肾间叶性软骨肉瘤1例报告并文献复习北大核心CSCD

0引言 间叶性软骨肉瘤是一种少见的恶性肿瘤,可发生于骨和骨外软组织,其中肾间叶性软骨肉瘤则更是极其罕见的一种,它具有明显的未分化的肿瘤细胞和分化良好的软骨成分。临床上比较少见,需病理组织学及免疫组织化学明确诊断[1]。本文通过分析1例肾间叶性软骨肉瘤患者的临床病理特征,并对相关文献进行复习,以提高对肾间叶性软骨肉瘤的认识。1病例资料1.1临床资料患者45岁,女,绝经2年。     

颅内间叶性软骨肉瘤的临床病理分析及文献回顾

目的:报道1例发生于颅内右顶叶的间叶性软骨肉瘤,并结合文献探讨其临床及组织病理学特点. 方法:对1例发生于右顶叶的间叶性软骨肉瘤进行常规病理及免疫组化观察.结果:成年男性患者,左侧肢体乏力伴下肢间断性抽搐半年,头颅MRI示右顶叶占位.镜下可见肿瘤组织主要由弥漫性大小基本一致的小圆形瘤细胞及散在分布的小岛屿状的软骨细胞灶构成,核分裂像较少.免疫组化染色软骨区表达S-100( ),不表达CK(-)、EMA(-)、NF(-)、GFAP(-)、NSE(-)、CD34(-). 结论:原发于颅内的间叶性软骨肉瘤极为罕见,诊断时容易误诊,主要依赖于临床、影像学、病理学三方面的结合,并且需与少突胶质细胞瘤、血管外皮细胞瘤、滑膜肉瘤等相鉴别.     

间叶性软骨肉瘤3例临床病理观察

目的：探讨间叶性软骨肉瘤（mesenchymal chondrosarcoma，MC）的临床病理特征、诊断与鉴别诊断。方法：结合临床资料及影像学所见，分析3例MC患者的组织病理特点并复习相关文献。结果：患者均为成人，平均年龄42．3岁。主要临床表现为疼痛、肿胀、局部肿块伴或不伴肿瘤压迫症状。发病部位分别是小脑幕、左胸壁及骶椎。x线显示溶骨性破坏，其内可见斑驳钙化。巨检：肿瘤组织切面呈灰白色，质地软硬不均，点缀着不规则矿化沉积物。镜下肿瘤由密集的未分化细胞与散在的软骨岛构成。在蓝染的小细胞区，细胞较原始，呈圆形或短梭形，胞质稀少，排列成片层状或形成血管外皮瘤样；在软骨成分区，细胞相对成熟，分化良好。两种成分截然分界或逐渐混杂。免疫组织化学显示3例未分化细胞区vimentin呈弥漫阳性，部分CD99呈阳性；软骨分化区S-100呈阳性，而二者均表达Sox-9。结论：MC是一种罕见的高级别骨或软组织软骨源性恶性肿瘤。具有独特的双向组织学表现，预后不佳。     

骨外间叶性软骨肉瘤二例

骨外间叶性软骨肉瘤二例袁怀文广东总队武警医院病理科（５１０５０７）间叶性软骨肉瘤比较罕见。一般认为７０％的病例发生在骨，３０％起源于软组织。现将我们所见骨外间叶性软骨肉瘤２例报告如下。例１．女，３５岁。患者在无外伤时感颈右侧胀痛，左侧肢体乏力，右下肢...     

手足部内生软骨瘤及原发性软骨肉瘤的临床病理分析

目的 探讨手足短管状骨内生软骨瘤及原发性软骨肉瘤的影像学及临床病理学特征,为二者的诊断及鉴别诊断提供依据.方法 回顾性研究手足短管状骨的内生软骨瘤204例,年龄6～72岁（平均34岁）,高峰年龄20～40岁,女∶男约为2∶1.其中指骨166例,掌骨34例,足趾骨4例.原发性软骨肉瘤2例,均发生于指骨,年龄分别为52岁、76岁,男女各1例.对上述病例的临床影像学资料进行分析,病理学特征通过石蜡切片HE染色进行观察.结果 手足短管状骨的内生软骨瘤临床多无症状,影像学呈现膨胀性的骨破坏,组织学表现为分化较好的软骨组织伴不同程度黏液变、钙化及细胞轻度非典型性,未见浸润骨及软组织.软骨肉瘤临床多伴有疼痛,影像学可见皮质破坏、软组织包块形成.2例病理组织学分级均为II级,其肿瘤细胞较内生软骨瘤更为丰富,非典型性及黏液变显著,可见肿瘤性软骨浸润宿主骨、侵破皮质进入软组织.结论手足短管状骨以内生软骨瘤多见,尽管组织学具有非典型特征,但其呈现＂软骨岛＂结构及为宿主骨小梁包绕的生长方式与软骨肉瘤的浸润性生长不同,手足短管状骨软骨肉瘤罕见,其组织学显示显著的恶性特征,二者诊断时应结合临床、影像学特征及病理学特点进行综合分析.     

