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Collecting Duct Carcinoma of the Kidney: A Case Report and Review of the Literature
1096
CARTER AND ASSOCIATES
A, view of duodenal patch at dome of bladder with polypropylene suture and blood clot. Bullous edema is around graft site. B, polyprophylene suture at anastomosis of duodenal patch and bladder.
anterior placement of the orifice to allow room for the pancreaticoduodenal graft and the implanted ureter. This relatively new technique of pancreaticocystostomy adds several new sources of bleeding to the differential diagnosis of post-transplant hematuria. REFERENCES 1. Sutherland, D. E. R. and Kendall, D.: Clinical pancreas and islet transplant registry report. Transplant. Proc., 17: 307, 1985. 2. Gruessner, R. W. G., Dunn, D. L., Tzardis, P. J., Nakhleh, R. I., Najarian, J. S. and Sutherland, D. E. R.: Complications occurring after whole organ duodenopancreatic transplantation: relation to the allograft duodenal segment. Transplant. Proc., 22: 578, 1990.
3. Sollinger, H. W., Kalayoglu, M., Hoffmann, R. M., Deierhoi, M. H. and Belzer, F. 0.: Experience with pancreaticocystostomy in 24 consecutive pancreas transplants. Transplant. Proc., suppl. 2, 17: 141, 1985. 4. Prieto, M., Sutherland, D. E. R., Fernandez-Cruz, L., Heil, J. and Najarian, J. S.: Urinary amylase monitoring for early diagnosis of pancreas allograft rejection in dogs. J. Surg. Res., 40: 597, 1986. 5. Tom, W. W., Munda, R., First, M. R. and Alexander, J. W.: Autodigestion of the glans penis and urethra by activated transplant pancreatic exocrine enzymes. Surgery, 102: 99, 1987. 6. Konigsrainer, A., Feichtinger, H., Waitz, W., Ebner, A., Scheiber, K., Mack, D. and Margreiter, R.: Does pancreatic juice have a detrimental effect on bladder mucosa? Transplant. Proc., 22: 1600, 1990.
0022-534 7/92/1474-1096$03.00/0 THE JOURNAL OF UROLOGY Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Vol. 147, 1096-1098, April 1992
Printed in U.S.A.
COLLECTING DUCT CARCINOMA OF THE KIDNEY: A CASE REPORT AND REVIEW OF THE LITERATURE MICHAEL D. CARTER, SUSAN THA, MARTIN G. MCLOUGHLIN
AND
DAVID A. OWEN
From the Departments of Surgery (Urology) and Pathology, Vancouver General Hospital, Vancouver, British Columbia, Canada
ABSTRACT
Collecting duct carcinoma of the kidney is unique and rarely reported. The histological and ultrastructural characteristics of this tumor are well described in the pathological literature. However, there are few reports documenting the clinical presentation and long-term prognosis in patients with this unusual malignancy. Collecting duct carcinoma arises from the epithelium of the collecting tubules within the medulla and secondarily invades the renal cortex. The tumor occurs in a younger age group than is typical for renal cell carcinoma and most patients appear to have metastatic disease at presentation. We describe a case of this uncommon tumor and review the literature. KEY WORDS: kidney neoplasms, kidney tubules, carcinoma
Most primary epithelial malignancies of the renal parenchyma are believed to arise from the proximal convoluted Accepted for publication August 2, 1991.
tubule. Recent reports have suggested that some papillary renal cell carcinomas may, in fact, arise from the epithelium of the collecting duct (of Bellini) with secondary invasion of the renal
COLLECTING DUCT CARCINOMA OF KWNEY
1097
A section from apillary mass shows columnar to cuboidal1 eosinophil_ic cells ':l'it~ pro_minent nucleoli. Reduc~d from XlOO. B, section from med~lla shows sim~le tubules lined by malignant cells with acmar formation and mf1ltrat10n through desmoplastic stroma. Reduced from X200.
