Dedifferentiated chondrosarcoma

We reviewed 74 cases of dedifferentiated central and peripheral chondrosarcoma. Histologically these tumours consist of an underlying cartilaginous component (either benign or malignant) juxtaposed to a highgrade non-cartilaginous component, with a typically abrupt transition between the two tissue types. The noncartilaginous component may constitute a very small or a very large proportion of the tumour, so diagnosis often requires histological evaluation of the entire tumour. The diagnosis is often suspected on the basis of the clinical course and careful evaluation of the radiographie characteristics. Central dedifferentiated chondrosarcoma can be classified radiographically into three types. In type 1 (36 cases in our review) the radiographie features are the same as those of a central chondrosarcoma, with the addition of a region with very aggressive radiographie features. Type 2 lesions (20 cases) resemble the underlying benign enchondroma but also have destructive changes and/or a large soft tissue mass. Type 3 lesions (8 cases) are not distinctive radiographically and present as a very high grade destructive lesion of bone. These cases are diagnosed following biopsy or tumour resection. The prognosis of these tumours is extremely poor, with 13% overall 5-year survival in this series. Improved survival was found in those cases where diagnosis was prompt and surgical treatment with a wide or radical margin was attained. No benefit was found from the use of adjuvant chemotherapy or radiotherapy. Thus, early recognition of the characteristic radiographie features, adequate histological sampling, and wide or radical surgical margins are necessary for satisfactory management of this highly malignant variant of chondrosarcoma.     

Infantile fibrosarcoma: radiological and clinical features

Two cases of infantile fibrosarcoma are described. This rare childhood malignancy of mesodermal origin usually affects the lower limbs, as it did in both of our cases. Previously, the only treatment option available involved some form of radical and often mutilating surgery. More recently, combination chemotherapy has given good results, with the effect that various imaging modalities have become important in assessing both the initial extent of disease and the response to treatment. Computed tomography has the advantage of demonstrating the amount of osseous involvement, but at the expense of a considerable dose of ionizing radiation. On the other hand, magnetic resonance imaging, with its multiplanar capacity, gives superior demonstration of breaching of tissue planes, which has important implications for planning of surgery. However, as in other soft tissue tumours, changes in signal characteristics with treatment have proved less specific than was originally anticipated.     

Sclerosing epithelioid fiberosarcoma: short T2 on MR imaging

A case of sclerosing epithelioid fibrosarcoma and its appearance on MRI is presented. The tumor showed a zonal architecture on MRI with a large central core of very low signal intensity and a peripheral rim of intermediate to high signal intensity on T1- and T2-weighted spin echo pulse sequences. The core showed decreased cellularity with dense collagen deposition on histologic examination, and the peripheral zone increased cellularity with increased nuclear atypia. The presence of a prominent region of very low signal intensity on T1- and T2-weighted images can be seen with neural tumors, giant cell tumor of the tendon sheath, aggressive fibromatosis, and, in rare instances, with soft tissue sarcomas rich in collagen.     

Malignant fibrous histiocytoma: an ultrastructural perspective

Malignant fibrous histiocytoma is a frequent diagnosis, but the relationship of the tumors to histologically similar soft tissue neoplasms is controversial. In this study, 157 examples representing the 4 main subtypes were reviewed by light microscopy and each tumor was studied with the electron microscope. Immunohistochemical stains were performed on 77 tumors. Electron micrographs on 100 fibrosarcomas were reviewed for comparison. Malignant fibrous histiocytomas often closely resemble fibrosarcomas at the ultrastructural level and differences between the two are generally of degree only. Evidence for true histiocytic differentiation was not found. The immunohistochemical results did not contradict the authors' impression from electron microscopy that malignant fibrous histiocytoma forms part of the histologic spectrum of tumors of fibroblasts.     

Fibrosarcoma Mimicking Plasmacytoma or Carcinoma: An Ultrastructural Study of 4 Cases

Because the fibroblast has a remarkable capability of phenotypic modulations, reflected in both morphologic and immunohistochemical (IHC) changes, ultrastructural studiesare mandatory to identify the variants of fibroblasts. Myofibroblasts or histiofibroblasts are such examples, demonstrating chimeric ultrastructural features of fibroblastic cells in common with smooth muscle cells or with histiocytes, respectively. The presence of epithelioid fibroblastic cells sharing morphologic features with epithelial or plasma cells has not been yet characterized. The authors identified 4 cases of fibrosarcomas (FS) characterized by an unusual phenotype and associated with peculiar ultrastructural findings. The electron microscopic (EM) findings were correlated with the histologic appearance and immunoprofile. All tumors were located in the extremities, 3 in soft tissues and 1 in the bone. By light microscopy 2 cases were composed predominantly by round uniform cells with a striking plasmacytoid appearance. One case mimicked carcinoma, composed predominantly by epithelioid cells and scattered giant tumor cells. The fourth case showed a mixture of plasmacytoid-like and epithelioid cells. By IHC, tumor cells were positive for vimentin and in 2 cases also for epithelial membrane antigen. Kappa/lambda light chain and cytokeratins markers were negative. By EM all 4 tumors showed in addition to classic features of fibroblasts, unusual epithelial-type features, such as secretory granules of "neurosecretory-type" (3 cases), rudimentary cell junctions (3 cases), microvilli (2 cases), and lumen-like structures (1 case). One plasmacytoid-type tumor showed finely granular extracellular deposits. The study describe 4 examples of fibrosarcomas with unusual features at the ultrastructural level, which are associated microscopically with a peculiar phenotype, mimicking plasmacytoma or carcinoma. These findings broaden the spectrum of fibroblastic cell variants in neoplasia.     

