uROPATHOLOGY
URACHAL SIGNET-RING CELL CARCINOMA KARL T• K. CHEN, M.D. RONALD D. WORKMAN, M.D. GREG RAINWATER, M.D. From the Departments of Pathology and Urology, Fresno Community Hospital and Medical Center, Fresno, California
ABSTRACT--A case of urachal signet-ring cell carcinoma is reported and compared with the 18 i previously reported cases. This tumor appears aggressive, with a five-year survival rate of 27 per:cent. Only 1 patient achieved a five-year disease-free survival.
: Urachal carcinoma is an uncommon neoplasm ;associated with a poor prognosis, partly because ~;~~he location of the tumor favors a long silent ~: course before detection. 1 Although approxim ii a t e l y i 90 percent of the urachal malignancies ~i~lare adenocarcmomas, 2 only a small proportxon iiii!i~fthese are of signet-ring cell type. We report a ::: :~ase of signet-ring cell urachal carcinoma and ~,:rewew the hterature of this rare variant of ura:~i~hal carcinoma. ':
A
Case Report
seventy-three-year-old man presented with ten-day history of painless hematuria. In~!:;~i~!i't~ravenouspyelogram showed no evidence of renal or bladder tumor. Cystoscopy revealed a necrotic tumor at the dome of the bladder. :~,:!:i~:~ansurethralresection of the bladder tumor renet-ring cell carcinoma. Findings on ;trointestinal series, chest roentgenod barium enema studies were negae weeks later a partial cystectomy and of umbilicus and urachal remnant ormed. he surgery, progressive back pain, fatigue, and anemia developed. An marrow biopsy showed metastatic arcinoma. The patient was treated with com~ b i n a t i o n chemotherapy consisting of doxorubi~in h y d r o c h l o r i d e ( A d r i a m y c i n ) and 5~fluorouracil. He died seven months after initial :~ !~:diagnosis. i~i:.i~a ,0
/
O C T O B E R 1990
/
Pathologic findings The tumor was composed of relatively large cells with prominent intracellular and extracellular mucin accumulation• Many tumor cells had a signet-ring appearance (Fig. 1A). The nuclei were hyperchromatic and pleomorphic. There were frequent mitotic figures. In the transurethral resection specimen, portions of a tubular structure lined by dysplastic columnar epithelium were seen• The structure was surrounded by tumor tissue (Fig. 1B). The tubular structure was consistent with a urachal remnant. In the partial cystectomy specimen, the tumor showed transmural infiltration without significant intracystic gross tumor formation. Comment The urachus is a tapered cephalad extension of the urogenital sinus tract that is contiguous with the allantois. Following birth, it undergoes atrophy as it descends caudally with the urinary bladder. Microscopic urachal remnants have been found in autopsy cases in the bladder dome and midline anterior abdominal wall in 32 percent to 70 percent of adults204 In most cases, the remnants are lined by transitional epithelium with columnar cell metaplasia demonstrable in one third of the cases. 3'4 The configuration of the urachal remnants within the bladder wall varies from a narrow tube to those with microcystic dilatation or irregular lateral pouches. 4
V O L U M E XXXVI, N U M B E R 4
339
FIGURE 1. (A) High-power view showing signet-ring tumor cells. (B) Urachal remnant lined by dysplastic columnar epithelium. (Hematoxylin and eosin, original magnifications x 100 and x 25, respectively.) ~i!!!i
The first description of urachal cancer was made by Hue and Jaequin in 1863. 