原发性软组织恶性纤维组织细胞瘤的MRI影像诊断(附15例报告)

目的:探讨原发性软组织恶性纤维组织细胞瘤(MFH)的MRI影像诊断。方法:回顾性分析15例经手术病理证实的原发于软组织的恶性纤维组织细胞瘤患者的影像学资料,15例患者均行MRI检查,有7例行CT检查,8例行增强扫描。结果:原发于软组织恶性纤维组织细胞瘤15例,肿瘤形态多样,以卵圆形、分叶状多见。病变于T1WI呈低信号或等信号,T2WI呈高信号或等信号为主混杂信号影,7例病变内可见低信号之分隔影。13例病变内见坏死或黏液样改变,2例伴出血。明确有包膜3例。增强扫描均呈显著不均匀强化。结论:MRI对原发性软组织恶性纤维组织细胞瘤的诊断具有重要价值,是首选的影像学检查方法。     

骨原发性恶性纤维组织细胞瘤的临床分析（附15例报告）

骨恶性纤维组织细胞瘤是一种罕见的肿瘤,其特点是兼有组织细胞性和纤维性成分。男性发病率多于女性,无明显好发年龄,以中老年稍多。病损主要位于长骨骨端,约占全部病例的75%。病程较长,疼痛和逐渐长大的肿块是较早出现的临床症状。X线表现为溶骨性病变。诊断时要与骨纤维肉瘤鉴别。单纯手术治疗效果不满意,应辅以化疗或放疗。早期病例可在有效化疗控制下,行广泛病灶切除,保留肢体预后比骨肉瘤、骨纤维肉瘤好。     

肺原发性恶性纤维组织细胞瘤（附5例报告）

自1958—1995年底38年间我院共收治恶性肿瘤96704例,其中肺部恶性肿瘤13383例,在肺部恶性肿瘤中发现肺原发性恶性纤维组织细胞瘤5例,占肺部恶性肿瘤的0.37‰。 1 临床资料 本文5例均为男性,年龄49—68岁,平均59.4岁,其中4例吸烟20—48年,均无肿瘤家族史。临床症状以咳嗽、吐痰(或血丝痰),胸背痛为主,个别有气短、肢体疼痛。既往史包括高血压、冠心病、脑供血不足、慢性支气管炎、肺结核、胸膜炎 支气管扩张、胃溃疡和黄疸型肝炎等10余种疾病。X     

上颌骨原发性恶性纤维组织细胞瘤(附5例报告)

报告5例上颔骨原发性MFH,男2例,女3例,年龄17-39岁,平均29岁。临床主要症状为疼痛和肿块。X线表现为溶骨性破坏。结合文献讨论本瘤的诊断和组织起源。     

乳腺恶性纤维组织细胞瘤免疫组化研究（附8例报告）

应用ＡＢＣ法，对８例乳腺恶性纤维组织细胞瘤进行ＡＡＴ、ＡＡＣＴ、Ｖｉｍ、Ｄｅｓ、Ｋｅｒ、ＣＥＡ和ＥＭＡ免疫组化染色。结果ＡＡＴ、ＡＡＣＴ和Ｖｉｍ阳性率１００％，２例Ｄｅｓ阳性。作者同意恶纤组来源于原始间叶细胞，在不同情况下可向组织细胞、纤维母细胞和肌纤维母细胞等不同方向分化，构成了恶纤组复杂多样的细胞成分。     

恶性纤维组织细胞瘤：附4例报告

恶性纤维组织细胞瘤(Malignant Fibrous Histiocytomas,简称MFH)是一种主要由纤维细胞、组织细胞组成,并排列成席纹状结构的恶性肿瘤。MFH又名纤维组织细胞肉瘤、恶性纤维黄色瘤等。该肿瘤可发生于任何年龄,但以中老年多见。主要发生于肢体,尤以下肢为多见,也发生在其它少见的部位,如肺、鼻腔等。现     

纵隔原发性恶性纤维组织细胞瘤

为了研究原发性纵隔恶性纤维组织细胞瘤的临床病理特点。采用常规石蜡包埋ＨＥ 染色及免疫组织化学方法研究３例ＭＦＨ。３ 例ＭＦＨ 均位于中纵隔,肿瘤界限清楚有包膜或假包膜,术后无局部复发及转移,最长无瘤生存者已达９ ａ。纵隔原发ＭＦＨ的病理特点与软组织ＭＦＨ 相同,但局部复发率及转移率低,提示其预后好于软组织ＭＦＨ。     

