J of Evolution of Med and Dent Sci/ eissn , pissn / Vol. 3/ Issue 24/June 16, 2014 Page 6628

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ADENOCARCINOMA OF BLADDER-SIGNET RING CELL MUCINOUS VARIANT S. Senthil Kumar 1, D. Prem Charles 2, B. Krishnaswamy 3, P. Viswanathan 4, S. Sarath Chandran 5 HOW TO CITE THIS ARTICLE: S. Senthil Kumar, D. Prem Charles, B. Krishnaswamy, P. Viswanathan, S. Sarath Chandran. Adenocarcinoma of Bladder-Signet Ring Cell Mucinous Variant. Journal of Evolution of Medical and Dental Sciences 2014; Vol. 3, Issue 24, June 16; Page: 6628-6632, DOI: 10.14260/jemds/2014/2793 ABSTRACT: Mucinous adenocarcinoma of the urinary bladder is a rare variant constituting approximately 0.5% to 2.0% of all primary carcinomas of the bladder. This tumor initially presents as a high-grade, high-stage lesion and diffusely invades the bladder wall without forming intraluminal growth. The patients have no specific symptoms, which leads to delayed diagnosis and poor prognosis. A case of adenocarcinoma of bladder in a 32years old male was presented with clinical hematuria was reported. KEYWORDS: Signet ring cell, mucinous, adenocarcinoma. INTRODUCTION: Mucinous adenocarcinoma is a rare neoplasm of the urinary bladder. Less than 100 cases were described after Saphir reported the first two cases in 1955. 1 A case of mucinous adenocarcinoma of the urinary bladder had been reported with a review of the current literature. CASE HISTORY: A 32years old marketing executive was presented with a history of passing frank blood at the end of micturition for ten months duration. No similar complaints in the past. Complete hemogram, urine examination, renal function tests, coagulation profile, prothrombin time had been done and were found to be normal. Computerized tomography (CT), KUB plain and contrast reveals, a well-defined mildly enhancing lobulated polypoidal lesion with few calcific foci measuring 2.9 2.7cms noted arising from the anterior wall of the urinary bladder (Fig. 1). No significant pelvic lymphadenopathy was noted. Transurethral resection was done and the specimen was sent in bits measuring 3ml inaggregate for histological examination. Microscopic examination revealed the features consistent with the diagnosis of mucin secreting adenocarcinoma of bladder (Fig. 2) with significant presence of signet ring cells. DISCUSSION: Urothelial carcinomas constitute ninety percent of malignant bladder tumors. Remaining ten percent of tumors represent sarcomas and others. Primary adenocarcinomas of the bladder are rare, malignant neoplasms derived from transitional epithelium showing histologically pure glandular phenotype, representing 2.5% of all malignant bladder neoplasms. They arise through process of metaplasia of the urothelium and frequently associated with long standing local irritation 3 like schistosomiasis and renal stones. Ninety percent of carcinomas are associated with bladder exostrophy. 4 These tumors more commonly in males than females, with a peak incidence of sixth decade of life. 2 The site of origin was from the trigone and posterior wall. Patient typically presents with history of hematuria because of large dilated blood vessels (Fig. 3 & Fig. 4). Two thirds of adenocarcinoma is single discrete lesions where transitional cell carcinoma tends to be multifocal. 5 Grossly the tumor presents with papillary, nodular or flat and ulcerated. Microscopically the tumor composed of colonic type of glandular epithelium (Fig. 5) and often contains abundant extra J of Evolution of Med and Dent Sci/ eissn- 2278-4802, pissn- 2278-4748/ Vol. 3/ Issue 24/June 16, 2014 Page 6628

