髓内高分化骨肉瘤的诊断及鉴别诊断

目的研究探讨髓内高分化骨肉瘤的临床病理特点及鉴别诊断。方法通过光镜及免疫组化方法研究1例髓内高分化骨肉瘤。该患者复发了2次,最终转移到T12椎体,随访8年。结果1例28岁女性患者,1998年在左股骨远端患髓内高分化骨肉瘤。2002年和2003年复发2次,2004年转移到T12椎体,呈低分化肉瘤样变。结论髓内高分化骨肉瘤常多发生于中、青年,早期诊断较困难,了解其病理、免疫组化特点及鉴别诊断,结合临床及X线表现,将有助于做出正确的诊断。     

表面骨肉瘤

目的探讨表面骨肉瘤的组织类型、临床、病理及影像学特征。方法回顾性分析经病理证实的表面骨肉瘤19例,其中骨膜骨肉瘤4例,部位分别为胫骨干中上段(3例)和腓骨下段(1例);X线片及CT显示肿物位于骨旁,外缘不规则,可见放射状骨针影;镜下为低度或中度恶性软骨肉瘤样改变。高度恶性表面骨肉瘤2例,肿物均位于股骨内侧;X线片显示肿物以广泛基底附于患骨,混杂骨膜反应;镜下显示高度恶性,似髓内型。去分化骨旁骨肉瘤1例,肿物位于股骨下端;患者曾先后4次手术,术后病理显示高分化组织内出现低分化区。经典骨旁骨肉瘤12例,肿物75%位于股骨下端干骺端后部。结果骨膜骨肉瘤4例均行保肢手术,已无瘤生存1.1～6.5年。高度恶性表面骨肉瘤2例,其中1例拒绝手术,3个月后死于肺及肝转移;另1例应用大剂量化疗及保肢治疗,现已无瘤生存19个月。去分化骨旁骨肉瘤1例,行假体旁复发性肿瘤切除,现已无瘤生存6个月。经典骨旁骨肉瘤的初始治疗均为保肢手术,其中9例随访1.2～9.6年,除1例局部复发、1例死于肺转移外,另外7例均无瘤生存。结论表面骨肉瘤四种组织类型各具有特殊性,应区别对待。骨膜骨肉瘤及经典骨旁骨肉瘤应以局部广泛切除或瘤段切除为主,预后较好;去分化型和高度恶性表面骨肉瘤恶性程度较高,预后较差。保肢或截肢均应配合有     

老年骨肉瘤10例治疗总结

目的探讨老年骨肉瘤（≥60岁）的临床特点和治疗效果。 方法回顾性分析2007年1月至2017年3月期间山东大学齐鲁医院骨科收治的≥60岁的骨肉瘤患者10例,女性8例,男性2例,年龄60~76岁,平均（66±6）岁,病变位于股骨5例、踝关节软组织1例、多中心1例、骨盆（Ⅲ区）1例、肱骨1例、距骨1例。确诊后行截肢术2例,截肢术结合化疗2例,广泛切除术1例,广泛切除术结合化疗3例,边缘切除结合放疗1例,单纯化疗1例。 结果10例患者中,4例就诊时病史已超过6个月,2例就诊时已出现肺转移,5例合并基础疾病,2例血清ALP水平升高（>125 U/L）。影像学方面,7例为混合型,1例为溶骨型,1例为成骨型,1例位于软组织。肿瘤组织类型方面,普通型骨肉瘤7例,骨旁骨肉瘤2例,髓内高分化性骨肉瘤1例。10例患者随访3.5~124个月,平均（40±37）个月。1例接受单纯化疗的肺转移患者于明确诊断后3.5个月死亡,另1例肺转移患者接受转移灶射频消融术,随访58个月带瘤生存,1例距骨骨肉瘤复发后行截肢术。其余7例均未出现肿瘤复发或远处转移。 结论老年骨肉瘤临床较为少见,具有不同于青少年骨肉瘤的临床特点和预后,应早期明确诊断,采取适合患者自身情况的治疗方法,以尽可能降低复发和转移的发生率,延长生存期。     