发生颅外转移的脑膜血管周细胞瘤临床病理观察

目的探讨脑膜血管周细胞瘤发生颅外转移的临床病理特征、诊断与鉴别诊断要点。方法对9例发生颅外转移的脑膜血管周细胞瘤进行临床病理分析、组织形态学及免疫组化染色观察,结合文献对其,临床表现、病理形态特点及鉴别诊断进行探讨。结果患者年龄32～59岁,男性3例,女性6例。转移部位有肝、肺、骨、腹腔、皮下等。转移间隔时间0．5～10年不等,光镜下瘤细胞为形态大小较一致的短梭形细胞,瘤细胞间富于小血管,呈裂隙状或“鹿角状”,核卵圆形,具有非典型性,核分裂象多少不等,WHOII级或III级。免疫组化瘤细胞波形蛋白均呈强阳性表达,CD34阳性程度不等,弥漫阳性或局灶阳性。结论脑膜血管周细胞瘤临床呈侵袭性,易复发和转移到中枢神经系统外,鉴别诊断包括孤立性纤维性肿瘤、脑膜瘤及间叶性软骨肉瘤等。     

乳腺腺泡型横纹肌肉瘤1例及文献复习

目的：探讨乳腺腺泡型横纹肌肉瘤的病理诊断及鉴别诊断。方法：对1例乳腺腺泡型横纹肌肉瘤进行HE切片、免疫组化染色等观察并复习文献进行讨论。结果：镜检肿瘤细胞排列杂乱，可见部分呈腺泡及假腺管状，细胞呈短梭及圆形，胞浆丰富红染，核大偏位。肿瘤细胞特异性表达MyoD1。结论：乳腺腺泡型横纹肌肉瘤是非常罕见的恶性肿瘤，其肿瘤结构、细胞形态都有一定的特征，确诊需免疫组化染色的帮助，应与小细胞类型以及乳腺间叶组织可能发生的其他肉瘤相鉴别，同时横纹肌肉瘤的亚型区分对治疗也很重要。     

软组织间叶性软骨肉瘤一例

间叶性软骨肉瘤少见,发生于软组织者更少;我院诊治一例原发于软组织的间叶性软骨肉瘤,报告如下: 患者男,42岁,住院号1633;于1988年初发现右上臂上1/3段内侧有一黄豆大肿物,无痛,渐增大,1988年底在当地医院手术切除,病理诊断“纤维肉瘤”。术后约三个月,右上臂原发部位再次发现一肿瘤,增大迅速,半年增至成人拳头大。于1989年9月4日转我院治疗。检查见肿物位于右上臂上1/3至中1/3段内侧,约9×4×3 cm,表面凹凸不平,边缘不整,     

腹腔内促结缔组织增生性小圆细胞瘤临床病理分析

目的：探讨腹腔内促结缔组织增生性小圆细胞瘤临床病理特征。方法：对2例原发于腹腔的促结缔组织增生性小圆细胞瘤进行HE、免疫组织化学观察并复习相关文献。结果：肿瘤细胞为小圆形，呈巢团状排列，周围结缔组织及血管明显增生。免疫组织化学表达上皮性、间叶性、神经性标记物。结论：促结缔组织增生性小圆细胞瘤是一种特殊的高度恶性肿瘤；预后极差，主要应与小细胞未分化癌、尤文氏肉瘤、PNET、神经母细胞瘤等鉴别。     

