Case Report
Neuroendocrine Carcinoma Associated to Mucinous Urachal Adenocarcinoma - Case Report
Rodrigo Beserra Sousa*, Marcos Francisco Dall’Oglio, Alexandre Stievano Carlos, Sander
Tessaro Rocha, Cassius Martins e Silva and Luiz Jorge Budib
Department of Urology, Santa Marcelina Hospital, Brazil
*Corresponding author: Rodrigo Beserra Sousa, Department of Urology, Santa Marcelina Hospital, Rua Santa Marcelina, 177, Itaquera, São Paulo/SP, Brasil, 2º Andar (Chefia da Urologia), CEP 08270-070, Brazil
Published: 16 Mar, 2017
Cite this article as: Sousa RB, Dall’Oglio MF, Carlos AS,
Rocha ST, Martins e Silva C, Budib LJ.
Neuroendocrine Carcinoma Associated
to Mucinous Urachal Adenocarcinoma -
Case Report. Ann Clin Case Rep. 2017;
2: 1303.
Abstract
Malignant urachal transformation is extremely rare, representing less than 1% of bladder tumors. Adenocarcinomas are the most frequent subtype. Mucinous urachal tumors are far less common. The most usual symptom of urachal tumors is hematuria due to erosion of the vesical dome. Standard treatment is surgery. This is a case report about a 63-year-old patient diagnosed with a urachal tumor and the conduct after diagnosis with follow-up until the patient’s death.
Keywords: Malignantneoplasia; Urachus; Surgical treatment
Introduction
The urachus is a fibrous remnant ofthe urogenital sinus and the allantois, which connects the
vesical dome to the umbilicus. It is found in the middle line between the posterior rectus sheath,
anteriorly, and the peritoneum posteriorly. Clinically, it remains undetectable, except if it becomes
symptomatic or is identified through imaging [1-4].
Involution of the urachus occurs in the antenatal period, between the 6th and 12th week.
However, complete obliteration may not occur, forming cysts in the urachus– more frequent - or
patenturachus. Remaining tracescan also suffer metaplasia and the formation of tumors [1,5].
Malignant urachal transformation is quite rare, representing less than 1% of bladder tumors,
and is more commonly found in males. Adenocarcinomas are the most frequentamong the
subtypes. Urachal mucinous tumors are much less common and can be the cause of peritoneal
pseudomyxomas [1,3,6]. The most common symptom of urachal tumors is hematuria due to the
erosion of the vesical dome. Standard treatment is surgery, preferably partial cystectomy, associated
to pelvic bilateral lymphadenectomy [3,7].
The present study aims at reporting the case of a patient with a urachal lesion, from diagnosis to
surgical treatment and conduct after the anatomopathological findings.
Case Presentation
A male patient, 63 years of age, smoked, began with about 4 months of urinary storage symptoms,
associated to macroscopic hematuria, when he sought medical attention. The kidney and urinary
tract ultrasound showed: focal parietal thickening of the bladder, parietal nodular formation on the
right lateral wall measuring 1.6 x 0.8 cm, formation of a cystic aspect and thickened walls adjacent to
the vesical dome extending towards the umbilicus, measuring around 10 x 5.5 x 3.8 cm. Due to these
findings he was submitted to a contrast-enhanced abdominal and pelvic CT-scan, which showed an
elongated cystic image with parietal calcifications based on the vesicle dome (Figure 1).
A bulge was observed in the vesical dome during the cystoscopy that appeared to be extrinsic
compression without signs of lesions in the vesical mucosa. A resection of this area was carried
out to obtain a sample for an anatomopathological study and results were conclusive only after the
immunohistochemical study: malignant neoplasia of small cells disposed in blocks extending below
the typically scanty urothelium.
A block resection of the urachal tumor was performedwith partial cystectomy and a bilateral pelvic
lymphadenectomy, limited by the bifurcation of the iliac vessels (Figure 2-5). There was satisfactory
and uneventful progress in the postoperative phase. The anatomopathological exam showed a solid
undifferentiated predominant carcinoma (90%) with an associated mucinous adenocarcinoma
(10%), involving the perivesical adipose tissue, perineural invasion with margins affected in extent
and depth; 10 lymphnodes free of lesions; staging pT3N0. The immune-histochemical study revealed a neuroendocrine invasive bladder carcinoma (negative CDX 2 and
p63; positive CK7, CK20, cromogranine, Ki67) with a high rate of
cellular proliferation. The systemic staging did not exhibit lesions,
and adjuvant radiotherapy and chemotherapy (with cisplatin and
etoposide) was indicated.
