Retroperitoneal malignant fibrous histiocytoma

Authors review the case history and follow-up of a rare malignant fibrous histiocytoma patient, based on the relevant literary data. The tumor filled the retroperitoneum on the right side, in front of the right kidney. Intravenous urography and computer tomography revealed a 10 x 15 cm sized mass, suspect of being a kidney tumor. Upon surgery, the tumor was found to be a retroperitoneal malignant fibrous histiocytoma. In connection to the case, a brief review is given of the storiform type of malignant fibrous histiocytoma, regarding its etiological, clinical and pathological aspects, the difficulties in diagnosis, as well as the therapeutic possibilities. Authors regard their case worthy of publication because of the retroperitoneal location and significant size of the tumor, and because of the unproven diagnosis prior to surgery. Even after 4 years the patient is symptom- and complaint-free, and CT has revealed no metastases.     

Angiomatoid fibrous histiocytoma: rare tumor of intermediate malignancy

Angiomatoid fibrous histiocytoma is a rare tumour affecting young adults. Unlike conventional malignant fibrous histiocytoma, its extension is only local thus giving a good prognosis. We report the cases of a 9 year-old girl and a 16 year-old boy presenting respectively, with an axillary tumour 5 cm of diameter and a paravertebral subcutaneous tumour 1.5 cm of diameter. In both cases, the diagnosis was not initially suspected. The treatment consisted in surgical resection.     

Malignant fibrous histiocytoma of the breast

In connection with the mammary tumor of a 41-year-old woman the authors draw attention to the rare occurrence of malignant fibrous histiocytoma in the mamma. The histology of the mammary malignant fibrous histiocytoma, role of immunohistochemical reactions in the diagnosis are described. The literature dealing with the malignant fibrous histiocytoma in the mamma is reviewed. This is the 11th case reported in the literature. The authors discuss the possibilities of the therapy of the malignant fibrous histiocytoma in the mamma.     

前列腺恶性纤维组织细胞瘤1例报告并文献复习

目的探讨前列腺恶性纤维组织细胞瘤（MFH）的临床和病理特征。方法我科收治的1例前列腺MFH患者,经尿道前列腺肿瘤部分切除术,并结合文献进行讨论。结果术后2个月肿瘤复发,6个月死于全身衰竭。结论前列腺MFH为高度恶性、预后差,早期手术切除加长期化疗可提高生存率。     

腹膜后恶性纤维组织细胞瘤的螺旋CT诊断价值

目的探讨腹膜后恶性纤维组织细胞瘤（MFH）的螺旋CT表现及诊断价值。材料和方法回顾性分析20例经手术或活检病理证实的后腹膜恶性纤维组织细胞瘤的临床和CT检查资料。结果 20例中,肿瘤最大平均直径为10.5cm。肿瘤形态不规则伴明显分叶,坏死部分占肿瘤体积的大部分,病灶呈浸润性生长。5例肿瘤内可见钙化,3例肿瘤见出血灶,3例肿瘤有脂肪成分。增强扫描肿瘤强化较明显,所谓轨道样强化4例。重建后肿瘤定位准确18例。结论螺旋CT对腹膜后恶性纤维组织细胞瘤（MFH）有一定的诊断价值。     

带骨干的人工关节置换术治疗骨端骨肿瘤

目的 探讨采用带骨干的人工关节置换术治疗骨端骨肿瘤的手术方法及效果。方法 骨肿瘤患者28例，年龄18～76岁，骨肉瘤ll例，骨巨细胞瘤10例，软骨肉瘤2例，骨转移瘤2例，恶性纤维组织细胞瘤l例，动脉瘤样骨囊肿l例，骨软骨瘤l例。其中股骨上段7例，股骨下段6例，胫骨上段l0例，肱骨上段5例。经正常组织切除肿块，行带骨干的人工关节置换术。对放化疗敏感的予以相应治疗。结果 本组28例经16个月至15年随访，18例死亡，9例无瘤存活，l例带瘤存活。多数患者术后获得了较好关节功能及生活质量。结论 带骨干的人工关节置换术适用于骨端骨肿瘤的治疗，骨干假体的设计有待改进。     

Malignant fibrous histiocytoma of the aortic arch; a case report

A 42 year old female patient with an unusual vascular tumor, a malignant fibrous histiocytoma of the aortic arch is described. The clinical, radiological and pathological features of this case are presented.     

