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Appendiceal Carcinoma Masquerading as Primary Bladder Carcinoma
KARL T. K. CHEN* AND ROBERT W. From the Department of Pathology, Saint Agnes Medical Center, and Department of Surgery (Urology), Fresno Community Hospital and Medical Center, Fresno, California
ABSTRACT
We report 2 cases of appendiceal adenocarcinoma invading the bladder. Both tumors as primary bladder carcinomas. Cystoscopic biopsies obtained adenocarcinomatous tissue and the secondary nature of the bladder lesion became apparent at laparotomy in both cases. One patient was treated with surgical resection of the appendix, the adjacent cecal wall and the bladder wall, and postoperative irradiation. She was well 10 years later. The other patient was treated with right colectomy and segmental bladder resection. She died of diffuse peritoneal recurrence 6 years later. VV"Or\,-,rv".n'"..nr".nril
KEY
WORDS: appendiceal neoplasms, adenocarcinoma, bladder neoplasms
Secondary tumors account for about 15% of malignant bladder tumors. l Of these tumors cases of direct bladder extension from carcinoma of adjacent organs outnumber cases of bladder metastasis from distant primaries. About a third of the secondary bladder tumors result from direct extension from carcinoma of the colon. 1 Appendiceal carcinoma with bladder extension is extremely rare with only 6 cases reported in the literature. 2- 7 We describe the clinicopathological features of 2 such cases and compare them with those of previously reported cases.
The tumor invaded the cecal and bladder wall. Small bowel obstruction developed 6 years postoperatively. At the entire peritoneal cavity was distended mucinous material. Biopsy revealed mucinous adenocarcinoma. The patient died 3 months later. aV'l"\If'\l"<:It-lf'\'Y'I
CASE REPORTS
Case 1. A 70-year-old woman was evaluated after an episode
of gross hematuria. Pelvic examination showed a right pelvic mass and cystoscopy revealed a mass in the right bladder wall. A biopsy demonstrated mucosal tissue with fibrosis and chronic inflammation, and separate fragments of mucinous material that contained nests of well differentiated adenocarcinoma (fig. 1). An upper gastrointestinal series, barium enema and excreurography were normal. exploration a mucinous lesion of the dix was found. The communicated with the The adherent cecal wall wall were the was well tiated mucinous of the received site. remained Case 2. gross hematuria. IVP showed slight indentation of right posterior bladder wall and cystoscopy revealed a 6 em. area of bullous edema in which a central 2.5 em. area was covered by mucus. Biopsy of the central area led to a cavity. The biopsy specimen showed mucosal tissue with chronic inflammation and separate fragments of mucinous adenocarcinoma (fig. 2). The abdominal roentgenograms demonstrated a 5 em. area of calcification in the right pelvis. A barium enema showed an area of external compression of the cecum. At exploration a 5 em. mass was found between the cecum and bladder. Excision of the mass, right colectomy and segmental bladder resection were done. Histologically the mass was well differentiated mucinous adenocarcinoma of the appendix. 'UL ............ 'U ..... 'U ........
Accepted for publication September 19, 1990. *Requests for reprints: Department of Pathology, Saint Agnes Medical Center, 1303 E. Herndon Ave., Fresno, California 93720.
