血管肉瘤的临床分析

目的 分析血管肉瘤治疗后的生存情况及影响预后的因素。方法 对中国医学科学院中国协和医科大学肿瘤医院收治批病理证实的４１例血管肉瘤进行分析。其中综合治疗（手术＋放射治疗;手术＋化疗;手术＋放射治疗＋化疗）２３例,非综合治疗（手术：放射治疗）１８例,生存率用Ｋａｐｌａｎ－Ｍｅｉｅｒ法计算,用Ｌｏｇｒａｎｋ法进行生存曲线比较,用Ｃｏｘ模型进行多因素回归分析。结果 出现复发或转移的中位时间为１０个月。局部复发１４例,占３８．９％;远地转移１６例,占４４．４％。转移部位依次为淋巴结８例、肺８例、肝脏５例、骨４例、其它４例。全组生存期５个月至２４．５年（中位３０个月）,１、３、５、１０年生存率分别为６７．５％、４６．１％、３４．５％和１４．４％。综合治疗组５年生存率为４２．１％。非综合治疗组为１０．４％（Ｘ＾２＝７．３７,     

The importance of surgery in scalp angiosarcomas

Background

Scalp angiosarcomas (SA) are rare, representing <1% of soft tissue sarcomas. The optimal management of these tumors is unknown, with management based on small case series. We sought to assess the impact of different therapies on overall survival (OS), the practice patterns nationally, and identify factors associated with OS for non-metastatic scalp angiosarcomas.

angiosarcoma arising in the breast following breast-conserving surgery with radiation for breast carcinoma

We report a case of angiosarcoma arising in the breast following breast-conserving surgery with radiation therapy for breast carcinoma. The patient, a 49-year-old postmenopausal woman, had undergone breast-conserving surgery for invasive ductal carcinoma of the left breast (pT2 pN0 M0 Stage IIA). Adjuvant radiotherapy (50 Gy with a booster dose to the tumor bed of 10 Gy) was then performed for the residual breast tissue and the patient was treated with hormone therapy (tamoxifen, 20 mg daily) for 5 years. She presented with skin erosion with bleeding 10 years after the initial operation. Incisional biopsy revealed angiosarcoma of the breast, and total mastectomy was subsequently performed. The patient was the treated with chemotherapy (weekly paclitaxel, 80 mg/m2 x cycles) and has remained well without evidence of local or distant recurrence.     

Recurrent angiosarcoma effectively treated with superselective continuous intra-arterial injection of recombinant interleukin-2 combined with radiotherapy

We report a case of recurrent angiosarcoma (AS) in a 63-year-old man, effectively treated with a superselective continuous intra-arterial injection of recombinant interleukin-2 (rIL-2) combined with electron beam radiotherapy. The patient, who had undergone surgical resection for AS on his face near the nasal bridge, was admitted to our hospital because of recurrent AS on his bilateral cheeks. We placed 4-French catheters in the right external carotid artery (ECA) and superselectively in the left facial artery. A continuous intra-arterial injection of rIL-2 was administered in combination with radiotherapy using a 6 MeV electron beam. The lesion rapidly decreased in size. The total dose of rIL-2 was 3.72 × 10⁷ IU (International Unit) in the right ECA and 2.96 × 10⁷ IU in the left facial artery, while the total radiation dose was 50 Gy, in 25 fractions, for the tumor on the right cheek and 46 Gy, in 23 fractions, for the erythematous plaque on the left cheek. The patient was discharged with almost no cutaneous lesion on his cheeks. Unfortunately, there was another recurrence of AS and the patient died of respiratory failure due to lung metastases. However, as the lesion could be completely controlled even for a while, we believe this treatment method will contribute to the future management of AS.     

First-line anthracycline-based chemotherapy for angiosarcoma and other soft tissue sarcoma subtypes: Pooled analysis of eleven European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group trials