MRI在评价骨肉瘤侵袭骨骺中的价值

目的：探讨NRI在评价骨肉瘤侵袭骨骺中的价值。方法：复习13例骺板未闭骨肉瘤的NRI、X线平片所见，与病理组织学相对比。男3例，女10例；年龄平均11．2(5—15)岁。股骨下端9例，胫骨上端4例。8例化疗结束后再次拍X线平片行NIll检查。结果：所有患者的骺板未闭合，与病理组织学所见对比分析发现X线平片诊断骨肉瘤侵蚀骨骺的准确率为50％，MR的准确率为1000%。8例化疗后已侵蚀骨骺的骨肉瘤并不会完全消失。结论：NRI的检查与病理检查结果相一致。对于指导保留骨骺的保肢术选择具有重要的意义。     

毛细血管扩张型骨肉瘤误诊分析

目的 探讨毛细血管扩张型骨肉瘤的临床、影像及病理学特点.方法 结合文献回顾,对2例毛细血管扩张型骨肉瘤患者的诊断与治疗过程进行分析.结果 毛细血管扩张型骨肉瘤是多见于青壮年、发病迅速的恶性骨肿瘤,影像学上表现为广泛溶骨性破坏,组织学上显示为动脉瘤样扩张的含血腔隙,而瘤细胞的异型性及较多的核分裂像是毛细血管扩张型骨肉瘤区别于动脉瘤样骨囊肿的特征性病理组织学特征.电镜检查与免疫组织学检查起重要补充作用.结论 临床特征、影像学,特别是组织病理学检查是毛细血管扩张型骨肉瘤避免误诊的关键.     

骨肉瘤髓腔内侵袭范围MRI测量与确定合理截骨平面的相关研究

[目的]探讨磁共振成像(MR I)确定肢体骨肉瘤在髓腔内侵袭范围的准确性及据此决定保肢术中合理截骨平面的可靠性。[方法]25例骨肉瘤患者,股骨远端15例,胫骨近端10例,均行瘤段切除定制人工假体置换术,截骨平面为MR I所示肿瘤骨干侧边界外30 mm处。术后对所有患者进行随访,对患者下肢的功能进行评估。通过术前MR I、X线和术后标本的肉眼及病理测量确定肿瘤在髓腔内的侵袭范围。对所得数据进行统计学分析。[结果]25例患者随访5~36个月,平均15.5个月,膝关节活动度0~120°,股骨假体的活动度优于胫骨假体活动度。按Ennek ing肌肉骨骼肿瘤术后下肢功能评分标准,平均功能恢复率为80%。所测得肿瘤在髓腔内的侵袭范围分别为(107.4±34.5)、(78.6±25.6)、(92.6±20.5)、(104.3±32.8)mm。统计学分析表明磁共振图像所确定的肿瘤髓内侵袭范围与实际范围2组数据之间无显著性差异。[结论]应用MR I确定骨肉瘤髓内侵袭范围有较高准确性,以此作为判断骨肉瘤手术时切除边界的参考是可靠的。经短期随访证实,在MR I确定的肿瘤髓内边界外30 mm处作为保肢术中的截骨平面是安全和有利于肢体功能重建的。     

原发性骨肉瘤影像学表现与病理分型的关系

目的探讨原发性骨肉瘤影像学表现与病理分型的关系。方法分析82例原发性骨肉瘤患者X线、CT、MRI等影像学表现与病理分型的关系。结果82例原发性骨肉瘤患者病理分型:普通型67例,小细胞型6例,髓内高分化型2例,毛细血管扩张型2例,高级别骨表面型1例,骨膜型2例,骨旁型2例。不同病理分型的原发性骨肉瘤X线、CT、MRI等影像学表现均存在差异。结论原发性骨肉瘤的病理分型以普通型较常见,骨高密度成骨灶是各病理分型均具有的影像学表现,通过X线、CT、MRI等影像学表现可对不同病理分型的原发性骨肉瘤作出鉴别,可为制定合适的临床治疗方案提供重要的参考和依据。     

