Primary Fibrosarcoma of the Liver Immunohistochemical and Electron Microscopic Studies

An autopsy case of primary fibrosarcoma occurring in the liver of a 75-year old female is presented. The tumor, arising from the lateral upper portion of the right lobe, measured 14 cm in greatest dimension. Light microscopy of the tumor revealed homogeneous, spindle-shaped cells with abundant collagen fibers showing a classic herringbone pattern. Immunoreactive vimentin was found in the tumor cells. Electron microscopically, the tumor cells were rich in rough endoplasmic reticulum without a basement membrane, and were surrounded by large amounts of collagen fibers. The fibroblastic character of the tumor cells was suggested by light and electron microscopy. This may be the first reported case of primary hepatic fibrosarcoma examined by immunohistochemistry and electron microscopy. Acta Pathol Jpn 39: 814 820, 1989.     

Primary fibrosarcoma and malignant fibrous histiocytoma of bone--a comparative ultrastructural study: evidence of a spectrum of fibroblastic differentiation

As primary bone fibrosarcoma (FS) and malignant fibrous histiocytoma (MFH) have similar clinical, radiographic, or survival manifestations, ultrastructural and immunohistochemical studies were undertaken to determine the differentiation pathways of constituent malignant cells. Twelve cases of primary intraosseous FS and MFH were selected for this ultrastructural comparative study and were analyzed for fibroblastic or modified fibroblastic differentiation. There were 4 FS cases and 8 MFH cases, of which 5 were storiform-pleomorphic, 2 were giant cell, and 1 was myxoid type. All FS consisted of spindle fibroblasts with a prominent rough endoplasmic reticulum and Golgi apparatus, variable amounts of vimentin intermediate filaments, and extracellular collagen fibrils. The MFH were composed of a mixture of spindle and pleomorphic fibroblasts (8/8), histiofibroblasts (4/8), and myofibroblasts (3/8). Variable numbers of undifferentiated cells were found in both tumors. In conclusion, fibroblastic differentiation and collagen production was noted in all cases. The often pleomorphic histiofibroblasts present in some MFH cases most likely represent "modified fibroblasts," similar to myofibroblasts. These findings support the hypothesis that the fibroblast and its variants are the predominant cell types found in these tumors, suggesting that the diagnostic entity MFH should be classified as a pleomorphic fibrosarcoma.     

Pseudosarcoma in Paget's disease

The authors describe the case of a male patient who had had Paget' s disease since 1973 which led to the diffuse swelling of the proximal femur in 1993. The elevated alkaline phosphatase level and the destructive lytic lesions on the X-ray of the femur raised the possibility of malignancy. The bone scan showed increased accumulation in the proximal part of the left femur. On the MR the low T1 and the elevated T2 signal intensity suggested malignity despite that in some regions signals typical of adipose tissue could also be seen, which is usually interpreted as a sign of benignity. In the histological specimens no signs of malignant tumour cells were identified. The patient received bisphosphonate treatment which decreased his pain and swelling and also the alkaline phosphatase level. The patient died because of a rapidly progressing retroperitoneal fibrosarcoma and lung metastasis in 1996. The autopsy did not prove any malignant transformation of Paget's disease of bone in the proximal femur. Received: 3 August 1999; Revised: 16 December 1999; Accepted: 30 March 2000     

Transplant immunity in hamsters treated with ultrasound

Hamsters with subcutaneous fibrosarcomas were treated by excision and by exposure to 5-MHz ultrasound for six minutes at an intensity of 3 W/sq cm. Rates for successful elimination of the tumors were between 80% and 90% and were not significantly different for the two methods of treatment. After 30 days, the animals were challenged with the same tumor cells. Animals successfully treated with ultrasound or excision required at least 10 times as many cells to produce a tumor as controls, indicating that eradication of the tumor by ultrasound induced a level of transplant immunity at least as good as that resulting from surgical excision.     

Fibrosarcoma of the jaws

The records of 14 patients with fibrosarcoma of the jaws — 10 in the mandible and four in the maxilla — from the files of the Netherlands Committee on Bone Tumours were studied. The mean age of the patients was 30 years; more men than women were involved; pain and especially swelling were the symptoms most frequently present. The radiographic appearance often indicated the malignant nature of the lesion. Histologically 42% of the tumours were low-grade malignant. The 5-year survival rate was 71%, which is more favourable than either fibrosarcoma located else-where in the skeleton or osteosarcoma of the jaws. Radical surgery is the therapy of choice. All cases with an unfavourable course acquired lung metastases.     

