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Mucinous cystic tumor of low malignant potential (MCTLMP) is a very rare disease.The patient was a 50-year-old man. Contrast-enhanced computed tomography and magnetic resonance imaging showed a multiocular cystic lesion on the top of the bladder. We performed surgery to remove the tumor for definitive diagnosis. Intraoperative rapid pathological diagnosis of the cyst wall showed no malignant findings, so we performed combined resection of the peritoneum and part of the bladder wall.Based on imaging findings, tumor location, and pathological and immunostaining findings (CK20 and CDX2 positivity; β-catenin negativity), the patient was diagnosed with MCTLMP thought to be of urachal origin. After the operation, recurrence was not observed.

The laparoscopic management of urachal remnants has gradually become a common practice. Recently, laparoscopic single-site surgery (LESS), a minimally invasive approach that provides excellent cosmetic results, has been adopted in several surgical procedures for treating urachal remnants. However, when suturing the bladder wall or peritoneal defect during LESS it may be difficult to conduct the procedure manually, and such cases require an additional port for suturing. Our strategy, however, employs a knot pusher to perform the suturing without the need for an additional port. We compared and examined the perioperative parameters of the patients with the additional port and one without it (knot-pusher group). For the additional-port and knot-pusher groups, the average operative time, was 146.8 and 161.7 minutes respectively, pneumoperitoneal surgery time was 90.8 and 88.0 minutes, respectively, suturing time for the bladder wall was 577 and 502 seconds, respectively suturing time for peritoneal defect was 758 vs 779 seconds, respectively, and estimated blood loss was 19 and 9.6 ml, respectively ; there being no significant difference between the two groups. We report our knot-pusher method because it can achieve comparable results without compromising the surgical outcome.

(Objective) Although laparoscopic excision of urachal remnants has been applied widely, the standard surgical method has yet to be established. The aim of this study, therefore, was to evaluate perioperative outcome and surgical techniques, including the trocar placement at our institution. (Subjects and methods) A total of 20 patients with urachal remnant were treated using laparoscopic surgery between October 2002 and August 2016. 3 trocars were inserted in the peritoneal cavity. Camera trocar was placed at right side of umbilicus and two working trocars were placed at the upper side of umbilicus and right lower quandrant. A 5 mm, 30° angled lens camera was used during operation. (Results) The mean age was 27 years old. There were 16 males and 4 females. The classifications of urachal remnants were urachal sinus (n=18) and urachal diverticulum (n=2). Umbilical excision was performed in 18 case and partial cystectomy were required in 4 cases. 4 trocars were inserted in only one case due to severe adhesion in the peritoneal cavity. Laparoscopic excision was successfully completed in all cases. The mean operative time was 220 minutes and the mean blood loss was minimal. The complications occurred in 5 cases including small intestinal injury (n=2), peritonitis (n=1), wound infection (n=1), intestinal obstruction (n=1) and acute renal failure (n=1) (intestinal obstruction and acute renal failure were occurred in the same case). (Conclusion) Although there are problems such as shortening the operative time and reducing complications, our unique port placement enables good surgical view and easy suturing during partial cystectomy.

A 64-year-old woman presented to our hospital with the chief complaints of abdominal pain and appetite loss, and she was admitted to the internal medicine department. Kidney, ureter and bladder X-ray revealed intrapelvic calcification near the bladder, and so, the patient consulted our department. Computed tomography and magnetic resonance imaging revealed an urachal abscess accompanied by a stone. Open surgery was performed under general anesthesia. The mass adhered tightly to the intestine and bladder. The urachal abscess ruptured during the operation, and pus leaked into the intraabdominal cavity. Partial cystectomy was performed to remove the mass completely. The stone existed in the urachal abscess, and its constituents were CaOxa (51%) and CaP (49%). The pathological diagnosis was urachal abscess without malignancy.

A 39-year-old man visited our clinic with gross hematuria. Cystoscopy revealed a papillary tumor at the urinary bladder dome. Abdominal magnetic resonance imaging (MRI) and computed tomography(CT) demonstrated a tumor extending from the umbilicus to the bladder dome. Transurethral resection of bladder tumor (TUR-Bt) was performed and histopathological findings revealed adenocarcinoma. Chest CT and examination of the upper gastrointestinal did not reveal any abnormal findings. The tumor was diagnosed as stage IIIA urachal carcinoma, and en bloc segmental resection was performed. About 10 months later, chest CT demonstrated multiple lung metastases. After two courses of combination chemotherapy with methotrexate (MTX), 5-fluorouracil (5-FU), epirubicin (epiADM), and cisplatin (CDDP), the multiple lung metastases completely disappeared. The patient has survived 23 months to date with no evidence of disease and is receiving adjuvant chemotherapy with tegafur uracil.

