University Journal of Surgery and Surgical Specialities

ISSN 2455-2860 2019, Vol. 5(3)

Urachal mucinous in a young women - A Case Report SENTHILKUMAR P Department of Urology, KILPAUK MEDICAL COLLEGE AND HOSPITAL

Abstract : Urachal is an uncommon associated with poor prognosis. No concensus has been reached regarding diagnostic criteria so far. The management of urachal carcinoma is controversial, too. In this case presentation, we reported a 45 year old female with urachal cancer which was managed by extended partial cystectomy.For cases with asmall tumor or minimal involvement of the bladder wall, extendedpartial cystectomy can be considered in order to improve the patientsquality of life without affecting the survival rate. CYSTOSCOPY- 5*4 CM GROWTH BLADDER DOME Keyword :urachal adenocarcinoma,partial cystectomy. Patient was taken up for surgery. On laparotomy , a mass Case History : involving urachus with infiltration of dome of bladder was found A 45-year-old female patient was admitted with abdominal out. Since there was no findings of local or distant , pain and dysuria. She did not have or abnormal Extended partial cystectomy was done by resecting tumor, umbilical discharge. Her clinical examination findings and urachus along with 2 cm normal peritoneam, transversalis fasia, blood investigation reports were normal. The chest connective tissue in the space of Retzius , adjacent 2 cm normal radiography and urine cytology were normal USG showed a bladder wall. lesion of mixed echogenicity and foci of I creased echogenicity involving the dome of the bladder. Computerized tomography revealed enhancing soft tissue lesion 5 x 4.3 cm., involving anterior wall with calcification from bladder to anterior abdominal wall; urachal remnant seen. There were no regional or non – regional lymphnode enlargement.

EXTENDED PARTIAL CYSTECTOMY SPECIMEN Histopathological Examination of the specimen revealed features of urachal mucinous adenocarcinoma with negative resection margin

CECT PELVIS- HETERODENSE BLADDER DOME MASS WITH CALCIFICATION Cystoscopy showed 5 x 4 cm proliferative growth in dome of bladder. On pressing the suprapubic region, the growth was found to be protruding towards the bladder cavity. We did Cystoscopic biopsy of the bladder growth which revealed features of urachal mucinous adenocarcinoma. HPE- URACHAL MUCINOUS ADENOCARCINOMA

An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Surgery and Surgical Specialities

