Genitourinary cancer Urachal cancer part 3

Proper management of urachal carcinoma surgical requires that the diagnosis was made preoperatively on the basis of recognition of this possibility in the appropriate clinical setting. Before read this part, please read Genitourinary cancer Urachal cancer part 2 . Cross-sectional images are the key to recognize the diagnosis. On CT, urachal carcinoma usually appears as a mass of low attenuation in the dome of the bladder, usually in the midline or slightly to one side. Due to the relatively high rate of recurrence after treatment of this disease, the resection of the umbilicus, urachus, covering the peritoneum and posterior rectus fascia lateral to the medial umbilical ligament, bladder, and lymph nodes of the pelvis is now normal. The recognition that urachal tumors are predominantly extravesical and is not associated with a defect in the camp suggested to surgeons that in most cases, even bulky tumors could be completely resected with adequate margins and en bloc dissection only a partial cystectomy. Contemporary series report that neither local recurrence nor result threatened by this approach. On the contrary, survival is rather linked to the stage of presentation, presence of metastases in lymph nodes, and the ability to achieve a negative surgical margin with the completion of a partial or total cystectomy. Radical cystectomy is indicated for salvage surgery to treat a positive surgical margin, or delete a ligament from inadequate control of urachus - which occurs when the diagnosis was made before the operation. In a series of patients referred to MD Anderson Cancer Center is remarkable that only 19 of 35 patients undergoing primary surgical treatment was resection of the urachal ligament and umbilicus. The importance of adequate surgical treatment was reinforced by the finding that 13 of the 16 long-term survivors referred to in this series were treated with en bloc resection including umbilectomy.

Unfortunately, patients with lymph node or peritoneal discovered in surgery have a median survival of about 25 months, and demonstrate a clinical course that is virtually indistinguishable from that of patients with clinically evident metastases at diagnosis. In view of this finding, and demonstrated benefit of perioperative chemotherapy for colorectal cancer, the use of adjuvant or neoadjuvant chemotherapy for urachal cancer appears to be a reasonable consideration. Unfortunately, there are essentially not affect the data on this point directly, so we were left to extrapolate from our experience with other intestinal adenocarcinomas. Since we have some systemic therapies with clinically relevant response rates (see below), it seems appropriate to discuss adjuvant therapy in patients at high risk of recurrence, even those with tumors in the lymph nodes or peritoneal surface, or in the environment of inadequate surgery, including the presence of positive margins and in the scenario where the urachal ligament was not controlled.

As expected, few long-term survivors were observed after developing metastases. The sites most frequently involved are the bones, lungs, liver, lymph nodes and brain. Peritoneal carcinomatosis is common, especially in the context of positive surgical margins and when the peritoneal implants are present in the cystectomy.

Historically, chemotherapy has had little impact on the treatment of urachal cancer. This is particularly true in the context of traditionally used chemotherapy regimens for TCC. More recently, the responses have been reported in the establishment of schemes of 5-fluorouracil-based chemotherapy. Today, they are enrolling patients in a Phase II trial of combination chemotherapy with 5-fluorouracil, leucovorin, gemcitabine and cisplatin. The results in the first 20 patients showed an objective response in just over a third of patients. According to the clinical manifestations of this disease is so closely related to colorectal cancer, anecdotal responses have been observed in patients treated with capecitabine and irinotecan-based regimens, and the antibody cetuximab antiepidermal growth factor.

Genitourinary cancer Urachal cancer part 2

Before read this part, please read Genitourinary cancer Urachal cancer part 1. Most patients have locally advanced disease at diagnosis, usually presenting with gross hematuria and irritative voiding symptoms, but often occurs without urinary complaints at all. Patients may report voiding mucoid material, a feature consistent with typical histology. Umbilical erythema and discharge have also been reported, and we have seen patients with initial diagnosis of an "umbilical infection." The presence of a midline cystic mass with calcifications in the dome of the bladder in the radiographic image is almost pathognomonic. In practice, all patients with intestinal-type adenocarcinoma involvement of the dome of the bladder should be considered an urachal cancer until proven otherwise. However, it is important to recognize that these tumors can occur along the urachal ligament, and may produce a palpable mass anywhere between the navel and the symphysis. Although the involvement of the bladder often occurs, is not a requirement for diagnosis.

Most patients present with locally advanced disease with a tumor that invades the bladder wall. The diagnosis is typically made by cystoscopy and biopsy. Besides the location in the dome of the bladder and unusual histology, an important key to the recognition of a urachal origin is the typical finding of tumor in the muscularis propria with smooth urothelium covering cancer. By contrast, adenocarcinomas arising from the urothelium to grow from the "inside out", and are frequently associated with urothelial dysplasia or focal areas of recognizable transitional cell cancer. The only other important differential consideration in the differential diagnosis is "drop metastases" of an ovary or upper gastrointestinal (or pancreas) primary tumor, although these tend to involve the dead end and not the dome of the bladder. The invasion of a primary urachal in the large intestine or bowel is quite common, and has seen several cases of colon cancer multifocal "or" metastatic bladder cancer of the colon, which turned out to be eroding urachal cancer in the intestine one or more places.

As with colon cancer, evaluation of tumor markers may be useful, especially in the context of assessing treatment response. We found CEA, CA125 and CA19-9 to be useful in some patients. As with other tumor types, the elevation of CA125 should suspect the presence of peritoneal carcinomatosis, which is very common in patients with urachal cancer.

Continue Genitourinary cancer Urachal cancer part 3

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.....