Three patients with hereditary multiple exostoses and malignant degeneration of an osteochondroma located in the pelvis

In three male patients with hereditary multiple exostoses (HME), aged 50, 29 and 31 years, peripheral low-grade chondrosarcoma in the pelvic region led to swelling or pain. In the first patient, curative resection was not feasible because of the size and extension of the tumour. However, rapid tumour growth and unbearable pain necessitated a debulking procedure 16 months later. Histopathologic examination revealed a highly malignant dedifferentiated chondrosarcoma. The patient died two years after initial presentation as a result of local tumour growth. In the second patient, treatment consisted of wide resection of the tumour. Five years after the surgery the patient was free of disease. The third patient was initially treated by intralesional resection, followed by partial hemipelvectomy because of residual tumour. Thirteen months later, a local recurrence occurred that was treated by wide excision. Four years after the partial hemipelvectomy the patient was both pain-free and disease-free. Patients with HME are at increased risk for malignant degeneration of pelvic osteochondroma to chondrosarcoma. Periodic control of patients with pelvic osteochondromas is advised, preferably once every two years.     

Postradiation sarcomas of bone

Fifty-nine patients with osteogenic sarcomas arising in bones following exposure to x rays and 20 patients with postradiation malignant fibrous histiocytomas of bone arising as a direct consequence of irradiation were studied. These represent 5.5% of all osteogenic sarcomas and 4.9% of all malignant fibrous histiocytomas of bones. The sarcomas may affect any skeletal site, but most commonly they arose in bones of the pelvic and shoulder girdles or the distal end of the femur. Grounds for irradiation were either nonosseous conditions or preexistent skeletal lesions. Reasons for incidental osseous irradiation included Hodgkin's disease, carcinoma of cervix, breast or lung; bilateral retinoblastoma and others, and giant cell tumor predominated among the irradiated skeletal lesions. The mean and the median radiation doses were 6,040 cGy (rad) and 5,700 cGy (rad), respectively. The period of latency between irradiation and the appearance of the bone sarcoma ranged from 3.5 to 47 y with a mean of 16.5 and median of 14.5, respectively. The cumulative disease-free survival rate for malignant fibrous histiocytoma patients at 3 y was 58%. The cumulative disease-free survival rate at 5 y for patients with osteogenic sarcoma was 17%, with a median survival estimate of 1 y. Although all patients with malignant fibrous histiocytoma who received their radiation therapy for a preexistent bone lesion survived, only 27% of the patients whose bone was normal at the time of irradiation are alive and well at the 3-y mark.     

Inaccuracies in certification of nonmelanoma skin cancer deaths.

Nonmelanoma skin cancer is the most common cancer site in the United States, yet mortality from this cause is poorly understood. We sought medical records of the 16 reported deaths during 1979 through 1987 from this cause (International Classification of Diseases, 9th version [ICD-9], code 173) among Rhode Island residents to evaluate the accuracy of the reported cause of death. Of the 110 cases for which the cause of death could be classified as correct or incorrect, 59 (54%) were misclassified, 49 (83%) of which were mucous-membrane, squamous-cell carcinomas of the head and neck. For most of these, the written death certificate diagnosis was squamous-cell carcinoma of the head and neck, which was coded 173.4. Other problematic diagnoses were cancer of the head and neck and malignant fibrous histiocytoma. In response to a mailed survey, most health departments replied that squamous-cell carcinoma of the head and neck was coded under rubric 173 and malignant fibrous histiocytoma was coded under rubric 171, but there was no unanimity. The misclassification of other causes of death to ICD-9 rubric 173 is substantial. The vast majority were coded to rubric 173.4 and were due to a small number of diagnoses that are recognizable on examination of the death certificate.     

