Postradiation sarcomas: 20 cases and a literature review

PURPOSE: To analyze 20 cases of postradiation sarcoma (PRS) to determine dose levels at which this condition developed, the pathology of the initial and postradiation tumors, latency period, and outcome; and to review the literature and propose modified criteria for diagnosis. METHODS AND MATERIALS: Patient records were reviewed. Previous radiation fields and isodose charts were reconstructed to determine the dose received in the tissue in which the PRS subsequently developed. RESULTS: There were 16 female patients and 4 male patients. Mean age at the time of initial radiotherapy was 28 years. Mean latency was 14 years, with no difference in latency between the adult and pediatric group (t = 0.45, p = 0.37), but shorter latency in the retinoblastoma than in the nonretinoblastoma patients (t = 3.18, p = 0.003). The outcome was poor; 2 patients were alive and disease-free at 2 and 5 years. The 18 patients who died as a result of PRS had a median survival of 1 year. CONCLUSION: PRSs are rare. Unnecessary radiation must be avoided. Cases should be reported with full details so that risk factors can be ascertained. PRSs usually arise in moderate to higher dose areas. Diagnostic criteria should allow soft tissue tumors and short latency.     

Metaplastic breast carcinoma with extensive osseous differentiation: a report of two cases and review of the literature

Invasive breast cancer with osseous metaplasia is rare. Here we report two cases of metaplastic breast carcinoma with extensive osseous differentiation. Case 1: The patient was a 60-year-old woman with a right breast tumor, about 4 cm in diameter. Mammogram and ultrasound presented an irregular-shaped mass suspected for malignancy. Core needle biopsy confirmed invasive carcinoma and the patient underwent a modified radical mastectomy. Case 2: The patient was a 48-year-old woman with a left breast tumor, about 3 cm in diameter. Mammogram demonstrated a well-circumscribed mass with extensive dense calcifications. Frozen section biopsy confirmed invasive carcinoma and a modified radical mastectomy was performed. The two patients had no metastatic carcinoma in the axillary lymph nodes and remained free of recurrence and systemic metastases in a 13- and 4-month follow-up period, respectively. Histopathologically, patient 1 had an adenocarcinoma with prominent sarcomatous (osteosarcomatous) differentiation with intervening spindle cells. The sarcomatous areas showed high nuclear atypia, pleomorphism and a high Ki-67 index. In Case 2, the neoplasm consisted of invasive ductal carcinoma of no special type with an osseous metaplasia component and showed a direct transition from the carcinoma to the osseous elements. The distinction between the different types of metaplastic carcinomas, specifically the distinction between benign and malignant metaplastic (osteoid) elements, should be taken into consideration.     

Soft tissue sarcomas in Osaka, Japan (1962-1985): review of 290 cases

Two hundred and ninety patients with soft tissue sarcomas (STS) in Osaka, Japan, were reviewed. The patients' ages ranged from one month to 84 years (mean 51 years) with a male to female ratio of 1.23:1. The tumors were located in the extremities (120 cases), the trunk (76 cases), the abdominal cavity (40 cases), and the head and neck (43 cases). Histologically the tumors were classified as malignant fibrous histiocytoma (101 cases, 34.8%), liposarcoma (28 cases, 9.7%), synovial sarcoma (24 cases, 8.3%), rhabdomyosarcoma (23 cases, 7.9%), neurogenic sarcoma (20 cases, 6.9%), fibrosarcoma (16 cases, 5.5%), leiomyosarcoma (16 cases, 5.5%) and others. From the present study, it appears that there are no significant differences between STS in Japan and Western countries with regard to the distributions of histological type and primary site.     

Clinical factors and treatment parameters affecting prognosis in adult high-grade soft tissue sarcomas: a retrospective review of 267 cases

Data on 267 adults with high-grade soft tissue sarcomas were reviewed. Male sex, large tumor size, Stage IIIC, IV A and sarcomatous skin invasion, as well as marginal excision, amputation, postoperative fever and wound infection, were found to be associated with shorter survival time. Head and neck location, multifocal growth of sarcoma. Stage IIIC, malignant skin infiltration, locally recurrent tumor as well as marginal excision and limb-sparing resection, were found to influence local control unfavorably in single factor analyses. Each of the significant variables were entered into a multivariate proportional hazards model in a stepwise manner. Stage, postoperative fever, the surgical margin and type of surgery, and sarcomatous skin changes significantly affected survival time. Local recurrence was significantly affected by the surgical margin and type of surgery, the status of tumor (primary or recurrent), stage and malignant skin infiltration.     

