核仁组成区蛋白在软组织肿瘤及瘤样病变中的表达与意义

应用胶银技术,对软组织肿瘤特别是纤维源性肿瘤核仁组成区蛋白进行了定量研究。在软组织一些恶性肿瘤中,以血管肉瘤、横纹肌肉瘤核仁组成区蛋白均值最高,并以此排列了它们的恶性度顺序。在纤维源性肿瘤中,纤维瘤、瘤样纤维组织增生、纤维肉瘤及其亚类核仁组成区蛋白均值有显著与高度显著性差异(P<0.05、P<0.001),其中随访的34例纤维肉瘤患者,具有高核仁组成区蛋白均值(≥5)与低核仁组成区蛋白均值(<5)的5年生存率分别是20％和53％(P<0.05)。因此,作者认为核仁组成区蛋白定量在判定软组织肿瘤的恶性度、鉴别其良恶性及预测患者预后中均有一定实用价值。     

Fascin蛋白在软骨肉瘤中的表达及其临床意义

目的：分析Fascin蛋白在软骨肉瘤组织中的表达及其与软骨肉瘤临床病理因素的关系。方法：应用免疫组化EnlivionTM法分别检测和比较70例软骨肉瘤和20例软骨瘤中Fascin蛋白的表达情况。结果：（1）Fascin在软骨肉瘤组和软骨瘤组中的阳性表达率分别为64．29％和5．00％,两组相比差异有统计学意义（X2=21．88,P=0．00）;（2）Fascin蛋白的表达与患者的年龄、性别无显著相关性（Xk0．37,P=0．54;X2=-0．29,P=0．59）;Fascin蛋白在肺转移组中的表达为76．32％,显著高于无肺转移组（50．00％）,两组相比差异有统计学意义（XZ=5．24,P=0．02）;Fascin蛋白在间叶性软骨肉瘤、透明细胞软骨肉瘤的中表达分别与普通型软骨肉瘤相比,无统计学意义（P〉0．05）,但去分化软骨肉瘤与普通型软骨肉瘤相比,有统计学意义（P〈0．05）;Fascin在普通型软骨肉瘤的I-III级中的阳性表达率分别为11．1l％、61．90％、75．00％,三者之间的差异有统计学意义（P〈0．05）。结论：Fascin参与了软骨肉瘤恶变的过程,其高表达与软骨肉瘤的分化和转移有关。     

MMP-9和PTEN在胶质瘤中的表达及相关性分析

目的：检测脑胶质瘤组织中MMP-9和PTEN表达情况并探讨其意义。方法：采用免疫组织化学S-P法检测50例脑胶质瘤、10例正常脑组织中两者的表达。结果：脑胶质瘤组织中MMP一9和PTEN表达定位于细胞浆,其阳性表达率分别为74．00％（37／50）和60．00％（30／60）与正常脑组织0、100％有差异（P〈0．05）。两者的表达与胶质瘤患者的年龄、性别无明显相关性（P〉O．05）,但高级别组与低级别组之间表达有差异,具有统计学意义（P〈0．05）;在脑胶质瘤组织中MMP-9蛋白的阳性表达与PTEN蛋白的阳性表达有相关性,二者呈负相关（P〈0．05）。结论：MMP．9蛋白在胶质瘤组织中的表达明显高于正常脑组织,并且高级别胶质瘤组的表达明显高于低级别胶质瘤组中的表达,提示MMP-9可能与胶质瘤的浸润、发展、转移有关,可作为一个预测胶质瘤侵袭转移能力的肿瘤标志物。PTEN蛋白在正常脑组织中的表达明显高于胶质瘤,且高级别组表达明显高于低级别组,提示PTEN基因突变或缺失在胶质瘤的发生发展中起重要作用,且与肿瘤恶性分化程度密切相关,表明PTEN的失活预示着一个特殊的进展性的临床行为,在胶质瘤恶性进展中属于较晚发生的分子事件。     

