Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery

OBJECTIVE: To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968. PATIENTS AND METHODS: From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5-18) had RMS of bladder/prostate or vagina. Twenty of the patients received primary polychemotherapy (vincristine, actinomycin D, cyclophosphamide, adriamycin, and more recently including etoposide and ifosfamide), two had primary surgery and seven had additional radiotherapy. Fourteen patients had radical cystoprostatectomy, with continent cutaneous urinary diversion with an ileocaecal pouch in seven, in one each a transverse colonic pouch, orthotopic ileocaecal bladder substitution, a rectal reservoir and rectosigmoid pouch and a colonic conduit diversion in two patients. RESULTS: After a mean (range) follow-up of 8.6 (1.0-26) years, 17 patients had no evidence of disease. Five patients presenting initially with advanced tumour stages died from progressive RMS. Two patients with a continent urinary diversion required ureteric reimplantation for stenosis. In two patients severe bladder contraction after radiotherapy required bladder augmentation. CONCLUSION: Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.     

Transitional cell carcinoma of the prostate: Diagnosis,staging and management

Summary Over a 5-year period, 21 patients were diagnosed with transitional cell carcinoma (TCC) of the prostate. Tumor was primary to the prostate in 3 patients, but was associated with TCC of the bladder in the remaining 18. Ten patients developed TCC of the prostate following intravesical therapy for TCC of the bladder. Patients were staged according to local tumor extent, with 2 having disease confined to the prostatic urothelium, 7 showing invasion into ducts and 12 with stromal involvement. None of the 21 patients had metastatic disease at diagnosis. A total of 14 patients underwent radical cystoprostatectomy as primary therapy, while the remaining 7 were managed without radical surgery, receiving intravesical therapy, transurethral resection, systemic chemotherapy, and radiotherapy, singly or in combination. Minimum follow-up was 22 months, with an average of 35 months. Twelve patients (57%) are alive with no evidence of disease, 3 (14%) are alive with tumor and 6 (29%) have died — 5 (24%) of progressive tumor.     

Bladder tumors in children

Our experience with bladder tumors in children is presented and the pertinent literature reviewed. Nonepithelial tumors are more common than epithelial ones, and the most common symptom is bladder outlet obstruction. Papilloma, the common epithelial tumor, usually presents with hematuria and is successfully treated by transurethral methods. Treatment of bladder neurofibroma will depend on the site and size of the tumor and on resultant symptoms and obstruction; small or moderate-size nodules may be amenable to transurethral resection, local excision, or segmental resection, but for diffuse involvement of the bladder, total cystectomy may be necessary. Hemangiomas are the most common benign connective tissue tumor, and their common location in the upper part of the bladder usually permits successful treatment by open excision or segmental resection. Rhabdomyosarcoma is the most common bladder tumor in children. On present evidence radical cystectomy is the treatment of choice. The contributions of adjunct radio- and chemotherapy are not well defined, but such treatment is recommended since it has contributed to the control of tumor in nonresectable cases. Four of 8 patients in this series are alive and well five and one-half to seventeen years, 3 having had radical cystectomy (with radiotherapy in 2 and chemotherapy in 1) and 1 segmental resection with radio- and chemotherapy. Leiomyosarcoma of the bladder has been controlled by segmental resection, but it is logical to believe that radical cystectomy would extend the possibilities of surgical control in some cases; adjunct irradiation and chemotherapy may deserve a place in treatment as well.     

Treatment of medulloblastoma in children: Long-term results following surgery,radiotherapy and chemotherapy