直肠腺癌伴骨化生1例并文献复习

目的：探讨直肠腺癌骨化生的病理学特征、发生机制及临床意义。方法：报道1例直肠腺癌伴有骨化生的病例,并复习文献。结果：在直肠腺癌中骨化生机制很多,目前没有明确。结论：骨化生只是一个组织学现象,和预后没有相关性。     

直肠腺癌伴骨化生1例并文献复习

目的:探讨直肠腺癌骨化生的病理学特征、发生机制及临床意义。方法:报道1例直肠腺癌伴有骨化生的病例,并复习文献。结果:在直肠腺癌中骨化生机制很多,目前没有明确。结论:骨化生只是一个组织学现象,和预后没有相关性。     

796例软组织肉瘤分析

目的:了解软组织肉瘤各病种发病情况及相对频率,并调查软组织肉瘤发病趋势。方法:对本院1993年1月至2003年12月所有收治的原发软组织肉瘤796例发病情况进行回顾性分析,从而初步得到各病种发病情况和相对频率,并推测其发病趋势。结果:依据病理分型发病率最高者为恶性纤维组织细胞瘤、滑膜肉瘤、脂肪肉瘤、横纹肌肉瘤等,分别占所收治例数的31.5%,16.8%,16.8%和16.2%。软组织肉瘤可见于各个年龄阶段,可发生于全身任何部位,但各亚型均有各自的特点。结论:软组织肉瘤发病率较低。恶性纤维组织细胞瘤在软组织肉瘤中发病率最高。软组织肉瘤的发病率呈逐年上升趋势。     

Mesenchymal breast sarcomas. Apropos of 25 cases

Breast sarcoma are rare, representing 1% of all malignant breast tumours. This is a retrospective study of 25 patients with a breast sarcoma, treated at Institut Gustave Roussy from 1954 to 1981. Thirty six per cent of these arose in a cystosarcoma phyllodes. A variety of histologies were found, the main one being malignant fibrohistiocytoma (44%). Nodal involvement was rare (4%) and, as in other sarcoma, hematogenous spread of metastases was more usual. Local recurrence occurred in 44% of cases and distant metastases (usually pulmonary) in 24%. The 3 year disease-free survival was 60% and the major prognostic factor was the mitotic index. Surgery is the treatment of choice of these tumours, supplemented by local irradiation in those cases where only a tumorectomy has been performed. The role of adjuvant chemotherapy remains undefined.     

Extrapleural pneumonectomy for sarcomas report of two cases

AIMS AND BACKGROUND: Extrapleural pneumonectomy (EPP), which is a very uncommon surgical procedure, is electively indicated only in patients with early stages of malignant pleural mesothelioma, a rare condition. Two adults suffering from sarcomas and treated with EPP are described here. METHODS: A 29-year-old male with four left-sided lung metastases and ipsilateral pleural effusion from a chondrosarcoma of the mandibula and a 64-year old woman with a megamass in the left chest due to a local recurrence of a hemangiopericytoma underwent EPP. RESULTS: Extra-EPP-field multiorgan progression was diagnosed 14 months following surgery in the first patient who died at the 24th postoperative month but remained free of disease at the site of surgery. The second patient had a chest wall relapse at the forty-third month following EPP, which was treated by partial resection of the second and third ribs. She is alive and disease-free at the twelfth postoperative month. DISCUSSION: EPP may be considered for salvage treatment in selected patients with intrathoracic sarcomas not amenable to other effective therapies to achieve mid- to long-term disease control, even in the case of advanced spread.     

骨原发性腺泡状肉瘤X线病理诊断：附三例报告

Three cases of primary osseous alveolar sarcoma are reported. The tumor was of two different types as shown on X-ray films. Osteolytic type: the lesions occurred in the flat and short tubular bones with areas of patchy osteolytic destruction. Mixed type: the lesions, located in the long tubular bones, appeared as irregular compact shadow in the bone marrow without periosteal reaction or soft-tissue swelling besides areas of patchy osteolytic destruction. The pathological diagnosis was made according to the following: (1) tumor cells aggregating to form solid nest-like or alveolar structures; (2) antidiastase PAS positive granules in the tumor cell cytoplasm; (3) specific crystals in the tumor cell cytoplasm.     

