侵袭性纤维瘤病恶变为纤维肉瘤1例报告并文献复习

目的 探讨侵袭性纤雏瘤病的临床表现、病理特点、治疗及促使其恶变为纤维肉瘤的相关因素.方法 报告1例上肢侵袭性纤维瘤病恶变为纤维肉瘤的临床资料并复习相关文献.结果 侵袭性纤维瘤病是一种介于良性和恶性之间的纤维增生性肿瘤,具有局部漫润性和破坏性生长的生物特性;病理学表现为成纤维细胞单克隆性增生,由分化良好的成纤维细胞和肌成纤维细胞组成,缺乏恶性细胞学特征,电镜下见多形态细胞被增生的胶原网包绕,免疫组化波形蛋白和肌动蛋白阳性;手术切除为主要治疗措施,术后复发率高迭10%～70%,因此多数患者需多次手术并配合放射治疗和化学治疗;本例患者接受手术切除并经放射治疗1疗程后的13个月时复发,再次手术病理示纤维肉瘤.结论 侵袭性纤维瘤病是一种少见的纤维增生性肿瘤,其治疗以手术切除为主,具有高度复发倾向,术后常需配合放射治疗、化学治疗,向纤维肉瘤转变罕见,手术刺激、放射治疗可能是促使其恶变的主要因素.     

Aggressive fibromatosis of the larynx: case report and brief review

Aggressive fibromatosis is a rare, benign, fibroblastic neoplasm, characterized by local invasion and a relatively high rate of recurrence. Here a case of laryngeal aggressive fibromatosis in a 47-year old man is reported. The patient presented with worsening dyspnoea and hoarseness and was hospitalized for treatment with partial laryngectomy. Final pathological evaluation of the tumour confirmed a diagnosis of aggressive fibromatosis. The patient has remained disease-free without further treatment for 5 years. This study demonstrated that aggressive fibromatosis may occur around the larynx and can be managed by partial laryngectomy alone. It is, therefore, important to include this rare disease entity in the routine differential diagnosis of laryngeal masses.     

Gene expression in aggressive fibromatosis

Aggressive fibromatosis represents a group of tumors with heterogeneous patterns of biologic behavior. In this study, gene expression in 12 samples of aggressive fibromatosis, as well as that in samples of normal skeletal muscle and a variety of normal tissues, was determined at Gene Logic Inc (Gaithersburg, MD), with the use of Affymetrix GeneChip U_133 arrays containing approximately 33,000 genes. Gene-expression analysis was performed with the Gene Logic Gene Express software system. Differences in gene expression were quantified as the fold change in gene expression between the sets of fibromatosis tissue and normal skeletal muscle. A set of genes was then identified that was significantly overexpressed in aggressive fibromatosis compared with expression in normal muscle. This set of genes was then further examined for expression in a variety of normal tissues. We identified genes that were selectively overexpressed in aggressive fibromatosis compared with expression in 448 samples comprising 16 different nonneoplastic tissues. In particular, ADAM12, WISP-1, SOX-11, and fibroblast activation protein-alpha were uniquely overexpressed in aggressive fibromatosis compared with expression in normal tissues. In addition, the technique of Eisen clustering identified 2 distinct subgroups of aggressive fibromatosis with regard to gene expression. We conclude that gene-expression patterns may be useful in the further classification of subtypes of aggressive fibromatosis and that such classification could have clinical significance.     

高频彩色多普勒超声对腹壁纤维瘤病的诊断价值

目的总结腹壁纤维瘤病的声像图特征，探讨超声对腹壁纤维瘤病的诊断价值。方法分析总结了12例经手术及穿刺活检病理证实的腹壁纤维瘤病的二维和彩色多普勒超声特征。结果腹壁纤维瘤病声像图及血流图特征，腹肌内长条形实性低回声包块，无包膜，边界欠清，周边及内部见少许血流信号。结论高频彩色多普勒超声对腹壁纤维瘤病的诊断与鉴别诊断具有重要价值，可为临床诊断及治疗提供可靠的信息。     

彩色多普勒超声在纤维瘤病诊断中的应用研究

目的探讨彩色多普勒超声在纤维瘤病诊断中的应用价值。方法对42例纤维瘤病患者(共44个肿瘤结节)术前的声像图及术前、术中及术后病理结果进行回顾性分析,评价彩色多普勒超声在纤维瘤病诊断中的应用价值。结果21例新发病例中,12例经超声引导下活检、外科切除活检或术中冰冻检查取得病理诊断。21例复发病例均为肿瘤原位复发,其中除1例肿瘤发生恶变外,其余肿瘤病理类型与上次手术相同。超声上显示26个肿瘤(59.1%)边界清晰、形态规则,余肿瘤边界不清、形态不规则;32个肿瘤(72.7%)内血流信号稀疏,12个肿瘤内可见丰富的血流信号。8例肿瘤可见侵及周围骨骼肌或骨质,或包绕血管、神经或肌腱等周围组织。结论超声检查在纤维瘤病的术前评估、术后随访中具有较大的应用价值;超声引导下粗针切割活检可用于术前的病理诊断。     