骨膜骨肉瘤与高度恶性表面骨肉瘤

目的报告表面骨肉瘤的两种少见亚型,即骨膜骨肉瘤和高度恶性表面骨肉瘤的诊治经验。方法回顾 5例骨膜骨肉瘤和 4例高度恶性表面骨肉瘤患者的影像学和病理学特点,并对临床治疗结果进行分析。 5例骨膜骨肉瘤患者,男 1例,女 4例;年龄 28～ 42岁,平均 35岁;肿瘤位于胫骨上段者 4例,股骨下段者 1例。 4例高度恶性表面骨肉瘤患者,男 3例,女 1例;年龄 17～ 23岁,平均 19.25岁; 4例患者肿瘤均位于股骨下段。结果 5例骨膜骨肉瘤均予以广泛切除,除 1例因局部复发行截肢术,现无瘤生存 1年 3个月外,余 4例已无瘤生存 3～ 9年,平均 5年 9个月。 4例高度恶性表面骨肉瘤患者,虽经积极综合治疗,但仅 1例无瘤生存 7年; 1例术后 2年 5个月复发而截肢,现无瘤生存 1年 9个月; 1例于术后 2年 4个月死于肺转移;另 1例在确诊后 3个月死亡。结论骨膜骨肉瘤和高度恶性表面骨肉瘤各具鲜明的影像学和病理学特点,骨膜骨肉瘤恶性程度较低,应采用以局部广泛切除为主的手术治疗,预后相对较好;而高度恶性表面骨肉瘤的生物学行为则与经典的髓内骨肉瘤相似,预后较差,必须采用手术与化疗相结合的综合治疗方法。     

Ki-67蛋白在人骨肉瘤中表达的研究

目的:探讨ki-67蛋白在人骨肉瘤中的表达及其与骨肉瘤发生、发展和病理分型的关系。方法:采用免疫组化S-P蛋白的表达情况。结果:ki-67蛋白在骨肉瘤中阳性表达率为66.67％,在骨软骨瘤中阳性表达率为10％,比较骨肉瘤和骨软骨瘤染色阳性率和染色强度,差异均有显著性(P<0.05)。ki-67蛋白在骨肉瘤高分化组阳性表达率为28.57％,在骨肉瘤低分化组的阳性表达率为78.26％,两组染色阳性率及染色强度的差异均有显著性(P<0.05)。而ki-67蛋白在骨肉瘤各病理亚型的染色结果,差异无显著性(P>0.05)。结论:ki-67蛋白表达与骨肉瘤发生、发展密切相关,其阳性表达率和表达程度是判断骨肉瘤恶性程度的一个指标。     

骨肉瘤多药耐药表型的分化特性及矛盾相关性分析

目的：探讨肿瘤的多药耐药性和恶性程度的关系,主要研究骨肉瘤细胞在向耐药表型的转化过程中,是否出现如骨源性干细胞在成熟分化过程中相似的生物学行为,或者是更复杂的生物学表型。方法：采用不同表达程度Pgp的骨肉瘤耐药亚型细胞,并针对增殖、分化及转移关键性基因Stathmin及VEGF,应用原位杂交和RT-PCR来衡量Mdr1同Stathmin、VEGF表达的相关性。结果：Mdr1/P170表型的骨肉瘤细胞的碱性磷酸酶的活性明显高于亲本细胞,说明Mdr表型的骨肉瘤细胞出现了分化。INH和RT-PCR结果揭示Mdr1mRNA同VEGF、Stathmin mRNA表达呈负相关。结论：Mdr1/P170的表达预示着骨肉瘤细胞向成熟状态分化,为研究Mdr及其逆转开拓了新的思路,为正确认识Pgp的功能提供了新的研究方法。     

Evaluating marrow margins for resection of osteosarcoma. A modern approach.