5 Begg, in 1930, 6 contended that all apical tumors of the bladder must be considered urachal tumors unless proved otherwise. Mostofi, Thomson, and Dean in 1955, 7 stated that a malignant epithelial tumor situated in the dome or anterior wall of the urinary bladder is uraehal if the tumor is mostly intramural, and is demonstrated not to be a secondary carcinoma. These criteria have been used in most of the subsequent publications of this subject. In the present case, the bladder dome location, the transmural tumor infiltration, and the absence of intraeystic tumor mass were compatible with the designation of urachal carcinoma. A unique finding in our case was that a tubular urachal remnant was identified in the tumor, confirming the urachal origin of the tumor. Urachal carcinoma has been estimated to account for 0.17 to 0.34 percent of all bladder cancers and 20 to 39 percent of primary bladder adenoearcinoma.1 Approximately 90 percent of the urachal malignancies are adenocarcinoma. ~ Only 6.7 percent of the urachal adenoearcinomas belong to the rare variant of signet-ring cell carcinoma. 2 Kondo, Ogisu, and Mitsuya in 19818 reviewed 21 cases of signet-ring cell carcinoma of the urinary bladder. Twelve of these 21 eases (59 % ) were judged to be of urachal origin. Our review of the literature revealed 2 additional cases9,16 which were not included in their review. Four additional eases were reported after 1981.1L12 Our case appears to be the nineteenth case of uraehal signet-ring cell carcinoma. Of these 19 patients, there were 14 men and five women. The ages ranged from twenty to seventy-three years, with an average of forty-eight years. The most common presenting symptom was hematuria. All except 1 were 340
treated with partial or total eysteetomy; in ad, dition 7 received irradiation and 3 others r e . : delved chemotherapy. Fourteen patients died in:f} less than two years; 3 died between two five years; and 4 died after five years. One was alive with tumor in seventeen months. Four pa' tients w e r e alive and w e l l , t w e n t y - t w o , : i seventy-two, thirty, and six months, respee~ ~; tively, after diagnosis. The five-year s u r v i v a l rate was 27 percent (4 of 15). A five-year dis~ ~' ease-free survival so far has occurred only in 1 } patient.
and:
Department of Pathology Saint Agnes Medical Center 1303 E. Herndon ?i Fresno, Caifornia 93710 i~
(DR C EN) ;
References 1. Sheldon CA, et ah Malignant urachal lesion, J Urol 1 3 1 : 1
(1984).
J
)[
2. Petersen RO: Urologic Pathology, Philadelphia, J.B. Lip, pineott Company, 1986, pp 288-290, 357-860. 8. Bourne CW, and Mary ME: Uraehal remnants: benign or malignant? J Urol 118. 743 (1977). b 4 a r Sueha:bertreGEn P ~ i e MB, and Bethke-Bedurftig BA: TuN an " adult bladders, J Urol 127 40 (1982), 5. Hue L, and Jaequin M: Cancer eolloide de la lombille et de paroi abdominale anterieure ayant envahi la vessie, Union Med de la Siene-Inf Rouen 6:418 (1863). 6. Begg RC: The colloid adenoearcinoma of the bladder vault arising from the epithelium of the uraehal canal: with a critical survey of the tumors of the uraehus, Br J Surg 18:422 (1931). 7. Mostofi FK, Thomson RV, and Dean AL: Mucous a d e n o carcinoma of the urinary bladder, Cancer 8:741 (1955). 8. Kondo A, Ogisu B, and Mitsuya H: Signet-ring cell car: einoma involving the urinary bladder, report of a case and review of 21 eases, Urol Int 36:373 (1981). 9. McGeoy TJ, and Lewis CW Jr: Mucinous adenocarcinoma of the uraehus, J Urol 96:317 (1966). 10. Loening SA, Jacobo E, Hawtrey CE, and Culp DA: Adenocarcinoma of the urachus, J Urol 119:68 (1978). :: 11. Johnson DE, Hodge GB, Abdul-Kavim FW, and Ayala ! AG: Urachal carcinoma, Urology 26:218 (1985). 12. DeMay RM, and Grathwohl MA: Signet-ring-cell (colloid) carcinoma of the urinary bladder, cytologic, histologic and ultra' structural findings in 1 case, Aeta Cytol 29:132 (1985). ~:
UROLOGY
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OCTOBER1990
/
VOLUME XXXVI, NUMBER4 4t
URACHAL SIGNET-RING CELL CARCINOMA KARL T• K. CHEN, M.D. RONALD D. WORKMAN, M.D. GREG RAINWATER, M.D. From the Departments of Pathology and Urology, Fresno Community Hospital and Medical Center, Fresno, California
ABSTRACT--A case of urachal signet-ring cell carcinoma is reported and compared with the 18 i previously reported cases. This tumor appears aggressive, with a five-year survival rate of 27 per:cent. Only 1 patient achieved a five-year disease-free survival.