Tumor induction in mice locally irradiated with carbon ions: a retrospective analysis

Tumor induction in mice legs that were locally irradiated with carbon ions was compared to tumor induction by gamma rays after single and fractionated irradiation. A total of 250 tumors were induced in 1104 mice that received carbon-ion doses of 5 through 65 Gy. A total of 77 tumors were induced in 371 mice that received gamma-ray doses of 45 through 95 Gy. Of 91 carbon-ion induced tumors examined histologically, 97 percent were malignant, and sarcomas such as malignant fibrous histiocytoma (47%) and fibrosarcoma (32%) were most frequently observed. Malignant fibrous histiocytoma was also the most frequently observed tumor (12 out of 20 tumors; 60%) after gamma-ray irradiation, followed by carcinomas (25%) such as adenocarcinoma and squamous cell carcinoma. Neither dose fractionation nor linear energy transfer affected tumor induction for carbon ions and gamma rays. Dose responses were linear for carbon ions and gamma rays, and showed no saturation up to 65 Gy of carbon ions and 95 Gy of gamma rays. The relative biological effectiveness of carbon ions was 2.2 for tumor induction and 1.9 for early skin reaction. We conclude that risk of secondary tumor induction by carbon-ion radiotherapy would not be seriously higher than anticipated.     

原发性下腔静脉恶性肿瘤侵及右心房的外科治疗

目的探讨手术治疗原发性下腔静脉肿瘤侵及右心房的方法及下腔静脉多形性恶性纤维组织细胞瘤的外科治疗效果。方法2007年4月、8月,2例患者术前影像学检查除外远处转移并评估下腔静脉重建的可能和范围,在深低温体外循环下分别经右后外侧切口和胸部正中切口入路,切开下腔静脉近心段,切除了原发性下腔静脉肿瘤及延及右心房内的肿瘤,下腔静脉补片成型。结果患者术后症状缓解并康复出院,随访17～21个月,证实手术效果良好。其中1例术后病理诊断为下腔静脉多形性恶性纤维组织细胞瘤。结论下腔静脉原发肿瘤侵及右心房如未发现其它部位转移,在充分的术前准备及多学科的合作下行体外循环下积极手术治疗．可取得良好的近期效果。     

Chest wall resection in general thoracic surgery

To clarify the incidence, indications, and efficacy of chest wall resection, a comprehensive review is needed. Chest wall resection was performed in 23 of 162 operations for thoracic disease over a nine-year period. Eight surgeries requiring chest wall resection for benign disease (8/79) were classified as fenestration or thoracoplasty for empyema, or resection of a benign neoplasm. Fifteen patients who underwent chest wall resections for malignant disease (15/83) were classified as contiguous extension of neoplasms of neighboring organs, primary tumor, or local recurrence. The most common procedure in the malignant disease group was resection for contiguous spread of primary lung cancer (n = 7). The survival rate was 50% at 4 years. There were no serious postoperative complications. In some malignant diseases, complete local control with such a procedure may even lead to a long-term survival. This is a safe and an effective procedure for a variety of diseases.     

Malignant fibrous histiocytoma: the most common soft-tissue sarcoma

Although soft-tissue sarcomas account for less than 1% of all malignancies, over 55 different histological variants have been described. Consequently clinicians often find the classification both complex and confusing. A new variant, the malignant fibrous histiocytoma (MFH), is now the most common adult soft-tissue sarcoma, therefore a clear understanding of the diagnosis, its implications and the optimum treatment is desirable.     

软组织肉瘤中EGFR、HER-2及CD117蛋白的表达

[目的]探讨EGFR、HER-2及CD117蛋白在软组织肉瘤中的表达。[方法]应用免疫组织化学技术E-vision二步法检测108例软组织肉瘤中EGFR、HER-2及CD117蛋白的表达。[结果]108例软组织肉瘤中EGFR、HER-2和CD117蛋白的阳性表达率分别为18.5%、12.0%和4.6%,EGFR蛋白和HER-2蛋白过表达主要出现在滑膜肉瘤、皮肤隆突性纤维肉瘤和恶性纤维组织细胞瘤中;3例CD117蛋白过表达均为外周原始神经外胚层瘤。[结论]EGFR、HER-2及CD117蛋白在软组织肉瘤中的表达局限,用抑制剂治疗需警惕。     

