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Obstructive anuria and dermatomyositis: A unique association
ELSEVIER
OBSTRUCTIVE ANURIA A UNIQUE B. G. PARULKAR,
AND DERMATOMYOSITIS: ASSOCIATION J. KEN,
AND
D. TRIFILIO
ABSTRACT
This case is the first reported presentation of obstructive anuria due to extrinsic ureteral compression in a patient with dermatomyositis. Further evaluation revealed disseminated sigmoid colon adenocarcinoma. Conservative treatment of ureteral obstruction by internal double-J stents achieved appropriate palliation. UROLOGY 50: 135-l 37, 1997. 0 1997, Elsevier Science Inc. All rights reserved.
D
ermatomyositis (DM) is an inflammatory muscle disease characterized by symmetric proximal muscle weakness and typical skin lesions, such as heliotrope eruptions and Gottron’s sign.’ Malignancies can occur concurrently with DM, and quite often their course may parallel or complement each other.2 Obstructive anuria due to either idiopathic or secondary retroperitoneal fibrosis is a well-described entity in urologic literature. This paper reports a patient who presented with bilateral ureteral obstruction with features suggestive of retroperitoneal fibrosis (RPF). After thorough evaluation, an underlying disseminated adenocarcinoma of the sigmoid colon was detected and the patient succumbed to her disease a few weeks later. This case illustrates the rarity of the association of urologic disease in patients with DM and strongly emphasizes the importance of a thorough evaluation to rule out underlying secondary pathology in these patients, with special emphasis on the detection of malignancy. CASE REPORT A 64-year-old female retired health worker was diagnosed to have DM in November 1995. Prednisone therapy brought about a rapid improvement in the skin lesions but produced minimal change in fatigue symptoms.
From the Department of Urology, University of Massachusetts and the Department of Urology, St. Vincent’s Hospital, Worcester, Massachusetts Reprint requests: J. Isen, M.D., Department of Urology, FaIlon Clinic, 135 Goldstur Boulevard, Worcester, MA 01606 Submitted: August 1, 1996, accepted (with revisions): February 5, 1997 0 1997, ELSEVIER SCIENCE ALL RIGHTS RESERVED
INC.
In the first week of March 1996, she presented to the emergency department with a 2-week history of malaise, nausea, and bloating and a 24hour history of nonfecal vomiting, marked decrease in urine output, and puffiness of face. Physical examination was normal, except for significant gaseous distention coupled with minimal ascites. Rectal and pelvic examination was normal. Her laboratory evaluation revealed a normal white blood count of 6800/mm3 (normal 3800 to 10,100) and a hematocrit of 36.6% (normal 39.5 to 47.7). She was in acute renal failure, as evident from a creatinine of 11.4 mg/dL (normal 0.5 to 1.4) and a blood-urea nitrogen (BUN) of 76 mg/ dL (normal 5 to 25). Her serum KC level at presentation was 8.1 mmoVL (normal 3.5 to 5.3). Real-time ultrasound scanning of the abdomen and pelvis showed a moderate degree of bilateral hydroureteronephrosis, with well-preserved renal parenchyma and minimal ascites. Cystoscopy and bilateral retrograde ureteropyelogram was performed. This revealed bilateral concentric narrowing and slight medial deviation of the segment of ureters (4 cm long and 1 mm wide) overlapping the regions of the fifth lumbar vertebra and the sacrum (Fig. 1). The distal pelvic ureters were normal in course and caliber. Proximal ureters and both pelvicaliceal systems showed moderate to severe dilation (right greater than left). The tentative diagnosis of RPF of unknown etiology was arrived at. Floppy-tip guidewires (0.038 in.) were advanced in the renal pelves bilaterally under fluoroscopy. In spite of some difficulty with their passage, 6F double-J stents were positioned bilaterally over the guidewires. A significant postobstructive diuresis ensued, and she had a urine output of greater than 0090-4295/97/$17.00 PII SOO90-4295(97)00200-S
135
After appropriate discussion with the patient, a palliative loop transverse colostomy was performed. Minilaparotomy during colostomy showed abdominal carcinomatosis with diffuse secondary lesions. Since the diagnosis of disseminated malignancy was confirmed and the prognosis was evidently poor, further tests such as CTguided retroperitoneal needle biopsies were declined by the patient. She was placed on palliative cycled chemotherapy with 5-FU and appropriate pain medications. She died within 2 months. COMMENT
FIGURE 1. Bilateral retrograde pyelogram showing medial displacement and narrowing of the sacral segment of both ureters (arrows) and proximal dilation of both pelvicaliceal systems.