cortex. The histological, immunocytochemical and ultrastructural features of this tumor closely resemble those of the collecting ducts. This atypical renal malignancy has bee~ called collecting duct or Bellini's duct carcinoma to empha~1ze the unique histological origin. We des?rib~ a ~ase of coll~c~mg ~uct carcinoma recently treated at our mstitut10n. The chmcal fmdings and typical pathological features are described. CASE REPORT
A 26-year-old white man presented with a m?bile, nontend~r, superficial mass in the right submandibular triangle_ 6 weeks 1;11 duration. Medical history was unremarkable. Physical examination and ear nose and throat examination were unremarkable. Fine needle aspirate confirmed the presence of malignant cells. Right suprahyoid neck dissection revealed a firm, whitegray 2.5 x 2.0 X 1.5 cm. mass that proved to be a lymph n~de largely replaced by papillary adenocarcinoma. The surro1:mdmg lymph nodes were grossly negative as were the thyroid and submandibular glands. Computerized tomography (CT) scans of the chest and head were unremarkable. CT of the abdomen revealed a large tumor in the superior pole of the left kidney without r~nal vei_n involvement. The liver appeared to be normal. M1croscop1c examination of the urine and cytology studies were normal. The patient underwent left thoracoabdominal radical n~phrectomy. A smooth, tense, cystic 8 cm. mass wa~ present i_n the superior pole. Regional nodes were grossly negative. A cystic mass contiguous with renal capsule was present on gross examination, the contents of which were hemorrhagic. The adrenal, renal vein and perirenal adipose tissue were free of tumor. Projecting into the cyst inferiorly within t_he medulla. was a markedly papillary mass that on microscopic exammat10n revealed papillary adenocarcinoma with cuboidal to columnar cells, pleomorphic nuclei, prominent nucleol~ and abund~nt eosinophilic cytoplasm (part A of figure). Sect10ns of the adJacent medulla showed carcinoma in situ of the collecting ducts. Also seen in the medulla was a histological pattern of simple tubules lined by malignant cells and collections of cells with acinar formation infiltrating through a desmoplastic stroma (part B of figure). These histological features were indistinguishable from those of the lymph node in the neck, and are typical of collecting duct carcinoma as described by Kennedy et al 1 and Weiss and Mills. 2 The immediate postoperative cour;e was uneventful but there was evidence of recurrent disease in the inguinal lymph nodes 3 months postoperatively. Cerebral and bone metastases have since developed and the patient is receiving cisplatin-based chemotherapy. DISCUSSION
The proximal tubular cell is generally considered the_ cell type giving rise to renal cell carcinoma .. Recently, the epi~helium of the collecting ducts has been discussed as a possible
source of renal cell carcinoma. Of note is the differing embryological origin of the proximal tubules and collecting ducts, which are, respectively, the metanephros and mesonephros (ureteral bud). Collecting duct carcinoma has, in fact, been designated in the World Health Organization classification of kidney tumors as Bellini's duct carcinoma. 3 However, there are a paucity of clinical data concerning patients with these atypical . . malignancies. Mancilla-Jimenez et al studied 34 cases of papillary carcinoma of the kidney from 221 nephrectomy specimens performed for renal malignancy. 4 Of these 34 cases 3 were associated with atypical hyperplastic changes in the collecting duct epithelium, 1 of which was carcinoma in situ. The authors hypothesized that some of these papillary tumors have, in fact, arisen from the collecting tubules themselves. While the clinical data are not reported on these specific patients, they did identify 3 patients with a particularly aggressive course, unlike the typically favorable prognosis in the remaining patients with papillary renal cell carcinoma. These patients died with "'.idely disseminated metastases at 5, 8 and 18 months postoperatively. The age of these patients is not recorded but the youngest patient in their series was 27 years old. It would be interest~ng to know whether the patients with the particularly aggressive clinical course were the same as those with the atypical changes in the epithelium of the collecting ducts. Several case reports in the literature document atypical renal malignancies suggestive of collecting duct carcinoma. Cromie et al reported an unusual renal tumor with histological features that they suggested may have arisen from the collecting duct epithelium. 