition, so-called fibrous histiocytoma of orbit, a previous designation, has overlapping morphologic features with these tumors. To date, a large series of these collagen-rich fibroblastic tumors of the orbit has not been fully explored. Forty-one fibroblastic orbital tumors, originally diagnosed as hemangiopericytomas (n = 16), fibrous histiocytomas (n = 9), mixed tumors (hemangiopericytomas/fibrous histiocytoma) (n = 14), and giant cell angiofibromas of orbit (n = 2) between 1970 and 2009, were retrieved from our consultation files, the Ophthalmic Registry, at the Armed Forces Institute of Pathology. Slides and clinical records were reviewed, analyzed, and compared. Immunochemistry was performed for CD34, CD99, Bcl-2, Ki-67, and p53. Upon histologic review, all cases were reclassified as solitary fibrous tumor (41/41). The patients included 23 (56%) males, 17 (41%) females, and 1 unknown, with a mean age at presentation of 40.7 years (range, 16-70 years). The sites of involvement were the right orbit in 18 (44%) cases and the left in 16 (39%) cases. Tumors ranged in size from 0.4 to 5.0 cm (mean, 2.2 cm). Seventeen (41%) patients presented with an orbital mass, 8 (20%) with proptosis, 2 (5%) with painful mass, and 2 (5%) with painless mass. Duration of symptoms ranged from 3 to 96 months, with a mean of 23 months (median, 9 months). Microscopically, all lesions showed considerable similarity, varying in degree of cellularity, stromal collagen, and the presence of giant cells. Overlapping features with soft tissue giant cell fibroblastoma were observed. Immunochemistry revealed positivity for CD34 in all cases (100%), p53 in 85%, CD99 in 67.5%, and Bcl-2 in 47.5%. Although Ki-67 labeling was seen in all cases, it ranged from less than 1% in 54.3% of cases to 5% to 10% in 20% of cases. Taken together, the findings of this study suggest that orbital hemangiopericytoma and some cases previously designated as fibrous histiocytoma, giant cell angiofibroma of orbit, and solitary fibrous tumor have overlapping morphologic and immunohistochemical features and should be designated as solitary fibrous tumor. Adipocytes and unusual multivacuolated adipocytic cells may be present in these tumors, as well stromal myxoid change; and even stromal intramembranous ossification can be observed. There are overlapping features of orbital solitary fibrous tumor with another CD34-positive specialized fibroblastic tumor of soft tissue, giant cell fibroblastoma. Morphologic criteria for uncertain behavior to low-grade malignant ocular solitary fibrous tumors can be made by cytologic atypia and increased mitotic activity, but overall outcome for malignant solitary fibrous tumors of the eye should be further explored.     

Primary malignant giant cell tumor of bone: "dedifferentiated" giant cell tumor

Well documented examples of primary malignant giant cell tumor of bone (giant cell tumor and concurrent sarcoma arising de novo) are exceedingly rare in the literature. We report a case arising in the left ischium of a 44-yr-old man. He had no previous history of radiation therapy or multiple resections. Histologically, the tumor was a typical giant cell tumor of bone juxtaposed to a malignant fibrous histiocytoma (MFH). The juxtaposition of a high grade sarcoma (MFH) and a locally aggressive nonmalignant neoplasm such as giant cell tumor is analogous to several other tumors of bone and soft tissue in which a low grade malignant or locally aggressive tumor can be associated with MFH or fibrosarcoma de novo, namely chondrosarcoma, chordoma, liposarcoma, and well differentiated intraosseous and parosteal osteosarcoma. The presence of a high grade malignant component in each of the aforementioned neoplasms generally portends a more ominous prognosis, although this is not invariably true. Recognition of the phenomenon of "dedifferentiation" (or tumor progression) in some bone tumors and sarcomas is important to ensure appropriate treatment. Distinction from secondary malignant giant cell tumors which are usually radiation induced is also important, since the latter have a much worse prognosis than those with dedifferentiation occurring de novo.     

