Urologic sarcoma in adults. Memorial Sloan-Kettering Cancer Center experience based on a prospective database between 1982 and 1989

Urologic sarcoma is a group of rare tumors with a generally poor prognosis. These sarcomas share prognostic characteristics with soft-tissue sarcomas arising from other sites. A staging system that incorporates tumor grade and size is valuable in identifying patients at high risk of dying of disease. Patients with sarcomas that are high-grade, greater than 5 cm in diameter, or metastatic at the time of presentation (MSKCC stages 3 and 4) have a combined 3-year relapse-free survival rate of 26%. Complete surgical resection, preferably with negative margins, still offers the best chance of cure. For reasons that are not clear, multimodality therapy in rhabdomyosarcoma is less effective in adult than in pediatric disease. Present-day chemotherapeutic regimens are ineffective in controlling disseminated sarcoma. The adoption of a uniform staging system coupled with multi-institutional collaboration may allow progress to be made in this otherwise deadly form of genitourinary cancer.     

Adult genitourinary sarcoma: the 25-year Memorial Sloan-Kettering experience

PURPOSE: Urological sarcomas are rare. We describe a continued single institutional experience during 25 years. MATERIALS AND METHODS: The records from July 1977 to July 2003 were reviewed of all patients at our institution with sarcoma arising in the urinary tract or male genital system who were 16 years or older at diagnosis. RESULTS: The primary tumor site in 131 patients was the bladder in 20, the kidney in 26, paratesticular in 57, the prostate in 21 and other in 7. Median followup was 4.0 years. The most common histological subtypes were leiomyosarcoma in 29% of cases and liposarcoma in 26%. Median tumor size was 7 cm and 78% of lesions were high grade. Of the patients 28 (21%) presented with metastatic disease and their median survival was 1.4 years compared to 10.7 years in patients without metastatic disease (p < 0.0001). Disease specific survival was 56% and 42% at 5 and 10 years, respectively and median survival was 7.6 years. On univariate analysis unfavorable prognostic variables for disease specific survival were metastasis at presentation, high tumor grade, a lack of leiomyosarcoma and liposarcoma histological subtypes, prostate sarcoma and large tumor size, incomplete surgical resection and positive surgical margin. Patient sex, age and surgical margin status were not significant predictors. On multivariate analysis 2 variables remained significant predictors of disease specific survival, including tumor size (HR 1.1, 95% CI 1.02 to 1.17) and absent metastasis at diagnosis (HR 4.9, 95% CI 1.4 to 17.2). CONCLUSIONS: Predictors of disease specific survival include local disease at presentation, complete tumor resection and tumor grade, size, location and histological subtype. With adequate surgical treatment most patients who presented with primary disease and underwent complete surgical resection achieved prolonged disease specific survival.     

ADULT PROSTATE SARCOMA: THE M. D. ANDERSON CANCER CENTER EXPERIENCE

PURPOSE: Sarcoma of prostate origin is rare. Historically, long-term survival rates for adult patients with prostate sarcoma are poor. We analyzed the experience of 1 institution with prostate sarcoma during the last 3 decades. MATERIALS AND METHODS: The records of 21 patients with prostate sarcoma were reviewed to identify symptoms at presentation, diagnostic procedures, presence and development of metastases, staging evaluation, histological subtype, grade and size of the primary tumor, and treatment sequence, including surgery, and preoperative and postoperative therapies. Several clinicopathological variables were assessed for prognostic importance. RESULTS: Most patients presented with urinary obstruction. The diagnosis of prostate sarcoma was usually established with ultrasound guided biopsy or transurethral resection. Histological subtypes were leiomyosarcoma in 12, rhabdomyosarcoma in 4, malignant fibrous histiocytoma in 1 and unclassified sarcoma in 4 patients. At last followup, 8 patients had no evidence of disease after a median of 81.5 months (range 10 to 197). The remaining 13 patients died of sarcoma (median survival 18 months, range 3 to 94). The 1, 3 and 5-year actuarial survival rates for all 21 patients were 81%, 43% and 38%, respectively. Factors predictive of long-term survival were negative surgical margins (p = 0.0005) and absence of metastatic disease at presentation (p = 0.0004). Tumor size and grade, and the histological subtype of prostate sarcoma had no significant influence on actuarial survival. CONCLUSIONS: The long-term disease specific survival rate for adults with prostate sarcoma is poor. Early diagnosis and complete surgical resection offer patients the best chance for cure.     