表达CD19^＋CD56^-的多发性骨髓瘤浆细胞免疫表型的鉴别

目的：研究多发性骨髓瘤患者浆细胞和对照组正常浆细胞免疫表型,有效地识别表达CD19^＋CD56^-的多发性骨髓瘤恶性浆细胞。方法：采用四色流式细胞仪（BD,FACSCalibur）检测44例MM患者浆细胞以及25例健康骨髓捐献者正常浆细胞膜上的抗原表达。采用CellQuest软件分析结果。细胞膜表面抗原表达率大于20％定义为表达阳性。阳性率为表达阳性的患者及健康对照者所占百分比。结果：44例MM患者浆细胞免疫表型表达频率为：CD^138＋;97．72％（43／44）、CD38^＋：100％（44／44）、CD56^＋：63．64％（28／44）、CD19：84．09％（37／44）、CD200^＋：77．27％（34／44）、CD28^＋：38．64％（17／44）;25例对照组正常浆细胞免疫表型表达频率为：CD138^＋：100％（25／25）、CD38^＋：100％（25／25）、CD56^＋-：100％（25／25）、CD19^＋：96％（24／25）、CD200^＋：0％（0／25）、CD28^＋：0％（0／25）。在44例MM患者中,9％（4／44）患者表达CD19^＋CD56^-,利用CD200检测4例表达CD19^＋CD56^-的患者,有3例患者伴有CD200阳性表达,可与正常浆细胞鉴别。结论：CD200有利于鉴别表达CD19＋CD56-MM恶性浆细胞与正常浆细胞。     

HPV-16、EB病毒对细胞增殖的影响及与子宫颈癌发病的关系

目的 了解宫颈癌患者人乳头瘤病毒16（HPV 16）、EB病毒（EBV）感染情况,探讨HPV-16、EBV对细胞核增殖性抗原（PCNA）表达的影响及在子宫颈癌发病中的意义。方法 免疫组织化学SP法检测59例宫颈癌和20例非癌性子宫颈上皮细胞中HPV-16、EBV蛋白表达和PCNA表达的情况,并分析它们的表达与病理参数的关系。结果 子宫颈癌和非癌性子宫颈上皮细胞中HPV-16、EBV及PCNA的阳性表达率依次分别为69．49％、57．63％及77．97％和30％、25％及15％;子宫颈癌和非癌性子宫颈上皮细胞中HPV-16、EBV的共同阳性表达率分别为35．59％和0％,子宫颈癌中HPV-16、EBV及PCNA的阳性表达率均高于非癌性子宫颈上皮（P〈0．05）。病理Ⅰ级、Ⅱ级和Ⅲ级子宫颈癌中HPV-16、EBV、PCNA的阳性表达率分别为65．00％、55．00％、60．00％,69．57％、60．87％、82．61％和75．OO％、56．25％、93．75％。各级子宫颈癌间HPV-16、EBV的阳性表达率差异无显著性,但PCNA的表达率随病理分级的增加显著上升（P〈0．05）。不同期别子宫颈癌问HPV-16、EBV及PCNA的阳性表达率差异无显著性。HPV-16阳性与阴性组子宫颈上皮PCNA的阳性表达率分别为82．98％（39／47）与43．75％（14／32）,两者差异有显著性（P〈0．05）。EBV阳性及阴性组子宫颈上皮PCNA的阳性表达率分别为71．79％（28／39）及42．50％（17／40）,两者差异有显著性（P〈0．05）。HPV-16和EBV共同阳性表达的21例子宫颈癌PCNA阳性表达率均为100％。结论 HPV-16、EBV通过增加PCNA表达的促细胞增生作用可能是子宫颈癌的发生机制之一。     

