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| Bladder cancer histology - rare tumors |
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On this page: Correct pathology is of paramount importance when considering treatment options and approaches for rare bladder tumors. If possible, it is wise to go to a cancer center of excellence for a second opinion on diagnosis, pathology and/or treatment as major cancer centers are more experienced with non-TCC bladder cancer. Experts at MD Anderson Cancer Center in Houston, Texas are currently (2006) conducting clinical trials for small cell and urachal bladder cancers (below). Rare histologic cell types found in bladder cancer: Squamous cell carcinoma, This type of cancer affects the flat, scale-like cells on the surface of the skin called squamous cells. SCC of the bladder cancer typically forms after many years of chronic infection or irritation (such as by indwelling catheters). By the time squamous cell bladder cancer is detected it is usually at an advanced stage.2 Although this is the second most common form of bladder cancer, it only accounts for 2-5% of cases in industrialized countries. 3 SCC bladder cancer is more often found in tropical climates and is associated bilharzial parasites. These parasites cause a condition called urinary schistosoma haematobium which can lead to bilharzial bladder cancer. This tumor is the most common form of cancer found in adult males in Egypt, due to the prevalence of bilharzial infestation in the countryside. The stage and grade of the tumor, as well as lymph node involvement, have the most significant impact on survival. 4 Although metastases in bilharzial associated bladder cancer are uncommon, bone is the most common site of metastasis. 5 Adenocarcinoma of the bladder- 2% of all bladder cancer diagnoses, adenocarcinoma cells have glandular characteristics. Adenocarcimomas may occur as solid tumors, or they may be papillary (wart-like) in nature. Adenocarcinoma can be grouped into one of three categories: ---- primary vesical (involving the bladder proper) ---- urachal - involving the urachus, outside the bladder, can include the navel ---- metastatic - cancer cells that have broken away from the original tumor to grow within the bladder. The most common metastatic adenocarcinomas come from prostate, ovary and colon cancer. Many adenocarcinomas, urachal or primary, are advanced at the time of diagnosis. According to UK researchers, radiation therapyis not effective against urachal and primary adenocarcinomas, and radical cystectomy is the treatment of choice. The prognosis after radical surgery is often favorable for primary adenocarcinoma of the bladder. 6 Stage and grade are powerful predictors of outcome. 7 Urachal carcinoma of the bladder is a rare tumor that affects the outside of the bladder. Urachal tumours are more commonly found in women as well as younger patients. Tumors may be composed of tissue classified as adenocarcinoma, squamous cell carcinoma (SCC), or even sarcoma. Urachal tumors may cause mucous or bloody discharges in the urine, and they may produce dotted or "stippled" images on X-ray. Urachal tumors often are wider and deeper than expected, difficult to treat and they tend to metastasize and/or recur. 8 Urachal carcinomas are often locally advanced at presentation, which brings with it a risk of distant metastases. However, long-term survival following radical resection occurs in a significant fraction of patients (16 of 35 in the series from MDAnderson), supporting an attempt at margin-negative resection if at all possible. Chemotherapy appropriate for primary - type adeno-carcinomas can induce objective responses but definite increases in survival are as yet unproven. 9 MD Anderson Cancer Center in Houston is conducting a clinical trial for adenocarcinoma/urachal bladder cancer: Phase II Trial of 5-Fluorouracil, Leucovorin, Gemcitabine, and Cisplatin for Adenocarcinomas of the Urothelial Tract and Urachal Remnant: Principal Investigator: Arlene Siefker-Radtke Protocol Number ID03-011