Hepatocellular adenoma, a tumour particularly seen in mostly young women

A 37-year-old woman was examined because of temporary diarrhoea. On CT scan, there was an abnormality of the liver. MRI showed a tumour that was suspicious for adenoma. Biopsy confirmed the diagnosis of hepatocellular adenoma. The patient then discontinued the use of oral contraceptives. The tumour regressed and was resected after two years. A 22-year-old woman presented with abdominal pain. A tumour was found in the upper right quadrant of the abdomen. In the laboratory, liver function tests were abnormal. Ultrasound and a CT scan of the liver showed an adenoma. After withdrawal of oral contraceptives, abdominal complaints lessened, but no regression of the tumour was detected. Surgical resection was uncomplicated. Hepatocellular adenoma is a rare, benign tumour of the liver, most often seen in young healthy women. Its incidence is rising due to the prolonged use of oral contraceptives. Not rarely, benign liver tumours are incidental findings on echography. If symptomatic, the presentation usually consists of vague abdominal complaints. Spontaneous rupture and malignant degeneration have been reported for adenoma. A reliable diagnosis is mandatory for the decision whether to apply surgery or continue observation. Radiological investigations play a key role in the detection and diagnosis of hepatocellular adenoma. Due to the risk of bleeding and malignant degeneration, elective surgical resection is indicated in symptomatic adenomas, asymptomatic adenomas larger than 5 cm in diameter, and smaller adenomas without regression after discontinuation of oral contraceptives.     

Diffuse intestinal malignant lymphoma

The authors describe a patient with malignant lymphoma presenting with clinically severe malabsorption due to diffuse involvement of the small intestine and with intestinal obstruction. The histologic diagnosis was based on the association of (1) diffuse involvement of the small intestine showing lymphoid tissue expansion and (2) a non-classified form of highly malignant lymphoma. After surgical resection of a stenotic part of the small intestine, abdominal irradiation therapy was done, and the patient improved.     

Anorectal malignant melanoma. Report of three cases

The authors report three cases of primary anorectal malignant melanoma in order to discuss the various diagnostic problems, therapeutic modalities and to remind of the prognostic factors of this rare and unknown affliction. The diagnosis is unfortunately realized in the advanced stage. Mrs B.O, 55 years old, presented rectal hemorrhages and false meeds since a year, the clinical examination showed rectal tumor that bleeds with touch. The mass has been biopsed during the rectoscopy and the diagnosis of the malignant melamoma has been confirmed. Abdominoperitoneal amputation had been realized. Mr F.K, 35 years old, hospitalized because of constipation and rectal hemorrhages that evolve since 7 months with loss of weight and alteration of the general state. The rectal touch emphasizes a budy rectal polypoid tumor about 6 cm that the biopsies confirmed the diagnosis of invasive malignant. A Hartman's operation has been realized. A resection of the tumoral bud has been realized 3 months later, the patient died 4 months after that. Mrs F.K, 50 years old, presented since 50 days relapsing rectorrhages. The rectal touch showed a rectal tumor far about 6 cm from the amal margin, the biopsy during the rectoscopy confirmed the diagnosis of the pigmented and little invasive malignant melanoma. The abdominal exhography showed hepatic metastases and a resection by endo-mal way in order to reduce the tumor has been realized. The inclusion of the primary anorectal malignant melanoma in the diagnosis of the afflictins of the anorectal region would permit an improvement of this affliction prognosis, this is still unfortunate when the diagnosis is late. Its treatment is still surgical, the role of the other therapies still needs to be defined.     

原发性腹膜后肿瘤外科治疗

目的 探讨原发性腹膜后肿瘤的诊断、治疗和总结其手术治疗经验。方法 回顾性分析手术治疗的26例原发性腹膜后肿瘤病人的临床资料。结果 13例良性肿瘤和10例恶性肿瘤均能完整切除，只有3例恶性肿瘤行非完整性切除，其中1例两周内复发于术后28d死亡。结论 原发性腹膜后肿瘤IVP、BUS、CT和MR检查对手术前的准备和受累器官的正确估计非常重要，对复发性肿瘤再次或多次手术可以提高治愈率甚至治愈。     

原发性腹膜后肿瘤46例临床分析

目的探讨原发性腹膜后肿瘤的诊断及治疗.方法回顾性分析1990～2001年术前有影像学资料经手术治疗病理学证实的原发性腹膜后肿瘤46例进行临床分析.结果 46例中恶性31例,恶性肿瘤病程短平均仅3.6个月,瘤体较大平均直径为13cm,完整切除率51.6%,5年生存率41%;良性15例,良性肿瘤病程长平均达16个月,瘤体直径平均7cm,完整切除率91%.结论 BUS及CT对术前评估有较大作用,手术完整切除是治疗的关键.