VEGF—A和VEGF—C在皮肤恶性黑色素瘤表达的临床病理意义

目的：内皮细胞生长因子（Vascularendothelialgrowthfactor,VEGF）与恶性肿瘤转移密切相关,研究发现VEGF过度表达与恶性黑色素瘤转移有关,在本研究中通过研究VEGF在恶性黑色素瘤中的表达及与临床病理指标的相关性,为以VEGF为靶的抗转移治疗提供依据。方法：应用免疫组织化学技术检测恶性黑色素瘤中VEGF-A和VEGF-C表达,及与临床病理特点和生存状态的关系。结果：VEGF—A在皮肤恶性黑色素瘤中的阳性表达率是83．33％（30／36）,在色素痣中阳性表达率是15％（3／20）,两组间有显著性差异（P〈O．05）。VEGF—C在皮肤恶性黑色素瘤中的阳性表达率是88．9％（32／36）,在色素痣中阳性表达率是10％（2/20）,两组间有显著性差异（P〈0．01）。VEGF-A和VEGF—C表达与年龄、性别、肿瘤形态、肿瘤大小无显著关系,但与淋巴结转移和封闭血管环形成有关,VEGF-A和VEGF—C阳性病例淋巴结转移率和封闭血管环出现率显著高于VEGF-A和VEGF-C阴性病例。有统计学意义。对VEGF-A和VEGF-C表达与恶性黑色素瘤生存状态的关系分析显示,VEGF-A和VEGF-C表达阴性的病例的生存期和生存率均显著高于VEGF-A和VEGF-C表达阴性的病例,有统计学意义。结论：VEGF-A和VEGF-C表达与恶性黑色素瘤的淋巴结转移、血管形成和生存期相关,这两种蛋白过度表达反映黑色素瘤处于进展状态和预后差,可以作为黑色素瘤诊断、预后和复发预测的指标和靶向治疗的靶蛋白。     

胶质瘤组织中PTEN、p57／Kip2蛋白的表达及临床意义研究

探讨PTEN、p57／Kip2在脑胶质瘤中的表达情况和临床意义。选取54例胶质瘤手术切除标本,采用sP法进行免疫组织化学染色。PTEN、p57／Kip2阳性表达率分别为48．1％（26／54）和44．4％（24／54）,两者在高度恶性组（Ⅲ～Ⅳ级）的表达强度明显低于低度恶性组（Ⅰ—Ⅱ级）,且随着胶质瘤的恶性程度不同表现出明显的统计学差异（P〈0．05）。PTEN与p57／Kip2的低表达可能参与肿瘤的生长分化和进展,可能提示预后不良。     

survivin在非小细胞肺癌中的表达及其与bcl-2、p63表达的关系

目的：探讨survivin在非小细胞肺癌组织（non small cell lung cancer,NSCLC）中的表达,及其与bcl2、p63蛋白表达的相关性。方法：应用二步法免疫组织化法,检测survivin、bcl-2、p63蛋白在60例NSCLC组织和20例正常肺组织中的表达。结果：肺癌组织中的survivin蛋白阳性率（56．67％）明显高于正常肺组织15％）,有显著性差异;（p〈0．05）Ⅲ期surviving蛋白阳性表达率72．73％（16／22）明显高于Ⅰ＋Ⅲ期survivin47．37％（18／38）。有显著差异;（p〈0．05）survivin蛋白表达与患者年龄、病理类型、组织分化程度,淋巴结转移情况无关（P〉0．05）NSCLC组织bc 1-2蛋白表达阳性、阴性组中,survivin蛋白阳性表达率分别为66．67％（18／27）和48．48％（16／33）,两者比较,差异有显著性（P〈0．05）;p63蛋白表达阳性、阴性组中,survivin蛋白阳性表达率分别为53-33％（16／30）和60％（18／30）,两者比较,差异有显著性（P〈0．05）survivin,蛋白与bc1．2蛋白的表达呈正相关。survivin蛋白与p63蛋白的表达呈正相关。结论：survivin在NSCLC组织中表达上调,通过抑制细胞凋亡,在NSCLC的发生和发展中起到重要作用。survivin,bcl-2与p63它们分别在肺癌发生发展过程中不同途径上抑制肺癌细胞的凋亡,对肺癌早期诊断有一定的意义。对3种蛋白进行联合检测,更有利于肺癌的早期诊断和判断肺癌的分化程度、临床分期、淋巴结是否转移及病人的预后。survivin与bcl-2及survivin与p63可能起协同作用、并可能会成为NSCLC基因治疗的新靶点。     