5 The patient was a 61-year-old man and followup clinical data were not provided. O'Brien and Bedard reported on a 13-year-old girl with a renal cell carcinoma suggestive of collecting duct carcinoma. 6 Hai and Diaz- Perez also described an atypical renal cell carcinoma in a 22-year-old man with features strongly suggestive of a collecting duct origin. 7 This unfortunate man had a poor clinical course after nephrectomy and he died 3 months postoperatively with lung and bone metastases. Fleming and Lewi reviewed 225 nephrectomies performed for neoplastic disease and found 6 patients with features consistent with a collecting duct origin, for an overall incidence of approximately 2%. 8 All were papillary lesions arising in the medulla and all showed infiltrating tubules with an associated desmoplastic reaction. Again, clinical data on these patients were not provided. Kennedy et al identified 6 cases of collecting duct carcinoma. 1 Mean patient age at presentation was 34 years. Interestingly, 2 patients had cervical adenopathy at presentation, as did our patient. Clinical data revealed a poor outcome, with 4 patients dead of metastatic disease within 2 years postoperatively. One patient was alive with metastatic disease and 1 had no evidence of disease at 9 months postoperatively. In summary, collecting duct carcinoma is a rare, pathologically distinct tumor possibly comprising 2 to 3 % of all renal
1098
HOHENFELLNER AND ASSOCIATES
cell carcinomas. Grossly, the tumor is noted to arise from the medulla with secondary invasion of the cortex. The typical histological appearance is that of a papillary adenocarcinoma with infiltrating tubules and an associated desmoplastic reaction. Atypical hyperplastic changes are present in the collecting duct epithelium. There are little consistent clinical data. However, the disease appears to affect a younger age group than is typical for renal cell carcinoma and it seems to have a poorer prognosis, with most patients having metastatic disease at presentation. Specific recommendations with regard to therapy cannot be made but it is obvious that an operation alone in the majority of these patients is inadequate treatment. The proposed embryological origin of the tumor is the ureteral bud and this may influence the selection of chemotherapeutic agents. Those witnactivityaga:inst urothelial neoplasms would be a rational choice. REFERENCES
of the kidney. Hum. Path., 21: 449, 1990. 2. Weiss, M. A. and Mills, S. E.: Atlas of Genitourinary Tract Disorders. Philadelphia: J.B. Lippincott Co., pp. 11.25-11.26, 1988. 3. Mostofi, F. K., Sesterhenn, I. A. and Sobin, L. H.: Histological
4. 5. 6. 7.
typing of kidney tumours. In: International Histological Classification of Tumours, No. 25. Geneva: World Health Organization, 1981. Mancilla-Jimenez, R., Stanley, R. J. and Blath, R. A.: Papillary renal cell carcinoma: a clinical, radiologic, and pathologic study of 34 cases. Cancer, 38: 2469, 1976. Cromie, W. J., Davis, C. J. and DeTure, F. A.: Atypical carcinoma of kidney. Possibly originating from collecting duct epithelium. Urology, 13: 315, 1979. O'Brien, P. K. and Bedard, Y. C.: A papillary adenocarcinoma of the renal pelvis in a young girl. A light- and electron-microscopic -study.-Amer. J-. Cli-n-. Path.-, '7-3:--427,-1980. Hai, M. A. and Diaz-Perez, R.: Atypical carcinoma of kidney originating from collecting duct epithelium. Urology, 19: 89, 1982.
1. Kennedy, S. M., Merino, M. J., Linehan, W. M., Roberts, J. R.,
Robertson, C. N. and Neumann, R. D.: Collecting duct carcinoma
8. Fleming, S. and Lewi, H. J.: Collecting duct carcinoma of the kidney. Histopathology, 10: 1131, 1986.
0022-534 7/92/14 7 4-1098$03.00/0 THE JOURNAL OF UROLOGY Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Vol. 147, 1098-1102, April 1992
Printed in U.S.A.
