Wang et al. BMC Pediatrics(2024) 24:554
https://doi.org/10.1186/s12887-024-05021-5

BMC Pediatrics

CASE REPORTOpen AccessUrachal adenocarcinoma in an adolescentboy: a case report
Chuan Wang1†, Ting An1† and Xue Ma1*
Abstract
Background Urachal carcinoma is an extremely rare malignant tumor originating from the urachus. Urachal
adenocarcinoma has never been reported in patients under 20 years of age. In this case, we describe a 15-year-old
patient with urachal adenocarcinoma and propose possible risk factors.
Case presentation The patient presented with hematuria for two months and dysuria for one month, and had
a history of smoking and alcohol consumption for three years. Ultrasonography showed an irregular mass on
the anterior wall of the bladder. Contrast-enhanced computed tomography revealed a pedicled soft tissue massmeasuring 2.6×2.4 cm within the bladder, showing significant enhancement. Partial cystectomy was conducted, anda histopathological diagnosis of urachal adenocarcinoma (T2N0M0) was made. During eight months of follow-up, thepatient remained asymptomatic with no evidence of recurrence.
Conclusions Urachal remnants may lead to urinary symptoms and the development of urachal carcinoma. A historyof smoking and alcohol consumption could be possible risk factors for urachal adenocarcinoma in this case. It is
possible that urachal remnants can undergo malignant transformation, even at ages as young as 15 years. Regularfollow-up should be recommended for patients whose urachal remnants persist beyond childhood.
Keywords Urachal carcinoma, Adenocarcinoma, Urachus, Adolescent

BackgroundThe urachus is a three-layered tubular structure as aremnant of an embryonic structure. Urachal remnants
result from failure of allantois lumen obliteration, andmay lead to urinary symptoms and the development ofurachal carcinoma at an older age. This anomaly is relatively uncommon, with a prevalence of 1% in children[1]. Urachal carcinoma is an extremely rare malignant
tumor originating from the urachus, with an incidence†Chuan Wang and Ting An contributed equally to this work.

*Correspondence:
Xue Maimamaxue@163.com
1
Department of Pediatric Surgery, West China Hospital, Sichuan
University, #37 Guo-Xue-Xiang, Chengdu 610041, Chinaof 0.3% among all bladder cancers [2]. The majority ofcases are diagnosed in individuals in their 50s and 60s,
with a median age at diagnosis of 52 years, according to a
study reviewing 1,010 cases [3]. Urachal adenocarcinoma
has never been reported in patients under 20 years ofage. Here, we describe a case of 15-year-old with urachal
adenocarcinoma.

Case presentation
A 15-year-old Chinese boy, previously healthy with anunremarkable past medical and family history, complained of hematuria for two months and dysuria for onemonth. The boy had a history of smoking and alcohol consumption for three years, smoking approximately 20 cigarettes per day and drinking alcohol approximately oncea week. The physical examination was unremarkable.

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Wang et al. BMC Pediatrics(2024) 24:554

Ultrasonography showed a hypoechoic, obscurelydemarcated, irregular mass on the anterior wall of thebladder (Fig. 1A). Doppler assessment detected vascular flow within the mass. Contrast-enhanced computed
tomography revealed a pedicled soft tissue mass measuring 2.6×2.4 cm within the bladder, showing significant
enhancement (Fig. 1B). A high-density nodular image
was observed in front of the bladder (Fig. 1C).
A bladder tumor was suspected, and open partial cystectomy was conducted. The entire mass, along with partof the bladder wall and the distal segment of the urachus, was resected. The tumor involved the submucosal
and muscular layers, but not the peritoneum. The surgical margin was negative. A histopathological diagnosis
of urachal adenocarcinoma was made. The high-density
nodule in front of the bladder was confirmed to be astone in the urachus. Mucinous adenocarcinoma cells
were identified via hematoxylin and eosin staining, andsome cells were signet-ring cells (Fig. 1D). Immunohistochemical analysis showed positive staining for CK (pan),
CK20, CDX2 and β-catenin (Fig. 1E, F, G and H). The
patient was diagnosed with T2N0M0 urachal adenocarcinoma. An indwelling catheter was placed in the bladderfor 5 days after the operation. He recovered uneventfully
and was discharged six days after the operation. A CT
scan performed six months after discharge showed noevidence of recurrence. During eight months of followup after discharge, the patient remained asymptomatic.