Summary Forty-seven children with histologically confirmed medulloblastoma are considered. Forty-five cases were surgically treated by direct approach to the tumour, while two cases were treated only with a shunt. A shunt was inserted preoperatively in 14 cases, postoperatively in 4 cases. Surgical resection was total or subtotal in 52% of cases, partial in 35%, and limited to a biopsy in 13%. Radiation treatment to the entire neuraxis was done in 37 cases: 10 of these cases received additional chemotherapy—mostly with CCNU—as primary treatment for medulloblastoma.Ten patients died within 30 days after surgery. Twenty-two patients died months after treatment, mainly from tumour recurrence (19 cases). One patient was lost to follow-up. Thirteen patients are survivors from 10 months to 20 years after treatment. As a whole, the one year survival rate has been 67%, 3 year survival 43%, and 5 year survival 27%. Complications affecting prognosis have been presented by tumour recurrence and metastases. CSF shunting and lack of prophylactic irradiation to the cerebral hemispheres have been considered responsible for the high incidence of supratentorial metastases in our series.Factors influencing prognosis have been the extent of tumour resection and association of primary chemotherapy with radiotherapy. Within 3 years after surgery survival has been 52% in cases with total resection against 31% in cases with partial resection of tumour. As regards chemotherapy, 3 year survival has been 60% for patients with combined treatment (chemo- and radiotherapy) against 37% in patients with radiotherapy alone. It is concluded that the best results in children with medulloblastoma are achieved by a radical resection, associated with a combined primary treatment of radiotherapy and chemotherapy.     

Genitourinary small cell carcinoma: a retrospective review of treatment and survival patterns at The Ottawa Hospital Regional Cancer Center

OBJECTIVE: To review patients with genitourinary (GU) small cell carcinoma (SCC) treated at a regional cancer centre, as due to its rarity and aggressive nature, GU SCC remains a therapeutic challenge. PATIENTS AND METHODS: The charts of patients managed at a regional cancer centre between 1991 and 2002 for any GU diagnosis were manually reviewed to identify GU SCC. Demographic, staging, treatment and outcome data were extracted. The Veterans Administration small cell lung cancer staging classification of "limited" or "extensive" disease was adapted for SCC of the prostate and bladder (with "limited" defined as disease localized to the true and false pelvis, and "extensive" as disease beyond the pelvis). RESULTS: In all, 555, 858 and 5066 new cases of primary renal, bladder and prostate cancer, respectively, were identified. Of these patients, 22 had GU SCC (12 bladder and 10 prostate; there were no cases of SCC of the kidney). Eight of 12 patients with bladder SCC had limited disease; five of 12 are alive (all with limited disease at diagnosis), and the median survival was 19.8 months. Surviving patients received similar therapy, with transurethral resection of the bladder tumour, platinum-based chemotherapy, etoposide (4-6 cycles), and radical radiotherapy (56-60 Gy). Two of 10 patients with SCC of the prostate had limited-stage disease, but all 10 died, the median survival being 9.5 months. Survival by stage for both types combined was 59 months for limited disease and 8 months for extensive disease. CONCLUSIONS: These results indicate that GU SCC is an aggressive cancer; limited-stage SCC of the bladder or prostate, when treated with platinum/etoposide chemotherapy and radical radiotherapy, has a more favourable outcome than that of extensive GU SCC.     

Paediatric lower urinary tract rhabdomyosarcoma: a single-centre experience of 30 patients

OBJECTIVE: To retrospectively analyse the long-term outcome of children with bladder and/or prostate rhabdomyosarcoma who were diagnosed at the authors' institution over the last 17 years. PATIENTS AND METHODS: The study comprised 30 children (26 boys and four girls, mean age 5 years, range 15 days to 15 years); 23 had stage III and seven had stage II disease. The initial biopsy showed an embryonal variant in 27 and round-cell sarcoma in three patients. All patients received eight weekly doses of vincristine, actinomycin D and cyclophosphamide (VAC). Subsequent treatment depended upon the response to chemotherapy. RESULTS: Fourteen patients had a complete or partial response to chemotherapy (> 50% reduction in tumour size); they were maintained on VAC chemotherapy for 2 years. Twelve patients in this group survived with no evidence of disease for 7 months to 10 years. Additional therapies were used in three patients, i.e. radical cystectomy in one and external irradiation in two. Sixteen patients had a minimal response to chemotherapy; in six, radical cystectomy was feasible and was followed by one year of chemotherapy. All patients were free of disease for 4-11 years. Radiotherapy was given to the remaining 10 patients; thereafter radical cystectomy became feasible in five while partial cystectomy was possible in three. Only three of these 10 patients survived for 4-11 years. CONCLUSION: The tumour response to initial chemotherapy can be used to stratify patients into two risk-groups, i.e. low-risk patients with a complete or partial response in whom the bladder could be salvaged, and high-risk patients with a minimal response, in whom intensive treatment should be pursued, with no attempt at bladder salvage.     