A 49-year-old male was referred to our hospital with the chief complaint of gross hematuria. We performed cystoscopy, and found a solid tumor at the dome of the bladder. Sagittal magnetic resonance imaging revealed an extravesical tumor at the position of the urachus. Transurethral resection of bladder tumor was performed. A solid tumor at the dome and two papillary tumors at the right lateral region of the bladder were observed. A pathological examination revealed adenocarcinoma from the solid tumor and urothelial carcinoma from the papillary tumors. Under a diagnosis of double cancer, adenocarcinoma of the urachus and urothelial carcinoma of bladder, we performed a total cystectomy, neobladder and total resection of the urachus.

A 61-year-old woman was admitted to our hospital because of abdominal pain. Computed tomography showed an abscess in the anterior abdominal wall and an intraabdominal tumor. Biopsy from bladder dome presented adenocarcinoma. We diagnosed the tumor as urachal carcinoma and operation was performed. The resected specimen showed transverse fistula. In Japan, there is no report of urachal carcinoma with fistula to digestive duct. We think it is a cause of strong infiltration of the carcinoma. Curative resection for urachal carcinoma with fistula can lead to a better prognosis, in spite of its invasive character.

A 52-year-old woman was referred to our hospital for treatment of urachal cancer. She complained of supurapubic dull pain and gross hematuria. Computed tomography and magnetic resonance imaging showed a non-papillary sessile tumor, which was located on the dome of the bladder and invaded the small intestine. The tumor was diagnosed as Sheldon's stage IIIC urachal cancer. After three courses of neoadjuvant chemotherapy with FOLFOX4 (oxaliplatin, 5-FU and leukovolin), the tumor was reduced from 7 x 6 cm to 5.5 x 5 cm in size. Consequently, the patient underwent an en-bloc resection of the urachal tumor with the dome of the bladder and the parts of the ileum invaded by the tumor. One course of adjuvant chemotherapy (FOLFOX4) was performed. Surgical specimen revealed histologically well differentiated squamous carcinoma and invasion to the propria of the ileum. The surgical margins were negative for the cancer. For 1.5 years after the surgery, no local recurrence or distant metastasis has been observed.

The authors presented a patient with metastatic brain tumor originating from urachal carcinoma. A 64-year-old female was admitted to our hospital with complaints of memory disturbance, indifference and apathy of 3 months duration. Head CT and MRI on admission showed a round mass with perifocal edema in the right frontal lobe. After administration of Gd-DTPA, the mass lesion showed ringed enhancement effect. Pelvic MRI scan revealed a bladder tumor, which was diagnosed as urachal carcinoma. The brain lesion was suspected to have metasta sized metastatic from urachal carcinoma, and was excised by craniotomy. Histology of the brain tumor was identical to that of urachal carcinoma. Postoperatively the patient received local radiation therapy, but died of multiple metastasis to lung and local recurrence, 18 months later. Urachal carcinoma is an extremely rare tumor, comprising 0.17-0.34% of all bladder tumors. Though this rare tumor carries a poor prognosis, it may be effective for longer survival of a patient to treat the metastatic brain lesion with surgery and radiation.

A 34-year-old married woman complaining of macrohematuria was admitted to our institute. Cystoscopy revealed a broad-stalk, nonpapillary tumor at the urinary bladder dome, and cold-punch biopsy proved it to be a mucus-producing adenocarcinoma. Abdominal managnetic resonance imaging demonstrated a tumor extending from the umbilicus to the bladder dome, and chest computed tomography (CT) demonstrated a small lung tumor with calcification. Examination of the upper gostroinstestinal tract, barium enema, and colon fiberscopy did not reveal abnormalities. We therefore diagnosed an urachal carcinoma with lung metastases. Total cystectomy, umbilical-urachal resection, hysterectomy, ileal neobladder, and partial resection of lung were performed, followed by partial resection of the left lung using thoracoscopy. About 6 months later, chest CT demonstrated multiple metastases in the right lung. After treatment with three courses of chemotherapy (paclitaxel and carboplatin), the right lung was partially resected. Serum CEA and CA19-9 levels were used to follow her disease, since both were elevated before the surgery and at the recurrence. Both indicators returned to their normal ranges after treatment. Such cases require careful observation using imaging modalities and tumor markers.

