骨良性纤维组织细胞瘤2例报告并文献复习

目的 探讨骨的良性纤维组织细胞瘤(benign fibrous histiocytoma,BFH)的临床病理特征、诊断及鉴别诊断要点与治疗方案.方法 对2例发生于胫骨的BFH的临床表现、影像学特征、组织学形态进行分析,并复习相关文献.结果 临床表现、CT及MRI图像与组织学特征均支持骨BFH的诊断,骨BFH并无特异性的免疫标记.结论 BFH在骨关节系统是一种鲜见的良性肿瘤,须密切结合临床资料、影像学特点及组织形态学特点,才能做出明确的病理诊断,并要与干骺端纤维缺损/非骨化性纤维瘤、骨巨细胞瘤等相鉴别.     

心原发性恶性纤维组织细胞瘤2例报道并文献复习

目的探讨心原发性恶性纤维组织细胞瘤(MFH)的病理组织学形态、诊断及鉴别诊断。方法复习2例原发于心MFH的相关临床资料，并对其进行组织学、免疫组化染色及电镜观察。结果2例MFH无特异性临床症状与体征。肿瘤细胞常排列呈席纹状，形态多样，主要由梭形的纤维母细胞样细胞和圆形、卵圆形组织细胞样细胞组成，并有不等量的多形性巨细胞及炎细胞。瘤细胞vimentin、CD68、Lysozyme和α-AT( )。电镜下瘤细胞呈梭形，胞质内有丰富的粗面内质网、溶酶体、中间型微丝和少量脂质。结论原发于心的MFH极为罕见，是一种起源于原始间叶细胞的高度恶性肉瘤，诊断依赖于病理组织学、免疫组化染色和电镜检查。     

Malignant fibrous histiocytoma of the spleen: report of a case and literature review

There is a marked paucity of reports on malignant fibrous histiocytoma (MFH) of the spleen in the literature, and there are no previous reports of its color Doppler sonographic (US) and contrast-enhanced US findings. We report on an 82-year-old male with splenic MFH (inflammatory subtype), with an emphasis on color Doppler and contrast-enhanced US findings.     

胰腺纤维组织细胞瘤1例

患者女,12岁,上腹部阵发性疼痛36小时．加重12小时．伴呕吐,呕吐物为黄绿色。入院查体：中腹部脐右侧局部略膨隆,腹肌略紧,可扪及10cm×10cm肿物,质韧,轻压痛,无反跳痛,不活动,与皮肤无粘连。相关化验检查：血淀粉酶30u／L,尿淀粉酶377u／L。血常规示白细胞14．5×10^9／L,粒细胞84．4％。影像学检查：腹部超声示胰腺前方异常回声．考虑胰腺囊肿。     

Malignant fibrous histiocytoma of the spermatic cord: case report and literature review

This case study reports on a rare case of malignant fibrous histiocytoma arising in the right spermatic cord. An 80-year old male presented with a 2-week history of a right inguinal mass, with mild pain in the same region. Ultrasonography and computed tomography showed a mass arising from the right spermatic cord. A right radical orchiectomy was performed with wide dissection of the mass; the right testicle and epididymis were found not to be grossly involved. The patient had an uneventful postoperative course and recovered well. The patient and his family refused further adjuvant radiotherapy. There was no evidence of recurrence or metastasis during 20 months of regular follow-up. The diagnosis, histological classification, treatment and prognosis of this case are presented, together with a review of the literature.     