Nephrectomy during operative management of retroperitoneal sarcoma

Background: Complete resection of a retroperitoneal sarcoma often requires removal of adjacent organs. In this study we evaluated the role of nephrectomy during operation for retroperitoneal sarcoma. Methods: Between July 1982 and July 1995, 75 of the 371 (20%) patients who underwent resection of retroperitoneal sarcoma at MSKCC underwent concommitant nephrectomy. Data concerning the reasons for nephrectomy, degree of sarcomatous renal involvement, and survival were retrospectively analyzed. Results: Fifty-four patients (72%) underwent nephrectomy during the initial resection, and 21 (28%) during a resection of a recurrent or persistent tumor. The most common reason for nephrectomy was total encasement by sarcoma (n=40; 53%), followed by dense adherence of the tumor to the kidney (n=21; 28%), and the direct invasion of the kidney by tumor (n=2; 3%). Pathology demonstrated an absence of kidney invasion in the majority of cases (55 of 75; 73%). Renal capsular invasion was present in 11 of 75 (15%), renal parenchymal invasion in 7 of 75 (9%), and renal vein invasion in 2 of 75 (3%) of cases. There were no significant differences in survival based on degree of sarcoma involvement of the kidney, tumor grade, or whether the resection was for primary or recurrent disease. The 53 patients who underwent a complete gross resection of all tumor had a significantly improved long-term survival compared to the 20 patients who did not (50% versus 20% DFS at 5 years, respectively; p<0.001). Conclusions: Decisions for concomitant nephrectomy during resection of retroperitoneal sarcoma should be based on whether this maneuver will provide a complete resection of all gross tumor, in which case the long-term disease-free survival of 50% is comparable to the reported 5-year survival of all patients with retroperitoneal sarcoma who are completely resected. Presented at the 49th Annual Cancer Symposium of the Society of Surgical Oncology, Atlanta, Georgia, March 21–24, 1996.     

Paratesticular rhabdomyosarcoma

Summary Even though rhabdomyosarcoma is the most common soft tissue sarcoma in children, accounting for 5–10% of all malignant disease in children under 15 years of age, so few cases are seen in a single institution that only the combined efforts of multicentre prospective trials made it possible fro adequate treatment schedules to be devised. Thank to this cooperation, survival rates have increased dramatically in recent decades; risk factors have been identified and treatment can now be adapted accordingly. This is especially true for the paratesticular rhabdomyosarcoma (PTRM), which now has a good prognosis in all stages. The striking similarity of tumor behavior and metastatic pathways to those in germ-cell tumors in young male adults can provide us with more extensive data derived from a much larger group of patients. Recent data are gathered and evaluated in this review. Only in this way will it be possible to eliminate all treatment modalities known to be followed by severe sequelae, thus avoiding exposure of the patients to a therapy that carries more risks than the primary tumor itself.     

Sarcomas paratesticulares en el paciente adulto. Manejo y evolución de la enfermedad

ObjectiveIdentify and analyze the clinic pathologic characteristics, therapeutic, and the evolution of the disease in adult patients with a diagnosis of paratesticular sarcoma.Material and MethodsWe reviewed retrospectively the medical records of patients diagnosed with sarcoma paratesticular location of the department of urology at the National Institute of Neoplastic Diseases from 1952 to 2003. Data was collected affiliation, background, symptoms, diagnostic methods, pathological diagnosis, treatment and status of the disease. We analyzed statistically evaluated and compared with information obtained in the literature.ResultsSixteen paratesticular sarcomas cases were found in patients greater than 16 years old; 9 rhabdomyosarcomas, 3 leiomyosarcomas, 3 liposarcomas, and 1 nonclassifiable sarcoma. There were two thresholds of presentation, one among 16 to 20 years and another one in greater than 60 years. Six had metastatic disease at diagnosis. The average survival was 32.18 months with a range among 1 and 142, only two patients are alive, a case of rhabdomyosarcoma without disease evidence and another one of leiomyosarcoma with inguinal recurrence.ConclusionsParatesticular sarcoma is a rare malignant neoplasia of poor prognostic. with variable history of disease in relation a histology subtype, but in general of bad evolution with fatal ending. The biological behavior of the tumor and history of the disease vary according to histological subtype, but in general the trend is poor with fatal outcome.     

