Saturday, May 7, 2011

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.