Malignant fibrous histiocytoma of the bone in a traumatic amputation stump: A case report and review of the literature

BACKGROUNDMalignant fibrous histiocytoma (MFH) is one of the most common soft tissue sarcomas among adults. It is characterized by large size, high grade, and biological aggressiveness. There are many reports of MFH after local stimulation, such as bone fracture, implants, and chronic osteomyelitis. In this paper, we report a patient who developed MFH 6 years after amputation, suggesting that wound healing and mechanical force play a role in the local stimulation of this disease.CASE SUMMARYA 66-year-old man complained of persistent pain in his residual mid-thigh. He had undergone amputation surgery due to a traffic accident 6 years prior. Physical examination showed tenderness but no abnormalities in appearance. X-ray radiographs and magnetic resonance imaging supported the diagnosis of a tumor, and a biopsy confirmed that the lesion was MFH. The patient received neoadjuvant chemotherapy and left hip disarticulation. During the 6-mo follow-up, there were no symptoms of recurrence.CONCLUSIONPostsurgery MFH has been reported before, and many studies have attributed it to the biological effects of implants. Our case report shows that this disease can develop without an implant and thus highlights the importance of local stimulation. The wound-healing process and mechanical force can both promote this tumor, but whether they directly cause MFH needs further investigation.     

股骨良性纤维组织细胞瘤1例

良性纤维组织细胞瘤绝大多数发生于肌肉、纤维组织与脂肪组织，原发于骨内者极为罕见。本文报道经手术病理证实的股骨良性纤维组织细胞瘤１例。病例女，１９岁，以“右臀部阵发性疼痛２０余天，加重伴膝痛７天”为主诉入院。查体：患侧臀部及下肢未见异常。X线所见：右股骨下段内侧可见３．０cm×２．５cm偏心性囊性分叶状骨破坏，破坏区与正常骨分界清楚，可见硬化缘，其内有不规则骨间隔使病变内呈多发小囊状。外侧亦可见相同改变的骨破坏区。周围无骨膜增生及软组织肿块影（图１）。术中所见：股骨下段内侧后面切除长３cm宽１cm骨皮质，…     

Angio-immunoblastic lymphadenopathy: report of ten cases and review of the literature.

Angio-immunoblastic lymphadenopathy (AIL) is a disease of unknown aetiology and pathogenesis. It has features of hyperimmunity, immune deficiency and can behave like a malignant lymphoma. An underlying abnormality of T-cell regulatory function has been proposed. We report ten patients with AIL followed prospectively and review 200 cases from the literature. As well as showing the typical features described in previous retrospective series namely: constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, skin eruptions, hypergammaglobulinaemia and characteristic lymph node histology; four patients had oedema with ascites or pleural effusions associated with hyponatraemia and hypoalbuminaemia. We have also observed low free thyroxine indices in three patients with elevated TSH levels in two, but without clinical features of hypothyroidism. Seven of the patients in this study were treated with prednisone and cyclophosphamide. Three achieved complete remission but only one patient has survived longer than two years. Failure to achieve complete remission has been associated with a 90 per cent mortality within one year of the onset of disease in the reports reviewed. Corticosteroids alone have produced only a 40 per cent rate of remission. The management of poor responders, non-responders and many relapse cases in unsatisfactory. More instensive chemotherapy is very hazardous, increasing the already high risk of severe infections, and is often unsuccessful. Histological features recently reported to represent co-existent immunoblastic lymphoma at diagnosis were recognized in three of our patients, two of which went on to develop definite lymphoma.     

肾原发性孤立性纤维性肿瘤临床病理观察

目的 探讨原发于肾的孤立性纤维性肿瘤的临床病理特点及鉴别诊断要点.方法 复习2例肾孤立性纤维性肿瘤患者的临床资料,并对其进行组织学观察和免疫组化标记.结果 2例肾原发性孤立性纤维性肿瘤患者均为女性,年龄分别为33岁和63岁.临床主要表现为肾区钝痛,无尿频、尿急、尿痛,无血尿.CT检查示肾占位.组织学特征为瘤组织呈束状、波纹状排列,瘤细胞呈梭形,局部富于瘤细胞,间质血管丰富,部分呈血管瘤样或血管外皮瘤样结构,有明显的束状胶原.免疫表型：瘤细胞CD34、CD99和bcl-2（＋）,SMA局灶性（＋）;而HMB45和CD10（-）.结论 孤立性纤维性肿瘤具有低度恶性潜能,发生在肾极少见,以手术治疗为主,预后较好.确诊主要依靠组织病理学,并辅以免疫组化标记.     