: Urachal carcinoma is an uncommon neoplasm ;associated with a poor prognosis, partly because ~;~~he location of the tumor favors a long silent ~: course before detection. 1 Although approxim ii a t e l y i 90 percent of the urachal malignancies ~i~lare adenocarcmomas, 2 only a small proportxon iiii!i~fthese are of signet-ring cell type. We report a ::: :~ase of signet-ring cell urachal carcinoma and ~,:rewew the hterature of this rare variant of ura:~i~hal carcinoma. ':
A
Case Report
seventy-three-year-old man presented with ten-day history of painless hematuria. In~!:;~i~!i't~ravenouspyelogram showed no evidence of renal or bladder tumor. Cystoscopy revealed a necrotic tumor at the dome of the bladder. :~,:!:i~:~ansurethralresection of the bladder tumor renet-ring cell carcinoma. Findings on ;trointestinal series, chest roentgenod barium enema studies were negae weeks later a partial cystectomy and of umbilicus and urachal remnant ormed. he surgery, progressive back pain, fatigue, and anemia developed. An marrow biopsy showed metastatic arcinoma. The patient was treated with com~ b i n a t i o n chemotherapy consisting of doxorubi~in h y d r o c h l o r i d e ( A d r i a m y c i n ) and 5~fluorouracil. He died seven months after initial :~ !~:diagnosis. i~i:.i~a ,0
/
O C T O B E R 1990
/
Pathologic findings The tumor was composed of relatively large cells with prominent intracellular and extracellular mucin accumulation• Many tumor cells had a signet-ring appearance (Fig. 1A). The nuclei were hyperchromatic and pleomorphic. There were frequent mitotic figures. In the transurethral resection specimen, portions of a tubular structure lined by dysplastic columnar epithelium were seen• The structure was surrounded by tumor tissue (Fig. 1B). The tubular structure was consistent with a urachal remnant. In the partial cystectomy specimen, the tumor showed transmural infiltration without significant intracystic gross tumor formation. Comment The urachus is a tapered cephalad extension of the urogenital sinus tract that is contiguous with the allantois. Following birth, it undergoes atrophy as it descends caudally with the urinary bladder. Microscopic urachal remnants have been found in autopsy cases in the bladder dome and midline anterior abdominal wall in 32 percent to 70 percent of adults204 In most cases, the remnants are lined by transitional epithelium with columnar cell metaplasia demonstrable in one third of the cases. 3'4 The configuration of the urachal remnants within the bladder wall varies from a narrow tube to those with microcystic dilatation or irregular lateral pouches. 4
V O L U M E XXXVI, N U M B E R 4
339
FIGURE 1. (A) High-power view showing signet-ring tumor cells. (B) Urachal remnant lined by dysplastic columnar epithelium. (Hematoxylin and eosin, original magnifications x 100 and x 25, respectively.) ~i!!!i
The first description of urachal cancer was made by Hue and Jaequin in 1863. 