Recurrence and progression of T1G3 transitional cell carcinoma of the bladder treated with intravesical bacillus Calmette-Guérin

OBJECTIVE: To examine the incidence of recurrence and progression in patients with stage T1, grade-3 carcinoma of the bladder treated with endovesical bacillus Calmette-Guérin (BCG) after complete transurethral resection. MATERIAL AND METHODS: From May 1995 to June 2002, 937 patients with superficial bladder cancer underwent transurethral resection. 46 patients (4.9%) had T1G3 tumors. All patients received endovesical BCG therapy 2-3 weeks after transurethral resection, given in 6 sessions as weekly instillations of 120 ml Pasteur strain BCG in 50 ml saline. Success was defined by normal cytology and cystoscopy, and normal bladder biopsies. Recurrent tumors were resected and a second or third cycle of therapy was given according to pathological status. Progressive tumors were managed by radical cystectomy, radiotherapy and/or chemotherapy depending on the nature of the tumor or clinical status of the patient. RESULTS: During follow-up 60.7% of the patients (28 of 46) remained tumor free after only 1 BCG cycle and 73.9% (34 of 46) after the third BCG cycle, and the bladder was preserved in all. Muscle-invasive progression was noted in 10 (21.7%) patients at the end of the BCG cycles. Radical cystectomy was done in 10 patients. The tumor-free survival rate of all patients including those who underwent cystectomy is 84.8% (39 of 46) with a median follow-up of 61 (range 39-118) months. CONCLUSION: Adjuvant immunotherapy with BCG after complete transurethral resection of the bladder tumor represents a highly effective treatment for bladder preservation in stage pT1, grade-3 carcinoma of the bladder. pT1G3 tumors with early high-grade recurrence after failed immunotherapy should be regarded as candidates for early radical cystectomy.     

Malignant thymoma in children: a 20-year review

Malignant thymomas are extremely rare in children, with only 27 cases reported thus far in the pediatric surgical literature. We report four additional cases diagnosed at this institution over the past 20 years (ages 3 to 14 years). Clinical presentations included superior vena cava syndrome, cough, dyspnea, cyanosis, enlarging mediastinal mass, spontaneous pneumothorax, and pleuropericardial effusion. Three patients underwent incomplete resection of the mass or biopsy because of "unresectability" and were treated with radiotherapy and adjunctive chemotherapy. One patient underwent near complete macroscopic resection as well as radiotherapy and chemotherapy. All patients died at intervals ranging from 6 months to 2 1/2 years after diagnosis. Three patients were found to have metastatic disease prior to death or at autopsy. In one case, the initial pathological diagnosis was lymphocytic thymoma. After ultrastructural studies were performed, the diagnosis was changed to thymic T-cell lymphoma. This patient subsequently developed acute lymphoblastic leukemia 3 months after surgical resection followed by radiotherapy. Malignant thymomas are highly aggressive tumors in children. A radical surgical approach with complete excision of the tumor and contiguous structures in continuity, with adjunctive radiotherapy and chemotherapy remains the only hope for survival in children with these rare lesions.     

对肌层浸润性膀胱癌不同分期行个体化治疗的讨论

肌层浸润性膀胱癌(MIBC)治疗的经典方案为根治性全膀胱切除+盆腔淋巴结清扫术,但近些年来国内外研究报道对MIBC患者采用保留膀胱手术+综合治疗,可获得相似或超过RC之疗效。分析保留膀胱手术可取得较好疗效与MIBC患者之相对分期低、病理分级属低级别性质肿瘤以及采用新辅助、辅助化、放疗等综合治疗密切相关。MIBC包括3个分期(T2、T3、T4),如能对不同分期制定相应的个体化治疗方案,而不是一律采用RC,相信会避免过度治疗且会进而提高MIBC患者生存率和生活质量。为此本文提出对MIBC中不同分期患者采用个体化综合治疗方案的设想:T2a期采用TURBT或根治性TURBT+术后膀胱灌注化疗;对T3a期新辅助化疗1～2疗程后行PR+盆腔淋巴清扫术,术后膀胱灌注化疗+辅助性化、放疗等综合治疗。余不同分期方案见文内。以上方案之设想仅供研讨,期盼进一步完善,提高疗效。     