原发性肾脏恶性纤维组织细胞瘤(附4例报告)

目的：探讨原发性肾脏恶性纤维组织细胞瘤的临床、病理特点,以及影响预后的相关因素。方法：分析临床所见4例肾恶性纤维组织细胞瘤（MFH）,并总结国内外39例病例文献资料。结果：消瘦、肿块是肾MFH的常见临床症状,免疫组化检查有助于提高肾MFH病理诊断率,肾MFH预后与肿瘤病理类型及转移有关,与肿瘤大小无关。结论：肾MFH是极罕见的高度恶性肾原发肿瘤,盲目扩大手术范围对预后无明显改善。     

骨原发性恶性纤维组织细胞瘤的临床病理研究（附3例报告）

目的：探讨骨原发性恶性纤维组织细胞瘤的临床病理特征，提高对本病的认识。方法：对3例骨原发性恶性纤维组织细胞瘤进行临床病理分析，并作免疫组化加以证实。结果：3例中2例为轮辐状多形性恶性纤维组织细胞瘤，坏死明显；l例为粘液样型恶性纤维组织细胞瘤。3例均发生于胫骨上端。免疫组织化学示：AAT( )、Lysozyme( )、S-100(-)、Desmine(-)、AKP(-)。结论：骨的恶性纤维组织细胞瘤好发于胫骨上端和股骨下端，各年龄组均可发生；肿瘤细胞多表现为多形性、核大深染，轮辐状排列，坏死明显。免疫组化：AAT和Lysozyme阳性，未形成类骨组织和免疫组织化学标记的不同，有利于与骨肉瘤的鉴别。     

恶性纤维组织细胞瘤的治疗--附24例报告

恶性纤维组织细胞瘤 (恶纤组 )临床较少见 ,其中以个案报道较多 ,以往以手术治疗为主 ,近些年对此病采取综合治疗 ,取得了一定疗效。我科于 1980年～ 1998年 9月共收治恶纤组病人 2 4例 ,本文对该病的治疗探讨如下。临床资料　本组恶纤组病人 2 4例 ,其中男性 15例、女性 9例、年龄 15岁～ 76岁、中位年龄 47.6岁。发生部位 :下肢 7例 ,上肢 5例 ,腹膜后 3例 ,乳腺 3例 ,躯干 2例、臀部、腹股沟、腮腺及肺脏各 1例。病程 :1年以内者 10例 ,1～ 5年 8例 ,5～10年 5例 ,10年以上 1例。肿瘤大小 :肿瘤大小为 3～ 2 7ｃｍ ,其中小于 5ｃｍ 13例 …     

Postirradiation malignant fibrous histiocytoma

Three patients who were treated successfully with postoperative external radiation therapy for ovarian carcinoma, endometrial adenocarcinoma and bilateral retinoblastoma respectively developed years later malignant fibrous histiocytoma (MFH) within the irradiated field. MFH is a recently described soft tissue sarcoma known for its dual fibroblastic and histiocytic differentiation resulting in a pleomorphic histologic appearance.     

Malignant fibrous histiocytoma

A malignant fibrous histiocytoma of the shoulder in an elderly woman is reported. This heterogenous tumor is thought to be the most common soft tissue sarcoma of late adult life. Typically, the tumor appears as an enlarging soft tissue mass in the proximal portion of an extremity. On histological examination, the tumor contains a bimodal cell population of fibroblast and histiocytelike cells. The location, size, and depth of the tumor have a significant effect upon survival. The biological behavior of malignant fibrous histiocytoma is usually one of local recurrence followed by metastasis. At the present time complete surgical removal offers the best hope for cure.     