Aggressive fibromatosis of the right colon mimicking a gastrointestinal stromal tumour: a case report

Aggressive fibromatosis is a rare type of intra-abdominal desmoid tumour that usually involves the small bowel mesentery. It is a locally-invasive lesion, with a high rate of recurrence, but without metastatic potential. Aggressive fibromatosis is seen more often in young female patients. This case report presents the radiological, intraoperative and histopathological findings from a 37-year-old female patient that presented with epigastric pain and a palpable mass in the right hemiabdomen. Histological and immunohistochemical examinations of the resected tumour, including positive staining for beta-catenin, confirmed a postoperative diagnosis of desmoid type fibromatosis. This specific case showed that desmoid type fibromatosis of the colon can mimic gastrointestinal stromal tumours (GIST) based on its clinical presentation, computed tomography and magnetic resonance imaging findings. Differential diagnosis between desmoid type fibromatosis and GIST is clinically very important due to the different treatments and follow-up protocols that are implemented for these lesions.     

Aggressive fibromatosis in the submandibular region of a child

Aggressive fibromatosis is proliferation of well‐differentiated fibroblasts. Submandibular region is rare location for fibromatosis. We report a case of a rapidly growing mass in submandibular region of 5‐year‐old girl, excised surgically and ascertained to be aggressive fibromatosis on histological examination. Recovery was uneventful, and she was disease‐free in 6‐month follow‐up.     

韧带样型纤维瘤病的影像表现及其病理基础

目的 探讨韧带样型纤维瘤（DF）的影像学特征及病理学基础.方法 回顾性分析经手术病理证实的韧带样型纤维瘤14例,6例行CT检查,其中 3 例增强检查;8例行 MRI 检查,其中7例增强.分析CT及MRI表现,并对肿瘤的病理特点进行分析.结果 肿瘤平均直径6.2cm.肿瘤形态为不规则形或分叶状11例（11/16）.肿瘤与周围结构分界不清或部分分界不清的9例（9/16）.7个肿瘤在CT上呈稍低或等密度,3例增强后呈中度或高度强化.9例病灶在T1WI图像上呈等或稍低信号,在T2WI上呈稍高信号,8个病灶呈中度或高度强化.9个病灶内有条索状、片状的长T1、短T2区域,增强后未见强化.光镜下瘤细胞长梭形,无明显异型性,核分裂少见,呈侵袭性生长,浸润周围的横纹肌及脂肪组织,伴胶原纤维增生及程度不等的透明变性、黏液变性.结论 韧带样型纤维瘤影像学变现具有一定的特异性,认识其病理学改变有助于解释该病的影像学表现,有助于提高术前诊断率,磁共振对手术切除范围的判断具有优势.     

Des douleurs plantaires révèlent une maladie de Ledderhose

We report the case of a 21-year-old soldier with plantar pains associated with swellings of the two plantar arches. We suspect a Ledderhose's disease (or plantar fibromatosis), which is confirmed by ultrasound. It is a rare benign fibroblastic proliferation, which can be associated with other fibromatoses (Dupuytren or Lapeyronie's diseases). The origin of this pathology is unknown and the tolerance is variable. The initial treatment is based on orthopedic plantar orthosis. In our case, this treatment was sufficient to treat the pain completely.     

Large soft tissue tumor of the chest occurring after trauma.

The unusual soft tissue tumor we have described may be the largest yet reported. The tumor was considered benign, and its histologic features were most consistent with fibromatosis arising in a region that had been previously traumatized. Although the patient is doing well, it is premature (2 years later) to conclude that the tumor will not recur.     

Desmoid Tumors: A Review of Their Natural History,Imaging, and Treatment

Desmoid tumors are uncommon neoplasms that are also termed desmoid-type fibromatosis and aggressive fibromatosis. Although desmoids lack metastatic potential, they often exhibit aggressive local invasion. Desmoid tumors have a typical but nonspecific appearance on multiple imaging modalities, including computed tomography, magnetic resonance imaging, and ultrasound. Radiology plays an integral role with imaging and intervention in the management of these tumors as their location and relationship to adjacent structures determines the optimal treatment pathway. Currently, the standard of care for the management of desmoid tumors begins with a conservative observational approach, with additional interventions, such as surgery, radiation, systemic therapy, or alternative therapies used depending on the behavior of the individual desmoid tumor. Although not malignant, desmoid tumors can have serious medical and psychological implications for patients.     

Periesophageal mediastinal fibromatosis

We report two cases of aggressive fibromatosis of the mediastinum with esophageal involvement in adults. CT showed the posterior mediastinal mass involving the esophagus. In both cases, surgical resection was not feasible. Histopathologic examination showed a fibrous tissue. The patients died two years later.     