5 Begg, in 1930, 6 contended that all apical tumors of the bladder must be considered urachal tumors unless proved otherwise. Mostofi, Thomson, and Dean in 1955, 7 stated that a malignant epithelial tumor situated in the dome or anterior wall of the urinary bladder is uraehal if the tumor is mostly intramural, and is demonstrated not to be a secondary carcinoma. These criteria have been used in most of the subsequent publications of this subject. In the present case, the bladder dome location, the transmural tumor infiltration, and the absence of intraeystic tumor mass were compatible with the designation of urachal carcinoma. A unique finding in our case was that a tubular urachal remnant was identified in the tumor, confirming the urachal origin of the tumor. Urachal carcinoma has been estimated to account for 0.17 to 0.34 percent of all bladder cancers and 20 to 39 percent of primary bladder adenoearcinoma.1 Approximately 90 percent of the urachal malignancies are adenocarcinoma. ~ Only 6.7 percent of the urachal adenoearcinomas belong to the rare variant of signet-ring cell carcinoma. 2 Kondo, Ogisu, and Mitsuya in 19818 reviewed 21 cases of signet-ring cell carcinoma of the urinary bladder. Twelve of these 21 eases (59 % ) were judged to be of urachal origin. Our review of the literature revealed 2 additional cases9,16 which were not included in their review. Four additional eases were reported after 1981.1L12 Our case appears to be the nineteenth case of uraehal signet-ring cell carcinoma. Of these 19 patients, there were 14 men and five women. The ages ranged from twenty to seventy-three years, with an average of forty-eight years. The most common presenting symptom was hematuria. All except 1 were 340
treated with partial or total eysteetomy; in ad, dition 7 received irradiation and 3 others r e . : delved chemotherapy. Fourteen patients died in:f} less than two years; 3 died between two five years; and 4 died after five years. One was alive with tumor in seventeen months. Four pa' tients w e r e alive and w e l l , t w e n t y - t w o , : i seventy-two, thirty, and six months, respee~ ~; tively, after diagnosis. The five-year s u r v i v a l rate was 27 percent (4 of 15). A five-year dis~ ~' ease-free survival so far has occurred only in 1 } patient.
and:
Department of Pathology Saint Agnes Medical Center 1303 E. Herndon ?i Fresno, Caifornia 93710 i~
(DR C EN) ;
References 1. Sheldon CA, et ah Malignant urachal lesion, J Urol 1 3 1 : 1
(1984).
J
)[
2. Petersen RO: Urologic Pathology, Philadelphia, J.B. Lip, pineott Company, 1986, pp 288-290, 357-860. 8. Bourne CW, and Mary ME: Uraehal remnants: benign or malignant? J Urol 118. 743 (1977). b 4 a r Sueha:bertreGEn P ~ i e MB, and Bethke-Bedurftig BA: TuN an " adult bladders, J Urol 127 40 (1982), 5. Hue L, and Jaequin M: Cancer eolloide de la lombille et de paroi abdominale anterieure ayant envahi la vessie, Union Med de la Siene-Inf Rouen 6:418 (1863). 6. Begg RC: The colloid adenoearcinoma of the bladder vault arising from the epithelium of the uraehal canal: with a critical survey of the tumors of the uraehus, Br J Surg 18:422 (1931). 7. Mostofi FK, Thomson RV, and Dean AL: Mucous a d e n o carcinoma of the urinary bladder, Cancer 8:741 (1955). 8. Kondo A, Ogisu B, and Mitsuya H: Signet-ring cell car: einoma involving the urinary bladder, report of a case and review of 21 eases, Urol Int 36:373 (1981). 9. McGeoy TJ, and Lewis CW Jr: Mucinous adenocarcinoma of the uraehus, J Urol 96:317 (1966). 10. Loening SA, Jacobo E, Hawtrey CE, and Culp DA: Adenocarcinoma of the urachus, J Urol 119:68 (1978). :: 11. Johnson DE, Hodge GB, Abdul-Kavim FW, and Ayala ! AG: Urachal carcinoma, Urology 26:218 (1985). 12. DeMay RM, and Grathwohl MA: Signet-ring-cell (colloid) carcinoma of the urinary bladder, cytologic, histologic and ultra' structural findings in 1 case, Aeta Cytol 29:132 (1985). ~:
UROLOGY
/
OCTOBER1990
/
VOLUME XXXVI, NUMBER4 4t