Clinico-pathological study of primary malignant chest wall tumors

Clinicopathological studies of primary malignant chest wall tumor on 16 cases operated between 1962 and 1988 were made. Of 9 osteogenic sarcomas, 8 cases were chondrosarcoma and 1 case was Ewing's sarcoma. Of 7 soft part sarcomas, 2 cases were fibrosarcoma, liposarcoma and neurogenic sarcoma respectively, and one case was hemangiosarcoma. Most of the cases had symptoms, such as chest mass and/or chest pain comprehend symptoms for more than one year. The intrathoracic growth of tumor is common, especially in osteogenic sarcoma. The maximum size of tumor was 8.2 cm in a mean diameter. Preoperative histological diagnosis is difficult to make even though various radiologic diagnosis or pathological technique as biopsy or cytology were assessed. And true rate of preoperative diagnosis is limited only 43.8%. Wide resection combined with the tissue distant more than 3 cm length from tumor is recommended and 6 cases underwent combined resection of diaphragm, pericardium or lung. 3 cases underwent chest wall reconstruction using the Marlex mesh and 10 cases were able to direct closure, in 13 cases with ribs resection. The 5 year survival rate of endurable cases was 62.2%, and that of soft tissue sarcoma (68.6%) is better than that of osteogenic sarcoma (41.7%). The recurrent or metastatic rate in high, 7 cases (43.8%), but reoperation was added for 5 cases of local recurrence or for a case of lung metastasis. 5 year survival rate of cases with recurrence or metastases is relatively good, 46.8%, especially excellent in 4 cases with recurred lesions after more than 2 years of tumor free interval.     

Impact of surgical resection on survival in patients with metastatic soft tissue sarcoma and comparison between synchronous and metachronous metastases

BackgroundThe role of local surgery in patients with metastatic soft tissue sarcoma (STS) remains unknown. The study aims to assess the clinical outcomes and impact of surgical resection on survival in patients with metastatic STS and elucidate the survival differences between synchronous and metachronous metastatic groups.MethodsAmong the 272 patients with STS treated between 2000 and 2018, 84 with synchronous or metachronous metastasis were included. Associations between overall survival and primary tumor resection and metastasectomy were assessed using multivariate Cox regression analyses to adjust for baseline differences between surgically and non-surgically treated patients. Propensity score matching was applied to compare synchronous and metachronous metastasis.ResultsAmong the 84 patients included, 69 (82%) and 41 (49%) underwent primary tumor resection and metastasectomy, respectively. The 2- and 5-year overall survivals of all patients after first detection of metastasis were 51.1% and 24.4%, respectively. Multivariate analysis showed that size <8 cm, grade <3, and number of metastases <4 were associated with longer overall survival. After adjusting for baseline demographic and tumor characteristics, primary tumor resection and metastasectomy still had favorable effects on survival. Tumor subtypes, grade, and number of metastases differed significantly between synchronous and metachronous groups. However, after adjusting for these valuables, both groups exhibited comparable survival.ConclusionsApproximately one fourth of the patients with metastatic STS survived for >5 years. Our results showed that surgical resection of primary tumors or metastatic lesions had favorable impact on survival even after adjusting for patient backgrounds, with comparable survival observed between those with synchronous and metachronous metastases.     