Small-cell carcinoma - SCC is more commonly a malignancy of the bronchus, the cells take on endocrine-like characteristics. Primary small cell carcinomas of the bladder are rare and aggressive. Much of the new understanding about small cell bladder cancer came from reviewing treatment records of 88 patients with the disease treated at M. D. Anderson between 1985 and 2002, when expert researchers reported that patients who responded to neo-adjuvant chemotherapy, and were subsequently downstaged to stage pT2 or less, had a near 100% cure rate. Due to the rapid growth rate and the threat of understaging, there is clear evidence that neoadjuvant chemotherapy is important. Small cell bladder cancer has been shown to be highly responsive to chemotherapy; multi-therapy treatments are beneficial. 10 Page discussing the clincical trials at MDAnderson for small cell and urachal bladder cancer: Click here Rhabdomyosarcoma This tumor type begins in the soft tissues or connective tissue (e.g., tendon or cartilage) and can occur anywhere in the body. Rhabdomyosarcoma is the most common tumor of the lower genitourinary tract in children (mean appearance at five years of age), and rarely, is found in adults. In 2006 the Journal of Urology reported great improvements seen over the last 20 years, with cure rates in children as high as 80% and bladder preservation as high as 60% reported. The treatment of bladder-prostate rhabdomyosarcoma has evolved into multimodal therapy, including chemotherapy, radiotherapy and organ sparing surgery with bladder preservation. 11
Sarcomatoid carcinoma tumors arise mainly from connective tissue, which includes skin, tendons, muscles, bones, and cartilage. Radical surgery with adjuvant systemic therapy may be indicated for focal muscle invasive LELC. 22
Lymphoepithelioma-like carcinoma, whether in pure or mixed form, has a similar prognosis to ordinary urothelial carcinoma when treated by cystectomy.29 Urothelial papilloma of the bladder is another rare entity that represents less than 3% bladder tumors. The biologic potential of urothelial papilloma of the bladder is uncertain as there are only limited studies published on this issue. Patients with urothelial papillomas have a low incidence of recurrence and rarely progress to develop urothelial carcinoma. It seems reasonable to avoid labeling these patients as having cancer. It remains to be studied whether and when patients with papillomas who have no evidence of recurrence or progression no longer need to be followed. 24
Plasmacytoid Carcinoma The clinical presentation of this type of bladder cancer is frequently late because there is often no hematuria present. Cases showed coarse and indurated mucosal folds and thickened bladder walls, with no grossly identifiable tumor. Plasmacytoid bladder cancer is considered a very rare and aggressive form of bladder cancer.25 Clinical trial using chemotherapy for rare bladder tumors: 1: Urology. 2007 Feb;69(2):255-9. Related Articles, Links Prospective trial of ifosfamide, paclitaxel, and cisplatin in patients with advanced non-transitional cell carcinoma of the urothelial tract. Galsky MD, Iasonos A, Mironov S, Scattergood J, Donat SM, Bochner BH, Herr HW, Russo P, Boyle MG, Bajorin DF. Genitourinary Oncology Service, Division of Solid Tumor Oncology, Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA. This e-mail address is being protected from spam bots, you need JavaScript enabled to view it OBJECTIVES: Non-transitional cell carcinomas account for 5% to 10% of urothelial tract tumors and are each characterized by unique demographics, risk factors, and patterns of spread. A unifying feature of these malignancies is their aggressive course and poor outcome with standard chemotherapeutic regimens. Given the rarity of these tumors, no prospective data are available to guide management. METHODS: Patients with unresectable/metastatic adenocarcinoma or squamous cell, small cell, sarcomatoid, or poorly differentiated carcinoma of the urothelial tract were eligible for enrollment. Treatment consisted of paclitaxel 200 mg/m2 intravenously on day 1, cisplatin 70 mg/m2 intravenously on day 1, ifosfamide 1500 mg/m2 intravenously on days 1 to 3 plus mesna. Granulocyte colony-stimulating factor was administered with each cycle. The treatment was started again every 3 to 4 weeks for a maximum of six cycles. RESULTS: A total of 20 patients were enrolled. They had the following histologic types: adenocarcinoma in 11, squamous cell carcinoma in 8, and small cell carcinoma in 1. Patients received a median of four cycles (range one to six). The treatment was generally well tolerated, and the toxicity was predominantly hematologic. Overall, 7 (35%) of 20 patients (95% confidence interval 15% to 59%) achieved a major response (3 partial and 4 complete). The median survival for patients with adenocarcinoma was 24.8 months (95% confidence interval 10.2 to 32.3), and for those with squamous cell carcinoma it was 8.9 months (95% confidence interval 5.4 to not yet reached). CONCLUSIONS: The results of our study have shown that this regimen (ifosfamide, paclitaxel, and cisplatin) is active in patients with advanced non-transitional cell carcinoma of the urothelial tract. To our knowledge, this is the first prospective study of a chemotherapeutic regimen in this patient population. PMID: 17320659 [PubMed - in process]
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| Last Updated ( Friday, 14 November 2008 ) |