1. Case 1. Well differentiated adE~nocal'CHlonlatlous tissue mucinous background of cystoscopic biopsy. from x160.
FIG. 2. Case 2. Well differentiated adenocarcinomatous tissue in mucinous background of cystoscopic biopsy. H & E, reduced from X160. 821
822
CHEN AND SPAULDING DISCUSSION
Although gastrointestinal carcinomas not infrequently spread directly to the bladder, 1 they are almost exclusively colonic, especially rectos~gmoid, carcinomas. Appendiceal carcinomas that initially present with bladder symptoms secondary to direct tumor extension to the bladder wall are extremely rare. The first case was reported in 1977 by Richie. 2 A 50-yearold man presented with a 6-month history of pyuria and dysuria with negative cultures. Cystoscopy was negative. Repeat cystoscopy 6 months later for hematuria revealed a fungating mass in the right bladder wall. Biopsy showed adenocarcinoma without mucosal involvement and a colonic primary tumor was suspected. A barium enema revealed an appendiceal mass. The patient was well 2 years after cystectomy, right colectomy and irradiation. Since 1977, 4 more cases have been reported. 3- 6 An additional case was mentioned but no details were given. 7 Our 2 patients represent cases 7 and 8 in the literature. Of the 7 patients for whom clinical details were available 4 were women and 3 were men. Patient age ranged from 48 to 82 years with a mean of 60 years. One patient was asymptomatic and the bladder lesion was found at cystoscopy performed for carcinoma in situ of the uterine cervix. 3 Of the remaining cases the presenting symptoms were hematuria (4), pyuria (3), ~ys uria or urinary frequency (2), right lower quadrant abdomInal pain and fever (1), and multiorganism urosepsis (1). Physical examination showed a right pelvic mass in 2 cases. Cystoscopy revealed a bladder mass in all cases, although in 1 case the initial examination was negative but a repeat study 6 months later was positive. The cystoscopic biopsy was positive for adenocarcinoma initially in 5 cases. In the remaining 2 cases repeat cystoscopic biopsy was positive for adenocarcinoma. !he mass was located in the right bladder wall in 6 cases. BarIum enema showed an appendiceal mass in 1 case, extrinsic compression of the cecum in 1, a lesion at the tip of cecum in 1 and an enterovesical fistula in 1. All patients were treated with appendectomy, segmental bladder resection or cystectomy, and segmental cecal resection or right colectomy. Two patients were treated further with irradiation. Followup information was available in 4 patients, of whom 3 were alive with no
evidence of disease 2, 6 and 10 years, respectively, after diagnosis, and 1 died of diffuse peritoneal recurrence 6 years after diagnosis. A high index of suspicion is required for a preoperative diagnosis of an appendiceal primary tumor. In patients who initially present with bladder adenocarcinomas the following findings suggest the possibility of an appendiceal primary: multiorganism urosepsis, right pelvic mass, right bladder wall location, and abnormal barium enema findings in the region of cecum and appendix. The main mode of treatment is surgical resection of tumor-bearing areas. The prognosis is relatively good because most tumors are localized and resectable despite direct tumor extension to the bladder. Radiation therapy has been discouraged and systemic chemotherapy generally is of little use. 8- 10 REFERENCES
1. Melicow, M. M.: Tumors of the urinary bladder: a clinicopathological analysis of over 2500 specimens and biopsies. J. Urol., 74: 498,1955.
2. Richie, J. P.: Primary adenocarcinoma of the appendix masquerading as a bladder tumor. Arch. Surg., 112: 666, 1977. 3. Bischoff, W. and B6hm, N.: Adenocarcinoma of the appendix penetrating the bladder. J. Urol., 123: 123, 1980. 4. Henry, R., Bracken, R. B. and Ayala, A.: Appendiceal carcinoma mimicking primary bladder cancer. J. Urol., 123: 590, 1980. 5. Bartholomew, L. G., Farrow, G. M. and DeWeerd, J. H.: Adenocarcinoma of the appendix simulating primary bladder carcinoma. Dig. Dis. Sci., 29: 371, 1984. 6. Dalton, D. P., Dalkin, B. L., Sener, S. F., Pappas, P. S. and Blum, M. D.: Enterovesical fistula secondary to mucinous adenocarcinoma of appendix. J. Urol., 138: 617,1987. 7. Young, R. H.: Unusual variants of primary bladder carcinoma and secondary tumors of the bladder. In: Pathology of the Urinary Bladder. New York: Churchill Livingstone, pp. 103-138,1989. 8. Wolff, M. and Ahmed, N.: Epithelial neoplasms of the vermiform appendix (exclusive of carcinoid). 1. Adenocarcinoma of the appendix. Cancer, 37: 2493,1976. 9. Limber, G. K., King, R. E. and Silverberg, S. G.: Pseudomyxoma peritonaei: a report of ten cases. Ann. Surg., 178: 587, 1973. 10. Hesketh, K. T.: The management of primary adenocarcinoma of the vermiform appendix. Gut, 4: 158, 1968.