Multiple inflammatory fibrosarcoma of the abdominal cavity in a child

Inflammatory fibrosarcoma is a rare condition in childhood. In the abdominal location, its behaviour is often aggressive and potentially metastasizing. We report a case of a 3-year-old female with abdominal inflammatory fibrosarcoma who relapsed after 1 month from radical surgery. Chemotherapy was ineffective, and we registered a brief stabilisation of disease only with α-IFN. Our case confirms the potential malignancy of this tumour and its resistance to treatment. It is noteworthy that the therapy with α-IFN improved the quality of life in this child for 4 months. © 1996 Wiley-Liss, Inc.     

DMBA诱导大鼠乳腺癌与纤维肉瘤中cyclin D1及p53蛋白的表达及其意义

目的 探讨cyclinD1和p53蛋白在实验性大鼠同时性乳腺癌与纤维肉瘤中的表达及其意义。方法 通过改良给药的方法复制大鼠同时性乳腺癌与纤维肉瘤动物模型,应用SP免疫组化技术检测cyclinD1和p53蛋白在乳腺癌、纤维肉瘤及瘤旁组织中的表达。结果 在正常乳腺组织中无 D1和p53蛋白表达,在不典型增生乳腺组织中7／14有cyclinD1表达,8／18的乳腺癌与9／14的纤维肉瘤中有cyclin D1的表达,7／18的乳腺癌与5／14的纤维肉瘤中有p53蛋白的过度表达;肉瘤旁组织无cyclinD1和p53蛋白表达。cyclinD1和p53蛋白表达与乳腺癌和纤维肉瘤组织学分级有关,同时二者的表达呈负相关。结论 在大鼠同时性乳腺癌与纤维肉瘤中有cyclinD1和p53蛋白过度表达,cyclinD1蛋白可能参与肿瘤发生过程,而p53蛋白表达与肿瘤的恶性程度相关。     

Infantile fibrosarcoma: Magnetic resonance imaging findings in six cases

Purpose

To retrospectively review magnetic resonance (MR) imaging features in a series of six infantile fibrosarcomas to find out if MR can suggest this unusual diagnosis and to highlight the value of MR during and following treatment.

Materials and methods

The records of six cases of histologically proven infantile fibrosarcoma were retrieved from the files of our cancer center. All imaging data available were consensually reviewed by two radiologists.

Results

There were five females and one male (age range at diagnosis, 0-12 months; mean, 6 months). The most common finding was a well-circumscribed single mass in five patients (83%). All tumors had arisen on limbs; at their proximal or distal extremity or at the root of the limb. The masses were 9 cm large in mean diameter. The initial tumor signal was isointense to muscle on T1-weighted and hyperintense on T2-weighted sequences. All masses were well circumscribed and half of them contained internal fibrous septa. The internal signal was homogeneous in three patients and heterogeneous in the three others. An intense enhancement was seen in all three contrast-enhanced exams available; heterogeneous in two cases and homogeneous in one. Osseous erosion was observed in only one patient who was the only one with distant metastasis. After treatment (chemotherapy and very limited surgery), tumors had totally disappeared, leaving muscle fat infiltration in two patients and subcutaneous fat hypertrophy in one patient.

Conclusion

Although imaging findings are not specific of infantile fibrosarcoma, this diagnosis could be suggested when MR imaging depicts a large well-circumscribed mass arising in a limb at birth or during the neonatal period. This mass is sometimes heterogeneous and septate and exhibits an isointense T1- and hyperintense T2-weighted signals and strong enhancement. MR is also the technique of choice for follow-up during treatment which consists nowadays almost exclusively in chemotherapy.     

肾纤维肉瘤1例报告并文献复习

目的探讨肾纤维肉瘤的临床特征及诊疗方案。方法对我院1例左肾纤维肉瘤患者的临床资料进行整理和总结,并通过检索Pubmed数据库对肾纤维肉瘤的相关文献进行复习和讨论。结果患者在我院行左肾肿瘤根治术,肾组织病理诊断为纤维肉瘤,未行放、化疗,门诊随访5个月仍健存。结论肾纤维肉瘤起病一般以腰部疼痛为主,可伴肉眼血尿,临床症状不特异,术前不易明确诊断,确诊需借助免疫组织化学染色。根治性肾切除是目前唯一有效的治疗措施,放、化疗对患者的生存期影响尚不明确。