MMP-7和Survivin在结直肠癌中的表达及其临床意义

目的探讨Wnt信号通路中基质金属蛋白酶7（matrix melluoproteinases7,MMP-7）和凋亡抑制基因Survivin在结直肠癌组织中表达及其与临床病理特征的关系。方法应用免疫组织化学染色（Elivision）方法检测100例结直肠癌组织和60例癌旁正常黏膜组织中MMP-7和Survivin蛋白的表达。结果MMP-7蛋白在100例结直肠癌组织和60例癌旁正常黏膜组织中的阳性表达率分别为77．00％（77／100）和13．33％（8／60）,两组问差异有统计学意义（P〈0．01）;Survivin蛋白在100例结直肠癌组织和60例癌旁正常黏膜组织中的阳性表达率分别为65．00％（65／lOO）和15．00％（9／60）,两组问差异有统计学意义（P〈0．01）。MMP-7与Survivin蛋白阳性表达均与肿瘤的淋巴结转移和Dukes分期有关（P〈0．05）,此外,MMP-7蛋白在结直肠癌中的阳性表达也与肿瘤的浸润深度有关（P〈0．05）。而MMP-7与Survivin蛋白的阳性表达无相关性（r=0．097,P〉O．05）。结论MMP-7和Survivin在结直肠癌中的高表达可能与结直肠癌的发生、发展、浸润和转移等相关,检测癌组织中MMP-7和Survivin的表达有助于为结直肠癌的病情进展及预后判断提供帮助。     

Stathmin／Oncoprotein 18（Op18）基因在人骨肉瘤中的表达及其意义

目的：探讨stathmin／oncoprotein18（Op18）基因在人骨肉瘤中的表达规律及意义。方法：分别采用免疫组化法和Western blot法检测10例正常骨组织和56例骨肉瘤中stathmin蛋白的表达。结果：免疫组化法检测stathmin蛋白在正常骨组织、低级别骨肉瘤（G1级组）及高级别骨肉瘤（G2级组）中阳性表述率分别为20％、65％、100％。正常骨组织分别与G1级组、G2级组比较,均有显著性差异（P〈0．05）;G1级组与G2级组比较,有显著差异（P〈0．05）。Westernblot法检测显示,stathmin蛋白在正常骨组织、G1级组、G2级组表达相对值分别为（O．34±0．15）、（0．68±0．21）、（O．90±0．17）.正常骨组织分别与G1级级组、G2级组比较,差异均有显著性（P〈0．01）,G1级组与G2级组比较,差异有显著性（P〈0．01）。结论：Stathmin在骨肉瘤中过表达,与骨肉瘤的发生及发展密切相关。     

Prognostic significance of augmented metallothionein (MT) expression correlated with Ki-67 antigen expression in selected soft tissue sarcomas

In soft tissue sarcomas, the most important prognostic criteria include extent of malignancy (G), size of the tumour and intensity of Ki-67 antigen expression. In recent times expression of metallothionein (MT) in cells of some malignant processes of epithelial origin was found to correlate with intensity of Ki-67 antigen expression and to carry a possible prognostic significance. The present study aimed at a demonstration of prognostic value of MT expression and at comparing it with Ki-67 antigen expression and G grade in selected soft tissue sarcomas. Immunohistochemical studies were performed on paraffin sections in 54 cases of malignant fibrous histiocytoma (MFH), 18 cases of liposarcoma and 20 cases of synovial sarcoma. The extent of MT and Ki-67 antigen expression was evaluated and an attempt was made to correlate the results with each other and with grade of the tumour. Expression of MT was evident both in the cytoplasm and in cell nuclei of all studied sarcomas. The most pronounced MT expression was noted in MFH-type tumours. The extent of Ki-67 antigen expression was similar in MFH and liposarcoma and was the lowest in synovial sarcoma. In MFH, liposarcoma and synovial sarcoma a pronounced positive correlation was documented between expression of MT and Ki-67 antigen (r=0.85; p<0.001; r=0.93, p<0.0001; r=0.79, p<0.0001). In all types of the tumours a positive relation was detected between MT expression, expression of Ki-67 and G grade of malignancy in the tumour. Moreover, patients with higher MT expression in the studied tumours demonstrated a shorter survival. MT expression in soft tissue tumours of MFH, liposarcoma and synovial sarcoma type strongly correlated with intensity of proliferation (Ki-67) and G grade and could be useful in defining the extent of malignancy and in prognostic appraisal in the tumours.     