ABSTRACT
We report 2 cases of appendiceal adenocarcinoma invading the bladder. Both tumors as primary bladder carcinomas. Cystoscopic biopsies obtained adenocarcinomatous tissue and the secondary nature of the bladder lesion became apparent at laparotomy in both cases. One patient was treated with surgical resection of the appendix, the adjacent cecal wall and the bladder wall, and postoperative irradiation. She was well 10 years later. The other patient was treated with right colectomy and segmental bladder resection. She died of diffuse peritoneal recurrence 6 years later. VV"Or\,-,rv".n'"..nr".nril
KEY
WORDS: appendiceal neoplasms, adenocarcinoma, bladder neoplasms
Secondary tumors account for about 15% of malignant bladder tumors. l Of these tumors cases of direct bladder extension from carcinoma of adjacent organs outnumber cases of bladder metastasis from distant primaries. About a third of the secondary bladder tumors result from direct extension from carcinoma of the colon. 1 Appendiceal carcinoma with bladder extension is extremely rare with only 6 cases reported in the literature. 2- 7 We describe the clinicopathological features of 2 such cases and compare them with those of previously reported cases.
The tumor invaded the cecal and bladder wall. Small bowel obstruction developed 6 years postoperatively. At the entire peritoneal cavity was distended mucinous material. Biopsy revealed mucinous adenocarcinoma. The patient died 3 months later. aV'l"\If'\l"<:It-lf'\'Y'I
CASE REPORTS
Case 1. A 70-year-old woman was evaluated after an episode
of gross hematuria. Pelvic examination showed a right pelvic mass and cystoscopy revealed a mass in the right bladder wall. A biopsy demonstrated mucosal tissue with fibrosis and chronic inflammation, and separate fragments of mucinous material that contained nests of well differentiated adenocarcinoma (fig. 1). An upper gastrointestinal series, barium enema and excreurography were normal. exploration a mucinous lesion of the dix was found. The communicated with the The adherent cecal wall wall were the was well tiated mucinous of the received site. remained Case 2. gross hematuria. IVP showed slight indentation of right posterior bladder wall and cystoscopy revealed a 6 em. area of bullous edema in which a central 2.5 em. area was covered by mucus. Biopsy of the central area led to a cavity. The biopsy specimen showed mucosal tissue with chronic inflammation and separate fragments of mucinous adenocarcinoma (fig. 2). The abdominal roentgenograms demonstrated a 5 em. area of calcification in the right pelvis. A barium enema showed an area of external compression of the cecum. At exploration a 5 em. mass was found between the cecum and bladder. Excision of the mass, right colectomy and segmental bladder resection were done. Histologically the mass was well differentiated mucinous adenocarcinoma of the appendix. 'UL ............ 'U ..... 'U ........
Accepted for publication September 19, 1990. *Requests for reprints: Department of Pathology, Saint Agnes Medical Center, 1303 E. Herndon Ave., Fresno, California 93720.