TUMOR IN THE HORSESHOE KIDNEY: CLINICAL IMPLICATIONS AND REVIEW OF EMBRYOGENESIS M. HOHENFELLNER, D. SCHULTZ-LAMPEL, A. LAMPEL, F. STEINBACH, B. M. CRAMER J. W. THUROFF
AND
From the Departments of Urology and Radiology, Klinikum Barmen Wuppertal, and Department of Urology, Johannes Gutenberg University, School of Medicine, Mainz, Federal Republic of Germany
ABSTRACT
We report on 3 patients with tumor in a horseshoe kidney, 1 of whom had bilateral tumor (renal cell cancer on the right side and urothelial cancer on the left side). Tumors that arise predominantly in the bridge of a horseshoe kidney can mimic the symptoms of an intra-abdominal disease process. Besides routine diagnostic procedures, angiography is essential to plan the surgical approach, which in principle should be organ-sparing. The literature of the embryology of the horseshoe kidney was reviewed for a relationship between the abnormal renal development and the site of tumorigenesis, and for development of a key for the wide variation of blood supply. Recently reported data suggest that the theory of a mechanical fusion is valid only for horseshoe kidneys with a fibrous isthmus but that an abnormal migration of the posterior nephrogenic area causes the majority of horseshoe kidneys in which the isthmus consists ofparenchyma. Development of the isthmus through abnormal migration could predispose this location for renal cell cancer and would explain the varying forms of blood supply. Additionally, this hypothesis supports the previously raised assumption that horseshoe kidneys may be the result of teratogenic factors, which also may be responsible for the known increased incidence of related congenital anomalies and of nephroblastoma. KEY WORDS: kidney neoplasms; carcinoma, renal cell; embryology
The horseshoe kidney was recognized as an anomaly of the upper urinary tract as early as the 16th century. 1- 4 In most cases the kidneys are linked at the lower poles by an isthmus of parenchyma. 5- 8 The incidence is 1 to 4 in 1,000 cases, being 2 to 3 times as frequent in male as in female patients. 9 The association with other congenital anomalies is reflected in the inverse relationship between incidence and patient age. 5 • 10- 12 Furthermore, the horseshoe kidney has a conceivably increased risk of nephroblastoma and an approximately 3 to 4-fold higher risk of cancer of the renal pelvis. 13- 16 During the last 3 years 3 patients with cancer in a horseshoe kidney were treated at our institutions, 1 of whom represents Accepted for publication August 9, 1991.
a case of simultaneous bilateral renal cell cancer and urothelial cancer. Preoperative findings, their implications and special aspects of the surgical approach are discussed. The literature was reviewed to determine if a relationship exists between the abnormal renal development in horseshoe kidneys and the site of tumorigenesis, and to explain the wide variation in blood supply. CASE REPORTS
Case 1. A 78-year-old man presented for right flank pain and
gross hematuria. History was relevant for diabetes mellitus and hyperthyroidism, both of which were well controlled pharmacologically. Physical examination showed a palpable mass in
CARTER AND ASSOCIATES
A, view of duodenal patch at dome of bladder with polypropylene suture and blood clot. Bullous edema is around graft site. B, polyprophylene suture at anastomosis of duodenal patch and bladder.
anterior placement of the orifice to allow room for the pancreaticoduodenal graft and the implanted ureter. This relatively new technique of pancreaticocystostomy adds several new sources of bleeding to the differential diagnosis of post-transplant hematuria. REFERENCES 1. Sutherland, D. E. R. and Kendall, D.: Clinical pancreas and islet transplant registry report. Transplant. Proc., 17: 307, 1985. 2. Gruessner, R. W. G., Dunn, D. L., Tzardis, P. J., Nakhleh, R. I., Najarian, J. S. and Sutherland, D. E. R.: Complications occurring after whole organ duodenopancreatic transplantation: relation to the allograft duodenal segment. Transplant. Proc., 22: 578, 1990.
3. Sollinger, H. W., Kalayoglu, M., Hoffmann, R. M., Deierhoi, M. H. and Belzer, F. 0.: Experience with pancreaticocystostomy in 24 consecutive pancreas transplants. Transplant. Proc., suppl. 2, 17: 141, 1985. 4. Prieto, M., Sutherland, D. E. R., Fernandez-Cruz, L., Heil, J. and Najarian, J. S.: Urinary amylase monitoring for early diagnosis of pancreas allograft rejection in dogs. J. Surg. Res., 40: 597, 1986. 5. Tom, W. W., Munda, R., First, M. R. and Alexander, J. W.: Autodigestion of the glans penis and urethra by activated transplant pancreatic exocrine enzymes. Surgery, 102: 99, 1987. 6. Konigsrainer, A., Feichtinger, H., Waitz, W., Ebner, A., Scheiber, K., Mack, D. and Margreiter, R.: Does pancreatic juice have a detrimental effect on bladder mucosa? Transplant. Proc., 22: 1600, 1990.
0022-534 7/92/1474-1096$03.00/0 THE JOURNAL OF UROLOGY Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Vol. 147, 1096-1098, April 1992
Printed in U.S.A.
