Long-term survival after gemcitabine and cisplatin in patients with locally advanced transitional cell carcinoma of the bladder: focus on supplementary treatment strategies

OBJECTIVE: The objective was to evaluate response and survival, as well as efficacy of subsequent supplementary treatment and follow-up strategy in patients with locally advanced transitional cell carcinoma of the bladder following combination chemotherapy with gemcitabine and cisplatin (GC). METHODS: A total of 84 patients with locally advanced (T4b, Nx, M0 or Tx, N2-3, M0) received GC. After chemotherapy, the strategy was close surveillance in patients with complete response, and supplementary radical cystectomy or radiotherapy whenever possible in patients with partial response. RESULTS: A total of 25 patients (29.8%) with complete response to chemotherapy were followed by close surveillance. This group achieved a median overall survival of 47.6 mo. Another 25 patients had partial response to chemotherapy. Of these patients, 16 had supplementary treatment, with 10 achieving "no evidence of disease" (NED). Thus, a total of 35 patients achieved NED with a median overall survival of 48.7 mo versus 10.2 mo in patients not achieving NED (hazard ratio=0.10; 95%CI, 0.05-0.20; p<0.0001). The rate of NED was higher in the group of patients who had a cystectomy compared with the group who received radiotherapy as supplementary treatment. CONCLUSIONS: In patients with locally advanced bladder cancer, NED following chemotherapy alone or chemotherapy plus supplementary cystectomy or radiotherapy is essential to achieve long-term survival. Patients with a partial response should be offered radical cystectomy whenever possible, which seems to be superior to radiotherapy. Close surveillance may be an alternative to immediate cystectomy in patients with complete response following chemotherapy.     

Non-seminoma germ cell tumours (malignant teratoma) of the testis. Results of treatment and an analysis of prognostic factors

The results of treatment of 126 patients with non-seminoma germ cell tumours (malignant teratoma) of the testis are presented. Of this group 81% are alive and 70% disease-free. Of 98 patients who had had no prior treatment 83% are alive and 76.5% disease-free. Of 43 early-stage patients 41 (95%) are alive and tumour-free despite a 21% relapse rate after radiotherapy. The disease-free rate in patients with advanced disease treated with chemotherapy, and in some cases radiotherapy and/or surgery, ranged from 100 to 9% and was strongly dependent upon the volume of metastatic tumour and the histology. Patients with malignant teratoma undifferentiated (embryonal carcinoma) showed a significantly higher disease-free survival rate than those with malignant teratoma intermediate (teratocarcinoma). Twenty-six men with advanced disease received radiotherapy after chemotherapy and of these 23 (88%) are alive and 21 (81%) are disease-free. No difference in disease-free survival rate was seen between advanced-stage patients receiving vinblastine and bleomycin and those receiving, in addition, cis-platinum. The overall drug-related mortality was 4.5%. Serum alpha-fetoprotein (AFP) and beta-human chorionic gonadotrophin (beta-HCG) levels were valuable monitors in advanced disease but not prognostically significant if allowance was made for tumour volume. Fifteen patients had surgical resection of residual masses and one died post-operatively.     

Salvage surgery for recurrent nasopharyngeal carcinoma.

Objectives: To present our experience of salvage surgery for recurrent nasopharyngeal carcinoma after primary treatment by radiotherapy. Patients and methods: Eleven of 25 patient treated for nasopharyngeal carcinoma between 1990 and 2003 with radiotherapy had either residual or recurrent disease and underwent salvage surgery. The type C infratemporal fossa approach was used to access residual tumor. The patients' progress was followed by clinical examination and interval magnetic resonance scans. Outcome measures and results: The results were analyzed in terms of morbidity and oncological outcome; patients were recorded as NED (no existing disease), AWD (alive with disease), and DOD (died of disease). A disease-free survival rate of 72% was achieved in the salvage surgery group of patients and an overall disease-free survival rate of 56% applied to the initial cohort of 25 patients, following both the single mode and combined treatment. Conclusion: Salvage surgery is feasible for patients with recurrent nasopharyngeal carcinoma and may be achieved with minimal morbidity using the type C infratemporal fossa approach.     

