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RARE ADENOCARCINOMA OF URACHUS MANIFESTING AS A RETROPERITONEAL TUMOR: A CASE REPORT
Apresentação do caso
Male, 69 years old, referred to the oncology service presenting a retroperitoneal tumor (TPR) with dysuria and pain in the hypogastric region with 1 month of evolution, associated with urinary obstruction. Upon examination, a palpable mass in the left iliac region of cystic consistency, measuring 15 cm. CT and MRI were requested, showing a large, heterogeneous pelvic tumor, with extensive cystic areas, intermingled solid component, measuring 22x14x11cm, compressing bladder and ureters, severe bilateral ureterohydronephrosis, absence of lymph node enlargement. Underwent surgery showing a large mass in the pelvic region and TPR infiltrating the rectum and bladder, bilateral ureterohydronephrosis, sigmoid obstruction, absence of ascites, and peritoneal carcinomatosis. Performed total pelvic exenteration in oncology with resection of TPR, bladder, rectosigmoid, seminal vesicles, appendectomy, and pelvic lymphadenectomy and TPR, performed with a wet colostomy. Pathology (AP) revealed mucinous adenocarcinoma (AC) and suggested a lesion of urachal origin. Immunohistochemistry(IHC) showed positivity for cytokeratin 7 and 19, 34βE12 and GATA-3, favoring the diagnosis of uracal CA. Decided surgery complementation through resection of the abdominal wall segment with umbilicus excision. AP did not show neoplasia. Evaluated by clinical oncology, chemotherapy not indicated. The patient evolved without complaints for seven months, dying due to complications from COVID-19.
Urachus is an embryonic structure that connects the fetal bladder with the allantois. Physiologically, there is a channel obliteration that transforms into the median umbilical ligament, in Retzius space. However, in a small portion of the population, it persists. Urachal AC is a rare and aggressive malignancy, occurring in <1% of bladder cancers. The clinic is insidious with compressive urinary and abdominal symptoms, favoring a late and advanced presentation of the disease. The mucinous AC subtype is the most common. Thus, IHC plays a fundamental role in differentiating primary mucinous AC from other sites. Treatment is resection of the urachus, navel, bladder, and pelvic lymphadenectomy. The role of adjuvant is controversial.
As it is rare and with a poorly remembered structure, CA of urachus has a challenging diagnosis. The oncologic surgeon must have pathology knowledge to suspect pelvic and retroperitoneal masses and accomplish the appropriate treatment to provide better results and longer survival.
Urachus; Surgical Oncology; Urologic Surgical Procedures
JOSE AMERICO DOS SANTOS COSTA NETO, ROSANE NAYARA DE MEDEIROS ALVES FERNANDES, LAURA CRISTINA COSTA SILVA, ANDRE LUIZ COSTA SILVA, ANA LUISA DA SILVA MAIA, MARINA ÁLVARES PRUDÊNCIO, INGRID PINHEIRO MOLINA, EDUARDA MORGANA ANDRADE DE ALMEIDA, LUCIANO LUIZ SILVA JUNIOR, ISAAC BRAULLIO MAIA DELFINO OLIVEIRA