Glycosaminoglycans and PDGF Signaling in Mesenchymal Cells

Platelet derived growth factor (PDGF) is involved in the autocrine growth stimulation of normal and malignant cells, the stimulation of angiogenesis, and the recruitment and regulation of tumor fibroblasts. PDGF has been shown to physically interact with glycosaminoglycans which are abundant in the extracellular microenvironment. The present review discusses the effects of glycosaminoglycans on the functions mediated by the PDGF on cells of mesenchymal origin. Recent studies have demonstrated that both soluble and surface bound glycosaminoglycan chains can modulate PDGF-BB isoform signaling depending on the cell type. These data demonstrated that the microenvironment rich in GAGs/PGs is able to significantly modify the cellular response to PDGF-BB signaling in a critical way for cell growth and differentiation.     

卵巢核分裂象活跃的富于细胞性纤维瘤的临床病理学观察

目的 探讨卵巢核分裂象活跃的富于细胞性纤维瘤的临床病理学特征及鉴别诊断.方法 收集2008至2012年间11例卵巢核分裂象活跃的富于细胞性纤维瘤患者的临床病理资料,光镜观察并进行免疫组织化学EnVision法染色.结果 11例患者发病年龄21～ 65岁,平均46岁.肿瘤最大径6 ～ 16 cm,平均9.8 cm.镜下梭形瘤细胞丰富,交叉成束或旋涡状排列,形态温和,细胞轻至中度异型性,核分裂象(4～20)/10 HPF,平均8.4/10 HPF,无坏死.8例进行了波形蛋白检测,均阳性表达.5例检测了WT-1,均有弥漫或灶性阳性表达.α-inhibin和calretinin在检测的6例中仅有1例弥漫表达.6例检测了Ki-67的阳性指数5％～30％,其中1例约10％,1例核分裂象20/10 HPF的Ki-67阳性指数为30％.此外,细胞角蛋白、AE1/AE3、CD117、CD34、CD99和结蛋白在5例检测病例中均呈阴性.2例行网状纤维染色,显示网状纤维包绕在每个瘤细胞周围.10例随访4 ～ 38个月无复发或进展,1例术后94个月复发后仍存活.结论 在卵巢富于细胞性纤维瘤中,当核分裂象≥4/10 HPF、细胞温和、缺乏显著异型时,诊断为核分裂象活跃的富于细胞性纤维瘤比纤维肉瘤更为恰当.绝大多数病例临床呈良性经过,少数病例可复发,提示它是一种具有低度恶性潜能的肿瘤.     

先天性婴儿型胆管纤维肉瘤一例

1 临床资料 患儿女,4岁.因全身皮肤及巩膜进行性黄染15 d,腹痛4d于2010年4月22日入院.患儿4月7日前无明显诱因出现全身皮肤及巩膜黄染,呈进行性加重,伴有小便颜色加深呈浓茶色及全身皮肤瘙痒,无畏寒、发热、恶心、呕吐、腹胀、腹痛等不适.于当地医院输液治疗(具体用药不详),患儿症状无明显缓解.4月18日患儿出现腹部隐痛,伴有腹胀、厌油、恶心、呕吐,为求进一步诊断和治疗收入我科.入院体格检查:患儿发育正常,营养中等,全身皮肤及巩膜中度黄染.腹部稍膨隆,腹软,右季肋区深压痛,无反跳痛及肌紧张.右肋缘下约3 cm可触及肝脏,表面光滑,右季肋区可同时扪及肿大胆囊,张力高,余腹未扪及明显包块.     

The fibroblastic nature of dermatofibrosarcoma protuberans

Summary Dermatofibrosarcoma protuberans has been considered to be of fibrohistiocytic or fibroblastic origin. The purpose of this paper is to identify the original cell strain from which this neoplasm derives, using tissue culture and electron microscopic methods. Thirteen cases of DFSP characterised by clinical, topographical, histological and behavioral criteria were explanted. The emigrating cells were bipolar with two opposed processed and showed a radial arrangement in respect to the expiants. After the second week the distal processes tended to curve back towards the cell body forming flame-like structures. This cell morphology and cellular orientation persisted during the whole life of the culture. Electron microscopy was performed in three cases; the newly grown cells maintained an electron microscopic picture similar to that found in the original tumors.This pattern of behaviour is characteristic of fibroblastic tumors and has been found in explants of normal fibroblasts, of fibromatosis and of fibrosarcomas used as controls. On this basis, we believe that DFSP is a fibrosarcoma of the skin of low grade malignancy.     

Infantile fibrosarcoma associated with urticaria pigmentosa

The authors report the case of an infant with urticaria pigmentosa who developed infantile fibrosarcoma. The tumor was successfully resected but the skin lesions have persisted. This is the first report of a fibrosarcoma in association with urticaria pigmentosa. A brief review is presented of the available medical literature on associations of solid tumors with mastocytosis.