In rare cancers Sarcoma of The Bladder

Sarcomas are very rare in the urinary tract. Many patients referred to our institution with tumors diagnosed as sarcomas of arriving at epithelial components in the review, and therefore are best classified as carcinomas, as described above. However, sarcomas that appear to arise within the bladder without a detectable epithelial component occur. In our opinion, radiotherapy is the only recognized risk factor, and the typical delay of two or three decades of exposure to radiation of secondary cancer seems to apply.

In adults, the most common histological subtype is leiomyosarcoma, but in our own record, osteogenic sarcoma is the most common. Of course, many tumors show areas with more than one pattern of differentiation. Apart from the setting of prior pelvic radiation, no features of clinical presentation. It is our impression that patients with sarcoma are more susceptible to tumor implantation in the urethra after transurethral resection (TUR), but this has not been formally studied or reported by other centers. Clinical management follows the principles of sarcoma management in other sites. In general, surgery is the mainstay of therapy. If the primary tumor is quite large, and shows that histology may be a reasonable response to chemotherapy provided (such as osteogenic sarcoma), then, neoadjuvant chemotherapy is administered. As expected, the result is driven largely by the stage.

Genitourinary cancer Urachal cancer part 1

It is estimated that one case of urachal carcinoma for every 600 patients treated for bladder cancer. The urachus is a vestigial structure, which while important in some species, has no role in the development of human beings. The precursor initially urachal ligament arises from the cloaca at the end of the large intestine. During embryogenesis, the cloaca is divided to form the urogenital sinus, which develops in the bladder and sexual organs, and the anorectal canal, which becomes the rectum. The bladder is formed from the medial part of the urogenital sinus. Superior to this, in light of the allantois is obliterated to form the urachus. In adulthood, the urachus is attached to the obliterated umbilical arteries to form the commune of ligament. Although urachal ligament is most often connected with the dome of the bladder, can also attach to the back wall of the bladder or earlier, usually in the midline. A light remnant may persist in the bladder wall in the form of small tubular or cystic structures, and can communicate with the light of the bladder in up to one third of adults. Columnar cells, glandular islands, and the transitional cell epithelium may be present in a urachal remnant. When the malignancy is found to arise from this remnant, histology is the overwhelming intestinal adenocarcinoma.

Two theories have been proposed for the development of tumors of the urachus. One of them is that they come from intestinal adenocarcinomas is based on the left behind the cloaca during embryological development. This explains the histological similarity to adenocarcinomas of the rectum. An alternative hypothesis is that these tumors arise from metaplasia. Supportive evidence includes the occurrence of adenocarcinomas of the bladder epithelium despite exstrophic transition at birth, the occasional development of other tumors of intestinal type in the ureter and renal pelvis, which are not of sewage, and observation of adenocarcinoma arising from glandular cystitis.

What the details of its origin, it is clear that these types of cancer have a clinical manifestation that is very different from typical urothelial cancer. No risk factors have been identified, and in particular, smoking and other environmental factors figure prominently in the typical CTP do not seem relevant. Patients with cancer of the urachus are usually much younger, with a mean age of 47 to 57 years reported at diagnosis, with many cases reported in the third and fourth decades. In addition, these cancers occur equally in men and women (male / female ratio of TCC is approximately 3: 1), and show less susceptibility to cisplatin-based chemotherapy.

Most urachal tumors intestinal histology type display, similar to adenocarcinomas of the colon and rectum. These tumors are usually glandular structures with mucin production, colloid and / or histology of signet ring cells can be present.More rarely sarcomatoid carcinoma, and transitional cell histology have been reported. The epithelium remains normal to the surface or focally ulcerative tumor may overlap. Normal epithelium overlying the tumor strongly supports the diagnosis of urachal carcinoma. However, the destruction of this layer by the tumor can make the distinction between adenocarcinoma of the urachus and urachal bladder difficult. The presence of cystic glandular cystitis or cystitis transition to malignancy favors the diagnosis of adenocarcinoma of the bladder proper, as opposed to that of urachal origin.

To be continue.....

Lymphoepithelioma in rare cancers of bladder

The descriptive term lymphoepithelioma was originally applied to a characteristic tumor occurring in the nasopharynx consists of a poorly differentiated epithelial component showing syncytial features, and a second component consists of a prominent lymphoid stromal infiltration. In the nasopharynx and neck cancer is associated with Epstein-Barr virus, but no such association has been found that this pattern was observed in the bladder. These cancers were also found to be very sensitive to radiation therapy. More recently, morphologically similar cancers have been reported from a large number of sites, including thyroid, skin, cervix, lung and gastrointestinal tract. The first report of this histological variant of bladder came from researchers at Harvard in 1991. Subsequently, the case series MD Anderson Cancer Center, Sweden, Spain and Greece have appeared, but the total number of reported cases is still well below 100.

