Urachal Carcinoma, An Unusual Possibility of Hematuria; Case Report and Literature Review

Abstract

Urachal cancer is very rare, accounting for only 0.5–2% of bladder-associated malignancies and 0.01% of all cancers in adults. It has an insidious appearance, an aggressive behavior and a poor prognosis. The most common symptoms are hematuria and the presence of a palpable hypogastric mass. The scarcity of cases and the low number of studies carried out explains the lack of an evidence-based management strategy, but it seems that surgical treatment (open, laparoscopy or robot-assisted) represents the gold standard, while neoadjuvant and adjuvant chemotherapy or radiotherapy has a limited impact on overall survival. Since mucinous cystadenocarcinoma of urachal origin is a very uncommon pathological condition the differential diagnosis may be difficult and pathological investigations have to elucidate this disorder. It is worth mentioning the psychological impact on the patient in addition to the medical aspects. A rare condition is associated with heightened risk for mental health and psychosocial difficulties and this must be taken into account in the subsequent follow-up of the patient. In order to increase awareness of this rare entity we report a case of a 40-year-old male with a urachal adenocarcinoma who was treated surgically, with a favorable outcome. We also perform a brief literature review about this type of tumor.

Case Report: Intravesical and extravesical urachal cyst in children with lower abdominal pain and hematuria

“…Generally, most urachal cysts are not connected to the bladder or umbilicus; they are exogenous to the bladder. However, urachal cysts inside the bladder protrude into the bladder, making this type of cyst very rare (1,6). In 2013, Metwalli et al ( 6) first proposed the concept of "intravesical urachal cyst" and described its color ultrasound manifestations: An intravesical urachal cyst presents as a thin-walled oval cystic structure protruding into the lumen above the anterior midline of the bladder wall.…”

“…The urachus, also known as the median umbilical ligament, is a tubular structure that connects the umbilical cord to the front wall of the bladder (1). During the fourth to fifth month of pregnancy, the bladder gradually descends into the pelvis, and the urachal lumen closes and disappears, thus forming a permanent fibromuscular cord (2).…”

Acute Glomerulonephritis as a Paraneoplastic Syndrome Secondary to Urachal Adenocarcinoma: An Unknown Entity

“…In the same way as the peculiarity of the characterization of urachal adenocarcinomas, the clinical presentation constitutes a diagnostic challenge at the beginning, due to both the anatomical location of this structure, as well as the wide variability in the manifestation of symptoms (hematuria, dysuria, mucosuria, abdominal pain, among others like pollakiuria, urinary tract infection, umbilical discharge, and nausea) and the temporality of the same. As an effect of this, the establishment of a definitive diagnosis and therefore the start of treatment is delayed in most cases, representing a worse prognosis [ 11 ].…”

Ganglioneuroma of the Bladder in Association with Neurofibromatosis Type 1

“…In radiological investigations, bladder involvement is seen as a mass invading the bladder wall or as a better-defined tumor. The CT findings of bladder neurofibroma, ganglioneuromas, or other tumors can be similar and include a lobulated round shape mass with low density and uniform appearance, without increases in density with enhanced CT scan [ 1 , 9 ]. The presented patient showed a large asymmetric and heterogeneous infiltrative tumor in abdominal ultrasound and Uro-CT scans, and even with the help of cystoscopy, the diagnosis of certainty could not be established.…”

“…Adolescents with unusual diseases may face challenges different from those with frequent medical disorders, especially if they come from disadvantaged social backgrounds or disorganized families [ 9 , 21 ]. The clinical approach of the medical multidisciplinary team involved should help the patient in managing her medical, surgical, and social situations [ 9 , 22 ].…”