DISCUSSION a urachal origin. Most data suggest better outcomes for Urachal carcinoma is a rare entity, accounting for 0.5% to 2.0 % of urachal than nonurachal adenocarcinoma of the bladder. bladder tumors ( Henly et al., 1993)and 20.0 % to 39.0 % of bladder But when comparing bladder adenocarcinoma of urachal adenocarcinoma. It mostly occurs in males of 40 to 70 years of age. and non-urachal origin, there are no immunohistochemical The histological classification of urachal carcinoma is mostly markers to differentiate the two with confidence. Thus, the adenocarcinoma (94.0 %), but rarely , best predictor for survival is clinical and pathological transitional cell carcinoma or undifferentiated carcinoma. Typically, staging. urachal carcinoma spreads locally, but also carries a high risk of Diagnosis and Staging distant metastases. In the early stages, it often forms a mass within Sheldon et al staging system for urachal carcinoma as the urachus, then spreads locally in the space of Retzius, or the follows: space between the bladder dome and the umbilicus, or the space Stage I no invasion beyond the urachal mucosa; between the transversalis fascia and the peritoneum, or into the Stage II- invasion confined to the urachus; rectus abdominis muscle.( Scabini et al 2009) Pelvic lymph nodes Stage III- local extension to the (a) bladder, (b) abdominal are often involved and distant metastases can occur to the lung, wall, and (c) viscera other than the bladder; bone, liver, lymph nodes, skin, brain, and so on. Bone metastasis Stage IV- metastasis to (a) regional lymph nodes and (b) usually occurs as a part of widespread metastases; and isolated distant sites bone metastasis is rare. Thali- Schwab et al. reported that 28.0% of Mayo staging system for urachal carcinoma the urachal carcinoma patients had metastases at the time of Stage Definition presentation. Due to its obscure anatomic position, urachal I Tumors confined to the urachus and or bladder carcinoma is often misdiagnosed at the early stage and many II Tumors extending beyond the muscular layer of the patients have progressed to advanced states upon presentation. urachus or the bladder The most common clinical symptoms are hematuria, urinary III Tumors infiltrating the regional lymph nodes frequency and mucinuria, similar to other bladder tumors. Patients IV Tumors infiltrating non-regional lymph nodes or other may present with a mass in the lower abdomen. (Sheldon et distant sites all,1984) The presence of an inhomogeneous solid mass between Treatments for urachal carcinoma. the anterior wall or the dome of bladder and the abdominal wall in Urachal carcinoma is mostly managed with surgery. It the image is highly suggestive of urachal carcinoma. In ultrasound, remains debatable that which one is more efficacious: the tumor often manifests as a solid mass with an uneven surface extended partial cystectomy or radical total cystectomy. and echogenicity, sometimes with calcification. CT or MRI often Radical total cystectomy has been regarded as the first reveals a hypodense or low signal mass between the anterior dome choice for the treatment of urachal carcinoma. It is of the bladder and rectus abdominis, located either mostly within or combined with urachectomy for a large tumor or outside the bladder. CT and MRI scans are essential for squamous cell carcinoma. In recent years, extended pre-operative staging assessment of urachal carcinoma. ( Nese et al partial cystectomy has been recognized as an important 2003, Fancher et al, 2010) PET scanning would provide more timely surgical treatment for urachal carcinoma. (Bratu et al., and accurate information for the preoperative staging and decision 2009; Herr, 1994). There is a report of successful of treatment plans than traditional imaging studies. It also helps with laparoscopic resection of urachal carcinoma with good early evaluation on the operative effect and determining the short-term follow-up results. The incidence of local necessity of adjuvant treatment. The radiologic differential diagnosis recurrence was higher in patients who underwent a partial includes benign urachal tumors that may mimic malignancy, like cystectomy (37.5%) than in those who had more radical adenomas, fibromas, fibromyomas, and hamartomas, infected surgery(27%) (Gopalan et al 2009) The impact of urachal remnants, and other malignant tumors like radiotherapy and/or chemotherapy on prevention and of non-urachal origin, transitional cell carcinomas, treatments for postoperative recurrence remains and metastases originating from primary lesions of the colon, controversial. There is no randomized , prospective study prostate, or female genital tract Cystoscopic findings often reveal a comparing approaches of surgical treatment. Therefore, broad-based ulcerative mass in the dome or at the anterior wall of treatment approach can be decided in the light of literature bladder. The tumor may sometimes be covered by mucosa. On and according to clinicopathological features and also by pressing the suprapubic area, mucus-like substance can be discussing with the patient. observed flowing out from the surface of the tumor. However, there Summary may not be any obvious lesions in the bladder. (1) Surgery should be able to completely remove the Pathology: tumor. Partial cystectomy, which may leave positive Sheldon et al proposed the following criteria for the diagnosis of margins, increases the risk of short-term recurrence. urachal carcinoma: ‘‘1)tumor located in the dome of the bladder, (2) Radical surgery yields a large wound, resulting in a 2) absence of cystitis glandularis and cystitis cystica, variety of complications in the following urinary diversion. 3) predominant invasion of the muscularis or deeper tissues For cases with a small tumor or minimal involvement of the with a sharp demarcation between the tumor and the surface bladder wall, extended partial cystectomy can be epithelium, which is free of glandular or polypoid proliferation, considered in order to improve the patient’s quality of life 4) presence of a urachal remnant in association with the without affecting the survival rate. The most important neoplasm and 5) ramifications of tumor in the bladder wall with thing is to excise sufficient areas, including not only the extension to the space of Retzius, anterior abdominal wall or bladder dome, but also the covering peritoneum, the umbilicus.’’ The majority of urachal carcinomas are connective tissues in the space of Retzius and urachus, adenocarcinoma, most of which are mucin producing. Other plus dissection of conventional bilateral pelvic lymph subtypes include , squamous cell carcinoma and nodes for staging purposes. transitional cell carcinoma. Urachal adenocarcinoma is similar (3)For tumors spreading outwards into the peritoneal wall, histologically to colonic adenocarcinoma with both the affected part of the peritoneal wall should also be sharing several immunohistochemical markers. Gopalan et al excised. suggest 2 markers that may differentiate urachal and colonic (4) Radical cystectomy plus urachectomy is indicated for adenocarcinoma with strong reactivity for 34BE12 and lack of large tumors or tumors with positive margins found diffuse nuclear reactivity for _beta - catenin being more indicative of intraoperatively.

An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Surgery and Surgical Specialities

(5) combined chemo and Radio therapy has some impact on the survival of patients with advanced disease and post-operative recurrence. (6) The prognosis of urachal carcinoma is very poor with a five-year survival of 5.6% to 29.0%.A recent study reported a five-year survival of 61.9%. (7) radical removal of the tumor during the first treatment, and comprehensive therapies for advanced cancer patients and patients with recurrence or metastasis after operation are critical to improve the treatment efficacy of urachal carcinoma. REFERENCES 1.Brunicardi FC, Andersen DK, Billiar TR et al: Schwartz’s, Principles of Surgery, 9th ed. New York: McGraw Hill 2010; chapter 35. 2. Cappele O, Sibert L, Descargues J et al: A study of the anatomic features of the duct of the urachus. Surg Radiol Anat 2001; 23:229. 3. Cilento BG Jr, Bauer SB, Retik AB et al: Urachal anomalies: defining the best diagnostic modality. Urology 1998; 52:120. 4. Chan ES, Ng CF, Chui KL et al: Novel approach of laparo-scopic transperitoneal en bloc resection of urachal tumo and umbilectomy with a comparison of various techniques.J Laparoendosc Adv Surg Tech A 2009;19:423. 5. Gopalan A, Sharp DS, Fine SW et al: Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation. Am J Surg Pathol 2009; 33:659. 6. Copp HL, Wong IY, Krishnan C et al: Clinical presentation and urachal remnant pathology: implications for treatment. J Urol, suppl., 2009;182:1921.

An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Surgery and Surgical Specialities

An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Surgery and Surgical Specialities