There seem no particular distinctive features of the clinical presentation of patients with lymphoepithelioma. The clinical importance of recognizing this subgroup is, first, be aware of the possibility of diagnosing this entity as an extranodal lymphoma, and secondly, to recognize the significantly better prognosis than people enjoy this pattern as the predominant ( or exclusive) histology. In several series, including our own experience, these cancers have been observed to be more sensitive to chemotherapy and more radiosensitive than conventional TCC.

Surprisingly however, only patients with "focal" pattern of expression of a lymphoepithelioma have a poor prognosis. This clinical observation was made first in the series of 11 patients reported in MD Anderson's record. At present, we find an additional 18 patients in the register of MD Anderson. This additional experience confirms the aggressive nature of this subset (and the prognosis is excellent for people with lymphoepithelioma predominantly or exclusively).

Carcinoma is Uncommon Cancer Of The Bladder

Predominantly squamous cell carcinoma of the bladder is most commonly found in the environment of schistosomiasis in the Middle East, a context that is outside the scope of this chapter.

In the western, central squamous differentiation are commonly found in patients with invasive, non-papillary TCC. As is the case with focal glandular differentiation, we know you do not have clinical significance of this finding. By contrast, pure squamous cell carcinomas are rare, and show a very particular clinical expression. The most common scenario for (non-schistosomiasis) is that the chronic irritation squamous cell cancer, usually either urolithiasis (particularly staghorn calculi) or chronic catheters in patients with paraplegia and neurogenic bladder diseases such as MS. It is typical to see keratinizing squamous metaplasia, dysplasia often in areas adjacent to these cancers.

Surgery is the mainstay of therapy for squamous cell cancer. Local control is often a bigger problem than the progression from a distance, marked difference to the situation with conventional TCC. The sensitivity to chemotherapy is universally reported to be lower for the conventional squamous cell cancer of the CTP, which further reinforces the importance of primary surgical management. Unfortunately, when these cancers are recurrent or metastatic chemotherapy expectations are limited. However, there are certainly patients have excellent responses, and there is therefore difficult to assess the risk-benefit of a trial of chemotherapy. In our limited experience, we find the combination of gemcitabine (900mgm-2 for 90 minutes), cisplatin (50mgm-2), and ifosfamide (1000mgm-2) given every 14 days to be the most attractive pattern no a clinical trial.

Interestingly, lung metastases of carcinoma of the bladder tend to cavitation, the behavior is not typical of other histologies. Our experience in trying to administer chemotherapy in the special context of patients with paraplegia (or other diseases causing neurogenic bladder) is uniformly unsatisfactory, and we can not support the use of conventional treatments such as MVAC in this context.

Adenocarcinoma rare Cancers Of The Bladder

Glandular metaplasia is common in the urothelium, and the appearance of cystitis cystica and glandular cystitis are known consequences of chronic infection, inflammation or irritation (as urolithiasis). In the transformed state, focal areas of glandular differentiation are reasonably common in invasive non-papillary TCC. To our knowledge, focal areas of glandular differentiation have no clinical significance with regard to natural history or response to commonly used systemic agents. Moreover, it is rare to see a remnant of glandular differentiation in a piece of cystectomy after neoadjuvant chemotherapy for high grade, papillary TCC, even when nothing is seen adenocarcinomatous before therapy. These relatively common clinical scenarios highlight the morphological repertoire urothelial histology, and illustrate the difficulty of defining precisely what is meant by "adenocarcinoma of the bladder." In this section we limit our discussion to cancers that have adenocarcinoma histology as the dominant model, and recognizing the CTP.

There are many variants of adenocarcinoma found in the bladder. Most authors (including the World Health classification of tumors of the bladder) include mucinous ring, seal, type enteric hepatoid, and clear cell (formerly mesonephric) as recognizable subsets. In addition, an adenoid cystic pattern can also be seen, especially in the context of the transformation of a preexisting cystic cystitis. Tumors that mixtures of these patterns are the rule. Enteric type histology is particularly found among the cancers that originate in a urachal remnant, and these are taken separately in the "urachal cancer" below. However, villous adenoma and intestinal-type adenocarcinoma do occur rarely in the bladder properly. The immunophenotype of these cancers tend to overlap with that observed in colon cancer, and most produce carcinoembryonic antigen (CEA).

Most of the adenocarcinomas arising in the bladder are of the variety suitable mucinous or seal ring. The male / female ratio is at least 2: 1, and age of onset is very similar to that observed in conventional CTP. Bladder exstrophy, a rare developmental anomaly affecting 1 in 50 000 births, is a well-established risk factor, like other non-physiological states as a urinary bladder out of place. Intestinal metaplasia long before the appearance of carcinoma is typical in these contexts. Adenocarcinomas also arise in the context of preexisting cystic cystitis (and glandular), sometimes in association with schistosomiasis (although squamous histology is more common in the context of the latter).

It is typical to be diffusely infiltrative mucinous adenocarcinomas, and present with irritative symptoms out of proportion with the results of cystoscopy. Cross-sectional imaging typically shows diffuse thickening of the bladder walls, and plastic linitis Frank is well known, particularly in the subgroup with predominant signet ring histology. We have encountered patients not only with plastic linitis, but also with the involvement of seminal vesicles, and the extension, even to the spermatic cords. Thus, while the appearance may be mediocre cystoscopy, examination under general anesthesia, is striking.