A 43-year-old man visited our clinic with gross hematuria. Ultrasonography and computed tomography demonstrated a tumor at the bladder dome. Cold punch biopsy revealed well-differentiated adenocarcinoma and stage III A urachal carcinoma was diagnosed. Neoadjuvant intra-arterial chemotherapy with cisplatin, adriamycin and angiotensin II was performed and 40% reduction of tumor size was noted 3 weeks after this therapy. En bloc segmental resection was performed. Augmentation ilealcystoplasty was subsequently performed to secure bladder capacity. Adjuvant chemotherapy (UFT) was given for 1 year. Cystolithotomy and closure of ventral hernia were required 10 years after radical surgery for postoperative complications. The patient has survived 12 years with no evidence of local recurrence or distant metastasis.

We report a case of urachal actinomycosis with bone formation. A 76-year-old woman was admitted to our hospital with a complaint of gross hematuria. Cystoscopy revealed bleeding from the dome of the bladder wall. Magnetic resonance imaging revealed a mass extending from the dome of the bladder to just below the umbilicus. A urachal tumor was clinically suspected, and the mass was resected together with urachal remnant and part of the bladder wall. Pathological diagnosis of the specimen was actinomycosis. The bone tissue was found in the abcess. Actinomycosis is a chronic suppurative infection caused by Actinomyces israelii. Although urachal actinomycosis is a rare desease, it is important to recognize this uncommon intrapelvic infection as the differential diagnosis of any intrapelvic mass.

A 76-year-old male patient complained of frequent urination. Computed tomographic and magnetic resonance imaging scans revealed a heterogeneous mass involving his bladder, prostate and Sigmoid colon. He was diagnosed with an invasive urachal tumor and surgery was performed due to severe tumor adhesion to adjacent organs. Partial resection of the tumor was performed with partial cystectomy and sigmoidectomy. A post-operative pathological diagnosis revealed the tumor was a desmoid abdominal wall tumor. The desmoid abdominal wall tumor was not easily differentiated preoperatively from an urachal tumor.

Purpose: This study was undertaken to determine the most appropriate type of operation for the improvement of the radical cure rate and QOL of patients with urachal cancer. We assessed the association between the stage, type of operation, and prognosis of cases we experienced and those reported in Japan., Patients and Method: The subjects included 15 cases of urachal cancer we have experienced in the past 14 years. While clarifying the clinical patterns of these cases, the association between stage, type of operation, and prognosis was studied. Of the cases of urachal cancer reported in Japan in the recent 20 years, 75 cases in which the stage, type of operation, and prognosis were documented were selected, and the association between the stage and outcome in each type of operation was studied., Results: In regard to stage, all the cases were rated as more than IIIA. As for prognosis, 9 cases (60%) are alive without cancer at the present time with a mean survival time of 7 years. Of the above patients, 3 underwent cystectomy and 6 underwent en bloc segmental resection (herein after referred to en bloc). Recurrence or cancer death was experienced in 5 patients, 2 of which were classified as stage IIIA and 3 as IIID. Of these patients, one underwent en bloc, 3 partial resection of the bladder, and one underwent exploratory laparotomy. As far as our study of the cases reported in Japan is concerned, the prognosis of the cases having undergone only partial resection of the bladder was poor, while of the cases having undergone en bloc or total cystectomy 88-100% were alive without cancer for more than 2 years if their stage was classified as IIIA or below. On the other hand, prognosis was very poor whatever the type of operation in the cases whose cancer was stage IIIC or above., Conclusions: It appears that the en bloc is most appropriate as the type of operation for the cases of urachal cancer and that the application of total cystectomy is limited to some cases. In performing the en bloc, an extensive resection of the peritoneum, resection of the posterior sheath of the rectus muscle of the abdomen, and dissection of the intrapelvic lymph nodes in addition to the conventional types of operation should be carried out positively.