Disseminated malignant fibrous histiocytoma (giant cell rich): a case report

Giant cell rich malignant fibrous histiocytoma accounts for 3 -15% of all malignant fibrous histiocytomas. Currently, the nomenclature giant cell malignant fibrous histiocytoma is reserved for undifferentiated pleomorphic sarcomas with prominent osteoclastic giant cells. It is considered to be synonymous with malignant giant cell tumor of soft parts. We report a case of disseminated giant cell malignant fibrous histiocytoma involving the scalp, cervical node, lungs, spine, abdominal wall, base of penis, gluteal cleft, paraspinal region and back. The diagnosis was established after staining for a panel of immunohistochemical markers namely cytokeratin, vimentin, S100, desmin, CD68 and smooth muscle actin. CD68 positivity in tumor cells helped in arriving at the final diagnosis. It is essential to recognize this tumor as a giant cell rich distinct entity and differentiate from other giant cell rich pleomorphic sarcomas since therapeutic and prognostic differences are being appreciated currently.     

Sarcomatoid carcinoma (so-called pseudosarcoma) of the larynx simulating malignant giant cell tumor of soft parts. A case report

A tumor of the larynx composed of a small infiltrating and well-differentiated squamous cell carcinoma and a large polypoid sarcomatous mass showing the histologic features of the malignant giant cell tumor of soft parts (MGCT) is reported. Other types of sarcomatous components have been described with squamous cell carcinoma (SCC) of the larynx and names such as pseudosarcoma, carcinosarcoma, and spindle cell carcinoma have been used for these peculiar tumors. Because most data support that the sarcomatous component is an unusual carcinoma transformation due to unknown factors the authors prefer the term sarcomatoid carcinoma. Sarcomatoid carcinomas having the features of MGCT have been described in multiple epithelial organs. Therefore, an extensive search for a carcinoma component always should be carried out in tumors arising in any organ, including the larynx, and showing the typical histologic features of MGCT.     

Malignant giant cell tumor of tendon sheath

Summary A benign, but aggressive, giant cell tumor of tendon sheath developed over a period of 20 years into a metastasizing, histologically malignant giant cell tumor. Ultrastructure of the malignant tumor showed the same five cell types as described in giant cell tumors of tendon sheath. Even the same crystals were identified in the osteoblast-like and osteoclast-like cells.It therefore appears reasonable to assume that giant cell tumors of tendon sheath indeed are neoplasms with a malignant potential and not an inflammatory reaction of synovial cells as previously suggested.Both the benign and malignant tumors seem to be of mesenchymal derivation with partial osseous differentiation. No ultrastructural similarities with fibrous histiocytoma were apparent.     

恶性颗粒细胞瘤10例临床病理学观察及文献复习

目的 探讨恶性颗粒细胞瘤的临床病理学特征,评价组织学上诊断恶性的标准。方法 对10例恶性颗粒细胞瘤的临床资料和组织学形态进行回顾性分析,并采用免疫组织化学(LSAB法)和电镜检测研究其免疫表型和超微结构。结果男性4例,女性6例,年龄范围为27～73岁(平均46岁)。临床上,9例表现为皮下或深部软组织内无痛性生长的孤立性肿块,其中1例伴有周围神经症状。肿瘤位于下肢3例,乳腺2例,项部2例,胸壁、右颈部和盆腔者各1例。肿瘤直径2～11cm,平均4．8cm。镜下由成巢或成片的多边形细胞组成,胞质呈嗜伊红色颗粒状,与良性性颗粒细胞瘤极为相似,但仔细观察发现,9例显示至少下述形态中的3种：核增大呈空泡状并可见明显的核仁;多形性瘤细胞;核质比增大;瘤细胞趋向梭形;可见核分裂象;凝固性坏死。除经典性形态外,1例尚可见散在的多核性瘤细胞。余1例除局部区域显示核增大呈空泡状并可见明显的核仁外,其余形态均不明显,但临床上却呈恶性经过。9例均强阳性表达S-100蛋白和神经元特异性烯醇化酶(NSE),7例尚表达CD68。电镜观察显示瘤细胞胞质内充满大量退变的复合性溶酶体。随访7例,5例复发,4例转移,2例死于肿瘤。结论 (1)Fangburg-Smith等的组织学恶性标准具有较高的可重复性,但在少数情形下,最终诊断仍需结合肿瘤的生物学行为;(2)提议将核分裂象的标准修订为＞5／50HPF;(3)广泛性局部切除加上必要的区域淋巴结清扫仍是目前最主要的治疗手段,辅助性化疗和(或)放疗并不能明显改善患者的预后;(4)描述一种尚未见报道的多核性瘤细胞形态亚型。     