The patient had an irregular follow-up, only finishing radiotherapy
and not the full chemotherapy regimen. Around 18 months after the
surgical procedure, he was admitted to the emergency room with
an acute intestinal obstruction. An exploratory laparotomy was
performed and the findings suggested a peritoneal carcinomatosis,
confirmed anatomopathologically. A loop ileostomy was performed
with palliative care procedures defined thereafter. The patient
subsequently died about one month after the second approach.
Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Discussion
Malignant urachal neoplasias are rare, with very few cases
reported, involving mainly men between 50 and 70 years of age
[3,4]. In most cases – up to80% of the time the lesion is found in the
junction with the vesical dome [3,4,7]. The most common symptom is
hematuria (90% of the cases), followed by suprapubic pain, irritative
symptoms and mucosuria [4,8].
Initial imaging is usually ultrasound, which can suggest the
diagnosis with a complex lesion found in the middle line in contact
with the bladder. However, CT-scans, and mainly, MRIs, provide
better information [1,4]. Cystoscopy is usually indicated to evaluate
the bladder, where lesions or bulging of the dome are commonly found
and a resection is performed to obtain material for a pathological
study and more accurate diagnosis [4,7].
The most frequent histological type is the adenocarcinoma,
mostlymucinous, but transitional cell as well as squamous cell
neoplasias and sarcomas can also be found [3,4]. There is an important
structural similarity between urachal and intestinal adenocarcinomas,
including the production of the carcinoembryonic antigen, and the
origin of the lesion is frequently defined (primary or secondary) after
an immunohistochemical study [3,4,8].
The gold standard treatment for malignant urachal tumors,
whenever possible, is partial surgical block resection of the bladder with the lesion up to the navel associated to bilateral
pelvic lymphadenectomy [3,4,7,8]. The open approach is more
frequent, although the procedure can be successfully performed
laparoscopically or also robotically, in spite of less long-term study
outcomes [8-10]. The prognosis for patients with adenocarcinomas
depends directly on resections with free margins and absence of
positive lymph nodes [4,8,11].
Currently, no standard protocols are available for adjuvant
treatment of locally advanced or metastatic urachal tumors. In
general, supplementary treatment considers the experience of the
assisting medical team that many times uses its knowledge of other
types of tumors with similar behavior-especially those in intestinal
neoplasia [4,8].
Neuroendocrine tumor of the bladder is an extremely rare
neoplasm and does not yet have standard treatment, being
extrapolated treatments of neoplasms with similar histopathological
findings for its treatment [12]. The neuroendocrine tumor of the
urachus is even rarer and, like tumors of similar histology of bladder
origin, its treatment is done with extrapolation of tumors from other
sites [13].
Chemotherapy similar to that indicated for gastrointestinal tumors
is frequently used-based on cisplatin and the FOLFOX regimen [14-
16]. In cases like ours, with a predominantly neuroendocrine variant,
some defend the use of chemotherapeutic agents like those used in
small cell lung tumors [14].
An adjuvant regimen resembling what was done with patients
who had endocrine intestinal neoplasia as per the service protocol
was used in the case reported.
A urachal neuroendocrine tumor is an extremely aggressive
variant and progression of the disease occurs even with adjuvant
therapy. Ebara et al recently showed the use of gemcitabine, cisplatin
and paclitaxel afterprogression of local and systemic disease following
radical cystectomy and obtained a good response [14]. Ismaili
observed that the best kind of therapy is multimodal, beginning
with a resection. He also showed that even the use of combination
therapies and a close follow-up the prognosis is still poor [17].
Conclusion
We report a rare case of neuroendocrine tumor of the urachus and how it was conducted to try to some add informationto the poor literature in these cases. This rare neoplasm does not have adjuvant therapy protocols and should be individualized according to each patient and experience of the assistant team. In addition, we suggest that in similar cases the patient be strictly monitored due to aggressive behavior of this tumor.
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