上皮样肉瘤二例临床病理分析

 目的　探讨上皮样肉瘤的临床、病理学特征及鉴别诊断要点。方法　收集2例上皮样肉瘤患者的临床病理资料,光学显微镜下观察和分析其组织学形态特征,并通过免疫组织化学方法染色分析。结果　2例患者中经典型和近端型上皮样肉瘤各1例。2例上皮样肉瘤均由两种细胞组成,一种为圆形或多角形的上皮样细胞,胞质丰富,嗜酸染色,另一种为肥大的梭形细胞,组织结构呈结节状,可见中心性坏死。免疫组织化学染色2例广谱CK、EMA、Vimentin均阳性表达,CK7、CD68、HMB45、Syn、SMA均阴性表达,其中1例少数细胞S-100阳性表达。结论　上皮样肉瘤是一种少见的软组织恶性肿瘤,需要临床特点、组织学特征及免疫组织化学染色结果相结合,才能做出准确的诊断。     

Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases

A series of 111 mesenchymal chondrosarcomas was reviewed. The ages of the patients ranged from 5 to 74 years, and approximately 60% of them were in the second and third decades of life. There was no significant sex predilection. Seventy-two tumors, including 5 that involved multiple skeletal sites, arose in bone. Thirty-eight tumors were found in extraskeletal sites. At initial diagnosis, multifocal involvement, both in bone and in soft tissue, was observed in one case. Roentgenographically, the lesions in bone frequently resembled ordinary chondrosarcomas, showing osteolytic and destructive appearances with stippled calcification. Tumors in extraskeletal sites were almost always identified as calcified masses. Histologically, a combination of cellular zones composed of undifferentiated small cells and chondroid zones typically presented a bimorphic appearance that was virtually pathognomonic in most cases. Ablative surgical treatment seemed to be the procedure of choice. The value of irradiation or chemotherapy (or both) was difficult to assess in the current study. Prognosis for patients with mesenchymal chondrosarcoma is usually poor, and long-term follow-up is necessary. In a group of 23 patients from the Mayo Clinic, the 5-year survival rate was 54.6% and the 10-year survival rate was 27.3%.     

The clinical approach towards chondrosarcoma

This review provides an overview of the histopathology, classification, diagnostic procedures, and therapy of skeletal chondrosarcoma. Chondrosarcomas that arise de novo are primary chondrosarcomas, whereas chondrosarcomas developing superimposed on pre-existing benign cartilage neoplasms such as enchondromas or osteochondromas are referred to as secondary chondrosarcomas. Conventional chondrosarcomas can be categorized according to their location in bone into central, peripheral, and juxtacortical chondrosarcomas. Histological grading is related to prognosis; however, it is also subject to interobserver variability. Rare subtypes of chondrosarcoma, including dedifferentiated, mesenchymal, and clear cell chondrosarcoma, are discussed as well. Magnetic resonance imaging is necessary to delineate the extent of the intraosseous and soft tissue involvement preoperatively. Computed tomography is especially recommended in the pelvis and other flat bones where it may be difficult to discern the pattern of bone destruction and the presence of matrix mineralization. Wide, en-bloc excision is the preferred surgical treatment in intermediate- and high-grade chondrosarcoma. In low-grade chondrosarcoma confined to the bone, extensive intralesional curettage followed by local adjuvant treatment and filling the cavity with bone graft has promising long-term clinical results and satisfactory local control. Chondrosarcomas are relatively radiotherapy resistant; therefore, doses >60 Gy are needed in attempts to achieve local control after incomplete resection. Irradiation with protons or other charged particles seems beneficial in this curative situation. Chemotherapy is only possibly effective in mesenchymal chondrosarcoma, and is of uncertain value in dedifferentiated chondrosarcoma. Potential new systemic treatment targets are being discussed.     