Soft-tissue sarcoma of the thigh: Surgical margin influences local recurrence but not survival in 152 patients

Between 1979 and 1998, 152 patients with a soft-tissue sarcoma arising in the thigh were surgically treated in the Sarcoma Center in Aarhus, Denmark. We studied clinicopathologic factors prognostic for local recurrence and survival. 27 patients (18%) had a low-grade tumor, 26 (17%) an intermediate-grade and 99 (65%) a high-grade tumor. 27 patients (18%) were amputated and 125 (82%) had a local resection. 21 (14%) underwent a marginal resection, 82 (54%) a wide resection and 49 (32%) a compartmental resection. 32 patients were also given radiotherapy, 11 of these had a marginal resection. The 5-year local recurrence-free rate was 91%. Multivariate analysis selected marginal resection and histological high grade as unfavorable prognostic factors for local recurrence. The 5-year survival rate was 68%. High age and histological high grade were unfavorable prognostic factors for survival in a multivariate analysis. Surgical margin influenced local recurrence, but not the overall survival.     

Soft-tissue sarcoma of the thigh: surgical margin influences local recurrence but not survival in 152 patients

Between 1979 and 1998, 152 patients with a soft-tissue sarcoma arising in the thigh were surgically treated in the Sarcoma Center in Aarhus, Denmark. We studied clinicopathologic factors prognostic for local recurrence and survival. 27 patients (18%) had a low-grade tumor, 26 (17%) an intermediate-grade and 99 (65%) a high-grade tumor. 27 patients (18%) were amputated and 125 (82%) had a local resection. 21 (14%) underwent a marginal resection, 82 (54%) a wide resection and 49 (32%) a compartmental resection. 32 patients were also given radiotherapy, 11 of these had a marginal resection. The 5-year local recurrence-free rate was 91%. Multivariate analysis selected marginal resection and histological high grade as unfavorable prognostic factors for local recurrence. The 5-year survival rate was 68%. High age and histological high grade were unfavorable prognostic factors for survival in a multivariate analysis. Surgical margin influenced local recurrence, but not the overall survival.     

Soft-tissue sarcoma of the thigh: Surgical margin influences local recurrence but not survival in 152 patients

Between 1979 and 1998, 152 patients with a soft-tissue sarcoma arising in the thigh were surgically treated in the Sarcoma Center in Aarhus, Denmark. We studied clinicopathologic factors prognostic for local recurrence and survival. 27 patients (18%) had a low-grade tumor, 26 (17%) an intermediate-grade and 99 (65%) a high-grade tumor.

27 patients (18%) were amputated and 125 (82%) had a local resection. 21 (14%) underwent a marginal resection, 82 (54%) a wide resection and 49 (32%) a compartmental resection. 32 patients were also given radiotherapy, 11 of these had a marginal resection.

The 5-year local recurrence-free rate was 91%. Multivariate analysis selected marginal resection and histological high grade as unfavorable prognostic factors for local recurrence. The 5-year survival rate was 68%. High age and histological high grade were unfavorable prognostic factors for survival in a multivariate analysis. Surgical margin influenced local recurrence, but not the overall survival.     

Synovial sarcoma: the importance of size and location for survival

A retrospective analysis was done on 135 consecutive patients with extremity and truncal synovial sarcomas seen at our institutions between 1961 and 1996. Ninety-nine patients (73%) presented with a newly diagnosed localized primary sarcoma, nine patients (7%) presented with a locally recurrent sarcoma previously treated at another institution, and 27 patients (20%) presented with metastatic disease. The median followup for surviving patients was 78 months (range, 20-420 months). The overall survival for patients with localized primary and locally recurrent synovial sarcoma was 51% at 10 years. Patients with localized synovial sarcomas less than 5 cm in longest diameter had a survival at 10 years of 88% compared with a 10-year survival of 38% and 8% for patients with sarcomas 5 to 10 cm and greater than 10 cm in longest diameter, respectively. Distal location (hands and feet) and histologic grade also were statistically significant predictors of patient survival for localized synovial sarcoma on univariate analysis. On multivariate analysis size greater than 10 cm and size 5 to 10 cm were associated with an 18-fold and a 3.1-fold increased risk of death compared with tumors smaller than 5 cm. Grade and location were not found to be independent predictors of survival on multivariate analysis. Primary tumor size and initial status at presentation were prognostic for survival and patients with synovial sarcomas larger than 5 cm or locally recurrent at presentation should be considered for more aggressive surgical and adjunctive radiotherapy or chemotherapy.     