Intraoperative evaluation of bone marrow margins by frozen section analysis is a common practice in the surgical treatment of osteogenic sarcoma. The purpose of this study was to assess the clinical use of intraoperative marrow margin evaluation to rule out occult intramedullary tumor extension in osteosarcoma surgery. One hundred twenty-eight consecutive patients with high grade osteosarcoma diagnosed between 1988 and 1996 (Group 1) were reviewed retrospectively and compared with 92 consecutive patients treated from 1979 to 1984 (Group 2). Eighty-five patients in Group 1 met the inclusion criteria of having high grade intramedullary lesions of the long bones observed on preoperative magnetic resonance imaging evaluation of the lesion and intraoperative frozen section analysis of the bone marrow margin. Thirty-three patients in Group 2 met the same inclusion criteria with the exception of having preoperative magnetic resonance imaging. Ninety-two marrow margins in Group 1 and 33 marrow margins in Group 2 were evaluated by frozen section. All 92 marrow margins in patients in Group 1 were negative by frozen section analysis and permanent histologic analysis. Of the 33 marrow margins in patients in Group 2, three (9.1%) were reported positive for tumor. Of these, one was found to be a false positive result on permanent pathologic examination. In addition, one false negative frozen section result was found, which was positive for tumor on permanent pathologic examination. The difference in true positive results of marrow margins between Group 1 and Group 2 was statistically significant. Intraoperative marrow margin evaluation by frozen section is not mandatory with modern imaging techniques. Preoperative evaluation of tumor extent using magnetic resonance imaging and intraoperative evaluation of the specimen by the pathologist (done by bivalving the specimen) are reliable methods to ensure adequate surgical margins in most cases of conventional osteosarcoma of the long bones.     

Parosteal osteosarcoma: case report and review of the literature

BACKGROUND: The majority of osteosarcoma cases of the head and neck are high-grade lesions. We present a case and discuss the diagnostic and therapeutic implications of a rare low-grade parosteal osteosarcoma of the maxilla. METHODS: A 32-year-old man presenting to the Head and Neck Surgical Oncology clinic with a 1-year history of a firm palatal mass. Evaluation clinically and radiographically raised the suspicion of an osteosarcoma. RESULTS: A partial maxillectomy revealed a parosteal osteosarcoma with negative margins. No adjuvant therapy was recommended, and the patient remains without evidence of local recurrence after 3 years. CONCLUSIONS: Parosteal osteosarcomas of the head and neck region are rare, low-grade variants of osteosarcoma, but have the potential to recur with simple local excision. Clinical and radiographic features are diagnostically helpful. Definitive diagnosis comes from histopathology, and wide local resection should be employed as the optimal treatment.     

Osteosarcoma of bone

The definition of osteosarcoma requires that a malignant tumor of bone produce osteoid or bony matrix. With this as a basic definition, osteosarcoma of bone can be divided into several clinicopathologic entities based on clinical, roentgenographic, and pathologic features. The tumors can be broadly divided into those arising within the bone and those arising on the surface of bone. Most intraosseous osteosarcomas are high-grade malignant tumors that occur in children and adolescents. A small number occur in older patients, and they may be related to a preexisting condition, such as Paget's disease or radiation. The site of the lesion has prognostic importance. Osteosarcoma of the jaws is associated with an especially good prognosis, whereas the same kind of tumor involving the skull has a very poor prognosis. Most osteosarcomas of the surface of bone are well or moderately differentiated and are associated with an excellent prognosis. Received for publication on March 17, 1998; accepted on April 28, 1998     