Malignant fibrous histiocytoma of the glans penis: a case report

BACKGROUND: Malignant fibrous histiocytoma has been regarded as the most common sarcoma of older adults. However, recent opinion regards pleomorphic malignant fibrous histiocytoma as an undifferentiated high grade pleomorphic sarcoma not otherwise classifiable utilizing current techniques available in surgical pathology. Notwithstanding controversy regarding its nomenclature, malignant fibrous histiocytoma involving the penis is exceedingly rare, with only 4 cases previously described, to our knowledge. CASE: An uncircumcised 73-year-old male presented with a painless, granular, partially necrotic lesion beneath the penile foreskin. There was no history of sexually transmitted disease, constitutional symptoms or dysuria. Examination of penile shaft, testicles, spermatic cord and inguinal lymph nodes were unremarkable. Biopsy revealed a markedly pleomorphic sarcoma. Subsequent, partial penectomy revealed the same lesion with an adjacent area of squamous cell carcinoma in situ. CONCLUSION: Malignant fibrous histiocytoma remains a diagnosis of exclusion. The investigation requires extensive tumor sampling in search of areas of differentiation and a complete battery of immunohistochemical markers. Therapeutically important entities in the differential diagnosis that must be ruled out include other poorly differentiated sarcomas, sarcomatoid squamous cell carcinoma and desmoplastic melanoma.     

Low grade fibromyxoid sarcoma: report of a case with fine needle aspiration cytology and histologic correlation

BACKGROUND: Low grade fibromyxoid sarcoma has been fully described histologically; however, the fine needle aspiration (FNA) cytologic findings are scantily defined, and the distinction from other benign and malignant soft tissue tumors can be difficult. CASE: We examined FNA cytologic material from a slowly growing, large chest wall mass in a 28-year-old woman. The surgical specimen was processed for routine histology and immunohistochemical studies. The cytologic smears were adequately cellular, showing spindly cells with uniform, elongated nuclei; small, inconspicuous nucleoli; and scanty, wispy cytoplasm associated with myxoid material. No significant nuclear pleomorphism or mitoses were noted. The excised tumor was well circumscribed, focally infiltrating the surrounding muscles. The cut surface was variable, featuring fibrous, solid, fleshy and myxoid areas. Microscopically, the solid, fibrous areas displayed increased cellularity with storiform, intersecting and parallel patterns. In the myxoid areas the cells grew in a haphazard fashion and appeared floating in abundant mucoid matrix associated with a capillary vascular network similar to the chicken-wire pattern seen in cases of myxoid liposarcoma. The tumor cells were spindly, with fusiform, uniform nuclei. Focal, moderate nuclear pleomorphism was noted. The mitotic index was low. The tumor cells were positive for vimentin, alpha-1-antitrypsin and lysozyme and negative for S-100, actin, desmin and CD34. CONCLUSION: Although low grade fibromyxoid sarcoma is a rare neoplasm, it should be recognized and distinguished from other soft tissue tumors because of its low malignant potential. The definitive FNA cytologic diagnosis can be challenging but is possible if the tumor is adequately sampled, with multiple passes from different areas. Clinical and radiologic correlations are of great help. All spindle cell tumors with myxoid changes, such as myxoid liposarcoma, myxofibrosarcoma, cellular myxoma, myxoid leiomyosarcoma and peripheral nerve sheath tumors, should be considered in the differential diagnosis. In contrast to the cytologic features, the histologic findings are characteristic and well established.     

Characterization of PD-1/PD-L1 immune checkpoint expression in soft tissue sarcomas

