Vol 13 No 04 July-August 2016 2800 CASE REPORT Intradiverticular Sarcomatoid Carcinoma of The Bladder: An Overview Starting From A Peculiar Case. Francesco Lembo,1 Enrica Subba,1 Antonio Simone Laganà,2 Salvatore Giovanni Vitale, 2* Gaetano Valenti,3 Carlo Magno1 Keywords: Carcinosarcoma; Urinary Bladder; Neoplasm Invasiveness; Fatal Outcome; Urologic Surgical Procedures, Male. INTRODUCTION Sarcomatoid carcinoma (SC) of the bladder is a rare and highly aggressive tumour, defined accord-ing to the World Health Organization (WHO) as a biphasic neoplasm exhibiting morphologic and/or im- munohistochemical evidence of epithelial and mesenchymal differentiation.(1) SC of the urinary bladder is an uncommon neoplasm, accounting for 0.1 to 0.3% of all bladder malignancies and is more frequent during the seventh decade.(2-4) As standard protocol of the university hospital in which the case was reported, the patient was informed at the admission and signed an informed consent allowing data collection for research purposes. This case report is in accordance with the Helsinki Declaration, conforms with the Committee on Publication Ethics 1 Unit of Urology, Department of Human Pathology, University of Messina, Messina 98125, Italy. 2 Unit of Gynecology and Obstetrics, Department of Human Pathology in Adulthood and Childhood "G. Barresi", University of Messi- na, Messina 98125, Italy. 3 Department of General Surgery and Medical Surgical Specialties, University of Catania, Catania 95123, Italy. *Correspondence: Unit of Gynecology and Obstetrics, Department of Human Pathology in Adulthood and Childhood "G. Barresi", University of Messina, Messina 98125, Italy. Phone: +39 3479354575. Fax: +39 0902937083. E-mail: vitalesalvatore@hotmail.com. Received January 2016 & Accepted June 2016 Figure 1. Suspicious lesion inside a large bladder diverticulum. Figure 2. Pathology of the specimen: a. Hematoxylin-eosin (H-E), 63X; b. (H-E), 100X; c. (H-E), 100X; d. (H-E),160X; e. (H-E), 200X; f. (H-E), 200X; g. (H-E), 200X; h-i-l-m: Immunoperoxidase for: Smooth Muscle Actin (h), CK20 (i), p53 (l), Mib-1 (m) nuclear counterstaining with Mayer's hemalum. (COPE) guidelines (http://publicationethics.org/) and was approved by the Institutional Review Board (IRB) of the university hospital in which it was reported. CASE REPORT A 77-year-old man was referred to our hospital for mac- roscopic hematuria, dysuria, and pelvic pain. Cystosco- py showed a suspicious papillary lesion inside a large bladder diverticulum. Chest and abdomen computed tomography (CT) (Figure 1) showed no evidence of distant visceral metastases. Cystoscopy confirmed the presence of a polypoid area within a bladder divertic- ulum. A transurethral resection of the tumor (TUR-T) was performed and SC was histopathologically suspect- ed. Since the patient refused cystectomy, we have opted for a less invasive surgery: bladder diverticulectomy. At gross examination, the specimen measured 13 x 10.5 x 7 cm. Surgical samples were formalin fixed, paraffin embedded and cut into 4 micron thick sections for the histological examination with haematoxylin-eosin stain and immunohistochemical procedures against Smooth Muscle Actin (SMA), cytokeratins 20, p53 and Ki-67 (DakoCytomation, Glostrup, Denmark). At the micro- scopic examination, the tumour had a biphasic appear- ance with an epithelial component represented by areas of squamous carcinoma with corneous pearls and areas of urothelial high grade carcinomas and a mesenchymal component with spindle, pleomorphic cells (Figure 2a- 2g). Frequent mitoses and necrosis were evidenced. The tumour infiltrated the whole thickness of the diverticu- lum wall into the perivisceral fat. At immunohistochem- istry, the spindle areas were positive for SMA (Figure 2h), while the epithelial (squamous and urothelial) ones displayed intense cytokeratin 20 staining (Figure 2i). P53 immunopositivity was found throughout the tu- mour (Figure 2l). High proliferation index (50%) was documented by immunohistochemistry against Ki-67 (Figure 2m). Based on the histological and immuno- histochemical findings, SC of the urinary bladder was diagnosed. No postoperative radiotherapy or chemo- therapy was performed. 40 days after, the patient’s clinical status worsened with haematuria and abdomi- nal pain and he died. Abdomen CT showed that pelvic cavity was largely occupied by a mass of heterogene- ous appearance of 15x15 cm (Figure 3), with several satellite nodules in the context of intraperitoneal fat, anterior abdominal wall, left paramedian and left obtu- rator sites, with various dimensions from 3 to 6 cm. The right ureter was encompassed by the sarcomatous mass. DISCUSSION SC of the urinary bladder is a rare neoplasm and his- togenesis is a controversial issue.