Adenocarcinoma of the bladder: study of 14 cases and review of the literature

Fourteen patients with adenocarcinoma of the bladder have been seen over a 14-year period; 6 tumours were of urachal origin and the remaining 8 were primary. All tumours were muscle-infiltrating. The 6 patients with urachal tumours were subjected to surgery; 3 underwent partial cystectomy and 3 radical total cystectomy. All patients who underwent partial cystectomy are alive and free of disease at 60 months, 12 months and 12 months respectively. The other 3 patients who underwent radical cystectomy suffered recurrence and 2 succumbed to their disease within 1 year. All patients with primary adenocarcinoma of the bladder underwent radical cystectomy, 5 of them after planned pre-operative radiotherapy. There were 3 post-operative deaths. One patient died of an unrelated cause 30 months after surgery and 2 died of disseminated disease 8 and 16 months after surgery. One patient developed inguinal lymph node metastases 4 months after surgery and is controlled at 12 months with radiotherapy. Only 1 of the 8 patients with primary adenocarcinoma of the bladder is alive and free of disease at 6 months.     

The role of surgical resection when combined with chemotherapy and radiation in the management of pelvic rhabdomyosarcoma.

With the development of combined chemotherapy and radiation therapy for embryonal rhabdomyosarcoma, the role and extent of surgical resection of these pelvic tumors need to be defined. Thirty-six children with pelvic genitourinary rhabdomyosarcoma seen at St. Jude Children's were managed on protocols combining surgical resection and radiation, and chemotherapy. Ten children presented with cervical-vaginal tumors, which were managed with combined therapy; the surgical resection was histovaginectomy in eight and pelvic exenteration in one. Eight of the ten are free of disease from 1 to 14 years. Twelve children presented with bladder and prostate tumors, which were resected with segmental cystectomy in four cases, biopsy in five, and pelvic exenteration in three. All received combination therapy and six of the twelve are surviving free of disease from 6 months to 16 years. Fourteen children presented with paratesticular rhabdomyosarcoma. Chemotherapy was combined with radical orchiectomy in all cases. Retroperitoneal node dissection was done in nine and five had inguinal node dissection. Nine of the 14 are surviving NED from 2 to 16 years. One patient died, free of disease, with complications of combination therapy. The results of this review supports the approach of combining chemotherapy, radiation, and complete surgical resection.     

Postchemotherapy retroperitoneal lymph node dissection is effective therapy in selected patients with elevated tumor markers after primary chemotherapy alone

Objectives

Elevated tumor markers after primary chemotherapy for metastatic testis cancer are usually an indication of persistent cancer. Subsequent treatment has usually been salvage chemotherapy. This article examines the possibility that selected patients can achieve long-term disease-free survival with surgery alone.

Methods

Using a computerized data base of 627 postinduction chemotherapy retroperitoneal lymph node dissections (PC-RPLND), 23 patients with elevated tumor markers who have undergone PC-RPLND after induction chemotherapy alone were identified. Of the 23 patients, 15 were considered candidates for salvage chemotherapy, but instead underwent salvage surgery. Case histories were reviewed to establish selection criteria for PC-RPLND.

Results

Eight patients originally presented as clinical Stage C, 6 as clinical Stage B-3, and 1 as clinical Stage B-2. All patients initially received cisplatin combination chemotherapy. Twelve patients had an elevated alpha-fetoprotein level and 3 patients had an elevated beta human chorionic gonadotropin level prior to PC-RPLND. Seven patients had rising markers at the time of PC-RPLND. Seven patients had teratoma only in their resected specimen and all have no evidence of disease [NED) at a median of 35 months. Two patients had necrosis only in their RPLND specimen and both are NED at 10 and 42 months. Six patients had cancer in their resected specimen and 2 are NED, 1 is alive with disease, and 3 are dead of disease. Five of the 6 patients with cancer in their resected specimen were the only patients who received postoperative chemotherapy.

Conclusions

Some patients with modest elevations of tumor markers after induction chemotherapy may only have teratoma or necrosis in the postchemotherapy resected specimen. These patients (n = 9) remain continuously NED. Patients who undergo salvage surgery and have cancer in the resected specimen do less well, but selected patients can be cured with this modality and thus avoid the morbidity of salvage chemotherapy.     