We have encountered 6 cases of urachal carcinoma in the past 7 years. They consisted of 4 females and 2 males, with the mean age of 48 years old. Of them, 2 cases were identified as gestational complications. Bladder irritating symptoms and extracystic symptoms other than hematuria were not observed as initial symptoms, and urinary cytology was positive in 2 cases. Cystoscopy is essential for diagnosis, but the spread of the tumor was clearly visualized by sagittal section patterns on magnetic resonance imaging. A correlation between the tumor and clinical course was suggested immunohistochemically with, carcinoembronic antigen and carbohydrate antigen 19-9. Cases of gestation concurrent with urachal carcinoma are very rare, but are expected to increase in the future. Thus appropriate medical treatment must be given to the maternal body. For the treatment, appropriateness of advanced surgery more than partial resection of the bladder combined with total urachoumbilical resection was recommended. However, an effective adjuvant chemotherapy after the first surgical resection needs to be established to improve the patient's quality of life.

A 30-year-old man was referred to our department with a complaint of bladder irritability and with development of high fever. Physical examination revealed a tender mass in the suprapubic area. Computed tomographic scan and magnetic resonance imaging indicated a cystic mass above the bladder dome, extending toward the umbilicus. Urachal abscess was suspected and the mass was excised en bloc with the urachus. The wall of the mass was thickened, and a linear foreign body was detected in the mass, which was considered to be a fish bone. Pathological diagnosis of the mass was xanthogranuloma. We speculated that a swallowed fish bone had penetrated the bowel and might have migrated into the urachal cyst, which induced a xanthogranulomatous change of the wall.

A 68-year-old male was admitted to our hospital with the chief complaints of lower abdominal pain and fever. There was a tender mass palpable in the lower abdomen. Plain abdominal X-ray film revealed multiple air-fluid levels with dilated small bowel loops, suggesting intestinal obstruction. Abdominal ultrasonography, computed tomography and magnetic resonance imaging revealed a solid mass extending from umbilicus to the bladder dome beneath the rectal muscle. There was normal mucosa of the bladder by cystoscopic examination. A urachal tumor was clinically suspected and en bloc removal of the mass, the remaining urachus, umbilicus, omentum and bladder dome was performed. The histological diagnosis was urachal xanthogranuloma. The patient has remained in good health without any recurrence for 6 months since the surgery. We discuss urachal xanthogranuloma in the literature.

Five cases of urachal carcinoma experienced in our hospital during the past 20 years are reported. Surgical resection is considered as the first treatment option of this disease, and other therapies to be less beneficial. Complete surgical extirpation and detection of recurrence in the early stage are considered to be important since local recurrence occurs frequently. We enforced the bladder preserving operation for 4 patients with urachal carcinoma except for 1 case with peritonitis carcinomatosa in the initial diagnosis, and multiple surgical treatment was performed again for 2 patients with recurrence. The bladder was preserved with no evidence of malignancy in three patients for 24, 19 and 5 years, respectively. In the initial management of urachal carcinoma, we believe that bladder-preserving surgery should be considered in selected cases though close follow-up is demanded. Herein, we also report the immunohistochemical study of paraffin-embedded specimens using anti-CEA, CA19-9, CA125 and p53 monoclonal antibodies. The positive reaction was observed in 100% (5/5) for CEA, 80% (4/5) for CA19-9, and 20% (1/5) for CA125. These results suggest that CEA may be a useful marker in the diagnosis of this neoplasm and early detection of its recurrence. Nuclear accumulation of p53 was observed in 80% (4/5), but it did not correlate with the disease progression.

A 33-year-old man who had been treated for chronic prostatitis was diagnosed to have urachal cysts by transabdominal ultrasonography. Cystoscopy revealed protuberance at the dome of the bladder. Computerized tomography scan and magnetic resonance imaging showed the mass to be mostly cystic but partly solid. Resection of the urachal cysts and partial cystectomy were performed. Histopathologically, most cysts had a normal cylindrical epithelium with retention of mucinous substance. However, several small cysts contained epithelial cells resembling tubulo-villous adenoma and showing mitotic figures. This case was concluded as urachal carcinoma detected in its very early stage.

We report a case of pelvic fibromatosis, which has been rarely found. A 36-year-old man referred to our hospital complaining of right abdominal pain and a swelling mass. Abdominal CT and transabdominal echogram revealed a tumor contacted to the bladder. A nonpapillary tumor (little finger sized) at the bladder dome was observed by cystoscopy, and the tumor was clinically considered as urachal tumor. Wide radical excision including the bladder and prostate was performed. Pathological diagnosis was pelvic fibromatosis arising from musculoaponeurotic structures in the pelvis: The fibromatosis is benign nonmetastatic tumor but the recurrence rate is considerably high. However, in the present case, 14 months after operation there is no recurrence yet.