BackgroundAngiosarcoma is a rare subtype of soft tissue sarcoma (STS). Doxorubicin is the standard first-line chemotherapy for advanced STS. It is not known whether angiosarcoma response to anthracycline-based chemotherapy is different to other STS subtypes.MethodsPooled data were analysed from 11 prospective randomised and non-randomised European Organisation for Research and Treatment of Cancer (EORTC) clinical trials of first-line anthracycline-based chemotherapy for advanced STS. Baseline patient characteristics, chemotherapy response, progression free survival (PFS) and overall survival (OS) of angiosarcoma patients were compared with other STS patients. Analysis was performed to identify factors prognostic for angiosarcoma response to chemotherapy, PFS and OS.ResultsWith a median follow-up of 4.2 years, data from 108 locally advanced and metastatic angiosarcoma patients and 2557 patients with other STS histologies were analysed. 25% of angiosarcoma patients had a complete or partial response to chemotherapy compared to 21% for other STS histotypes. The median PFS was 4.9 months and OS 9.9 months, which were not significantly different from other STS histotypes. In univariate analysis, bone metastases were an adverse prognostic factor for OS (hazard ratio (HR) 1.66, 95% confidence interval (CI) 1.03–2.67; p = 0.036). Tumour grade was as an adverse prognostic factor for PFS (HR 1.72, 95% CI 1.01–2.92; p = 0.044) and OS (HR 2.03; 95% CI 1.16–3.56; p = 0.011). Compared to single agent anthracyclines, doxorubicin + ifosfamide was associated with improved PFS (HR 0.53, 95% CI 0.33–0.86; p = 0.010) and OS (HR 0.53, 95% CI 0.32–0.90; p = 0.018).ConclusionsAngiosarcoma response and survival following first-line anthracycline-based chemotherapy was similar to other STS histotypes. Our analysis provides a useful measure of angiosarcoma response to chemotherapy for comparison with future clinical trials.     

Angiosarcoma of the scalp treated with curative radiotherapy plus recombinant interleukin-2 immunotherapy

PURPOSE: To evaluate the effectiveness of curative radiotherapy (RT) plus recombinant interleukin-2 (rIL-2) immunotherapy regarding the treatment results for angiosarcoma of the scalp. Curative resection of angiosarcoma of the scalp is usually difficult because of the diffuse, clinically undetectable local spread. RT is a rational therapeutic approach, because a wide region of the dermis can be treated, while sparing the underlying normal tissues. Recently, the effectiveness of immunotherapy with rIL-2 has also been reported in the treatment of angiosarcoma of the scalp. METHODS AND MATERIALS: The data of 20 patients with angiosarcoma of the scalp treated with curative RT plus rIL-2 immunotherapy between January 1988 and June 2002 were retrospectively analyzed. The total radiation dose was 70.3 +/- 6.9 Gy. The fractions were 2-3 Gy daily, given 5 d/wk. rIL-2 immunotherapy was performed by transcatheter arterial administration in 10 patients, systemic administration in 11 during the course of RT, and intratumoral injection in 10 during and/or after RT; 12 patients received a combination of two. Five patients underwent limited surgery, and concomitant pacilitaxel chemotherapy was also used in 2 patients. RESULTS: The median survival time for overall, local recurrence-free, and distant metastasis-free survival was 36.2, 11.1, and 17.8 months, respectively. Local recurrence developed in 7 patients (35%), 4 of whom also had evidence of distant metastases. An additional 7 patients (35%) developed distant metastases alone. Recurrence within the radiation field was recognized in 2 patients with systemic rIL-2 administration alone (p < 0.05). Arterial or intratumoral administration combined with systemic administration of rIL-2 resulted in better distant metaststasis-free survival rates (p < 0.05). CONCLUSION: Curative RT plus rIL-2 immunotherapy provided an efficient, effective means of treating angiosarcoma of the scalp. Arterial or intratumoral administration combined with systemic administration of rIL-2 may prolong survival. Additional studies with detailed treatment protocols are recommended.     

Management of Localized Breast Angiosarcoma by North American Radiation and Medical Oncologists

Introduction

Primary breast angiosarcoma is a rare malignancy with no clinical trials to guide management. The current use of surgery, chemotherapy, and radiotherapy among North American oncologists is unknown.

