Small cell oesophageal carcinoma: an institutional experience and review of the literature

Primary small cell oesophageal carcinoma (SCOC) is rare, prognosis is poor and there is no established optimum treatment strategy. It shares many clinicopathologic features with small cell carcinoma of the lung; therefore, a similar staging and treatment strategy was adopted. Sixteen cases referred to Velindre hospital between 1998 and 2005 were identified. Patients received platinum-based combination chemotherapy if appropriate. Those with limited disease (LD) received radical radiotherapy (RT) to all sites of disease on completion of chemotherapy. Median survival of all patients was 13.2 months. Median survival of patients with LD was significantly longer than those with extensive disease (24.4 vs 9.1 months, P=0.034). This is one of the largest single institution series in the world literature. Combined modality therapy using platinum-based combination chemotherapy and radical RT may allow a nonsurgical approach to management, avoiding the morbidity of oesophagectomy. Prophylactic cranial irradiation is controversial, and should be discussed on an individual basis.     

Extrapulmonary small cell carcinoma: A single-institution experience and review of the literature

BACKGROUND: Small cell carcinoma (SCC) is a distinct pathologic entity thatmay also occur in extrapulmonary sites. In this report the retrospectiveresults of multimodal therapy of primitive extrapulmonary (E)SCC, in a single institution series, are presented. METHODS: Twenty-four patients (pts) with ESCC were referred to the Centrodi Riferimento Oncologico, Aviano, Italy, from 1986 to 1992.Clinico-therapeutic findings were evaluated in 20 pts. Theirages ranged from 20 to 87, with a median of 60.5 years. Primarytumor sites were urinary bladder (5 pts), prostate (4 pts),larynx (3 pts), kidney (2 pts), ovary, skin, oropharynx, trachea,uterine cervix, ethmoid, and stomach (1 pt each); lymph nodemetastases of unknown origin were observed in 3 pts. More than50% of pts presented extensive disease. RESULTS: Histologically, 16 cases were pure ESCCs and 8 cases were combined,4 of them with adenocarcinoma, 2 with transitional cell carcinoma,and 2 with squamous cell carcinoma. Immunohistochemical studies,performed in 7 cases, demonstrated the epithelial nature ofthese tumors. The cis-platin-VP16 (PE) regimen was used in 13pts, and 9 of them (69%) obtained objective responses afterchemotherapy (CT) alone, with 3 complete remissions (CR) and6 partial remissions (PR). Median CR and PR duration was 13+and 24 months, respectively. Radiotherapy was performed in 7/13pts after induction CT and before consolidation CT. The objectiveresponse rate was 100%, with 6 CR and 1 PR. No severe toxicside effects and no toxic deaths were reported. A patient treatedwith surgery alone for a urinary bladder tumor showed continuouslong-term survival, while 1 of 2 pts treated with radiotherapyalone obtained PR. CONCLUSIONS: The PE regimen has an activity similar to the one observed inpulmonary SCC. chemotherapy, extrapulmonary small cell carcinoma, pathology, radiotherapy     

Selective bladder preservation for muscle-invasive transitional cell carcinoma of the urinary bladder

Invasive transitional cell carcinoma (TCC) of the urinary bladder is traditionally treated with radical cystectomy. This approach results in great morbidity and lifestyle changes, and approximately half of the patients treated in this way will experience recurrent TCC despite surgery. An alternative approach using selective bladder-preservation techniques incorporates transurethral resection of bladder tumours, radiation therapy, and chemotherapy. Over the past 20 years, international experience has demonstrated that this approach is feasible, safe, and well tolerated. Furthermore, the long-term outcomes of overall survival and disease-free survival compare favourably with the outcomes from radical cystectomy. The most important predictor of response is stage, with significantly higher long-term survival in patients with T2 disease. Another important positive predictor of complete response to therapy is the ability of the urologic oncologist to remove all visible tumour through a transurethral approach prior to initiation of radiation therapy. A negative predictive factor is the presence of hydronephrosis, and age and gender do not affect disease-free survival. The majority of patients who enjoy long-term survival do so with an intact native bladder. Quality of life studies have demonstrated that the retained bladder functions well in nearly all of these patients. Selective bladder preservation will not entirely take the place of radical cystectomy, but should be offered as an important alternative to patients newly diagnosed with muscle-invasive TCC.     