Thirty years of experience with pediatric primary malignant liver tumors

Since 1950, 48 infants and children from 10 weeks to 16 years of age presented with primary hepatic malignancy. Signs and symptoms ranged from asymptomatic to those of malignant disease. All patients had a palpable abdominal mass. Jaundice was seen in five patients, four of whom had preexisting cirrhosis. Three male children had evidence of precocious puberty. Whereas liver function tests were usually normal, alpha-fetoprotein levels, when elevated, proved useful diagnostically and as a tumor marker in follow-up. Hepatic angiography and computed tomography (CT) scans have provided the most valuable preoperative assessment of hepatic architecture. Sixteen infants and children underwent resection for cure. Eleven of these patients are alive and disease free 6 months to 23 years later. Six additional patients had incomplete resection with subsequent radiotherapy and/or chemotherapy; only one such patient is disease-free past 3 years. Twenty-six tumors could only be biopsied; most of these patients died within 12 months regardless of what treatment they received. The histology was hepatoblastoma in 39 patients, hepatocellular carcinoma in 4, fibrolamellar carcinoma in 4, and malignant mesenchymal tumor (mesenchymoma) in 1. The patients with hepatocellular carcinoma and mesenchymoma all died. Three of four patients with fibrolamellar carcinoma are alive and disease-free following resection up to 3 years; this histology seems favorable. The other survivors had hepatoblastoma. The role of adjunctive chemotherapy and/or radiotherapy has not yet been determined.     

Local Recurrence After Mastectomy for Ductal Carcinoma In Situ

Abstract: Local recurrence after mastectomy for invasive cancer generally carries a poor prognosis. Local recurrence after mastectomy for ductal carcinoma in situ (DCIS) is rare and its impact on survival is unknown. Sixty-eight patients were treated with mastectomy for DCIS at the Fox Chase Cancer Center between 1985 and 1996, and only one of these developed a chest wall recurrence. An additional five patients treated with mastectomy at other institutions and referred to Fox Chase after local recurrence were also identified. These six patients had only DCIS as their primary pathology. Four of the six patients were premenopausal (median age 42 years). The median interval to local recurrence was 5.0 years (2.8–9.3 years). The median follow-up from initial diagnosis was 10.5 years (4.3–26.7 years) and 5.2 years (1.4–17.6 years) from recurrence. All of the recurrences were invasive and treatment included wide local excision with radiotherapy in all of the patients followed by adjuvant chemotherapy for the premenopausal patients. None of the patients had metastatic disease at presentation. The disease-free survival from initial recurrence was 83% and 63% at 5 and 10 years, respectively. The 5- and 10-year survival following local recurrence was 80%. One patient died 3.6 years after recurrence with metastatic disease, while a second patient developed metastatic disease 11.8 years after her initial recurrence. The remaining four patients are alive (NED), ranging from 1.4 to 10.7 years following their local recurrence. Local recurrence following mastectomy for DCIS is rare, usually invasive, and may have a long interval to failure. Salvage using conventional multimodality therapy appears to result in long-term survival.     

Concurrent platinum-based chemotherapy and radiotherapy for locally advanced bladder cancer

Eight patients with locally advanced bladder cancer who were not candidates for radical cystectomy or concurrent intra-arterial chemotherapy and radiotherapy were treated with combined platinum-based chemotherapy and radiation therapy. Six of the eight patients (75%) achieved a clinical complete response (CR). The effect of therapy in four patients whose histopathological responses were evaluated was effect grade 3. One of the eight patients died of treatment-related myelosuppression. The other two patients died of intercurrent disease, while the remaining five patients are alive with preservation of a functional bladder. The 2-year overall survival rate was 87.5%. Adverse events due to chemotherapy were mainly bone marrow suppression. Those caused by radiation therapy were rectal irritability. We considered concurrent platinum-based chemotherapy and radiotherapy useful for the treatment of locally advanced bladder cancer.     

Muscle-invasive transitional cell carcinoma of the bladder: strategies for bladder preservation

For patients with invasive bladder cancer, radical cystectomy remains the gold standard of treatment. However, based upon success with combination chemotherapy, physicians have begun to use this modality in an integrated approach with radiotherapy. This approach is of interest for elderly patients with poor medical conditions and for younger patients who may prefer to retain their bladders. Thorough transurethral resection of the bladder followed by chemotherapy with or without radiotherapy has become the focus of several studies. Bladder-conserving therapy may be offered to selected patients with invasive bladder cancer as a viable alternative to radical cystectomy.     

Stage B (P2/3A/N0) transitional cell carcinoma of bladder highly curable by radical cystectomy.