1. Case 1. Well differentiated adE~nocal'CHlonlatlous tissue mucinous background of cystoscopic biopsy. from x160.
FIG. 2. Case 2. Well differentiated adenocarcinomatous tissue in mucinous background of cystoscopic biopsy. H & E, reduced from X160. 821
822
CHEN AND SPAULDING DISCUSSION
Although gastrointestinal carcinomas not infrequently spread directly to the bladder, 1 they are almost exclusively colonic, especially rectos~gmoid, carcinomas. Appendiceal carcinomas that initially present with bladder symptoms secondary to direct tumor extension to the bladder wall are extremely rare. The first case was reported in 1977 by Richie. 2 A 50-yearold man presented with a 6-month history of pyuria and dysuria with negative cultures. Cystoscopy was negative. Repeat cystoscopy 6 months later for hematuria revealed a fungating mass in the right bladder wall. Biopsy showed adenocarcinoma without mucosal involvement and a colonic primary tumor was suspected. A barium enema revealed an appendiceal mass. The patient was well 2 years after cystectomy, right colectomy and irradiation. Since 1977, 4 more cases have been reported. 3- 6 An additional case was mentioned but no details were given. 7 Our 2 patients represent cases 7 and 8 in the literature. Of the 7 patients for whom clinical details were available 4 were women and 3 were men. Patient age ranged from 48 to 82 years with a mean of 60 years. One patient was asymptomatic and the bladder lesion was found at cystoscopy performed for carcinoma in situ of the uterine cervix. 3 Of the remaining cases the presenting symptoms were hematuria (4), pyuria (3), ~ys uria or urinary frequency (2), right lower quadrant abdomInal pain and fever (1), and multiorganism urosepsis (1). Physical examination showed a right pelvic mass in 2 cases. Cystoscopy revealed a bladder mass in all cases, although in 1 case the initial examination was negative but a repeat study 6 months later was positive. The cystoscopic biopsy was positive for adenocarcinoma initially in 5 cases. In the remaining 2 cases repeat cystoscopic biopsy was positive for adenocarcinoma. !he mass was located in the right bladder wall in 6 cases. BarIum enema showed an appendiceal mass in 1 case, extrinsic compression of the cecum in 1, a lesion at the tip of cecum in 1 and an enterovesical fistula in 1. All patients were treated with appendectomy, segmental bladder resection or cystectomy, and segmental cecal resection or right colectomy. Two patients were treated further with irradiation. Followup information was available in 4 patients, of whom 3 were alive with no
evidence of disease 2, 6 and 10 years, respectively, after diagnosis, and 1 died of diffuse peritoneal recurrence 6 years after diagnosis. A high index of suspicion is required for a preoperative diagnosis of an appendiceal primary tumor. In patients who initially present with bladder adenocarcinomas the following findings suggest the possibility of an appendiceal primary: multiorganism urosepsis, right pelvic mass, right bladder wall location, and abnormal barium enema findings in the region of cecum and appendix. The main mode of treatment is surgical resection of tumor-bearing areas. The prognosis is relatively good because most tumors are localized and resectable despite direct tumor extension to the bladder. Radiation therapy has been discouraged and systemic chemotherapy generally is of little use. 8- 10 REFERENCES
1. Melicow, M. M.: Tumors of the urinary bladder: a clinicopathological analysis of over 2500 specimens and biopsies. J. Urol., 74: 498,1955.
2. Richie, J. P.: Primary adenocarcinoma of the appendix masquerading as a bladder tumor. Arch. Surg., 112: 666, 1977. 3. Bischoff, W. and B6hm, N.: Adenocarcinoma of the appendix penetrating the bladder. J. Urol., 123: 123, 1980. 4. Henry, R., Bracken, R. B. and Ayala, A.: Appendiceal carcinoma mimicking primary bladder cancer. J. Urol., 123: 590, 1980. 5. Bartholomew, L. G., Farrow, G. M. and DeWeerd, J. H.: Adenocarcinoma of the appendix simulating primary bladder carcinoma. Dig. Dis. Sci., 29: 371, 1984. 6. Dalton, D. P., Dalkin, B. L., Sener, S. F., Pappas, P. S. and Blum, M. D.: Enterovesical fistula secondary to mucinous adenocarcinoma of appendix. J. Urol., 138: 617,1987. 7. Young, R. H.: Unusual variants of primary bladder carcinoma and secondary tumors of the bladder. In: Pathology of the Urinary Bladder. New York: Churchill Livingstone, pp. 103-138,1989. 8. Wolff, M. and Ahmed, N.: Epithelial neoplasms of the vermiform appendix (exclusive of carcinoid). 1. Adenocarcinoma of the appendix. Cancer, 37: 2493,1976. 9. Limber, G. K., King, R. E. and Silverberg, S. G.: Pseudomyxoma peritonaei: a report of ten cases. Ann. Surg., 178: 587, 1973. 10. Hesketh, K. T.: The management of primary adenocarcinoma of the vermiform appendix. Gut, 4: 158, 1968.