COLLECTING DUCT CARCINOMA OF THE KIDNEY: A CASE REPORT AND REVIEW OF THE LITERATURE MICHAEL D. CARTER, SUSAN THA, MARTIN G. MCLOUGHLIN
AND
DAVID A. OWEN
From the Departments of Surgery (Urology) and Pathology, Vancouver General Hospital, Vancouver, British Columbia, Canada
ABSTRACT
Collecting duct carcinoma of the kidney is unique and rarely reported. The histological and ultrastructural characteristics of this tumor are well described in the pathological literature. However, there are few reports documenting the clinical presentation and long-term prognosis in patients with this unusual malignancy. Collecting duct carcinoma arises from the epithelium of the collecting tubules within the medulla and secondarily invades the renal cortex. The tumor occurs in a younger age group than is typical for renal cell carcinoma and most patients appear to have metastatic disease at presentation. We describe a case of this uncommon tumor and review the literature. KEY WORDS: kidney neoplasms, kidney tubules, carcinoma
Most primary epithelial malignancies of the renal parenchyma are believed to arise from the proximal convoluted Accepted for publication August 2, 1991.
tubule. Recent reports have suggested that some papillary renal cell carcinomas may, in fact, arise from the epithelium of the collecting duct (of Bellini) with secondary invasion of the renal
COLLECTING DUCT CARCINOMA OF KWNEY
1097
A section from apillary mass shows columnar to cuboidal1 eosinophil_ic cells ':l'it~ pro_minent nucleoli. Reduc~d from XlOO. B, section from med~lla shows sim~le tubules lined by malignant cells with acmar formation and mf1ltrat10n through desmoplastic stroma. Reduced from X200.
cortex. The histological, immunocytochemical and ultrastructural features of this tumor closely resemble those of the collecting ducts. This atypical renal malignancy has bee~ called collecting duct or Bellini's duct carcinoma to empha~1ze the unique histological origin. We des?rib~ a ~ase of coll~c~mg ~uct carcinoma recently treated at our mstitut10n. The chmcal fmdings and typical pathological features are described. CASE REPORT
A 26-year-old white man presented with a m?bile, nontend~r, superficial mass in the right submandibular triangle_ 6 weeks 1;11 duration. Medical history was unremarkable. Physical examination and ear nose and throat examination were unremarkable. Fine needle aspirate confirmed the presence of malignant cells. Right suprahyoid neck dissection revealed a firm, whitegray 2.5 x 2.0 X 1.5 cm. mass that proved to be a lymph n~de largely replaced by papillary adenocarcinoma. The surro1:mdmg lymph nodes were grossly negative as were the thyroid and submandibular glands. Computerized tomography (CT) scans of the chest and head were unremarkable. CT of the abdomen revealed a large tumor in the superior pole of the left kidney without r~nal vei_n involvement. The liver appeared to be normal. M1croscop1c examination of the urine and cytology studies were normal. The patient underwent left thoracoabdominal radical n~phrectomy. A smooth, tense, cystic 8 cm. mass wa~ present i_n the superior pole. Regional nodes were grossly negative. A cystic mass contiguous with renal capsule was present on gross examination, the contents of which were hemorrhagic. The adrenal, renal vein and perirenal adipose tissue were free of tumor. Projecting into the cyst inferiorly within t_he medulla. was a markedly papillary mass that on microscopic exammat10n revealed papillary adenocarcinoma with cuboidal to columnar cells, pleomorphic nuclei, prominent nucleol~ and abund~nt eosinophilic cytoplasm (part A of figure). Sect10ns of the adJacent medulla showed carcinoma in situ of the collecting ducts. Also seen in the medulla was a histological pattern of simple tubules lined by malignant cells and collections of cells with acinar formation infiltrating through a desmoplastic stroma (part B of figure). These histological features were indistinguishable from those of the lymph node in the neck, and are typical of collecting duct carcinoma as described by Kennedy et al 1 and Weiss and Mills. 2 The immediate postoperative cour;e was uneventful but there was evidence of recurrent disease in the inguinal lymph nodes 3 months postoperatively. Cerebral and bone metastases have since developed and the patient is receiving cisplatin-based chemotherapy. DISCUSSION
The proximal tubular cell is generally considered the_ cell type giving rise to renal cell carcinoma .. Recently, the epi~helium of the collecting ducts has been discussed as a possible