Spindle and giant cell carcinoma of the bladder. Report of 3 cases

3 cases of spindle and giant cell carcinoma are reported. 2 patients with T3N0M0 and T3N1M0 tumors underwent radical cystectomy. They died 8 and 9 months after cystectomy for local recurrence and tumor metastases. Neither radiotherapy nor chemotherapy were effective in these 2 patients. 1 patient with T1NXM0 disease underwent resection of the tumor through cystotomy. Although she had neither adjuvant chemotherapy nor radiotherapy, she is well without recurrence 6 years after surgery. Early surgical resection of the tumor may be the only currently accepted therapy of spindle and giant cell carcinoma of the bladder.     

Long-Term Followup of all Patients with Muscle Invasive (Stages T2, T3 and T4) Bladder Carcinoma in a Geographical Region

Purpose

We studied the relationship between long-term survival and treatment of stages T2, T3 and T4 bladder carcinoma in an unselected patient population.

Materials and Methods

A total of 680 patients with the initial diagnosis of bladder carcinoma in 1987 to 1988 in Western Sweden was prospectively registered and followed until 1994. Of these patients 107 had stage T2 to T3 and 41 had stage T4 disease.

Results

Of the patients with stage T2 to T3 disease 30 (mean age 66) underwent radical cystectomy, 33 (mean age 75) full dose radiotherapy and 44 (mean age 81) nonradical therapy (mainly transurethral resection of the bladder). The 5-year crude survival rates were 33, 15 and 14%, respectively. Of the patients with stage T4 disease 6 (mean age 61) underwent radical cystectomy, 9 (mean age 73) full dose radiotherapy and 26 (mean age 81) nonradical therapy (mainly transurethral resection of the bladder). All except 1 patient died of disease within 4 years.

Conclusions

More than 60% of the patients in the cohort were considered unsuitable for radical cystectomy and their survival was poor, whether treated with full dose radiotherapy or transurethral resection of the bladder alone.     

泌尿系小细胞癌的诊断及治疗（附11例报告）

目的：探讨泌尿系小细胞癌的诊治方法、疗效及预后。方法：回顾性分析我院2003年1月～2012年12月收治的t1例泌尿系小细胞癌的病理、临床特征、诊断方法、治疗方法等资料,并对患者治疗效果、生存期等进行分析。结果：11例患者,男9例,女2例,平均年龄66．9（57～83）岁,肿瘤位于膀胱5例（1例为女性）,右肾3例（1例为女性）,左肾1例,前列腺2例。按TNM分期,膀胱肿瘤：T2bNnM01例,T2bN1M0 1例,2例均行根治性膀胱全切＋肠代膀胱术,分别于术后24个月及13个月死亡;T2bN2M1 1例,T3bN2M1 1例,此2例行根治性膀胱全切＋放疗＋化疗,分别于术后9个月、12个月死亡;T4N2M1 1例,行放疗＋化疗,3个月后死亡。肾脏肿瘤：T1aN0M01例,T1nN0M0 1例,T2bN1M0 1例,T3bN2M1 1例,均行根治性肾切除术＋放疗＋化疗,3例于术后6个月、13个月、24个月死亡,1例至今4年尚存活。前列腺肿瘤：T1cN0M1b 1例,行前列腺电切＋放疗＋化疗;T3bN1M1c 1例,行放疗＋化疗,随访时分别死于术后25个月及15个月。11例患者生存期最短3个月,目前最长48个月,平均17．5个月;1年生存率63．6％（7／11）,5年生存率目前为0。结论：泌尿系小细胞癌恶性度高,预后差,手术联合放化疗可能会延长患者生存期、改善其生活质量。     