原发性心脏肉瘤8例临床病理分析

目的:分析原发性心脏肉瘤的临床病理学特征及其预后与分级之间的关系。方法:回顾性分析8例原发性心脏肉瘤的临床资料、组织学形态及免疫组化结果,并进行随访。所有肉瘤均按照FNCLCC分级系统进行分级。结果:心脏肉瘤8例,年龄30~60岁（平均年龄45岁）,男女比例为1∶3,临床表现无特异性,发生部位:左心房4例,左心室1例,右心房1例,右房、右室及部分右室流出道1例,双侧心房1例。肿瘤直径从17 mm到93 mm。具体组织学类型:未分化多形性肉瘤3例,血管肉瘤、上皮样血管内皮瘤、平滑肌肉瘤、胚胎性横纹肌肉瘤、去分化脂肪肉瘤各1例。所有肿瘤被分级为1级（2例）、2级（1例）、3级（5例）。除2例失访外,其余6例均获得随访结果,随访时间4~84个月(中位时间40个月）,其中2例低级别（1级）肿瘤患者均未见复发及转移,4例中、高级别（2、3级）肿瘤患者在术后4~30个月死亡。结论:心脏肉瘤罕见,其中未分化多形性肉瘤与血管肉瘤多见,患者的总体预后差,与肿瘤的分级有关,由于本组样本量少,无法做统计学分析,需要更大的样本量来判断两者有无统计学相关性。     

Adult paratesticular sarcomas: A report of eight cases

Eight adult paratesticular sarcomas seen at a Regional Cancer Centre over a 7-year period are described. There were three cases of rhabdomyosarcoma (RMS), three cases of leiomyosarcoma (LMS), and two cases of liposarcoma (LS). The RMS occurred in a younger age group (3rd decade) than the LMS and LS (6th and 7th decades). Most of our patients presented with advanced disease after orchiectomy at other hospitals, three with recurrent or residual disease and four with metastasis. The single patient with RMS who received intensive adjuvant therapy is free of disease at 84 months in spite of the advanced stage at presentation. All three patients with LMS had an unfavourable clinical course. Both the patients with LS had well differentiated (WD) tumours and presented with recurrences, one over several years, following initial local excisions. The necessity for early adequate surgical and adjuvant therapy and the need for a uniform treatment policy are discussed. © 1994 Wiley-Liss, Inc.     

53例屈窝软组织肉瘤治疗经验

目的 探讨屈窝软组织肉瘤的治疗及预后。方法 53例屈窝软组织肉瘤，52例手术，其中4例以皮瓣覆盖血管、神经及修复皮肤缺损，放疗30例，化疗33例。结果伤口并发症8例，复发19例，转移21例，死亡22例。放疗、初治患者局部控制率明显优于未放疗、转诊患者，肿瘤大小及组织学级别与预后相关。结论 放疗可以提高屈窝软组织肉瘤局部控制率，肿瘤大小及组织学级别在其预后判断上有价值，使用皮瓣可以提高屈窝软组织肉瘤保肢率，减少并发症。     

Two cases of secondary soft tissue sarcomas after radiotherapy and radiochemotherapy

BACKGROUND: The development of secondary soft tissue sarcomas after chemo-radiotherapy is a rare and little known event, but its frequency is increasing. PATIENTS AND METHODS: We report two cases of secondary soft tissue sarcomas. The first is the case of a 51-year-old woman treated for Hodgkin's disease with chemotherapy and radiotherapy 15 years before she developed a high-grade malignant pleural sarcoma. The patient had no history of asbestos exposure. The second is the case of a 64-year-old woman with a giant cell malignant histiocytoma secondary to colorectal cancer treated with surgery and radiotherapy nine years before. The patients were not eligible for surgery or radiotherapy. Both were treated with chemotherapy (ifosfamide and epirubicin) without any relevant secondary effects; however, the response to therapy was poor. CONCLUSIONS: The causes of secondary malignancies are multifactorial, but radiation therapy and chemotherapy are certainly implicated in the development of post-therapy neoplasms that are difficult to treat.