Primary small cell bladder carcinoma: a case report and review of the current literature

Primary small cell bladder carcinoma is an extremely rare and highly aggressive tumor. Unfortunately, the optimal therapeutic strategy for the tumor is still unknown. Recently, a two-stage system for limited and extensive small cell bladder carcinoma has been suggested in analogy to the practiced staging and treatment of small cell lung carcinoma. We present a new case of small cell bladder carcinoma and discuss relevant current literature.     

An uncommon tumor of the urinary bladder: the small cell carcinoma

Primary small cell carcinoma of the urinary bladder is an uncommon tumor, compared to the frequency of urothelial tumors. Fifty percent of cases are combined with a non endocrine carcinomatous component. We report six new cases of this tumor, three of pure, and three associated with an urothelial carcinoma. Diagnosis is easy established by the immunohistochemical study which show the neuro-endocrine differentiation of these aggressive tumors. Pathologist needs to look for a neuro-endocrine part in all bladder cancer, as its presence modify the treatment. Chemotherapy is used in these cancers, due to their high metastatic power. Places of radical surgery and radiotherapy need to be specified.     

Symptomatic brain metastases from small-cell carcinoma of the urinary bladder: The Netherlands Cancer Institute experience and literature review

BackgroundThe incidence of symptomatic brain metastases in small-cell carcinoma of the urinary bladder (SCBC) is unknown. This precludes advice about prophylactic cranial irradiation (PCI).Patients and methodsThe medical records of all patients with SCBC seen at The Netherlands Cancer Institute from 1993 to 2009 (n = 51) were reviewed. Limited disease (LD) was defined as any pT, cN_0–1, and cM₀. Patients with LD were offered bladder-preserving treatment involving combined chemoradiotherapy. Patients with extensive disease (ED) were treated with palliative chemotherapy. PCI was not applied in any patient.ResultsAmong 39 patients with LD, median disease-specific survival was 35 months. Four developed symptomatic brain metastases after a median follow-up of 15 months (range 3–24) and were treated with whole-brain radiotherapy. No patient with ED developed symptomatic brain metastases during a median follow-up of 6 months. The reported incidence of brain metastases in SCBC in the literature ranges between 0% and 40%. On the basis of all reported series, the pooled estimate of the cumulative incidence of brain metastases is 10.5% (95% confidence interval 7.5% to 14.1%).ConclusionsThe incidence of symptomatic brain metastases from SCBC is significantly lower than that from small-cell lung cancer. Therefore, we do not routinely advise PCI in patients with SCBC.     

A case of small cell carcinoma of the urinary bladder

A case of small cell carcinoma with a high serum alpha-fetoprotein level of the urinary bladder is reported. Histologically, in addition to the small cell carcinoma, there were small areas of atypical carcinoids and larger epithelial cells. Immunohistochemically, the small cell carcinoma and the atypical carcinoids showed positivity for neuron-specific enolase, chromogranin, and vimentin. On the other hand, the larger epithelial cells showed positivity for alpha-fetoprotein, keratin, and carcinoembryonic antigen. Electron microscopically, a few neurosecretory granules were noted in the cytoplasm of the small cell carcinoma. After a total cystectomy, the serum alpha-fetoprotein level decreased to within a normal range. Postmortem examination of recurrent tumorous masses revealed only features of the small cell carcinoma. This case supports a theory that a small cell carcinoma of the urinary bladder develops from multipotential epithelial cells.     

Neurosecretory granules in small cell invasive carcinoma of the urinary bladder

A 69-year-old man showed symptoms of urinary bladder irritability and hematuria. Cystoscopy and radiographic studies revealed a tumor within a bladder diverticulum. The tumor shared histologic and ultrastructural features with oat cell carcinoma of the lung, including the presence of small numbers of neurosecretory type granules. In spite of conservative surgical treatment, there has been no evidence of recurrent or metastatic tumor over 14 months of follow-up. Hypophosphatemia was present preoperatively, but resolved spontaneously after tumor resection. In regard to histogenesis, a metaplastic origin is favored. This appears to be the first reported case of a neoplasm of Kultschitzky-type cells arising in the urinary bladder.     

