韧带样瘤的MRI表现

目的探讨韧带样瘤的MRI表现,提高MRI对该病的诊断符水平。方法回顾分析6例经手术病理证实的韧带样瘤的MRI表现。结果 6例病理证实的病例中,浸润性生长3例,膨胀性生长3例,MRI信号混杂信号,T1WI以等信号为主,接近邻近肌肉信号,T2WI以略高信号为主,压脂序列呈高信号,所有病例中可见片状或索条状低信号,增强扫描肿瘤不均匀强化。结论 MRI检查显示韧带样瘤的形态、信号、强化方式等方面有一定的特征性,具有鉴别诊断价值。     

Multicentric extra-abdominal ''myofibromatosis'' : report of a case with ultrastructural findings

A case of multicentric extra-abdominal fibromatosis, a rare entity, arising in a young female is presented. Three separate lesions developed in the same limb over a period of 11 years, one of which recurred on two occasions. Electron microscopy of the most recent tumour showed a predominance of myofibroblasts. The literature regarding extra-abdominal fibromatosis is reviewed and discussed. Redesignation of both abdominal and extra-abdominal fibromatoses as myofibromatoses is suggested.     

Hereditary gingival fibromatosis in a family with the Zimmermann Laband syndrome

Hereditary gingival fibromatosis is frequently an isolated condition of little consequence apart from a cosmetic problem and occasional associations with hypertrichosis and/or epilepsy. There are, however, several uncommon or rare eponymous syndromes described in which gingival fibromatosis can be a feature: these include the Zimmermann-Laband, Murray-Puretic-Drescher, Rutherfurd, Cowden and Cross syndromes. This paper describes two siblings with features of the rare Zimmermann-Laband syndrome and discusses the major aspects of this and other eponymous syndromes that may be associated with hereditary gingival fibromatosis.     

Control of aggressive fibromatosis by treatment with imatinib mesylate. A case report and review of the literature

Objective There has been only one report available that focuses on the treatment with imatinib mesylate of two individual persons with aggressive fibromatosis. The authors concluded that after long-term treatment, for 9 and 11 months, with imatinib mesylate, both patients demonstrated radiographic and clinical responses. The novel therapy should be considered as salvage in patients with aggressive fibromatosis expressed platelet-derived growth factor receptor—alfa, beta (PDGFR-alfa, PDGFR-beta), and/or c-kit, whose tumors are uncontrollable by the standard management. On the other hand, the number of kinases blocked by imatinib mesylate is notching up, for instance the tyrosine kinase, which is associated with macrophage-colony stimulating factor receptor (M-CSFR). Methods The patient was suffering from aggressive fibromatosis after prior therapy including surgery (R2), radiotherapy, and systemic treatment with combination of tamoxifen and sulindac. The tumor specimen was immunostained for PDGFR-beta and c-kit (CD117), and PDGFR-alfa and cytokines platelet-derived growth factor-alfa and beta were not assessed. The tests for both assessed molecules revealed negative results. In spite of this, the patient underwent a unique treatment with imatinib mesylate at the dose of 400 mg orally once daily for 3 years and 2 months. Results After three months of the therapy, radiographic (met criteria of SD but small decrease of the tumor was noted) and clinical responses were recorded for the first time. The same was seen after 6 and 13 months of therapy continuation with imatinib mesylate. Currently, the patient is treated with imatinib mesylate (400 mg orally once daily) without any toxicity effects. The last MRI revealed readily a smaller tumor (35 × 20 mm) after such a therapy lasted more than 3 years. Conclusions Treatment with imatinib mesyalte has been a well-accepted therapy for chronic myelagenous leukemia (CML) and gastrointestinal stromal tumors (GIST). There have been established four kinases (p210^bcr/abl, c-kit, PDGFR-alfa, PDGFR-beta) suggested as the target for imatinib mesylate. Other potential targets will be discovered as it has lately been determined that M-CSFR kinase activity was blocked by imatinib mesylate. The salvage therapy for aggressive fibromatosis with imatinib mesylate seems to be an attractive opportunity for patients with the advanced disease, whose prior therapy failed.     

Solitary intestinal fibromatosis associated with congenital ileal atresia

We report for the first time an association between congenital solitary intestinal fibromatosis and intestinal atresia. The spindle cell proliferation showed a high apoptotic index contrasting with a low proliferation rate, suggesting that the tumor may have undergone focal and spontaneous regression, leading to intestinal atresia.     

Continuous phenobarbital treatment leads to recurrent plantar fibromatosis

Despite contrary recommendations by expert opinion and international guidelines phenobarbital remains the most widely prescribed anticonvulsant worldwide. Although associated connective tissue disorders were described in a timely way after its introduction, the association between plantar fibromatosis--also called Ledderhose syndrome--and phenobarbital seems not to be well known in general. Our case series uniquely demonstrates that continuous phenobarbital treatment leads to recurrent plantar fibromatosis and may result in long-term disability and numerous unnecessary operations. In general, the association between connective tissue disorders and phenobarbital most prominently appears in adult patients of northern European descent. However, our case series and data from the literature suggest that patient groups less susceptible to connective tissue disorders may as well develop Ledderhose syndrome or other associated syndromes as Dupuytren's contractures, frozen shoulder, Peyronie's disease or complex regional pain syndrome in the course of phenobarbital treatment.     

Aggressive fibromatosis of the leg and sacrococcygeal region: a report of two cases

Aggressive fibromatosis is a rare soft tissue tumor that composes of myofibroblasts that arise from musculoaponeurotic structures. It usually affects the abdominal wall but may be also found in other less common sites including the head and neck, submucosa of the oral cavity, spinal, haunch and limbs, especially, the limbs and sacrococcygeal region are rare locations. We described two cases of aggressive fibromatosis. One was 3-year-old girl with aggressive fibromatosis arising from the right leg region. The other was 20-year-old female arising from in the sacrococcygeal region. They were resected with satisfied results. Pathological examination showed that they were composed of fibroblasts, fibrocytes and bundles of collagen fiber. The aggressive fibromatosis, although rare, should be differentiated from some other soft tissue tumors with similar histological features and different localizations of intra-abdominal, abdominal wall and extra-abdominal.     

Mesenteric desmoid fibromatosis with necrosis resembling sarcoma

Intra-abdominal desmoid fibromatosis is a benign but locally aggressive myofibroblastic neoplasm with no capacity to metastasize. Typical examples show an unremarkable homogeneous gross appearance with infiltrative borders and bland histology that pose little difficulty to the pathologist. Characteristically, fibromatoses lack worrisome features such as necrosis, hemorrhage and cavitation, which are usually seen in potentially higher grade tumors particularly gastrointestinal stromal tumor and other soft tissue sarcomas. Differentiating between these lesions is particularly important for prognostic and therapeutic implications. Herein a case of mesenteric desmoid fibromatosis in a 54-year-old woman is described, which demonstrated a pseudosarcomatous appearance both on radiology and gross examination because of the presence of central cavitation and necrosis.