Advanced chondrosarcomas: role of chemotherapy and survival

BackgroundThere are limited data about the role of chemotherapy in patients withadvanced chondrosarcomas.MethodsThe medical charts of 180 patients with advanced chondrosarcomas having received chemotherapy in 15 participating institutions between 1988 and 2011 were reviewed.ResultsMedian age was 52 years. Sixty-three percent of patients had conventional chondrosarcoma and 88% had metastatic disease. Combination chemotherapy was delivered in 98 cases (54.5%). One hundred and thirty-one patients (73%) received an anthracycline-containing regimen. Using RECIST, the objective response rate was significantly different according to histological subtype, being 31% for mesenchymal chondrosarcoma, 20.5% for dedifferentiated chondrosarcoma, 11.5% for conventional chondrosarcoma and 0% for clear-cell chondrosarcoma (P = 0.04). Median progression-free survival (PFS) was 4.7 months [95% confidence interval (CI) 3–6.5]. Performance status (PS) ≥2, number of metastatic sites ≥1 and single-agent regimen were independently associated with poor PFS. Median overall survival (OS) was 18 months (95% CI 14.5–21.6). PS, number of metastatic sites and palliative surgery were independently associated with OS.ConclusionsConventional chemotherapy have very limited efficacy in patients with advanced chondrosarcoma, the highest benefit being observed in mesenchymal and dedifferentiated chondrosarcoma. These data should be used as a reference for response and outcome in the assessment of investigational drugs in advanced chondrosarcoma.     

Extraskeletal myxoid chondrosarcoma in young children

Two extraskeletal myxoid chondrosarcomas with a solid soft tissue mass occurred on the right upper arm of a 4-year-old boy and on the chest wall of a 1-year-old boy. Microscopically, both tumors were characterized by lobular configuration and were sparsely cellular with a background of myxoid matrix. The cells were small and round, and appeared undifferentiated, sometimes with a narrow eosinophilic cytoplasm. They grew in nests or strands and sometimes in a single file. They were strongly positive for S-100 protein and vimentin. Ultrastructural features suggested that the cells had a poorly differentiated mesenchymal nature with chondrocytic differentiation. These are the sixth and seventh reported cases of extraskeletal myxoid chondrosarcoma occurring in children. There are definite differences between this tumor with immature features and the extraskeletal myxoid chondrosarcoma in adults. Problems of differential diagnoses from other small round cell sarcomas also are discussed.     

Immunohistochemical study of bone GLA protein in primary bone tumors.

METHODS. The immunoreactivity of bone GLA protein (BGP) in primary bone tumors, including osteosarcoma, chondrosarcoma, malignant fibrous histiocytoma of bone (MFH), and giant cell tumor of bone (GCT), was investigated with anti-BGP rabbit serum and peroxidase-antiperoxidase complex. RESULTS. As to intracellular localization, BGP antigenicity was detected in 33 of 35 cases of osteosarcoma and 12 of 25 cases of chondrosarcoma. However, there were no positive findings in all 15 cases of MFH or 20 cases of GCT. In chondrosarcoma, the frequency of positively stained cases increased according to pathologic grading (i.e., 3 of 14 cases of Grade 1, 7 of 9 cases of Grade 2, and 2 of 2 cases of Grade 3). Although the multinucleated cells in MFH or GCT were not immunostained, BGP antigenicity was observed in the multinucleated cells of osteosarcoma (12 of 15 cases). In the matrix of osteosarcoma, BGP immunoreactivity of the tumorous osteoid was observed in 28 of 32 cases. However, in the matrices of chondrosarcoma, MFH, and GCT, BGP immunoreactivity was not observed. CONCLUSION. These results suggest that the immunohistochemical study of BGP is useful for the differential diagnosis of bone tumors.     

The significance of bone and cartilage formation in malignant fibrous histiocytoma of soft tissue

Malignant fibrous histiocytoma of soft tissues (MFH), a mesenchymal tumor of varied morphologic patterns and cell types, sometimes contains bone and cartilage. Such bony and cartilaginous elements in a pleomorphic MFH may pose a difficult diagnostic challenge. An MFH with bone and cartilage can be distinguished from extraosseous osteogenic sarcoma and chondrosarcoma on the basis of qualitative and quantitative features of the osseous and chondroid elements. Five cases of soft tissue MFH containing bone and cartilage reported here showed: 1) that the bony and cartilaginous elements are mostly metaplastic and tend to be in the pseudo-capsule or fibrous septa of the tumor; and 2) that the bony elements may show a zoning pattern with peripheral maturation similar to myositis ossificans. In a single case, there was a small amount of "tumor osteoid." However, this was focal and lacked the diffuse and finely divided or ribbon-like pattern generally associated with osteogenic sarcoma. The probability that MFH with bone and cartilage is less aggressive than soft tissue osteogenic sarcoma or chondrosarcoma underscores the importance of accurate histologic diagnosis of these tumors.     