伴破骨样巨细胞平滑肌肉瘤的临床病理学观察

目的 探讨伴破骨样巨细胞平滑肌肉瘤的临床病理学特点及其鉴别诊断.方法 收集7例伴大量破骨样巨细胞平滑肌肉瘤病例的临床和影像学资料,行光镜观察、免疫组织化学(EnVision法)标记和电镜观察,并复习相关文献.结果 患者均为成年人,其中女性3例,男性4例,平均年龄 63岁.肿瘤位于大腿皮下软组织2例,左背部、腹膜后、小肠、乳腺和子宫各1例.组织学上,所有病例均由形态相对一致、呈交织条束状排列的梭形瘤细胞和大量的破骨样巨细胞组成,后者多散在分布于梭形细胞之间(6例),或形成巨细胞瘤样结节(1例).免疫组织化学标记显示,梭形细胞程度不等地表达平滑肌肌动蛋白、肌特异性肌动蛋白、结蛋白和高相对分子质量钙调结合蛋白,破骨样巨细胞表达CD68.电镜观察显示梭形细胞具平滑肌分化特征,破骨样巨细胞则具组织细胞分化.随访6例患者,均在术后发生复发或转移,其中3例带瘤生存,2例死亡.结论 (1)伴大量破骨样巨细胞的平滑肌肉瘤是平滑肌肉瘤的一种少见亚型,形态上与巨细胞型恶性纤维组织细胞瘤相似,诊断时应注意加以鉴别.(2)免疫组织化学标记和电镜观察提示肿瘤内的破骨样巨细胞起源于单核细胞/组织细胞系.(3)该肿瘤的恶性程度高,预后差.

Abstract：

Objective To study the clinicopathologic features of leiomyosarcoma with prominent osteoclast-like giant cells. Methods The clinical and pathologic features of 7 cases of leiomyosarcoma with prominent osteoclast-like giant cells were analyzed. Immunohistochemical and ultrastructural studies were performed. The literature was reviewed. Results All cases occurred in adults, with a mean age of 63 years. There was no significant soft tissue of thigh (number=2), left back (number=1), retroperitoneum (number=1), small intestine (number=1), breast (number=1) and uterus (number=1). Histologic examination showed that the tumor was composed of relatively uniform spindly cells arranged in interlacing fascicles. The hallmark was the presence of prominent osteoclast-like giant cells, either intimately admixed with the spindly cells (number=6) or forming giant cell tumor-like nodules (number=1). Immunohistochemically, the spindly cells expressed smooth muscle actin, muscle-specific actin, desmin and h-caldesmon in various degrees, whereas the osteoclast-like giant cells expressed CD68. Ultrastructural study showed smooth muscle differentiation in the spindly cells and histiocytic differentiation in the osteoclast-like giant cells. Follow-up data were available in 6 cases. There were local recurrences and/or metastases in all the 6 patients. Three patients were alive with unresectable or recurrent/metastatic disease and two patients died of the disease. Conclusions Leiomyosarcoma with prominent osteoclast-like giant cells is a rare variant of leiomyosarcoma which should be distinguished from the so-called giant cell variant of malignant fibrous histiocytoma. The osteoclast-like giant cells are of histiocytic differentiation. Surgical resection remains the mainstay of management of this high-grade sarcoma.