Clinical review - small cell carcinoma of the bladder

OBJECTIVES: To review the published literature on the diagnosis and management of small cell carcinoma of the bladder (SCCB). METHODS: Papers were identified by searches of PubMed using the terms "small cell", "bladder" and "carcinoma". Additional papers were identified from review of references of relevant articles. RESULTS: SCCB comprises less than 1% of bladder malignancies. It is an aggressive tumour that commonly presents at an advanced stage, in an elderly population. Consequently, patients are often not fit for anti-neoplastic therapy. In fit patients, the bedrock of treatment in the majority of cases is platinum-based systemic chemotherapy, which was the only factor predictive of improved outcome on multivariate analysis in one large review. The use of neoadjuvant chemotherapy has been associated with favourable results and may therefore be the preferred approach when scheduling treatment. Options for local management comprise surgery or radiotherapy (sequentially or concurrently with chemotherapy), both of which are potentially curative in selected cases. However, the subsequent frequent development of urothelial malignancies with bladder-sparing approaches should be considered when planning treatment, particularly in younger patients. Prognosis of SCCB overall is poor, the median survival of all cases varies from 4 to 23 months, and overall survival at 5 years from 10% to 40% of patients. CONCLUSIONS: SCCB is a rare and aggressive tumour with a poor prognosis. Future efforts should be directed at its early detection and the development of more effective systemic therapies.     

Small cell carcinoma of the urinary bladder. Report of two cases and review of the literature.

Undifferentiated small cell carcinoma of the bladder is a rare but aggressive subset of urinary tract neoplasms. Analogous to small-cell carcinoma of the lung, this tumor frequently exhibits neuroendocrine differentiation. We report the 92nd and 93rd case of small cell carcinoma of the bladder reported in the literature with characteristic cytologic, histologic, histochemical, and ultrastructural features. The patients were treated initially with chemotherapy, but after a brief clinical course died for progression of disease and for myocardial infarction, respectively. The pathologic and clinical features and therapeutic options of the cases described in the literature are reviewed.     

Merkel cell carcinoma: experience of 14 cases and literature review

Merkel cell carcinoma is an aggressive skin cancer, with a significant incidence of locoregional lymphnode involvement, which requires timely diagnosis, adequate staging and aggressive therapy based essentially on surgical procedures. The aim of this study is to report our experience and to compare our results with literature findings, in order to discuss the role of the procedures adopted and their influence on prognosis. From July 1995 to April 2005, 14 patients were treated and followed-up for MCC in the National Cancer Institute of Naples. Tumor location was: buttocks (43%), extremities (36%) head (7%), unknown (14%). There were 7 Stage I, 5 Stage II and 2 Stage III patients. Surgical treatment consisted in wide excision (WE) in Stage I cases, WE and regional lymphadenectomy followed by radio- or chemo-therapy in Stage II and combined surgical and pre- and post-operative medical treatments in Stage III. Overall disease specific survival rate was 64% (median follow up 44 months). Recurrence occurred in 86% of Stage I and 20% of Stage II patients and involved, in 83.3% of Stage I patients, the lymph nodal draining basin. The treatment of recurrence implied surgery and radio or radiochemotherapy. Overall survival rate of recurrent patients was 57% (median follow-up 37.2 months). Due to the particular lymphotrophism of MCC, major care should be set on investigation and treatment of tumor lymph nodal draining basin. As long as the disease remains surgically manageable the prognosis for patients with MCC is favourable. The role of radio and chemotherapy is not yet assessed.     

膀胱小细胞神经内分泌癌与鳞癌混合性癌1例并文献复习

目的:通过对1例膀胱小细胞神经内分泌癌与鳞癌的混合性癌患者病例的分析,探讨该病的临床特点、病理学特点、临床诊断、治疗方法及预后。方法:回顾性分析遵义医学院附属医院泌尿外科2017年10月收治的1例术后病理组织学诊断为膀胱小细胞神经内分泌癌与鳞癌混合性癌患者的临床资料并进行国内外文献复习。结果:49岁男患,因“发现肉眼血尿2月余”入院,全麻下行腹腔镜根治性膀胱全切+回肠新膀胱+扩大淋巴结清扫术,术后经病理组织学确诊为膀胱混合性癌（小细胞神经内分泌癌+角化型鳞癌）。结论:膀胱小细胞癌(包括其与移行细胞癌、腺癌和鳞癌等复合癌)临床表现主要以肉眼血尿为主,呈现高度的恶性生物学行为,具有发现晚、进展快、转移早、恶性程度高、预后差等特点,需尽早完善病理学诊断,明确肿瘤的临床分期以达到早期治疗与有效改善其预后的目的。     

Selective organ preservation in muscle-invasive bladder cancer: review of the literature