Primary intradural myxoid chondrosarcoma: a case report and review in the literature

Summary Objective and importance: Chondrosarcomas are extremely rare cartilaginous tumours that typically usually are associated with bone. Therapeutic experience with primary spinal mesenchymal chondrosarcomas is also extremely limited. The exact origin of rare intradural spinal chondrosarcomas remains obscure. We report the first case in the literature of a primary intradural myxoid chondrosarcoma. Clinical presentation: This 40-year-old man experienced a 3 month history with back pain. The results of his neurological examination were normal. Magnetic resonance imaging (MRI)demonstrated at the T12 level intradural tumour. We could not identify this lesion as chondrosarcoma in preoperative period. Intervention: At surgery, a mass found attached solely to pia mater, with a normal arachnoid and dura mater overlying was seen. The mass was excised completely and microscopic examination identified a myxomatous chondrosarcoma. The postoperative course was unremercable. But a histological examination revealed primary myxoid chondrosarcoma. Experience with primary spinal mesenchymal chondrosarcomas is also extremely limited. We especially discussed to the histological examination. Conclusion: The differential diagnosis considered in the present case included meningioma, plasmacytoma, and non-neoplastic intradural spinal cord lesion. We emphasize the benefit of surgical resection without radiotherapy and/or chemotherapy. This case presents the first case in the literature of an primary spinal intradural myxoid chondrosarcoma.     

Chondrosarcoma with dedifferentiated foci. A comparative and ultrastructural study.

The light and electron microscopic features of a well-differentiated chondrosarcoma with dedifferentiated foci (CDF) are compared with those of a poorly differentiated chondrosarcoma with spindle cell elements. The differentiation of this lesion from mesenchymal chondrosarcoma and from primitive multipotential primary sarcoma of bone is discussed. Ultrastructurally, the cells of the cartilaginous region of the CDF resembled those of the poorly differentiated chondrosarcoma and of the cartilaginous zones of an extraskeletal mesenchymal chondrosarcoma reported by Fu and Kay, and were characterised by an abundance of dilated endoplasmic reticulum and a scalloped and microvillous cell membrane. The stroma was devoid of mature crossbanded collagen fibers. The dedifferentiated portion was composed of mesenchymal-type cells surrounded by a relatively sparse matrix containing scanty mature collagen fibers; these cells resembled those in the cellular regions of the two previously documented mesenchymal chondrosarcomas but differed from the cartilaginous type cells in the cellular regions of the poorly differentiated chondrosarcoma.     

Clinicopathological Features of Extraskeletal Myxoid Chondrosarcoma:An Analysis of 9 Cases

Objective:To investigate the Clinicopathological characteristics of extraskeletal myxoid chondrosarcoma(EMC).Methods:Nine cases of extraskeletal myxoid chondrosarcoma were studied.Extensive immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas.Follow-up information was available for seven patients.Results:There were 7 males and 2 females whose ages ranged from 31 to 69 years(median 52.78 years).Local pain or tenderness and the presence of a palpable mass were the main complaints of the patients.The tumors were located mainly in the lower extremities(66.7%).Most tumors were deep-seated.They usually had a distinct multinodular configuration delineated by fibrous connective tissue.The tumor cells were arranged in delicate intersecting strands,rings,and garlands for the most part.The myxoid matrix was abundant in most cases.Immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas.EMC expressed vimentin(100%,9/9),neuron-specific enolase(77.8%,7/9),S-100 protein(66.7%,6/9),synaptophysin and chromogranin A(22.2%,2/9).None of the tumors expressed EMA and desmin.Ultrastructurally:EMC was characterized by distinct cords of cells immersed in a glycosaminoglycan rich matrix.The cells were rich in mitochondria,had well-developed Golgi apparatus and there were numerous smooth vesicles.In many cells,there were also prominent glycogen deposits and lipid droplets.Some tumor cells had intracisternal microtubules.In one of the 2 extraskeletal myxoid chondrosarcomas there were 140-180 nm diameter membrane-bound dense-core secretory granules in cell bodies.Conclusion:Extraskeletal myxoid chondrosarcoma(EMC)is a rare soft tissue sarcoma characterized by distinctive morphological and cytogenetical features.However,the chondroid nature has been a subject of controversy,and its line of differentiation remains to be determined.A substantial proportion of EMC shows immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation.EMC has high potential of local recurrence and metastasis,and a high disease-associated death rate.