source of renal cell carcinoma. Of note is the differing embryological origin of the proximal tubules and collecting ducts, which are, respectively, the metanephros and mesonephros (ureteral bud). Collecting duct carcinoma has, in fact, been designated in the World Health Organization classification of kidney tumors as Bellini's duct carcinoma. 3 However, there are a paucity of clinical data concerning patients with these atypical . . malignancies. Mancilla-Jimenez et al studied 34 cases of papillary carcinoma of the kidney from 221 nephrectomy specimens performed for renal malignancy. 4 Of these 34 cases 3 were associated with atypical hyperplastic changes in the collecting duct epithelium, 1 of which was carcinoma in situ. The authors hypothesized that some of these papillary tumors have, in fact, arisen from the collecting tubules themselves. While the clinical data are not reported on these specific patients, they did identify 3 patients with a particularly aggressive course, unlike the typically favorable prognosis in the remaining patients with papillary renal cell carcinoma. These patients died with "'.idely disseminated metastases at 5, 8 and 18 months postoperatively. The age of these patients is not recorded but the youngest patient in their series was 27 years old. It would be interest~ng to know whether the patients with the particularly aggressive clinical course were the same as those with the atypical changes in the epithelium of the collecting ducts. Several case reports in the literature document atypical renal malignancies suggestive of collecting duct carcinoma. Cromie et al reported an unusual renal tumor with histological features that they suggested may have arisen from the collecting duct epithelium. 5 The patient was a 61-year-old man and followup clinical data were not provided. O'Brien and Bedard reported on a 13-year-old girl with a renal cell carcinoma suggestive of collecting duct carcinoma. 6 Hai and Diaz- Perez also described an atypical renal cell carcinoma in a 22-year-old man with features strongly suggestive of a collecting duct origin. 7 This unfortunate man had a poor clinical course after nephrectomy and he died 3 months postoperatively with lung and bone metastases. Fleming and Lewi reviewed 225 nephrectomies performed for neoplastic disease and found 6 patients with features consistent with a collecting duct origin, for an overall incidence of approximately 2%. 8 All were papillary lesions arising in the medulla and all showed infiltrating tubules with an associated desmoplastic reaction. Again, clinical data on these patients were not provided. Kennedy et al identified 6 cases of collecting duct carcinoma. 1 Mean patient age at presentation was 34 years. Interestingly, 2 patients had cervical adenopathy at presentation, as did our patient. Clinical data revealed a poor outcome, with 4 patients dead of metastatic disease within 2 years postoperatively. One patient was alive with metastatic disease and 1 had no evidence of disease at 9 months postoperatively. In summary, collecting duct carcinoma is a rare, pathologically distinct tumor possibly comprising 2 to 3 % of all renal
1098
HOHENFELLNER AND ASSOCIATES
cell carcinomas. Grossly, the tumor is noted to arise from the medulla with secondary invasion of the cortex. The typical histological appearance is that of a papillary adenocarcinoma with infiltrating tubules and an associated desmoplastic reaction. Atypical hyperplastic changes are present in the collecting duct epithelium. There are little consistent clinical data. However, the disease appears to affect a younger age group than is typical for renal cell carcinoma and it seems to have a poorer prognosis, with most patients having metastatic disease at presentation. Specific recommendations with regard to therapy cannot be made but it is obvious that an operation alone in the majority of these patients is inadequate treatment. The proposed embryological origin of the tumor is the ureteral bud and this may influence the selection of chemotherapeutic agents. Those witnactivityaga:inst urothelial neoplasms would be a rational choice. REFERENCES
of the kidney. Hum. Path., 21: 449, 1990. 2. Weiss, M. A. and Mills, S. E.: Atlas of Genitourinary Tract Disorders. Philadelphia: J.B. Lippincott Co., pp. 11.25-11.26, 1988. 3. Mostofi, F. K., Sesterhenn, I. A. and Sobin, L. H.: Histological
4. 5. 6. 7.