Discussion
Urachal adenocarcinoma was previously thought tooccur in adults, whereas this case was an adolescentboy with a history of smoking and alcohol consumption. The risk factors for urachal adenocarcinoma remain
unclear. In this case, it seems that a history of smoking
and alcohol consumption may be a risk factor for urachal

Page 2 of 3

adenocarcinoma, which could help explain why urachal
adenocarcinoma is more common in men. Additionally, astone found in the urachus could cause urinary stasis andchronic irritation, which may also contribute to tumordevelopment. The most frequent clinical presentations ofurachal adenocarcinoma in adults are hematuria, abdominal pain and dysuria, which are similar to the symptoms
observed in this adolescent.
Approximately half of urachal carcinomas exhibit elevated levels of serum markers including CA125, CA19–9,
CEA [4, 5]. These makers serve as diagnostic indicatorsand predictors of therapeutic response and recurrence[4, 6]. We did not examine any of these serum markers
before surgery because we were unaware of the possibility of urachal carcinoma. Ultrasonography is widely used
to diagnose urinary system diseases and can often identify an irregular mass located at the dome or midline of
the anterior wall of the bladder in patients with urachal
carcinomas, although it lacks specificity. Mucin-producing urachal adenocarcinoma frequently contains calcification at the dome or midline of the anterior wall of thebladder, which is thought to be characteristic [7]. Calcification are present in about 32% of urachal carcinomas
and can be easily detected via computed tomography [3].
Cystoscopy is the most sensitive diagnostic method andshould be mandatory when urachal carcinoma is suspected [8].
For localized urachal carcinoma, the recommended
treatment is excision of the urachus, umbilicus and partial/radical cystectomy, along with removal of the bilateral pelvic lymph nodes [9]. In this case, we conducted apartial cystectomy without resecting the entire urachus,
the umbilicus or the bilateral pelvic lymph node, as we
did not initially consider the possibility of urachal adenocarcinoma and the CT scan did not reveal any enlarged
pelvic lymph nodes. Partial cystectomy is preferred overFig. 1 (A) Ultrasonography showed a hypoechoic, obscurely-demarcated, irregular mass with vascular flow on the anterior wall of the bladder. (B)Contrast-enhanced computed tomography revealed a pedicled soft tissue mass measuring 2.6×2.4 cm within the bladder, showing significant enhancement. (C) computed tomography showed a high-density nodular image in front of the bladder. (D) Histological image of hematoxylin and eosin staining.
(E) Positive staining for CK (pan). (F) Positive staining for CK20. (G) Positive staining for CDX2. (H) Positive staining for β-cateninWang et al. BMC Pediatrics(2024) 24:554

radical cystectomy because the survival rate in cases
undergoing partial cystectomy is similar to that in cases
undergoing radical cystectomy, and patients undergoingpartial cystectomy have a better quality of life. More than
20% of patients develop distant metastases at diagnosis[3]. For these patients, chemotherapy is the only treatment choice, and the 5-year survival rate is lower than20%.3 A cohort study indicates that distant metastasis is
more common in patients under 45 years old [10].
In conclusion, urachal remnants may undergo malignant transformation even at ages as young as 15 years.
Regular follow-up should be suggested for patients withurachal remnants persisting beyond childhood.
Acknowledgements
Not applicable.
Author contributions
C.W., T.A. and X.M. collected the data. C.W. and T.A. drafted the manuscript.
X.M. reviewed the manuscript.
Funding
Not applicable.
Data availability
The datasets used during the current study are available from thecorresponding author on reasonable request.

Declarations
Ethics approval and consent to participate
This study was approved by the Ethics Committee of the West China Hospital(2019(1085)) and informed consent was obtained from the patient’s parents.
Abbreviations
Not applicable.
Consent for publication
Written informed consent was obtained from the parents for the publicationof this case report and accompanying images.

Page 3 of 3

Competing interests
The authors declare no competing interests.
Received: 2 July 2024 / Accepted: 19 August 2024

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