Bladder-sparing protocols in the treatment of muscle-invasive bladder cancer

Bladder-sparing protocols (BSP) have been gaining widespread popularity as an attractive alternative to radical cystectomy (RC) for muscle-invasive bladder cancer. Unimodal therapies are inferior to multimodal regimens. The most promising regimen is trimodal therapy (TMT), which is a combination of maximal transurethral resection of bladder tumor (TURBT), radiotherapy, and chemotherapy. In appropriately selected patients (low volume unifocal T2 disease, complete TURBT, no hydronephrosis and no carcinoma-in-situ), comparable oncological outcomes to RC have been reported in large retrospective studies, with a potential improvement in overall quality of life (QOL). TMT also offers the possibility for definitive therapy for patients who are not surgically fit to undergo RC. Routine biopsy of previous tumor resection is recommended to assess response. Prompt salvage RC is required in non-responders and for recurrent muscle-invasive disease, while non-muscle-invasive recurrence can be managed conservatively with TURBT +/− intravesical BCG. Long-term follow-up consisting of routine cystoscopy, urine cytology, and cross-section imaging is required. Further studies are warranted to better define the role of neoadjuvant or adjuvant chemotherapy in the setting of TMT. Finally, future research on predictive markers of response to TMT and on the integration of immunotherapy in bladder sparing protocols is ongoing and is highly promising.     

新辅助治疗低位局部进展期直肠癌35例结果分析

目的 探讨新辅助治疗对低位局部进展期直肠癌的临床治疗价值。方法 对35例低位局部进展期直肠癌患者，采用新辅助治疗方案。常规分割放疗，放疗总剂量DT：46Gy，每次2Gy，每周5次。全身化疗2个疗程，每次予以奥沙利铂130mg／m^2，第1天静脉点滴；甲酰四氢叶酸钙(CF)200mg／m^3，第1～3天静脉点滴；氟尿嘧啶(5-FU)500mg／m^2，第1～3天静脉点滴。治疗结束后4～6周进行手术。结果 经新辅助治疗后，病理完全缓解7例，肿瘤平均缩小34．4％，65．7％的病例T分期下降，淋巴结阴转率为55．6％。根治切除34例，其中腹会阴联合切除18例，保肛手术16例，保肛率为45．7％。姑息性Hartmann术1例。随访至今，肝转移2例，根治切除术后无1例局部复发。保肛患者肛门功能良好。结论 对低位局部进展期直肠癌患者采用新辅助治疗，可使肿瘤分期降低，提高手术切除率和保肛率。     

前列腺尿路上皮癌的诊疗分析

目的:总结前列腺尿路上皮癌的诊断与治疗方法。方法:回顾性分析2011年1月至2019年11月郑州大学第一附属医院收治的25例前列腺尿路上皮癌患者的临床资料。平均年龄(63.4±11.2)(39~85)岁。就诊原因：肉眼血尿13例,排尿困难9例,膀胱刺激征3例。25例PSA均<4 ng/ml。17例MRI检查提示膀胱及前...     

Radical resection of rectal cancer primary tumor provides effective local therapy in patients with stage IV disease

Background The optimal use of radical surgery to palliate primary rectal cancers presenting with synchronous distant metastases is poorly defined. We have reviewed stage IV rectal cancer patients to evaluate the effectiveness of radical surgery without radiation as local therapy. Methods Eighty stage IV patients with resectable primary rectal tumors treated with radical rectal surgery without radiotherapy were identified. Sixty-one (76%) patients received chemotherapy; response information was available for 34 patients. Results Radical resection was accomplished by low anterior resection (n=65), abdominoperineal resection (n=11), and Hartmann’s resection (n=4). Surgical complications were seen in 12 patients (15%), with 1 death and 4 reoperations. The local recurrence rate was 6% (n=5), with a median time to local recurrence of 14 months. Only one patient received pelvic radiotherapy as salvage treatment. One patient required subsequent diverting colostomy. Median survival was 25 months. On multivariate analysis, the extent of metastasis and response to chemotherapy were determinants of prolonged survival. Conclusions For patients who present with distant metastases and resectable primary rectal cancers, radical surgery without radiotherapy can provide durable local control with acceptable morbidity. The extent of metastatic disease and the response to chemotherapy are the major determinants of survival. Effective systemic chemotherapy should be given high priority in the treatment of stage IV rectal cancer.     