原发性恶性纤维组织细胞瘤的影像学诊断(2例报告及文献复习)

目的探讨骨和软组织原发性恶性纤维组织细胞瘤（malignant fibrous histiocytoma,MFH）的影像学特征,提高对本病的认识。方法回顾分析了经手术、病理证实的右胫骨上端和左侧腰大肌恶性纤维组织细胞瘤各1例的X线、CT或MRI的影像学特征和表现。结果右胫骨上端1例的影像学表现为：溶骨性膨胀性骨质破坏,骨皮质破坏肿块累及软组织。术前术后诊断相符。左侧腰大肌1例表现为膨胀性肿块,中央坏死囊变,边缘厚层强化环壁,术前诊断为脓肿或神经源性肿瘤。结论原发性恶性纤维组织细胞瘤的X线、CT和MRI均虽有一定的特征性表现,但MFH因成份较复杂常导致影像学表现不典型,诊断不易,MRI辅以CT诊断优于常规X线。     

CT诊断骨原发性恶性纤维组织细胞瘤2例

骨原发性恶性纤维组织细胞瘤又称骨纤维组织细胞肉瘤，较少见，CT表现报告较少，本院遇到２例，报告如下。病例例１，女，７０岁，以左胫骨上端疼痛、逐渐加重３个月。药物治疗无效。查体：左胫骨上端偏外侧肿胀、局部压痛明显，皮肤色素沉着、无静脉怒张、无皮温升高。X线表现：左颈骨上端外侧４cm×５cm骨质破坏，边界清楚、有较厚硬化圈、无骨膜反应。CT扫描：左颈骨干骨上端不规则骨质破坏区，CT值２３Hu，破坏病灶大小约４cm×５cm，周围有硬化圈，骨皮质破坏，皮质破坏区周围可见软组织肿块，CT值约３６Hu（图１）。术中见瘤体呈…     

肺良性肌上皮瘤临床病理观察

目的报道1例肺的良性肌上皮瘤,并复习文献,对其临床病理特点及鉴别诊断进行讨论。方法对手术切除的肺及其肿瘤进行常规病理检查,HE及免疫组化(S-100、p63、CK、SMA、GFAP)染色,光镜检查。结果患者女性,58岁。左肺上叶舌段良性肌上皮瘤,突入支气管腔内,大小为1·8cm×1·5cm×1·2cm,瘤组织呈不规则片块状,由卵圆形细胞、透明细胞及少量梭形细胞构成,免疫组化显示瘤组织S-100、p63和CK(+)。结论肺良性肌上皮瘤是一种非常罕见的肿瘤,根据组织形态、结合免疫组化及电镜检查,可作出明确诊断及鉴别诊断。     

原发性良性纤维组织细胞瘤1例

患儿,男,7岁,主因外伤后间断性头痛8天入院.头颅CT检查:左顶骨可见类圆形骨质密度减低区,边缘硬化光滑,脑实质未见著变,脑室系统大小正常(图1).印象:左顶骨病变,性质多考虑为良性.     

肾上腺恶性纤维组织细胞瘤1例

患者女，68岁，高血压30余年、头昏、双下肢乏力1周。查体：左颈部可闻及3／6级收缩期吹风样杂音，双肺未闻及干湿罗音，心率72次／分，律齐，心音有力，各瓣膜区未闻及杂音。心脏彩超示左房扩大约41mm，室间隔及左室后壁增厚约12mm，符合高血压心脏病改变。Holter示房性早搏，胸片示心影增大，双肺纹理增强。入院后行改善循环、降压、对症治疗，患者血压控制可，头昏及双下肢乏力缓解。