The standard of care for transitional-cell carcinoma of the bladder with invasion to the muscularis propria is radical cystectomy with bilateral pelvic lymph node dissection. However, currently there is a tendency for organ preservation in selected cases of muscle-invasive bladder cancer. Trimodality treatment, including transurethral resection of the bladder tumor (TURBT), radiation therapy and chemotherapy, has been shown to produce 5-year and 10-year overall survival rates comparable to those of radical cystectomy. The current 5-year overall survival rates range from 50 to 67% with trimodality treatment, and approximately 75% of the surviving patients maintains their bladder. After trimodality treatment complete response is obtained in more than 70% of patients with muscle-invasive bladder cancer. Clinical criteria helpful in determining patients for bladder preservation include such variables as small tumor size (<2 cm), early tumor stage (T2-T3 disease), a visibly and microscopically complete TURBT, absence of ureteral obstruction, no evidence of pelvic lymph node metastases, and absence of carcinoma in situ (Tis). The close collaboration of urologists, radiation oncologists and medical oncologists is of paramount importance in succeeding in bladder preservation.     

Small cell carcinoma of the urinary bladder. The Mayo Clinic experience

BACKGROUND: Small cell carcinoma (SCC) of the urinary bladder accounts for 0.35-0.70% of all bladder tumors. There is no standard approach to the management of SCC of the urinary bladder. METHODS: The authors performed a retrospective study at Mayo Clinic (Rochester, MN) to characterize the clinical and pathologic features of patients with SCC of the urinary bladder diagnosed between 1975 and 2003 with emphasis on management. RESULTS: Forty-four patients were identified who had primary bladder SCC, 61.4% of whom had pure SCC. The male:female ratio was 3:1, the mean age was 66.9 years, and the mean follow-up was 3.2 years. Twelve patients (27.3%) had Stage II disease, 13 patients (29.6%) had Stage III disease, and 19 patients (43.2%) had Stage IV disease. The overall median survival was 1.7 years. The 5-year survival rates for patients with Stage II, III, and IV disease were 63.6%, 15.4%, and 10.5%, respectively. Six of eight patients with Stage II bladder SCC achieved a cure with radical cystectomy. Five patients with Stage IV disease had obvious metastases and received chemotherapy. Fourteen patients underwent radical cystectomy and were diagnosed later with locally advanced disease (T4b) or lymph node metastasis (N1-N3; Stage IV disease). Only 2 of 19 patients with Stage IV disease who received adjuvant chemotherapy were alive at 5 years. CONCLUSIONS: Patients with bladder SCC should undergo radical cystectomy except when metastatic disease is present (M1), in which case, systemic chemotherapy is indicated. Adjuvant treatment is not indicated for patients with Stage II disease after radical cystectomy but should be considered for patients with Stage III and IV disease. Chemotherapy should be a platinum-based regimen.     

Rectal small cell carcinoma: a case report and review of the literature

We present a rare case of small cell carcinoma of the rectum presenting with rectal bleeding and discomfort in a fit 51-year-old gentleman. Our patient was treated with a combination of chemotherapy and radiotherapy and remains alive and free of disease 6 years after diagnosis. Our patient experience and review of the literature is presented highlighting the uncertainties regarding the biological behaviour and management.     

原发膀胱平滑肌肉瘤临床病理分析及文献复习

目的:探讨原发性膀胱平滑肌肉瘤的临床病理诊断及鉴别诊断。方法:对1例膀胱平滑肌肉瘤的临床资料进行临床病理学分析及免疫组化观察,并复习相关文献。结果:组织特点:瘤细胞排列成束状或纵横交替呈编织状结构,瘤细胞呈短梭形或卵圆形,胞浆中度,核大、深染,异型性明显,核分裂相较多见,可见瘤巨细胞。免疫组化染色显示:Vim(+),SMA(+),Desmin(+),Myoglobin(-),Ckpan(-),MDM2(-),S-100(-)。结论:膀胱平滑肌肉瘤是一种罕见的膀胱肿瘤,其诊断主要依靠组织病理学和免疫组化标记。     

膀胱肉瘤样癌的诊断与治疗(2例报告及文献复习)

目的:探讨膀胱肉瘤样癌的组织学特性,提高对膀胱肉瘤样癌的诊治水平。方法:报告2例膀胱肉瘤样癌患者的临床、病理资料,复习相关文献进行讨论。结果:术后病理检查见上皮和肉瘤样间质2种恶性成分,癌与肉瘤样区有移行,诊断为膀胱肉瘤样癌。其中1例10个月后死于全身衰竭、多处转移。结论:膀胱肉瘤样癌具有浸润性生长的生物学特性,恶性程度高,预后不良,确诊依赖病理学及免疫组织化学检查;化疗、放疗都不太敏感[1],手术仍是首选治疗方式。