Clinical characteristics and overall survival in genitourinary sarcomas treated with curative intent: a multicenter study

OBJECTIVES: Adult soft tissue sarcomas in general, and those arising from the urological organs in particular, are a group of rare tumours with a generally poor prognosis, only a few studies are available. We report our experience with this type of tumours in a multicenter study carried out in a single region of Central Italy (Tuscany). METHODS: Pre-treatment and follow-up data were obtained from 22 adult patients, all residing in Tuscany, treated consecutively between 1984 and 2002 for primary or locally recurrent genito-urinary sarcomas in 8 urology departments in the area. All cases were classified according to the French Federation of Cancer Center System Grading Scheme for Adult Sarcomas (FFCC) and Broders System. The crude survival probability was estimated by using the Kaplan-Meier method and differences between patient sub-groups were assessed by the log rank test. RESULTS: The study series included 18 males and 4 females. The mean age at diagnosis was 61+/-21.5 years (range: 15.3-89.1). The most common site was paratesticular (n=9, 40.9%), followed by kidney (n=8, 36.4%), prostate (n=3, 13.6%) and penis and bladder (1 case each, 4.6%). 15 cases (68.2%) were classified as FFCC III, and 16 (72.7%) as Broders IV. The most common histological type was leiomyosarcoma (8 cases, 36.7%), followed by liposarcoma (6, 27.3%), rhabdomyosarcoma (3, 13.6%) and other histological types (5, 22.7%). At the last follow-up (mean: 3.66+/-3.25 years; range 0.15-10.0), 11 of the 22 patients (50%) were still alive. The overall survival rate at 1, 3 and 5 years was 85.9%, 62.0% and 48.8%, respectively. There were no significant differences in survival according to sex, age or histological type. When we compared paratesticular vs. kidney and prostate cancer cases, a significant difference in survival emerged (p=0.02). According to size and grade of the tumour we also found a significant difference in survival (p=0.0006 and p=0.01, respectively). CONCLUSIONS: In our representative series, 3 tumor parameters (site, size and grade) appeared to represent the most important prognostic factors in adult genitourinary sarcomas.     

Rhabdomyosarcoma involving the genitourinary organs, retroperitoneum, and pelvis

We report on 17 patients with rhabdomyosarcoma of the genitourinary organs, retroperitoneal, pelvic, or perineal regions. Treatment was refused by three patients with either bladder or prostatic lesions. The remaining patients were treated with combined modality therapy. Five of 6 patients with bladder lesions are free of tumor 24 to 108 months (mean 51) after diagnosis, while one patient died 4 months postoperatively; two patients with paratesticular lesions are free of disease 3 years and 10 months following diagnosis, respectively. Of two patients with perineal lesions, one patient died, probably due to metastases 21 months after diagnosis, while another patient with local tumor recurrence was treated by reexcision with follow-up for 12 months without evidence of tumor recurrence. The worst results were obtained with the four patients with retroperitoneal pelvic lesions; one patient died 3 months after gross resection of tumor, and the other three patients deteriorated in the 4 months after diagnosis. Overall, 7 of 8 operated patients with bladder or paratesticular lesions are free of disease with follow-up for 10 to 108 months. The biological behavior of embryonal rhabdomyosarcoma, with the sex and age distribution and the management are discussed.     