软组织肉瘤术后放射治疗疗效分析

目的 探讨软组织肉瘤术后放射治疗的疗效。方法 37例软组织肉瘤患者,术后及时放射治疗,27例术后局部复发再行扩大切除术及术后放射治疗,对两者的疗效进行对比分析。结果 术后及时行放射治疗者的3年和5年生存率分别为64.9%和43.2%;术后局部复发再行扩大切除术者的3年和5年的生存率分别为55.2%和37.9%。结论 软组织肉瘤术后及时行放射治疗可提高患者3、5年生存率,可减少肿瘤局部复发。     

Soft tissue sarcomas

Sarcomas are a heterogeneous group of rare tumors that arise predominantly from the embryonic mesoderm. They present most commonly as an asymptomatic mass originating in an extremity but can occur anywhere in the body, particularly the trunk, retroperitoneum, or the head and neck. Pretreatment radiologic imaging is critical for defining the local extent of a tumor, staging the disease, guiding biopsies, and aiding in diagnosis. Core-needle biopsy is the preferred biopsy technique for diagnosing soft tissue sarcomas. The American Joint Committee on Cancer (AJCC) staging system for soft tissue sarcomas is based on histologic grade, the tumor size and depth, and the presence of distant or nodal metastases. Despite improvements in local control rates with wide local resections and radiation therapy, metastasis and death remain a significant problem in 50% of patients who present with high-risk soft tissue sarcomas. The most common site of metastasis is the lungs, and metastasis generally occurs within two to three years after the completion of therapy. Progress in the molecular characteristics of these tumors should in the near future translate into molecularly based therapies that can be incorporated into standard treatment strategies.     

Soft tissue ameloblastoma

Two cases of oral soft tissue ameloblastoma are described and the relevant literature reviewed.     

Soft tissue aspiration cytopathology

BACKGROUND: Fine-needle aspiration (FNA) biopsy as a diagnostic modality for the pathologic evaluation of soft tissue neoplasms and non-neoplastic soft tissue mass lesions is uncommon and controversial. This procedure contrasts with more traditional diagnostic methods such as marginal excision, incisional (open) biopsy, or even core biopsy to procure tissue from somatic sites. METHODS: The authors reviewed the results of cytopathologic diagnoses obtained by fine-needle aspiration biopsy over a consecutive 11-month period in patients that presented primarily with a palpable soft tissue mass. A few patients with deep non-palpable soft tissue masses also were evaluated by radiologically guided FNA. Cytopathologic diagnoses were verified by different means including tissue examination either by concurrent cell block or subsequent surgical biopsy, flow cytometry, clinical outcome, or repetition of the FNA procedure. Patients were followed for a minimum of one year to evaluate the mass clinically, to determine whether any further therapy was administered, and to assess disease status. RESULTS: Eighty-two aspirates were performed without complications from 77 patients ranging from 12-88 years of age (mean = 50 yrs.) with men outnumbering women 1.5:1. Soft tissue masses were most common in the extremities (41 cases), followed by the trunk (34 cases), retroperitoneum (5 cases), and head and neck (2 cases). Fine-needle aspirates were diagnosed as malignant in 42 (51%), benign in 32 (39%), nondiagnostic in 6 (7%), and atypical in 2 (2%) cases. Malignant aspirates were comprised of 24 sarcomas (57%), 9 carcinomas (21%), 6 malignant lymphomas (14%), and 3 melanomas (7%). Twenty-two aspirates (52%) had an initial diagnosis of malignancy, whereas 18 (43%) represented metastatic and 2 (5%) recurrent neoplasms. Confirmation of the cytopathologic diagnosis was by concurrent or subsequent tissue examination in 57%, flow cytometry in 5%, clinical outcome in 34%, and repeat aspiration in 4%. One false negative and no false positive diagnoses were issued for a sensitivity and specificity of 100% and 97% respectively in distinguishing benign and malignant lesions by FNA. Of the malignant aspirates, 83% could be subtyped whereas 72% of benign aspirates were correctly subtyped. For primary soft tissue sarcomas, 12 of 19 (63%) were accurately subtyped. In 48% of cases a concurrent cell block was obtained and found diagnostically useful in 54% of them. CONCLUSIONS: Aspiration cytopathology of soft tissue mass lesions using FNA biopsy can be an accurate and minimally invasive method for the initial pathologic diagnosis of primary benign and malignant soft tissue masses, for the pathologic confirmation of metastatic tumors to soft tissue, and for the documentation of locally recurrent soft tissue neoplasms. FNA cytopathology is capable of specifically subtyping a large percentage of primary and metastatic soft tissue tumors if cellular material either in the form of a cell block or flow cytometry is obtained in addition to cell smears.     