cellular mucin. Regardless of histologic pattern, cystitis cystica et glandularis or surface glandular metaplasia is commonly present in the adjacent benign urothelium. Most adenocarcinomas had a tendency to infiltrate the muscularis (Fig. 8) at the time of initial diagnosis. 6 In differential diagnosis one must exclude the possibility of adenocarcinoma metastasis probably from rectum, prostate, appendix or endometrium. 7 HISTOLOGICAL VARIANTS OF ADENOCARCINOMA: 1. Adenocarcinoma of not otherwise specified. 2. Enteric (colonic) type. 3. Signet ring cell type. 4. Mucinous clear cell. 5. Hepatoid. 6. Mixed. 7. Urachal. SIGNET RING CELL (Fig. 6) CARCINOMA OF THE BLADDER 8 : Rare variant, fewer than 70 cases were reported. It composed entirely of signet ring or poorly differentiated round cells with intracytoplasmic mucin and without abundant intracellular mucin. Some signet ring cells show prominent plasmacytoid features. These tumors tend to infiltrate the bladder wall diffusely or extensively throughout the adjacent structures making primary resection impossible. MUCINOUS (Fig. 7) TYPE: Tumors that show abundant mucin and tumor cell clusters apparently floating in mucin are classified under mucinous variant. DIFFERENTIAL DIAGNOSIS: 1. Typical urothelial carcinoma with extensive glandular differentiation. 2. Nephrogenic adenoma. 3. Endometriosis and endocervicosis. 4. Metastatic adenocarcinoma. REFERENCES: 1. Saphir O: Signet ring cell carcinoma of the bladder: Am J Pathol 1955; 31: 223-231. 2. Bollito ER, Pacchioni D, Lopez-Boltron A, et al. Immunohistochemical study of Neuroendocrine differentiation in primary glandular lesions and tumors of the urinary bladder. Anal Quant cytol histol 2005; 27: 218-224. 3. Wheeler J D, Hill WT, Adenocarcinoma involving the urinary bladder. 1954; 7: 119-135. 4. Goyonna R, Emmett JL, Mc Donald JR: Exostrophy of the bladder complicated by the adenocarcinoma. Jurol 65: 391-400, 1951. 5. Mostofi Fk, Thomson RV, Dean AL. Mucous adenocarcinoma of the urinary bladder. Cancer 1955; 8: 741-758. 6. Rogers CG, Palapattu GS, Shariat SF, et al. Clinical outcomes following radical cystectomy for non- transitional cell carcinoma compared to transitional cell carcinoma of the bladder. Jurol 2006; 175 (6): 2048-2053; discussion 2053. J of Evolution of Med and Dent Sci/ eissn- 2278-4802, pissn- 2278-4748/ Vol. 3/ Issue 24/June 16, 2014 Page 6629

7. Dean AL, Mostofi FK, Thompson RV. Study of first four hundred cases in the bladder tumor registry.jurol 1954; 74: 571-590. 8. Braun EV, Ali M, Fayemi AO, et al. Primary signet ring cell carcinoma of the urinary bladder; review of the literature and report of a case. Cancer 1981; 47 (6): 1430-1435. Fig. 1: COMPUTERISED TOMOGRAPHY KUB PLAIN AND CONTRAST A well-defined mildly enhancing lobulated polypoidal lesion with few calcific foci measuring 2.9 2.7cms noted arising from the anterior wall of the urinary bladder. No significant pelvic lymphadenopathy noted. J of Evolution of Med and Dent Sci/ eissn- 2278-4802, pissn- 2278-4748/ Vol. 3/ Issue 24/June 16, 2014 Page 6630

MICROSCOPIC PICTURES: H&E SECTIONS Fig. 2: 4X Fig. 2: 10X Fig. 2: 40X Fig 2: Mucin secreting adenocarcinoma adjacent to transitionalepithelium. Fig. 3: 10X Fig. 3: 40X Fig. 4: 10X Fig. 4: 40X Transitional epithelium deeper to which adenocarcinoma with lake of mucin along with large dilated blood vessels are seen. Fig. 5: 40X Tumor cells tend to form glandularunits Fig. 6: 40X Collection of signet ring cells. J of Evolution of Med and Dent Sci/ eissn- 2278-4802, pissn- 2278-4748/ Vol. 3/ Issue 24/June 16, 2014 Page 6631

Fig. 7: 10X Pools of Mucin with calcified spherules. Fig. 8: 10X Mucin secreting adenocarcinoma present in the muscularis layer of the bladder. AUTHORS: 1. S. Senthil Kumar 2. D. Prem Charles 3. B. Krishnaswamy 4. P. Viswanathan 5. S. Sarath Chandran PARTICULARS OF CONTRIBUTORS: 1. 3 rd Year Post Graduate, Department of Pathology, Rajah Muthiah Medical College, Annamalai 2. 2 nd Year Post Graduate, Department of Pathology, Rajah Muthiah Medical College, Annamalai 3. Professor, Department of Pathology, Rajah 4. Professor, Department of Pathology, Rajah 5. Lecturer, Department of Surgery, Rajah NAME ADDRESS EMAIL ID OF THE CORRESPONDING AUTHOR: Dr. P. Vishwanathan, Professor, Department of Pathology, Faculty of Medicine, Rajah Muthiah Medical College, Annamalai University, Chidambaram-608002, Tamilnadu, India. E-mail: drpviswanathan2013@gmail.com Date of Submission: 23/05/2014. Date of Peer Review: 24/05/2014. Date of Acceptance: 04/06/2014. Date of Publishing: 12/06/2014. J of Evolution of Med and Dent Sci/ eissn- 2278-4802, pissn- 2278-4748/ Vol. 3/ Issue 24/June 16, 2014 Page 6632