Limb-saving surgery in osteosarcoma by vascularized fibular graft

Osteosarcoma is the most malignant bone tumor of the extremities that occurs in younger age groups; however, the prognosis of this disease has been improving. Limb-saving procedures have been successfully performed in more than half of the new cases of this disease. Because osteosarcoma occurs in young patients and their life expectancy is generally long, the rescued limbs must provide as full a service as possible for several decades. We have attempted the rescue of limbs in osteosarcoma patients, using the vascularized fibular graft (VFG). We performed a retrospective clinical and radiographic review of 12 patients with at least 3 years of follow up. The results were satisfactory in 11 out of the 12 patients from both the oncologic and functional point of view. There were no severe complications or donor site morbidity. Functional status, evaluated according to the system of Enneking et al. (Enneking WF, Dunham W, Gebhart MC, et al. A system for functional evaluation of reconstructive procedures after surgical treatment of tumors of the musculoskeletal system. Clin Orthop 1993;286:241–246), with some modifications exceeded 80%, except in one patient. The results of this study suggest that, because of the resulting durability of the reconstructed limb, VFG should be considered for limb rescue in young patients with osteosarcoma.     

Low-grade central osteosarcoma mimicking fibrous dysplasia: a report of two cases

Low-grade central osteosarcoma is an unusual variant of conventional osteosarcoma. We present here two rare cases of low-grade central osteosarcoma resembling fibrous dysplasia. A 24-year-old woman diagnosed as fibrous dysplasia was treated with intra-lesional excision and curettage of the tumor but tumor recurred at 4 months after surgery. Distal femoral en-bloc resection was performed followed by arthroplasty with mega-prosthesis of the knee. A 57-year-old man diagnosed as central osteosarcoma was treated with wide excision of the tumor, followed by reconstruction with the vascularized fibula graft combined with an autogenous irradiated bone graft. Because of the difficulty in distinguishing low-grade central osteosarcoma from a benign lesion, open biopsy is needed to obtain a large tumor sample. Careful clinical and pathological evaluation is required to obtain a definite diagnosis. The treatment of low-grade central osteosarcoma is en-block resection with wide surgical margins.     

Osteosarcoma of the meninges in association with glioblastoma

Summary Primary intracranial osteosarcoma not originating in the skull is a distinctly rare tumour, as is post-irradiation sarcoma of short latency. The authors report the case of a 56 year old caucasian male who underwent resection of a glioblastoma of the left temporal region and was subsequently administered partial field external beam radiation therapy (XRT) to a total dose of 5940 cGy. Seven months following the completion of XRT, an enhancing region adjacent to the surgical site was noted on followup magnetic resonance images (MRI), one which increased in size on serial studies. Initial biopsy of the dural lesion adjacent to the temporal resection site revealed a sarcoma with a suggestion of osseous differentiation. Subsequent reoperation with resection of the lesion showed it to be a primary meningeal tumour, and histological evaluation of the lesion demonstrated an osteosarcoma. Immunohistochemical staining for p53 protein performed on both the original glioblastoma and the subsequently resected osteosarcoma showed widespread nuclear positivity. The clinical, radiographic and pathologic features of this unusual case are discussed. Meningeal osteosarcoma should be included among the rare secondary sarcomas of the meninges which may be associated with malignant glioma.     

Epithelioid multinodular osteoblastoma: a clinicopathologic analysis of 26 cases

We describe the clinical, radiologic, and pathologic features of 26 osteoblastomas with a multinodular growth pattern (defined as multiple nidi in a single tumor) and primarily epithelioid-appearing cells. Clinical information and histologic slides for all 26 patients and radiologic studies for 9 were examined. Follow-up information was obtained from medical charts at our institution and from correspondence with pathologists who submitted cases in consultation. Of the 23 patients with osteoblastoma whose sex had been recorded, 18 were male and 5 were female (median age, 17.8 y). The most common site was the jawbones, although long bones were also involved. Radiologic studies suggested a benign lesion in 5 patients, a malignant lesion in 2, and indeterminate features in 2. Histologically, the tumors appeared to be multiple nodules of epithelioid cells, most with a lacy, blue-bone matrix. Frequently, there were sheets of cells with no matrix. Follow-up information was obtained for 14 of the 23 patients. One patient died in an automobile accident; all others were alive as long as 29 years after treatment. Epithelioid osteoblastomas mimic osteosarcoma because of similar cellularity and cytologic features. Although follow-up is limited, we are cautiously optimistic that these are benign lesions.     