原发肢体软组织肉瘤208例预后的影响因素分析

目的 探讨影响肢体软组织肉瘤预后的因素,特别是外科治疗对其预后的影响.方法 回顾性研究208例手术治疗的肢体软组织肉瘤患者,其中男性128例,女性80例,平均年龄46岁(9～98)岁.分析患者是否初治、肿瘤的大小(＜5 cm、5～10 crn、＞10 cm)、深度(深筋膜深层、浅层)、组织学分型(脂肪肉瘤、恶性纤维组织细胞瘤、滑膜肉瘤、纤维肉瘤、恶性神经鞘瘤、其他肿瘤)、病理分级(FNCLCC系统Ⅰ、Ⅱ、Ⅲ级)、外科边界(囊内切除、边缘切除、广泛切除、根治切除)以及辅助治疗等因素对患者预后的影响.结果 中位随访时间37.5个月(1.3 ～ 128.1个月),总体3年、5年生存率77％和75％;复发率28％和37％;转移率35％和43％.肿瘤大小、病理分级和术前是否有转移可以独立影响生存率(x2=18.813、24.849、21.107,均P＜0.05);是否为初治病例、组织学分型可以独立影响复发率(x2=21.915、12.192,P＜0.05);病理分级可以独立影响转移率(x2=7.714,P＜O.05).手术外科边界可以独立影响局部复发率和转移率(x2=19.610、9.272,P＜0.05).结论 外科边界独立影响局部复发率和远处转移率,从而间接影响生存率.尤其对无转移的初次治疗的软组织肉瘤,手术是首选方案,手术外科边界达到广泛切除或根治性切除将明显改善患者的预后.     

Urinary bladder leiomyosarcoma in adults

Introduction Non-urothelial neoplasms of the bladder account for fewer than 5% of all bladder tumors. Sarcoma constitutes the most usual mesenchymal malignancy of the bladder, with leiomyosarcomas being the most common type of sarcoma in adults. Patients and methods The records of seven patients presenting to two different institutions with bladder leiomyosarcomas between 2003 and 2007 and between 2000 and 2007, respectively, were examined. Cystoscopy, with transurethral resection of the bladder tumor was initially performed in all patients, with leiomyosarcoma being initially diagnosed on the basis of examination of the transurethral specimen. Results There were N = 5 men and N = 2 women with a median age of 64.8 years at presentation. All seven patients underwent a definitive surgical procedure. Complete resection with negative surgical margins was achieved in all seven patients (100%). MSKCC stage included 86% of patients with stage 3 (N = 6) and 14% with stage 2 (N = 1). A low-grade tumor was evident in one patient only, with the remaining 86% exhibiting a high-grade tumor. Discussion Leiomyosarcomas of the bladder have always been considered as a highly aggressive entity and little is known about their origin, clinicopathologic presentation, and the survival factors associated with them. Contemporary studies suggest that these tumors may have a better prognosis than once believed. Leiomyosarcomas require aggressive surgical extirpation and, when surgical resection is possible, radical cystectomy with wide margins is the rule and should be performed. Strict adherence to standard surgical technique has resulted in low rates of positive surgical margins and low rates of local tumor recurrence.     

Incidence and histological findings of unsuspected prostatic adenocarcinoma in radical cystoprostatectomy for transitional cell carcinoma of the bladder

OBJECTIVE: The present study is designed to evaluate the incidence, histological features and significance of prostatic adenocarcinoma in patients undergoing cystoprostatectomy for Transitional Cell Carcinoma (TCC) of the bladder. PATIENTS, MATERIAL AND METHODS: From January 1990 to December 1996, 59 male patients (mean age 66.5 years), with no evidence of prostatic malignancy on preoperative clinical and biochemical assessment, underwent cystoprostatectomy for TCC of the bladder. The bladder was adequately sampled and the entire prostate sectioned at 5-mm intervals and examined histologically, in order to identify unsuspected prostatic cancer (PCa). RESULTS: Sixteen out of 59 patients (27%) were found to have PCa, which was multifocal in 5 cases (31.25%). The mean tumor size was 0.24 cm. The tumors were equally distributed in the anterior and posterior parts of the prostate and in the peripheral and transition zones, with uniform distribution in both prostatic lobes. In 5 patients (31.25%), the single focus of the tumor was in the apex. All were grade I tumors except one, and all were organ-confined with no capsular penetration. The follow-up ranged from 12-74 months (mean 39 months). Within this period, 7 patients died from metastatic bladder cancer. One patient with PCa localized in the prostatic apex had recurrent prostatic disease in the urethro-ileal anastomosis of an orthotopic bladder substitute; he is alive and on androgen deprivation. The remaining patients are relapse-free. CONCLUSIONS: Incidental PCa is quite a common finding in cystoprostatectomy specimens of bladder cancer patients. Its tendency to appear in the apex of the prostate demands careful and complete excision of the organ.     

Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma

OBJECTIVE: To assess outcome and identify predictors of survival of adults with rhabdomyosarcoma. SUMMARY BACKGROUND DATA: The literature on adult rhabdomyosarcoma is limited. Few studies have identified predictors of long-term survival in this patient population. METHODS: Thirty-nine adults with rhabdomyosarcoma were treated between 1973 and 1996 and prospectively followed. Outcomes were assessed with respect to patient and tumor characteristics, local treatment, and response to chemotherapy. RESULTS: Twenty-six patients had localized/locoregional disease and 13 patients had metastatic disease at presentation. Twenty-one patients underwent attempted curative resection, 27 received radiotherapy, and 37 received chemotherapy. Median follow-up for surviving patients was 152 months. The overall 5- and 10-year survival rates were 31% and 27%, respectively. Five-year survival rates for patients with tumors less than 5 cm, 5 to 10 cm, and more than 10 cm were 60%, 14%, and 0%, respectively. Patients with localized/locoregional disease at presentation had a 44% 5-year survival rate; there were no 5-year survivors among patients with metastatic disease. Patients who had a complete response to chemotherapy had a 5-year survival rate of 57%, compared with a rate of only 7% for poor responders. Metastatic disease at presentation and poor response to chemotherapy were independent predictors of death on multivariate analysis. CONCLUSIONS: Age, location, nodal status, and histologic subtype do not appear be associated with survival in adults with rhabdomyosarcoma treated with multimodal therapy. Metastatic disease at presentation and poor response to chemotherapy are strongly associated with poor prognosis. Future systemic therapies should be targeted to patients with localized/locoregional disease and partial responders to conventional chemotherapy.     

Soft tissue carcinoma. Epidemiology, diagnostics and therapy

The treatment of soft tissue sarcoma requires an individually tailored, multimodal therapy due to the high variability in the clinical situation. Resection is the usual treatment for patients with superficial, low grade tumors with a diameter of <5 cm. For intermediate grade, differentiated lesions, resection with negative resection edges combined with radiotherapy attains an almost 80% total survival rate. For patients with high grade sarcoma of >5 cm, local control can be attained by resection and radiotherapy, however every second patient will develop metastases. Patients with a local recurrence should consider a new resection. Radiotherapy is the more effective the lower the remaining postoperative tumor burden.     

Clinicopathologic characteristics and survival for adult renal sarcoma: A population-based study

IntroductionTo analyze the association of clinicopathologic characteristics and treatment modality with survival among adult patients with renal sarcoma.MethodsWe identified 489 adults diagnosed with renal sarcoma from the Surveillance, Epidemiology and End Results registry between 1973 and 2011. Cancer-specific survival was estimated using the Kaplan-Meier method and was compared between groups with log rank and Cox models.ResultsMedian age at diagnosis was 61 years, while median tumor size was 11 cm. Tumor histology was leiomyosarcoma in 175, liposarcoma in 100, other subtypes in 129, and unknown in 85 cases. Tumor stage at diagnosis was nonmetastatic in 322 (67%) and metastatic in 167 (33%) cases. Treatment of nonmetastatic disease was surgical resection in 171 patients, radiation in 24, both in 35, neither in 18, and unknown in 74 cases. Treatment of metastatic disease was surgery in 39 patients, radiation in 27, both in 11, neither in 42, and unknown in 48. For nonmetastatic and metastatic disease, 5-year cancer-specific survival rates were 58% and 16%, respectively. On multivariable analysis, surgery was associated with decreased cancer-specific mortality among both patients with nonmetastatic disease (hazard ratio = 0.34; 95% CI: 0.14–0.85) and those with metastatic disease (hazard ratio = 0.38; 95% CI: 0.18–0.77). Age, race, tumor size, and tumor grade were independently associated with cancer death in nonmetastatic disease, whereas race and tumor histology remained associated with mortality in metastatic disease (all P< 0.05).ConclusionAlthough metastatic renal sarcoma has an ominous prognosis, durable survival may be achieved for localized tumors. Although we recognize the potential for selection bias, our results suggest an association between surgical resection and decreased mortality for both nonmetastatic and metastatic renal sarcoma.