Seventy-one patients with pathologic Stage B (P2/3a/N0) transitional cell carcinoma (TCC) of the bladder underwent radical cystectomy alone without preoperative radiotherapy or perioperative chemotherapy between 1983 and 1987 and have been followed a median of fifty months. The five-year actuarial survival and disease-free survival rates were 82 percent and 77 percent, respectively, and only 13 patients (18%) have relapsed. Histologic parameters were evaluated as to prognostic impact; none correlated with disease-free survival rates although the presence of vessel involvement portended a worse disease-free survival rate (68% versus 80%). During this same period, an additional 15 patients underwent radical cystectomy for pathologic Stage B disease but received adjuvant chemotherapy on the basis of vessel invasion. Their disease-free survival rate at five years was 80 percent, comparable to the disease-free survival rate for patients with vessel invasion treated by surgery alone (68%). Although the role of systemic chemotherapy in the management of invasive bladder cancer remains under investigation, it would appear that patients with Stage B TCC are best treated with radical cystectomy alone. Continued analysis of modern surgical results grouped by current pathologic staging criteria is needed to identify patients who have a relatively low risk of relapse and thus little need for additional therapeutic intervention. These results demonstrate that Stage P2/3a/N0 TCC of the bladder is highly curable by surgery.     

Improved survival in infants and children with primary malignant liver tumors

Forty-six infants and children with primary malignant liver tumors were studied. Thirty-three had hepatoblastomas, 9 hepatocellular carcinomas, and 4 had sarcomas. Thirty-eight patients underwent liver biopsy or excision of the tumor. Nineteen patients were treated between 1952 and 1971 (mean survival 5.5 months) and the remaining 19 were treated between 1972 and 1981 (mean length of survival 30 months, 2 year survival rate 53 percent). In 21 patients with an unresectable tumor, biopsy alone was performed (mean survival 7 months). Ten patients underwent primary resection of their liver tumor (mean length of survival 23 months, 2 year survival rate 37.5 percent). Seven other patients with an unresectable tumor were treated with chemotherapy alone or in combination with radiotherapy for a mean period of 6 months before second-look celiotomy was carried out. Six of these patients (all treated after 1975) had significant reduction in the size of their tumors. Mean length of survival in this group was 4 years 2 months, and the 2 year survival rate was 100 percent; at present, 5 of these patients are living without the disease 2.5 to 7 years after diagnosis. Improved techniques in liver resection and chemotherapy before second-look celiotomy is undertaken for an unresectable lesion have improved prognosis in children with a malignant liver tumor.     

Benefit of radical cystectomy in the elderly patient with significant co-morbidities

Although recent series have demonstrated that radical cystectomy can be safely performed in elderly patients, few if any, have examined the long-term success of this procedure. We sought to determine the long-term benefit and survival outcomes after radical cystectomy in the elderly, high operative risk patient. We reviewed the records of all patients undergoing radical cystectomy between July 1994 and January 2000. Of these 382 patients, we identified 38 patients with transitional cell carcinoma who met our predetermined selection criteria of elderly, high peri-operative risk patients [age > or = 75 years and American Society of Anesthesiologists (ASA) classification > or = 3]. We analyzed patient characteristics, presenting symptoms, pathology, outcomes, and survival. Median age was 79 years (75-87 years). All but a single patient underwent surgery for symptomatic disease. No patient died in the early perioperative period. At a mean follow-up of 22 months (3-90 months), 11/38 (29%) patients are alive. Of the patients with < or = pT2B pathology, 9/27 (33%) are alive and are disease-free. There are 2/11 patients (18%) with > or = pT3 pathology still alive with 1 of those patients (pT4a) alive with disease 34 months after his radical cystectomy. Kaplan-Meier survival curves demonstrate that patients with organ confined disease (< or = pT2B) had a significantly longer mean overall survival than patients with nonorgan confined disease (> or = pT3): 31 months vs. 18 months, P = 0.046. Cause of death was known in 17 patients, with the majority (14/17) because of bladder cancer. However, there were no local recurrences, and palliative goals were achieved in all patients. Our results validate radical cystectomy as a safe and effective treatment choice in the elderly patient with significant co-morbidities. These patients, most of whom are symptomatic, can achieve palliation of their symptoms, local control, and long term survival, especially if their bladder cancer is organ confined. Reluctance to offer timely, aggressive local therapy may compromise ultimate survival, even amongst high operative risk, elderly patients.     