The clear cell variant of adenocarcinoma of the bladder is very rare, and is also clinically distinct. These cancers often occur in women (at least 2: A female predominance) and the median age is younger. More than half of the cases appear to arise from the urethra or periurethral glands. Usually express CA125, and there are other lines of evidence supporting an etiology of M ullerian rest.

These cancers tend to be very sensitive to taxane-based therapy, such as those used for epithelial ovarian cancer. The optimal clinical management of adenocarcinoma is, of course, are not established. The stadium is the most important prognostic factor, and unfortunately the majority of patients with adenocarcinoma presented with locally advanced disease (cT3 or higher).

Therefore, for many patients, neoadjuvant systemic therapy is a reasonable consideration as historical results for surgery alone in this environment is poor. Most patients in our center are, in fact, treated with combination chemotherapy followed by radical surgery. We have seen the answers to a variety of chemotherapy regimens.

In the metastatic setting, we find many examples of patients who had an excellent response to chemotherapy, including the standard therapy for TCC. However, the overall response rate is lower than seen with conventional TCC, and the survival of more than 2 years is uncommon

Micropapillary BLADDER CANCER

The histomorphologic spectrum of many epithelial cancers is now recognized to include a subset of cells characterized by groups of nesting high in lacunar spaces that have a "micropapillary" architecture. This pattern was first reported in 1982 by The Henderickson et al. described an aggressive variant of endometrial adenocarcinoma. In this context, an infiltrating pattern, biologically aggressive expansion behavior remarkably reminiscent of ovarian serous papillary carcinoma was evident. Subsequently, a micropapillary variant has been recognized in cancers arising from breast, bladder, thyroid, lung and pancreas, and has therefore come to be seen as a general feature of epithelial cancer. It seems likely that this phenotype arises from a fundamental aspect of epithelial carcinogenesis, and the finding of a "genetic signature" recognizable that cuts across these different sites would not be surprising. Researchers at the MD Anderson Cancer Center were the first to report a subset of bladder cancers showing a micropapillary histomorphology, the publication of the first series of 18 patients in 1994.

As discussed in the context of sarcomatoid and small cell morphology is typical of a micropapillary component seen in a context that includes more typical of CBT. From this first series of patients, micropapillary variant was observed to have a more aggressive clinical course and a particular tendency for prominent lymphovascular invasion. Subsequently published a series of cases in Sweden, Harvard, Australia, Wayne State University, and the City of Mexico, along with many individual case reports. These reports established that micropapillary bladder cancer has a characteristic morphology that can be identified reliably. Moreover, the trend first reported to the clinical subclassification, aggressive behavior, and the relatively poor response to standard systemic chemotherapy has been fully confirmed. Ultrastructural studies in the context of micropapillary breast cancer suggested secretory granules along the basement membrane, ie, loss of normal cell polarity which is the secretory activity in the basal surface, not only on the surface apical.

This concept of "inside out" morphology has been reinforced by the demonstration that the gene product mucinous glycoprotein MUC1 is abnormally located at the basal surface micropapillary cancers, including bladder origin. Although not yet formally established, it seems likely that a mechanical connection is made between this type of abnormal phenotype, early submucosal infiltration and early access to the lymphatic vessels that characterize the clinical course of these cancers. In our experience, the unusual finding of a bladder cancer is the stage pT1N1 almost always associated with micropapillary histology. micropapillary bladder cancer has been reported to occur infrequently. In the series of cases of Sweden, on the basis of a population-based registry, the incidence of bladder cancer was 0.7%. That report-biased Mexico City put the incidence of 38/630 (6%). In a similar registry biased at the MD Anderson Cancer Center, we found 162 cases for an incidence of 4.2%. Of course, these reports come from genetic and environmental contexts very different, and it is very possible that the incidence of different geographic region. The clinical management of micropapillary bladder cancer should take into account the very real possibility that clinically understaged and can grow quickly. Therefore, we call for "early" cystectomy for any tumor that invades the lamina propria, and certainly calls for any patient with disease cT1 or higher after a trial of intravesical therapy will be guided to cystectomy without delay. For patients with locally advanced disease, the prognosis is worse than for patients with TCC conventional systems. In the MD Anderson Cancer Center experience, patients with cT4a cT3b or treated with a combination of systemic chemotherapy and surgery (in any order), had a lower overall cure rate compared with patients with conventional systems TCC. In the metastatic setting, both the response rate and overall survival of patients with micropapillary cancer are below our historical expectations. Despite the aggressive implementation of combination chemotherapy, patients with micropapillary cancer still have a relatively poor outcome. This has been methotrexate / vinblastine / doxorubicin / cisplatin (MVAC), with ifosfamide-based combinations, and gemcitabine / cisplatin. It is important to recognize, however, that
although the overall results are lower than those obtained for patients with conventional TCC, many patients do well, and therefore continue to offer conventional therapy for patients in this subgroup