(5-8) Macroscopically, these tumors are usually large, polypoid or nodular. Most of the reported cases contain high-grade papil- lary/undifferentiated urothelial carcinoma. In addition, other subtypes with epithelial origins, such as small-cell carcinoma, squamous carcinoma and adenocarcinoma have been reported. The most common sarcomatous elements are chondrosarcoma, leiomyosarcoma, and malignant fibrous histiocytoma.(4) According to recent data, the most common location of SC is the lateral wall of the bladder.(2) In our case the sarcomatous element was a leiomyosarcoma and was located in a bladder di- verticulum. Our patient presented with hematuria and pelvic pain. The only curative management of this kind of neoplasm could be early detection and aggressive surgery. The other preferred modalities of treatment include cystectomy or transurethral resection of the bladder (TURB) with or without radiation therapy and chemotherapy. Furthermore, chemotherapy and radio- therapy do not provide apparent survival advantages. Transurethral resection and partial cystectomy carry the risk of incomplete tumour resection. Neoadjuvant ra- diochemotherapy with radical cystectomy can provide 20 months of recurrence-free survival.(9) In a recent large retrospective study which analyzed 221 cases, the overall 5-year cancer-specific survival rate after radical cystectomy was 20.3%. The 1-, 5-, and 10-year sur- vival rates for SC of the urinary bladder were 53.9%, 28.4%, and 25.8%, respectively.(2) Treatment of bladder SC should be aggressive and multimodal but optional treatment is not still encoded. As already reported,(10) SC has a poor prognosis despite of all treatment modal- ities and a median cancer-specific survival of approx- imately 14 months. In particular, cancer-specific sur- vival was significantly better for those who underwent cystectomy instead of transurethral resection. On one hand, chemotherapy and radiotherapy do not provide apparent survival advantages;(11) on the other hand, neo- adjuvant/adjuvant radiochemotherapy has been used in many cases, and there were complete responses after neoadjuvant treatment.(12) Considering our experience and the most updated literature, we solicit future multi- centric large cohort analyses which may clarify the best evidence-based treatment of the reported condition. CONFLICT OF INTEREST None declared. REFERENCES 1. Lopez-Beltran A, Sauter G, Gasser T, et al. Infiltrating urothelial carcinoma. In: Eble JN, Sauter G, Epstein JI, Sesterhnn IA, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours Sarcomatoid Carcinoma of The Bladder-Lembo et al. Figure 3. Abdomen CT showing a mass of heterogeneous appear- ance of 15x15 cm which occupies the pelvic cavity. Case Report 2801 Vol 13 No 04 July-August 2016 2802 of the Urinary System and Male Genital Organs. Lyon: IARC Press; 2004. p. 93-109. 2. Wang J, Wang FW, Lagrange CA, Hemstreet Iii GP, Kessinger A. Clinical features of sarcomatoid carcinoma (carcinosarcoma) of the urinary bladder: analysis of 221 cases. Sarcoma. 2010;2010. pii: 454792. 3. Ikegami H, Iwasaki H, Ohjimi Y, Takeuchi T, Ariyoshi A, Kikuchi M. Sarcomatoid carcinoma of the urinary bladder: a clinicopathologic and immunohistochemical analysis of 14 patients. Hum Pathol. 2000;31:332-40. 4. Wright JL, Black PC, Brown GA, et al. Differences in survival among patients with sarcomatoid carcinoma, carcinosarcoma and urothelial carcinoma of the bladder. J Urol. 2007;178:2302-6. 5. Giannopoulos A, Alivizatos G, Kyriakou V, Mitropoulos D, Dimopoulos MA. Carcinosarcoma of the bladder. Br J Urol. 1991;67:106-7. 6. Völker HU, Zettl A, Schön G, et al. Molecular genetic findings in two cases of sarcomatoid carcinoma of the ureter: evidence for evolution from a common pluripotent progenitor cell? Virchows Arch. 2008;452:457-63. 7. Wick MR, Brown BA, Young RH, Mills SE. Spindle-cell proliferations of the urinary tract. An immunohistochemical study. Am J Surg Pathol. 1988;12:379-89. 8. Gorstein F, Anderson TL. Malignant mixed mesodermal tumors: carcinoma, sarcoma, or both? Hum Pathol. 1991;22:207-9. 9. Tazi FM, Ahallal Y, Benlemlih A, Khallouk A, Elfassi MJ, Farih MH. Urinary bladder carcinosarcoma: a complete pathological response after neoadjuvant chemotherapy. Turkish Journal of Urology. 2012;38:114-6. 10. Atılgan D, Gençten Y. Carcinosarcoma of the bladder: a case report and review of the literature. Case Rep Urol. 2013;2013:716704. 11. Lopez-Beltran A, Pacelli A, Rothenberg HJ, et al. Carcinosarcoma and sarcomatoid carcinoma of the bladder: clinicopathological study of 41 cases. J Urol. 1998;159:1497–503. 12. Hoshi S, Sasaki M, Muto A, et al. Case of carcinosarcoma of urinary bladder obtained a pathologically complete response by neoadjuvant chemoradiotherapy. Int J Urol. 2007;14:79–81. Sarcomatoid Carcinoma of The Bladder-Lembo et al.