Abstract
BackgroundUrachal carcinoma is a rare tumor that is usually associated with a poor prognosis, especially the pathological type, urachal mucinous adenocarcinoma. Surgery remains the primary treatment in prolonging the overall survival time of patients.Case presentationWe report on a 41-year-old woman with urachal mucinous adenocarcinoma who underwent three surgeries and several courses of chemotherapy over a 42-month period. The first surgery, involving en-bloc excision of the urachal mass, partial urinary bladder, urachal ligament, and umbilicus was performed in May 2007. It is well known that the correct surgical scheme plays a key role in preventing recurrence or metastasis. However, a second debulking surgery with only a single salpingo-oophorectomy may have contributed directly to the patient’s subsequent left ovarian metastasis. Therefore, we strongly recommend performing a bilateral salpingo-oophorectomy once ovarian metastasis has been detected, even if the metastasis is only present on one side. Although postoperative adjuvant chemotherapy regimens, first with Taxol, carboplatin, gemcitabine, and cisplatin, and then with IFO, EPI, and mesna were consecutively administered after the first and second surgeries, they seemed less effective, since recurrence and metastasis occurred shortly after each surgical treatment. After a third debulking surgery in June 2009, docetaxel, oxaliplatin, and capecitabine were administered. This chemotherapy regimen was chosen based on an immunohistochemical test that involved the multidrug resistance gene; this test indicated that the urachal mucinous adenocarcinoma was resistant to the two chemotherapy regimens used previously. Surprisingly, the patient exhibited a marker response to the new regimen and the metastatic foci entered into a stable disease stage. However, the patient still died of diffuse metastatic disease 1.5 years later. During the whole period of treatment, we found that serum tumor markers including CA724, CA125, CA19-9, and CEA were elevated in a linear pattern, with parallel increases in line with peritoneal carcinomatosis and parallel reductions in line with response to personalized chemotherapy.ConclusionPersonalized treatment can be given to those patients who experience a poor response to initial therapy. Moreover, an immunohistochemical test for the multidrug resistance gene and serum tumor markers may supply key information in the choice of reasonable chemotherapeutics.
Highlights
Urachal carcinoma is a rare tumor that is usually associated with a poor prognosis, especially the pathological type, urachal mucinous adenocarcinoma
Urachal carcinoma is a rare form of tumor that usually originates in the bladder, and represents 0.01% of all cancers
Urachal adenocarcinoma has been regarded as associated with poor prognosis, especially in the case of urachal mucinous adenocarcinoma [4,5]
Summary
It is sometimes difficult to differentiate between urachal and non-urachal carcinomas based only on symptoms. The criteria for making a diagnosis of urachal carcinoma are not uniform, but clinical findings and histological evidence are both key factors. Tumors have been staged using two different staging systems: the Sheldon staging system and the Mayo staging system Both systems have predicted cancer-specific survival well, but we recommend the use of the Mayo staging system in future studies owing to its simplicity (Table 1). Among the eight reported cases [15,16,17,18,19,20,21,22], three were identified simultaneously with the primary urachal carcinoma and one was detected as a primary tumor before urachal adenocarcinoma was confirmed.
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