Neoadjuvant chemotherapy before definitive treatment for stage III carcinoma of the breast

One hundred women with American Joint Committee (AJC) stage III (T2, N2; T3, N0/1/2; T4, N0/1/2) carcinoma of the breast were treated with combination chemotherapy following biopsy to confirm the diagnosis and determine hormone receptor status before any other treatment of the local disease (so-called neoadjuvant chemotherapy). Response was assessed after three cycles of treatment, and responders were treated until the tumor and/or axillary nodes failed to show further regression. Definitive surgery was then performed, usually radical mastectomy. Chemotherapy was resumed following surgery for a total of 12 cycles. Ninety patients are assessable, and 70% have responded to chemotherapy. Outcomes of both responders and nonresponders were analyzed. Radical mastectomy without postoperative radiotherapy seems to be the preferable surgical treatment for the responders. Median follow-up of the assessable patients was 27 months; projected five-year disease-free survival of the responders is greater than 65%, and projected overall five-year survival of this group is greater than 85%. Because the follow-up of these patients suggests a marked improvement in outcome compared with similar patients treated traditionally with mastectomy or radiotherapy followed by adjuvant chemotherapy, we advocate more widespread use of combination chemotherapy before definitive treatment for stage III carcinomas of the breast.     

Radiotherapy for adenoid cystic carcinoma of the palate

This is an analysis of 16 patients with adenoid cystic carcinoma of the palate who were treated with curative intent by radiotherapy alone (9) or surgery and radiotherapy (7). No patient received adjuvant chemotherapy. Follow-up ranged from 3 years to 19 years with 69% of the patients having follow-up of more than 5 years. Two patients in the radiotherapy alone group developed local recurrences, and both have had long-term surgical salvage. No local failures were seen in the combined therapy group. Twelve patients (75%) are alive and disease-free. Two patients died with distant disease, one within each treatment group. One patient in the combined therapy group is alive at 8 years with distant metastases, while one in the radiotherapy alone group died of intercurrent disease at 12 years. No significant complications were seen in either treatment group. The role of radiotherapy in the treatment of adenoid cystic carcinoma of the palate is reviewed.     

Long-term Results of Adjuvant Irradiation or Surveillance in Stage I Testicular Seminoma

Background : Excellent treatment results are obtained for stage I testicular seminoma treated with orchiectomy and prophylactic radiotherapy. In patients with stage I nonseminomatous testicular tumors, surveillance alone is successful, however, this treatment option for stage I testicular seminomas is controversial. There have been few reports of long-term follow-up of surveillance alone for patients with stage I testicular seminoma.
Methods : To assess the appropriateness of this treatment option, a retrospective survey of stage I testicular seminoma was undertaken. Twenty-seven patients who underwent prophylactic radiation therapy (RT group) and 41 patients followed only by surveillance (S group) after high orchiectomy were evaluated. Their follow-up consisted of frequent clinical examinations, abdominal CT scans, chest x-rays and serum tumor markers.
Results : In the RT group, with a median follow-up period of 15 years, 1 patient (3.6%) had a recurrence in the lung at 4 months after orchiectomy and died, but the remaining 26 are alive with no evidence of disease (NED). In the S group, with a median follow-up period of 7.3 years, 5 (12.2%) relapsed in the retroperitoneal lymph nodes, but all are alive with NED following chemotherapy. The remaining 36 are all alive without recurrence (follow-up period, 38 to 132 months). Although the relapse rate in the S group was relatively higher than in the RT group, there was no significant difference between the 2 groups.
Conclusion : If a frequent follow-up protocol is administered and followed by the patient, surveillance alone may be a recommended management for stage I testicular seminoma.     

Radical radiotherapy with salvage surgery for invasive bladder cancer: results following a reduction in radiation dose.

The management of invasive bladder cancer in Edinburgh, as in many other centres in the UK, has been by radical radiotherapy, with cystectomy reserved for local treatment failure or relapse. A review of the results of this policy in 1987 highlighted what was felt to be an unacceptably severe morbidity rate of 15%. The dose of radiation was therefore reduced from 55 Gy in 20 daily fractions to 52.5 Gy. Forty of 80 patients (50%) treated in this way have achieved a complete response at 6 months which is a similar response rate to that in previous reports. In addition, ten of 23 frail or elderly patients (43%) achieved a complete response with a lower dose of 50 Gy given as a split course over 7 weeks. Eighteen patients have had a salvage cystectomy, and 12 of 18 (67%) are alive and disease-free. Although follow-up is short, to date toxicity has been reduced, and our results support our decision to reduce the radiation dose.     