typing of kidney tumours. In: International Histological Classification of Tumours, No. 25. Geneva: World Health Organization, 1981. Mancilla-Jimenez, R., Stanley, R. J. and Blath, R. A.: Papillary renal cell carcinoma: a clinical, radiologic, and pathologic study of 34 cases. Cancer, 38: 2469, 1976. Cromie, W. J., Davis, C. J. and DeTure, F. A.: Atypical carcinoma of kidney. Possibly originating from collecting duct epithelium. Urology, 13: 315, 1979. O'Brien, P. K. and Bedard, Y. C.: A papillary adenocarcinoma of the renal pelvis in a young girl. A light- and electron-microscopic -study.-Amer. J-. Cli-n-. Path.-, '7-3:--427,-1980. Hai, M. A. and Diaz-Perez, R.: Atypical carcinoma of kidney originating from collecting duct epithelium. Urology, 19: 89, 1982.
1. Kennedy, S. M., Merino, M. J., Linehan, W. M., Roberts, J. R.,
Robertson, C. N. and Neumann, R. D.: Collecting duct carcinoma
8. Fleming, S. and Lewi, H. J.: Collecting duct carcinoma of the kidney. Histopathology, 10: 1131, 1986.
0022-534 7/92/14 7 4-1098$03.00/0 THE JOURNAL OF UROLOGY Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Vol. 147, 1098-1102, April 1992
Printed in U.S.A.
TUMOR IN THE HORSESHOE KIDNEY: CLINICAL IMPLICATIONS AND REVIEW OF EMBRYOGENESIS M. HOHENFELLNER, D. SCHULTZ-LAMPEL, A. LAMPEL, F. STEINBACH, B. M. CRAMER J. W. THUROFF
AND
From the Departments of Urology and Radiology, Klinikum Barmen Wuppertal, and Department of Urology, Johannes Gutenberg University, School of Medicine, Mainz, Federal Republic of Germany
ABSTRACT
We report on 3 patients with tumor in a horseshoe kidney, 1 of whom had bilateral tumor (renal cell cancer on the right side and urothelial cancer on the left side). Tumors that arise predominantly in the bridge of a horseshoe kidney can mimic the symptoms of an intra-abdominal disease process. Besides routine diagnostic procedures, angiography is essential to plan the surgical approach, which in principle should be organ-sparing. The literature of the embryology of the horseshoe kidney was reviewed for a relationship between the abnormal renal development and the site of tumorigenesis, and for development of a key for the wide variation of blood supply. Recently reported data suggest that the theory of a mechanical fusion is valid only for horseshoe kidneys with a fibrous isthmus but that an abnormal migration of the posterior nephrogenic area causes the majority of horseshoe kidneys in which the isthmus consists ofparenchyma. Development of the isthmus through abnormal migration could predispose this location for renal cell cancer and would explain the varying forms of blood supply. Additionally, this hypothesis supports the previously raised assumption that horseshoe kidneys may be the result of teratogenic factors, which also may be responsible for the known increased incidence of related congenital anomalies and of nephroblastoma. KEY WORDS: kidney neoplasms; carcinoma, renal cell; embryology
The horseshoe kidney was recognized as an anomaly of the upper urinary tract as early as the 16th century. 1- 4 In most cases the kidneys are linked at the lower poles by an isthmus of parenchyma. 5- 8 The incidence is 1 to 4 in 1,000 cases, being 2 to 3 times as frequent in male as in female patients. 9 The association with other congenital anomalies is reflected in the inverse relationship between incidence and patient age. 5 • 10- 12 Furthermore, the horseshoe kidney has a conceivably increased risk of nephroblastoma and an approximately 3 to 4-fold higher risk of cancer of the renal pelvis. 13- 16 During the last 3 years 3 patients with cancer in a horseshoe kidney were treated at our institutions, 1 of whom represents Accepted for publication August 9, 1991.
a case of simultaneous bilateral renal cell cancer and urothelial cancer. Preoperative findings, their implications and special aspects of the surgical approach are discussed. The literature was reviewed to determine if a relationship exists between the abnormal renal development in horseshoe kidneys and the site of tumorigenesis, and to explain the wide variation in blood supply. CASE REPORTS
Case 1. A 78-year-old man presented for right flank pain and
gross hematuria. History was relevant for diabetes mellitus and hyperthyroidism, both of which were well controlled pharmacologically. Physical examination showed a palpable mass in