A case of desmoid tumor of the abdominal wall which was preoperatively suspected as urachal tumor is presented. The patient was a 56-year-old man, who was referred to our clinic for further examination of the mass detected incidentally in the ventral region of the urinary bladder by computed tomography. Ultrasonography showed that the mass had a heterogenous and hypoechogenic content. An urachal tumor was suspected and surgery was performed to remove the tumor. During the operation we found that the tumor was completely separated from the urinary bladder and that it had originated from the left rectus abdominal muscle. The pathological diagnosis was desmoid tumor of the abdominal wall. Since urachal tumor has no characteristic findings on the imaging examinations, it is difficult to differentiate desmoid tumor of the lower abdominal wall from urachal tumor, preoperatively.

A 71-year-old female was seen initially with the complaint of gross hematuria. Cystoscopic examination revealed non-papillary tumor at the dome of the bladder and a transurethral biopsy showed signet ring cell carcinoma. En bloc segmental resection was performed, and the patient has been well without any evidence of progression. We report a case, which we believe to be the 6th in Japan, of signet ring cell carcinoma of the urachus.

Carcinoma of urachus accompanied with calcification is rarely encountered. A 37-year-old man presented with the complaint of mucus discharge on voiding. A plain X-ray film of his abdomen showed the presence of calcification at the urinary bladder. A hemispheric tumor at the top of the urinary bladder was seen on cystoscopic examination. The results of urine cytology and cold-punch biopsy supported the diagnosis; carcinoma of urachus. En bloc segmental resection was performed. Histopathologically, the tumor was composed of moderately differentiated adenocarcinoma producing mucus. Now, he is alive and has no evidence of recurrence. This is the 9th report on carcinoma of urachus with calcification in the Japanese literature.

Seven cases of urachal carcinoma experience in Yokohama City University in these 10 years are presented herein. The high incidence of local recurrence of this disease and the results of the treatments in the previously reported cases were reviewed. From these experiences, extensive radical surgery including total cystectomy, en bloc removal of entire urachus, pelvic lymphadenectomy, and adjuvant chemotherapy and or radiation are recommended for the treatment of urachal carcinoma.

Three cases of urachal carcinoma are presented. Chief complaints were passage of mucous urine in a 58-year-old man (case 1), hematuria and frequency in a 63-year-old woman (case 2) and hematuria in a 45-year-old man (case 3). Urine cytology were negative for all cases and serum CEA level was elevated in case 1. Mass in the area of the bladder dome were revealed by cystoscopic examination in all cases. CT scanning, TUR biopsy and cystogram were valuable diagnostic procedure. En bloc segmental resection were performed on all cases, and case 1 and case 2 have been well without disease for 36 and 40 months, respectively. Case 3 died 65 months after operation with disseminated carcinoma. Histologically mucin-producing adenocarcinoma were found in all cases. Statistic examination and discussion are made of 237 cases of urachal carcinoma reported in Japan.

We report a case of metastatic bladder tumor from gastric cancer, which was difficult to differentiate from urachal tumor preoperatively, especially computed tomographic scan, cystoscopy, and biopsy. A 51-year old man visited our hospital with the chief complaint of asymptomatic gross hematuria. He had received subtotal gastrectomy for gastric cancer two years earlier. Because the possibility of urachal tumor could not be excluded, en bloc segmental resection of the bladder, the urachus, and the umbilicus was carried out. This case was diagnosed as adenocarcinoma histologically, but it was difficult to determine whether the case was a metastatic bladder tumor from gastric cancer or urachal tumor by the routine staining method. High iron diamine-alcian blue and paradoxical concanavalin A (ConA) stainings were performed, on the surgical specimens of this case and other urachal tumor already diagnosed. In these cases, metastatic bladder tumor could be differentiated from an urachal one by mucohistochemically paradoxical Con A staining. Seventeen cases of metastatic bladder tumor from gastric cancer including our cases were collected from the Japanese literature and reviewed.

Carcinoma of the urachus is a rare disease with poor prognosis. This is probably due to the difficulty of preoperative diagnosis. Radiological modalities have been considered to be of little value, but in many cases, preoperative diagnosis can be established with the aid of CT scan. In patients with urachal carcinoma, CT scan visualize the primary tumor and its extension beyond the bladder wall into the space of Retzius. Although about 150 cases of urachal tumor have been reported in the English literature, computerized tomographic findings have not been established. We present two cases and emphasize the usefulness of CT scan in preoperative diagnosis.