Bladder carcinoma presenting with rectal obstruction

Two patients with bladder carcinoma that caused annular constriction of the rectum are described. A mechanism of local invasion by genitourinary malignancies (bladder and prostate) involves penetration of the rectovesical fascia of Denonvillier and circumferential rectal spread. Treatment of complete or partial obstructing lesions includes surgery, radiotherapy, or chemotherapy (or combination), as well as several new techniques, notably laser destruction and transrectal tumor resection.     

Intra-arterial chemotherapy in combination with radiotherapy for invasive bladder cancer and prostate cancer

Forty-five patients with muscle-invasive bladder cancer treated with intra-arterial doxorubicin chemotherapy plus low-dose radiotherapy between September 1979 and March 1990 were retrospectively studied. Twenty-eight (62%) patients achieved a complete response (CR) and in all of them, a functional bladder could be preserved. The 10-year cause-specific survival rate of patients with CR was 95.5%, but that of patients not achieving a CR was 39%. These results demonstrate that in patients who achieve a CR with this treatment, we may be able to preserve a functional bladder. In a prospective study, we designed a new intra-arterial chemotherapy regimen in order to achieve a higher degree of effectiveness and to preserve a functional bladder. Twenty-three patients were treated with concurrent pirarubicin/cisplatin intra-arterial chemotherapy and radiotherapy after complete transurethral resection. Twenty-one (91%) patients achieved CR. One of these patients had relapse with lung metastases and was treated surgically. Two patients who did not achieve a CR died of cancer, and 21 patients are alive with preservation of functional bladder. For treatment of prostate cancer, we now administer only adjuvant intra-arterial chemotherapy plus irradiation for patients after radical prostatectomy.     

新辅助动脉化疗在肌层浸润性大体积膀胱癌保留膀胱治疗中的价值

目的评估术前新辅助动脉化疗联合经尿道手术在直径超过3cm的肌层浸润性膀胱癌保留膀胱治疗的临床价值。方法对于较大体积(直径3cm)的28例肌层浸润性膀胱肿瘤(T2N0M0~T4aN0M0)采用新辅助动脉化疗联合手术治疗,观察动脉化疗效果,分析肿瘤降期率、保留膀胱率、肿瘤复发率,Kaplan-Meier法计算总体生存率、无肿瘤复发生存率,并绘制生存曲线。结果 26例(92.9%)患者动脉化疗有效,肿瘤可见明显缩小,经3~5次动脉介入治疗后行经尿道切除术+膀胱灌注完成保留膀胱治疗;动脉化疗无效2例,立即行根治性全膀胱切除术。26例完成保留膀胱治疗的患者,术后肿瘤病理分期降低19例(73.1%),无变化为7例。肿瘤复发8例(复发率为30.8%),其中,浅表性复发5例,局部浸润性复发2例,远处转移1例。28例患者总体生存率:3年69%,5年62.1%。无肿瘤复发生存率:5年44.07%。最终25例患者得到保留膀胱(保留膀胱率89.3%)。结论直径3cm的较大体积浸润性膀胱肿瘤采用术前新辅助动脉化疗治疗,可使肿瘤降期降级及体积缩小,有利于经尿道完全切除,可有效提高患者生存率,同时保留了膀胱,大大提高患者生存质量,对不愿或不宜行膀胱全切的患者是一个理想的选择。     

髂内动脉灌注化疗联合放疗治疗浸润性膀胱癌

目的：评估髂内动脉灌注化疗联合放疗治疗浸润性膀胱癌的有效性及安全性。方法：对18例无法耐受或不愿接受膀胱全切手术的浸润性膀胱癌患者进行保留膀胱治疗，包括髂内动脉灌注化疗联合盆腔放疗。化疗药物包括顺铂、表阿霉素、丝裂霉素。平均放疗剂量为30～40Gy。结果：经3次化疗、栓塞及一个疗程的盆腔放疗，完全缓解率44．4％(8／18)，部分缓解率55．6％(10／18)；对部分缓解患者进行经尿道膀胱肿瘤切除术(TURBT)或膀胱部分切除术。所有病例定期随访，中位随访期为24．3个月。对随访期间发现的肿瘤复发进行再次TURBt或膀胱部分切除术。2例死于肿瘤。结论：髂内动脉灌注化疗联合放疗作为浸润性膀胱癌患者保留膀胱手术前新的辅助治疗，在提高生存率的同时可改善生活质量。