Inhibitors of the programmed death-1/programmed death-ligand 1 (PD-1/PD-L1) immune checkpoint system are used for treating various malignancies. However, evidence on their use in soft tissue sarcomas (STS) is limited. This study aimed to retrospectively investigate the relationship between the expression of PD-1/PD-L1 and related antigens in STS, and their association with clinical characteristics. Immunostaining for CD4, CD8, PD-1, PD-L1, IL-2, and IFN-γ was performed using pathological specimens harvested at the time of biopsy from 10 patients with undifferentiated pleomorphic sarcoma (UPS), nine with myxofibrosarcoma (MFS), and three with malignant peripheral nerve sheath tumor (MPNST) who were treated at our hospital. Subsequently, the positive immunostaining cell rates were calculated. We also examined the correlation between each immune positive cell rate and age, tissue grade, size, and maximum standardized uptake (SUV-max) values. The 3-year event-free survival (EFS) and overall survival (OS) rates were compared between the positive and negative groups (positive rate >10%; negative <10%) for various immune stains. The positive rates were also compared between the presence and absence of events groups. There was positive staining for the immune checkpoint molecules in every STS type except for PD-1 in MPNST. CD4, CD8, and PD-1 stained lymphocytes in close proximity to the tumor in adjacent tissue sections. A positive correlation was observed between the positive cell rates of each immune component including inflammatory cytokines such as IL-2 and IFN-γ. Additionally, the clinical features positively correlated with the positive PD-1/PD-L1 expression rates. No significant differences in the 3-EFS and OS rates were observed between the PD-1/PD-L1 positive and negative groups. Our results suggest that an inducible immune checkpoint mechanism may be involved in UPS, MFS, and MPNST.Key words: Immune checkpoint inhibitors, PD-1/PD-L1, soft tissue sarcoma, programmed death-1, programmed death-ligand 1     

Evaluation of P-glycoprotein expression in soft tissue sarcomas of the extremities

Soft tissue sarcomas comprise a heterogeneous group of mesenchymal tumors accounting for less than one-percent of adult neoplasms. In the last few years, the use of adjuvant chemotorapy has been proposed for the treatment of these lesions in order to obain a better systemic control, but its usefulness is still controversial. In this study, we evaluated whether P-glycoprotein, a membrane protein strictly associated with multidrug resistance, is overexpressed in soft tissue sarcomas. By using human multidrug resistant sarcoma cell lines as controls, we analyzed P-glycoprotein expression in 34 primary and in 23 relapsed soft tissue sarcomas of the extremities. Overexpression of P-glycoprotein was found in 6 out of 34 primaries (18%) and in 8 out of 23 relapses (35%). In particular, in malignant fibrous histiocytoma, the most frequent soft tissue sarcoma of adults, P-glycoprotein overexpression was found in 23% of primary untreated cases, in agreement with the reported relapse rate of this tumor after surgery and chemotherapy. These data suggest that, in soft tissue sarcomas, overexpression of P-glycoprotein may be of prognostic value and that the assessment of P-glycoprotein expression may be useful for the design of chemotherapy protocols.Abbreviations MDR multidrug-resistance - STS soft tissue sarcomas     

Primary malignant fibrous histiocytoma of the lung. Fine needle aspiration cytologic features

A case of primary malignant fibrous histiocytoma of the lung occurring in a 71-year-old woman is presented. The preoperative aspiration cytology showed a large-cell, undifferentiated, malignant neoplasm suggestive of carcinoma. Subsequent histologic examination revealed a primary malignant fibrous histiocytoma. The diagnosis was confirmed by electron microscopic and immunohistochemical studies. Cytologic features of this rare primary pulmonary sarcoma are discussed.     

Malignant fibrous histiocytoma in a bonnet macaque (Macaca radiata)

Spontaneous sarcomas, not associated with an underlying disease, appear to be relatively rare in nonhuman primates. Since 1970, there have been few reported cases of naturally-occurring sarcomas of any kind in these species. A malignant histiocytoma and malignant fibrous histiocytoma have been described in a rhesus macaque and baboon, respectively. A malignant fibrous histiocytoma is defined as a sarcoma of varied pattern consisting of a mixture of histiocytic and fibroblastic elements. It is thought that the two cells types arise from a common precursor or that the fibroblastic elements are derived from the histiocytes. These tumors are relatively common in humans. Here we report a case of spontaneously-occurring malignant fibrous histiocytoma in an adult bonnet macaque.     

