Abstract
Urachal cancer (UrC) is a rare but aggressive cancer. Due to overlapping histomorphology, discrimination of urachal from primary bladder adenocarcinomas (PBAC) and adenocarcinomas secondarily involving the bladder (particularly colorectal adenocarcinomas, CRC) can be challenging. Therefore, we aimed to give an overview of helpful (immunohistochemical) biomarkers and clinicopathological factors in addition to survival analyses and included institutional data from 12 urachal adenocarcinomas. A PubMed search yielded 319 suitable studies since 1930 in the English literature with 1984 cases of UrC including 1834 adenocarcinomas (92%) and 150 nonadenocarcinomas (8%). UrC was more common in men (63%), showed a median age at diagnosis of 50.8 years and a median tumor size of 6.0 cm. No associations were noted for overall survival and progression-free survival (PFS) and clinicopathological factors beside a favorable PFS in male patients (p = 0.047). The immunohistochemical markers found to be potentially helpful in the differential diagnostic situation are AMACR and CK34βE12 (UrC versus CRC and PBAC), CK7, β-Catenin and CD15 (UrC and PBAC versus CRC), and CEA and GATA3 (UrC and CRC versus PBAC). Serum markers like CEA, CA19-9 and CA125 might additionally be useful in the follow-up and monitoring of UrC.
Highlights
The urachus is a remnant of the fetal structure connecting the allantois and the fetal bladder
We identified a total of 1984 cases of Urachal cancer (UrC) from 319 suitable studies with sufficient data from the English literature with overall 1834 adenocarcinomas (92%)
While only minor variations in clinicopathological factors such as gender distribution, age at diagnosis, tumor size, and adenocarcinoma subtypes were noted, none of these factors were associated with overall survival
Summary
The urachus is a remnant of the fetal structure connecting the allantois and the fetal bladder. The urachus usually regresses to form an obliterated fibromuscular canal, known as the median umbilical ligament [1–4]. Failure of complete luminal obliteration has been described in up to one-third of adults and can rarely lead to various anomalies including cysts, fistulas, and diverticula or rarely malignant transformation [2, 5]. UrC is a very rare but highly malignant tumor entity with an incidence of
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