Aggressive Osteoblastoma of the Maxilla: A Case Report and Review of the Literature

Aggressive osteoblastoma is a rare primary bone neoplasm with the potential for local invasion and recurrence. While the vertebrae or long bones are most commonly affected, few well-documented cases have been reported in the jaws. A 25-year-old man presented with a palatal mass of several months’ duration. He reported the lesion had undergone gradual enlargement and, while generally asymptomatic, had recently become increasingly painful. An incisional biopsy was interpreted as “osteoblastic neoplasm” most suggestive of osteoblastoma. However, final diagnosis was deferred until the resection specimen could be evaluated. Following partial maxillectomy, histopathologic examination revealed a proliferation of large epithelioid cells with eccentric nuclei and prominent nucleoli associated with broad, irregular deposits of osteoid and trabeculae of bone. The lesional cells exhibited minimal pleomorphism with infrequent, normal-appearing mitotic figures and numerous osteoclast-like giant cells were observed within an associated loose fibrovascular stroma. Transformation of “blue bone” to more organized eosinophilic trabeculae of woven bone was noted at the periphery of the lesion and there was no evidence of invasion. A diagnosis of aggressive osteoblastoma was made. Previous reports of gnathic aggressive osteoblastoma are reviewed and the features that distinguish this process from conventional osteoblastoma or osteoblastoma-like osteosarcoma are presented.     

Dedifferentiated chondrosarcomas arising in preexisting osteochondromas

BACKGROUND: Dedifferentiated chondrosarcomas that arise in osteochondromas are extremely rare lesions for which very little information on treatment and outcome is available in the literature. The purpose of the present study was to describe the specific clinical, radiographic, and histologic features of this lesion and to evaluate the oncologic outcome after different treatment strategies. METHODS: We reviewed the files of the Rizzoli Institute between 1970 and 2002 and identified eighteen patients for whom adequate records and histologic images were available and in whom a high-grade sarcoma had been diagnosed at the same location as a preexisting osteochondroma. Radiographic studies, histologic slides, and clinical records were reviewed, the features of those studies were tabulated, and prognostic features and the results of treatment were identified. RESULTS: The patients included twelve men and six women with an average age of forty-six years (range, twenty-two to seventy-four years). Eight lesions occurred in patients with multiple osteochondromas, and ten occurred in patients with solitary lesions. The most common locations were the pelvis (six lesions) and the femur (five lesions). Symptoms included pain, swelling, and a growing mass; the average duration of symptoms was eighteen months. Radiographically, ten lesions appeared as a conventional secondary chondrosarcoma arising in an exostosis, whereas eight showed typical signs of dedifferentiation. Histologic evaluation of the cartilage component demonstrated thirteen grade-1 and two grade-2 chondrosarcomas. In three cases, no cartilage component was recognized. The dedifferentiated component was considered to be an osteosarcoma in nine cases (including six cases in which it was osteoblastic and three in which it was fibroblastic), a malignant fibrous histiocytoma in eight, and a fibrosarcoma in one. The dedifferentiated component represented an average of 59% (range, 20% to 100%) of the lesion. For the fifteen patients who were managed at the authors' institution, the two and five-year survival rates were 47% and 29%, respectively; the median survival time was fourteen months. Patients who were managed with a combination of surgery and chemotherapy had a better overall survival rate than did those who were managed with surgery alone (p = 0.03). CONCLUSIONS: Dedifferentiated chondrosarcoma arising in a preexisting osteochondroma is an extremely rare lesion with a poor prognosis. In the present small series, overall survival was better when wide surgical resection was combined with adjuvant chemotherapy.