A population-based study of soft tissue sarcoma incidence and survival in Australia: An analysis of 26,970 cases

BackgroundSoft tissue sarcomas (STS) are rare, often fatal tumors, but little is known of the epidemiology and survival in the Australian population. This study aims to provide the first epidemiological analysis of incidence and survival rates of STS in the Australian population.MethodsA retrospective population-based observational study was conducted between 1982 and 2009 of all patients with a diagnosis of STS using the Australian Institute of Health and Welfare (AIHW) Australian Cancer Database. Incidence rates per 100,000; incidence rate ratios, age-standardized incidence rates, prevalence and incidence rates of subtypes of STS, median, one-year and 5-year survival rates were examined.ResultsA total of 26,970 patients were identified. Between 1982 and 2009 STS incidence rates significantly increased from 3.99 [95% CI 3.68–4.32] to 6.12 [95% CI 5.80–6.46] per 100,000 Australian population, with a peak incident rate ratio (IRR) of 1.59 [95% CI 1.51–1.69] (p < 0.0001) in 2001. Median age at diagnosis increased from 58 to 63 years. Incidence rates were stable across all 10-year age cohorts, except for people aged over 70 where it increased. Overall, age-standardized incidence rates increased from 4.70 [95% CI 4.42–5.00] in 1982 to 5.87 [95% CI 5.63–6.11] per 100 000 Australians in 2009. Leiomyosarcoma (20.43%), malignant fibrous histiocytoma (16.14%), and soft tissue tumors/sarcomas, not otherwise specified (10.18%) were the most common STS subtypes. Median survival from diagnosis increased from 5.80 years [95% CI 5.06–6.54] in 1985–1989 cohort to 8.18 years [95% CI 7.54–8.81] in the 2000–2004 cohort (log-rank test p < 0.0001).ConclusionThe incidence of STS is increasing in Australia, most noticeably in those aged over 70 years, with a small but statistically significant increase in overall survival rates.     

Diaphyseal medullary stenosis with malignant fibrous histiocytoma: a hereditary bone dysplasia/cancer syndrome maps to 9p21-22

Diaphyseal medullary stenosis with malignant fibrous histiocytoma (DMS-MFH) is an autosomal dominant bone dysplasia/cancer syndrome of unknown etiology. This rare hereditary cancer syndrome is characterized by bone infarctions, cortical growth abnormalities, pathological fractures, and eventual painful debilitation. Notably, 35% of individuals with DMS develop MFH, a highly malignant bone sarcoma. A genome scan for the DMS-MFH gene locus in three unrelated families with DMS-MFH linked the syndrome to a region of approximately 3 cM on chromosome 9p21-22, with a maximal two-point LOD score of 5.49 (marker D9S171 at recombination fraction [theta].05). Interestingly, this region had previously been shown to be the site of chromosomal abnormalities in several other malignancies and contains a number of genes whose protein products are involved in growth regulation. Identification of this rare familial sarcoma-causing gene would be expected to simultaneously define the cause of the more common nonfamilial, or sporadic, form of MFH-a tumor that constitutes approximately 6% of all bone cancers and is the most frequently occurring adult soft-tissue sarcoma.     

Cytology of vaginal and uterine sarcomas

OBJECTIVE: To retrospectively review, based on cytologic and histopathologic findings, the diagnoses of 13 patients with uterine sarcoma and 1 with vaginal sarcoma. STUDY DESIGN: There were 8 cases of uterine carcinosarcoma (CS), 2 of leiomyosarcoma, 2 of endometrial stromal sarcoma (ESS), 1 of endocervical stromal sarcoma (ECSS) and 1 of malignant fibrous histiocytoma (MFH) of the vagina. The presence of sarcomatous components was retrospectively investigated by microscopic observation of preoperative specimens from the endocervical canal and endometrial cells. Characteristic features of sarcomatous cells were then investigated by cytodiagnostic micrometry of malignant cells. RESULTS: Of the 14 patients, 1 with low grade ESS and 1 with homologous CS were diagnosed as negative for sarcomatous components. One case of high grade ESS had been overlooked, as were 4 cases of CS. Thus, 7 cases (50%) were diagnosed as positive for sarcomatous cells by preoperative cytologic observation. Based on these findings, 12 of the 14 cases (85.7%) were positive for sarcomatous elements on retrospective reexamination of the specimens. CONCLUSION: Careful attention should be paid to small sarcomatous cells since cases of ESS or ECSS with such cells show morphologic characteristics similar to those of stromatous cells. Furthermore, careful microscopic observation of an entire specimen is required to avoid misdiagnosis as carcinoma since it is easy to overlook sarcomatous elements in smears with carcinosarcoma if there are only a few sarcomatous cells.