Primary angiosarcomas of the breast

BACKGROUND: The purpose of the study was to describe the clinicopathologic characteristics and clinical outcomes of patients with primary breast angiosarcoma. METHODS: The institutional database was searched to identify breast angiosarcoma patients seen between 1965 and 2002. Survival outcomes were estimated by the Kaplan-Meier method. The log-rank test was used to compare groups. Cox proportional hazards models were used for multivariate analysis. RESULTS: In all, 69 patients were identified. Median follow-up was 40 months (range, 0-413 months). Median age was 46. Median tumor size at diagnosis was 5.5 cm. Thirteen (18.8%) patients received prior radiation for invasive breast carcinoma. Most patients underwent total mastectomy with (41%) or without (45%) axillary dissection. Regional metastasis to axillary lymph nodes was rare. There were 38 recurrences and 27 deaths. The 5-year overall (OS) and recurrence-free survival (RFS) rates were 61% (95% confidence interval [CI], 49%-76%) and 44% (95% CI, 33%-58%) with estimated medians of 100 and 37 months, respectively. In Cox proportional hazards models, OS and RFS were significantly associated only with T size and not with patient age, prior radiation, or chemotherapy administration. Of 29 patients treated with chemotherapy at recurrence, there were 4 complete and 10 partial responses (48%) with an anthracycline-ifosfamide or gemcitabine-taxane combination. CONCLUSIONS: Breast angiosarcoma is frequently advanced at diagnosis and has a tendency for local-regional recurrence. A significant number of responses to chemotherapy was observed in the metastatic setting. These data suggest that a multidisciplinary therapeutic approach should be employed in high-risk patients with large primary tumors.     

Malignant Soft Tissue Sarcoma of the Hypopharynx Successfully Treated by Radiotherapy Alone

Sarcoma of the hypopharynx has been reported very rarely inthe literature, only six cases having been found among all headand neck malignancies reported to SEER (Surveillance, Epidemiology,and End Results) during 1973–1987. We report a 14-year-oldboy with a huge malignant soft tissue sarcoma arising from thehypopharynx. Tracheostomy and feeding gastrostomy were performedas emergency life-saving procedures. Surgical resection hadbeen attempted, but abandoned. Because of the rapidity of tumorgrowth, we gave the patient a course of accelerated radiotherapy(170 cGy/fraction, two fractions per day) with a total doseof 7140 cGy within one month. A series of endoscopy and imagingstudies demonstrated complete regression of the tumor, and thepatient is currently alive without evidence of disease 3.5 yearsafter treatment. We conclude that for an unresectable tumorwithout distant metastasis, radiation therapy may be tried.The time, dose, and fractionation of radiotherapy should becarefully designed and individualized.     

A missing tissue compensator.

A method is described for the fabrication of missing tissue compensators used in radiation therapy. This method uses layers of styrofoam plastic and a hot-wire cutting machine to construct a mold in which the compensator is made from tissue equivalent material. When the patient contours are provided, no special equipment or materials are required beyond those normally found in a therapy clinic.     

Radiation-induced sarcoma of the breast: a systematic review

Introduction.

Radiation-induced sarcoma (RIS) is a rare, aggressive malignancy. Breast cancer survivors treated with radiotherapy constitute a large fraction of RIS patients. To evaluate evidenced-based practices for RIS treatment, we performed a systematic review of the published English-language literature.

Methods.

We performed a systematic keyword search of PubMed for original research articles pertaining to RIS of the breast. We classified and evaluated the articles based on hierarchal levels of scientific evidence.

Results.

We identified 124 original articles available for analysis, which included 1,831 patients. No randomized controlled trials involving RIS patients were found. We present the best available evidence for the etiology, comparative biology to primary sarcoma, prognostic factors, and treatment options for RIS of the breast.

Conclusion.

Although the evidence to guide clinical practice is limited to single institutional cohort studies, registry studies, case–control studies, and case reports, we applied the available evidence to address clinically relevant questions related to best practice in patient management. Surgery with widely negative margins remains the primary treatment of RIS. Unfortunately, the role of adjuvant and neoadjuvant chemotherapy remains uncertain. This systematic review highlights the need for additional well-designed studies to inform the management of RIS.     

软组织肉瘤的放射治疗

放疗是软组织肉瘤重要治疗手段之一,术前或术后放疗可降低肿瘤局部复发风险,局部扩大切除结合术前或术后放疗能达到与截肢术同等的长期生存率并且保存肢体功能,近些年来随着放疗技术的进步,软组织肉瘤放疗的正常组织损伤风险正在减少。