浅表软组织侵袭性纤维瘤病的超声诊断分析

目的:探讨浅表软组织部位侵袭性纤维瘤病的超声声像图特征.方法:回顾性分析经组织病理学证实的26例浅表软组织部位侵袭性纤维瘤病的超声声像图表现.结果:二维超声显示26例侵袭性纤维瘤病灶大小不等,其中13例(13/26,50.0％)最大径≥3 cm;23例(23/26,88.46％)边界不清晰、未见完整包膜;26例(26/...     

Infiltrating fibromatosis of the breast: a potential pitfall

Mammary fibromatosis is a rare condition. It presents a big dilemma for the clinician, because it mimics breast cancer clinically, radiologically and sometimes cytologically. We report a case of fibromatosis of the breast and highlight the diagnostic difficulties and management.     

Abdominal fibromatosis in a 58-year-old male patient

We report on a 58 year-old male patient, in whom abdominal fibromatosis occurred 3 years after laparotomy and irradiation on account of rectal cancer. Abdominal fibromatosis are very rare tumour-like fibrous lesions of the anterior muscular abdominal wall, mainly affecting fertile females. Men, however, are only rarely affected and without age preponderance. A hereditary or mutant generalized defect of growth regulation of fibrous tissue is considered to be the cause of this disease; moreover, trauma, hormonal effects and pregnancies often play a role as cofactors. Interestingly, there is a high correlation between abdominal fibromatoses and osseous lesions of the femur, mandible and thoracic, as well as lumbar spine. In our case thoraco-lumbar Scheuermann's disease was detected.     

腹壁外侵袭性纤维瘤病的手术治疗

目的:探讨腹壁外侵袭性纤维瘤的治疗,重点为手术治疗。方法:回顾分析34例腹壁外侵袭性纤维瘤,经手术治疗并病理确诊。结果:全组34例腹壁外侵袭性纤维瘤,局部姑息性切除复发率63．6％,广泛切除复发率20％,根治切除无复发。结论:腹壁外侵袭性纤维瘤复发率高,广泛或根治切除并保持切缘阴性,是减少术后复发的有效方法。     

腹部韧带样型纤维瘤病临床特点及治疗探讨

目的探讨腹部韧带样型纤维瘤病（desmoid-type fibromatosis,DTF）的临床特点及治疗方法。方法对我院2006年4月～2011年4月收治的26例腹部DTF临床资料进行回顾性分析。结果本组自行发现或体检时偶然发现腹部肿块24例（92.3%）,腹部不适2例（7.7%）。有腹部手术史10例（38.5%）。术前确诊DTF2例（7.7%）,诊断为腹壁肿瘤20例（76.9%）,余4例分别误诊为原发性肝癌、结肠癌、小肠肿瘤、盆腔肿瘤各1例（各占3.8%）。26例均行手术切除并经病理检查确诊,术后均恢复良好,无术后并发症发生,随访未见肿瘤局部复发及转移。结论 DTF多见于经产妇女,好发于既往有手术史者,以腹壁发病率为最高,肿瘤病理表现虽为良性,但临床上具有浸润性生长和易复发的特点,多发生于深部软组织,手术为该病主要治疗方法。     

侵袭性眼眶纤维瘤病及文献复习

目的：阐明眼眶侵袭性纤维瘤病的临床及病理学特征。方法：报导1例侵袭性眼眶纤维瘤病，结合献对该病的临床表现、影像学特点及病理形态进行探讨。结果：1例21岁女性患，眼眶肿瘤侵入颅内损伤嗅神经及视交叉，引起嗅觉及视力下降。肿物质韧，光镜下为胶原纤维结缔组织，可见胶原纤维玻璃样变性及断裂，间质有淋巴细胞为主的炎症细胞浸润。结论：眼眶侵袭性纤维瘤病为一种罕见的良性瘤样病变，但有明显类似恶性肿瘤的生物学行为，早发现、早手术，彻底切除肿物是预防本病术后复发的关键。     

乳腺纤维瘤病的临床病理特征

  目的  探讨乳腺纤维瘤病的临床病理特征及鉴别诊断要点。  方法  回顾性分析5例乳腺纤维瘤病患者的临床资料并进行组织形态学、免疫组织化学研究, 对其临床表现、病理形态特点及鉴别诊断进行总结。  结果  5例均为女性, 发病年龄24~75岁, 平均年龄53岁。3例行乳腺病灶活检术, 2例行乳腺单纯切除术。乳腺纤维瘤病由梭形细胞(纤维母细胞/肌纤维母细胞)构成, 背景是丰富的胶原纤维, 有程度不等的玻璃样变性。细胞数量不等, 没有或仅有轻度的细胞不典型性和多形性, 核分裂象少见。免疫组织化学染色5例vimentin均阳性, SMA均部分阳性, 3例β-catenin阳性, 而cytokeratin、desmin、EMA、S-100和CD68均阴性。  结论  乳腺纤维瘤病是一种罕见乳腺肿瘤, 呈进行性、侵袭性生长。