Vaginal neoplasms in infancy: the combined role of chemotherapy and conservative surgical resection

This retrospective review of nine infants treated for vaginal tumors between 1964 and 1986 identifies the spectrum of lesions and examines the trend toward more conservative surgical therapy. Our patients' ages ranged from 1 to 30 months. Symptoms of vaginal bleeding or protruding tissue prompted examination under general anesthesia in all cases. Two children had benign protruding polypoid masses treated by simple excision. Seven had malignant tumors, two with endodermal sinus lesions and five with embryonal rhabdomyosarcoma. Early in this series, surgical therapy for embryonal rhabdomyosarcoma consisted of either anterior or complete pelvic exenteration, with adjuvant chemotherapy given in two of the three patients. One patient was operated on prior to the development of effective chemotherapy, and died of recurrent tumor 14 months after surgery. A second patient died from cardiac failure secondary to adriamycin toxicity 6 years after initial therapy. A third patient underwent total pelvic exenteration followed by successful reanastomosis of the colon to the anal verge. This patient, a female, is the oldest survivor and is free of disease 14 years after therapy. The most recent therapeutic approach used in two patients with embryonal rhabdomyosarcoma consisted of local tumor excision followed by postoperative chemotherapy. Both patients are alive and disease-free 9 and 11 years after therapy. Two patients with endodermal sinus tumor were treated with chemotherapy after simple excisional biopsy. They were then followed with serial vaginal biopsies at 3-month intervals. One required a partial vaginectomy for local recurrence 21 months after initial presentation. Both patients are alive and disease-free 18 months and 4 years after surgery.     

Evidence supporting preoperative chemotherapy for small cell carcinoma of the bladder: a retrospective review of the M. D. Anderson cancer experience

PURPOSE: Neuroendocrine tumors of the bladder comprise a small subset of all bladder tumors. To improve our understanding of this tumor and define outcomes with current management, we performed a retrospective review of these cases. MATERIALS AND METHODS: We reviewed the records of 88 patients with small cell bladder carcinoma evaluated at our institution between 1985 and 2002. Of these patients 46 underwent cystectomy, including 25 who were treated with initial cystectomy and 21 who received preoperative chemotherapy. RESULTS: For patients treated with initial cystectomy median cancer specific survival (CSS) was 23 months, with 36% disease-free at 5 years. For patients receiving preoperative chemotherapy median CSS has not been reached (p = 0.026), although CSS at 5-years was 78% with no cancer related deaths observed beyond 2 years. Notably 7 of 25 patients treated with initial cystectomy received chemotherapy after surgery but their survival was no better than those treated with cystectomy alone. As others have observed, the pathological stage was higher than clinically appreciated for 56% of patients treated with initial cystectomy. Moreover, there were no cancer related deaths among patients with disease down staged to pT2 or less. CONCLUSIONS: Like other neuroendocrine tumors, small cell carcinoma of the bladder grows rapidly but is chemo-sensitive. Clinical under staging is the rule. Optimal results are achieved via integration of local and systemic treatment. Our results suggest that preoperative chemotherapy is the optimal strategy, even in the setting of clinically localized cancer. On the basis of these observations, we have initiated a trial in which 4 cycles of aggressive multiagent preoperative chemotherapy are followed by radical cystectomy.     

Genitourinary rhabdomyosarcoma

Rhabdomyosarcoma is the most common soft tissue malignant neoplasm involving the pelvis of children. Debate still exists over whether the best treatment is pelvic exenteration, radiation and chemotherapy or chemotherapy as the cornerstone to treatment, with diminished needs for extensive surgery and prolonged radiotherapy. Contrariwise, there is little debate over the combined treatment modality for paratesticular rhabdomyosarcoma. We have evaluated and treated 19 children with rhabdomyosarcoma, including 17 with pelvic rhabdomyosarcoma and 2 with paratesticular rhabdomyosarcoma. This retrospective study was done to evaluate treatment regimens for patients with genitourinary rhabdomyosarcoma. For patients with pelvic rhabdomyosarcoma the study was divided into 2 series. In an early series 6 of 7 children had a pelvic exenteration with or without chemotherapy. Of these 7 children 3 are well 15 to 27 years following diagnosis. In a later series of 10 children, when chemotherapy was used more commonly, none underwent pelvic exenteration. Of these 10 patients 7 had chemotherapy or chemotherapy and biopsy only. Only 1 child, who presented with stage IV disease, died in this series. Another child with stage III disease had progressive disease despite chemotherapy and subsequent cystoprostatectomy. Thus, of 9 remaining patients 8 are well from 1 to 8 years. It appears that pelvic rhabdomyosarcoma can be treated effectively with chemotherapy, and limited surgery and radiation. Fortunately, pelvic exenteration can now be limited to a select few.