Radiation-induced malignant fibrous histiocytoma of the brachial plexus

Summary Brachial plexopathy is a common and disabling complication in cancer patients most often attributed to metastasis or radiation-induced fibrosis. Occasionally, other rare but potentially treatable causes are found. A 73 year old woman had a left radical mastectomy followed by radiation to the chest wall and axilla 24 years ago. She recently presented with left arm pain, chronic, nonprogressive lymphedema, profound distal arm sensory loss and progressive severe hand weakness. There was moderate atrophy of all intrinsic hand muscles, anesthesia of the hypothenar eminence and 4th and 5th digits, and no adenopathy or palpable mass in the axilla. EMG confirmed a brachial plexopathy. MRI showed loss of tissue planes consistent with radiation fibrosis, but CT showed a discrete mass in the brachial plexus. Open biopsy showed pleomorphic spindle shaped cells with immunoperoxidase stains consistent with malignant fibrous histiocytoma.Radiation-induced malignant fibrous histiocytoma may present with a brachial plexopathy in the absence of a palpable mass and should be considered in the differential diagnosis of brachial plexus lesions in cancer patients. CT scanning through the plexus may be useful when MRI is normal or equivocal.     

Primary intrathoracic malignant fibrous histiocytoma and angiosarcoma

Primary intrathoracic malignant fibrous histiocytoma and angiosarcoma are rare sarcomas constituting less than 0.2% of lung cancers. The typical imaging appearance is a large, well-circumscribed, non-cavitating, non-calcified, peripheral lung mass without hilar or mediastinal lymphadenopathy. Bronchoscopy and percutaneous needle aspiration are of limited value in differential diagnosis, and thoracotomy is warranted for definitive diagnosis.     

Prolonged remission of metastatic malignant fibrous histiocytoma induced by combination chemotherapy

A patient with metastatic malignant fibrous histiocytoma (MFH) achieved a complete remission with polychemotherapy lasting 42+ months. In view of this long-term survival and two similar prior reports of long-term survival after aggressive treatment, the goal of therapy for metastatic MFH must be reconsidered.     

Primary Renal Malignant Fibrous Histiocytoma Four-Case Report and Review of the Literature

OBJECTIVE To evaluate the diagnosis and treatment for primary renal malignant fibrous histiocytoma, a rare tumor arising from the kidney.METHODS The clinical and pathological data from 4 cases of malignant fibrous histiocytoma of the kidney detected in our hospital are described.One case of special interest involved a giant cell subtype, the first to be reported in the oncology literature. The clinicopathologic features and prognostic factors of this tumor were analysed and summarized after reviewing 55 documented cases in the English and Chinese literature.RESULTS A palpable mass (71.2%), emaciation (54.2%) and pain (54,2%) were common manifestations in renal MFH, Of all the cases, 51 were identified as a storiform-pleomorphic subtype by pathologists. Inconsideration of all the prognosis related factors, the residual tumor and high TNM stage predicted a shortened survival duration, but the symptom of a fever served as a better prognostic factor.CONCLUSION Malignant fibrous histiocytoma which arises from the kidney is a rare pathologic type, and possesses a high tendency towards local recurrence and distant metastasis. Despite the poor prognosis, early detection and radical surgery can prolong survival in selected cases.     

39例恶性纤维组织细胞瘤临床分析

目的：探讨恶性纤维组织细胞瘤(MFH)的临床特点及合理治疗。方法：回顾性分析39例病例，其中6例为首程治疗病例，33例为院外局部手术后复发病例。按治疗方式分为两组：一组为单纯手术，共18例，另一组为手术加术后放疗，共2l例。结果：单纯手术组5年生存率为33％(6／18)，手术加术后放疗组为57％(12／21)。结论：MFH属于中高度恶性肿瘤，易出现局部复发和全身转移，术后放疗能降低局部复发率。     

软组织恶性纤维组织细胞瘤17例临床分析

目的　探讨软组织恶性纤维组织细胞瘤的合理治疗方法。方法　分析 17例病人的临床、病理特点 ,并总结其治疗结果。结果　男 13例 ,女 4例 ,平均年龄 5 5 .8岁 ,平均病程 8.7个月。发生于肢体 7例 ,占 41.2 %。 2年、3年和 5年生存率分别为 47.1%、2 9.4%和 17.6 %。发生远处转移 8例 ( 47.1% ) ,区域淋巴结转移 7例 ( 41.2 % )。首次术后复发率 10 0 %。结论　临床诊断困难 ,术后复发率高 ;根治性切除为其关键治疗手段。     

Scalp metastases as the initial presentation of malignant fibrous histiocytoma

A rare case of malignant fibrous histiocytoma of the retroperitoneum with scalp metastases as the initial presentation is described. The scalp lesions were mistaken for atypical schwannomas, however subsequent investigation revealed a large retroperitoneal tumor and multiple hepatic and pulmonary metastases.