3.5 L over the first 24 hours. Her serum creatinine improved to 1.0 mg/dL within the first 48 hours, and serum K+ decreased to 4.5 mmol/L. The urine culture was sterile. A noncontrast computed tomography (CT) scan of the abdomen and the pelvis showed streaking effect and an area of thickening in the retroperitoneal region between the fifth lumbar and the proximal sacral vertebrae. These findings conformed with the diagnosis of RPF. One of the treatment options, consistent with the therapy of RPF, considered at this time was ureterolysis and omental wrap. We pursued further evaluation of the ascites. An ultrasound-guided ascitic tap drained approximately 500 cc of straw-colored exudate. Ascitic fluid cytology was positive for a moderately differentiated adenocarcinoma. A transvaginal ultrasound study was normal. Colonoscopy showed a partially obstructing mass in the sigmoid colon 25 cm from the anal verge. Biopsy of the mass revealed adenocarcinoma. Laboratory studies showed very high levels of gastrointestinal malignancy tumor markers, including carcinoembryonic antigen (CEA): 817 ng/mL (normal 0.7 to 5.0); carbohydrate antigen type 125: 302 U/mL (normal 8.5 to 35); carbohydrate antigen 19-9: 52.1 U/mL (normal less than 40). 136
Urology literature is bereft of any report of the association of DM with extrinsic bilateral ureteral obstruction. Sporadic instances of ureteral obstruction arising from the association of DM with ureteral diverticulosis3 and ureteral necrosis in juvenile DM4,5 have been reported. DM has been temporally associated with malignancy. ’ The relative risk of cancer among DM cases is very high (standardized incidence ratio 26) in the first year after diagnosis of DM. Callen’ suggested that of the cancers associated with DM, about one third occur before, one third concurrent with, and one third after the diagnosis of DM. The risk is further enhanced in patients aged 50 years or older8 and in female patients.’ The types of neoplasms found in association with DM parallel those observed in the general population.2 Although no definite proof has been offered, it is believed that the myositis may be a generalized allergic phenomenon induced by the “antigens” on the malignant tumors or an immune complex-mediated vascu1opathy.l RPF has been broadly classified into two varieties: idiopathic (with no predisposing disease) and secondary (to a disease such as malignancy). Although the disease may be diffusely present in the retroperitoneum, it is mainly centered around the region from the fourth lumbar to the first sacral vertebra. Retroperitoneal malignancy was reported as the cause of RPF in 7.9% of 491 patients.” The malignancy-induced RPF may result from periureteral metastasis with fibrosis, primary retroperitoneal tumor such as lymphoma and sarcoma, or serotonin-producing carcinoid tum0rs.l’ Therapy for obstructive anuria due to disseminated malignancy-induced RPF is palliative. Conservative therapy and bypass of the obstructed system lead to a dramatic improvement in renal function.12 By contrast, ureteral obstruction secondary to idiopathic RPF may respond well to corticosteroids and azathioprine and to surgical ureterolysis.13 The patient in our study was on prednisone therapy for 5 months before the onset of obstructive anuria. Thus, a de facto trial of steroids for UROLOGY
50 (I), 1997
medical management of RPF had already failed at diagnosis. This case is uniquely different from other case reports of DM in urologic literature in that the urologic involvement was secondary to a bilateral extrinsic obstruction of the ureters, raising the possibility of association with RPF. The ureters were compressed to a cross section of about 1 mm over a 4-cm length. The urologist should be diligent in pursuing all causes of ureteral obstruction, including malignancy, in patients with bilateral extrinsic retroperitoneal ureteral obstruction leading to renal failure. DM is known to be associated with higher risk of malignancy, especially in patients over the age of 50 years. The heightened suspicion that led to the diagnosis of disseminated colonic malignancy spared this patient extensive laparotomy for ureterolysis. REFERENCES 1. Stonecipher MR, Callen JP, and Jorizzo JL: The red face: dermatomyositis. Clin Dermatol 11: 261-273, 1993.
UROLOGY
50 (11, 1997
2. Bernard P, and Bonnetblanc JM: Dermatomyositis and malignancy. J Invest Dermatol 100: 128S-132S, 1993. 3. Bernath AS, Garcias VA, Son YO, Fernandez R, Schutte H, and Nagamatsu G: Ureteral diverticulosis in a case of dermatomyositis. J Urol 124: 921-922, 1980. 4. Borrelli M, Prado MJ, Cordeiro P, Wroclawski ER, Junior JM, Kiss MHB, Saldanha LB, and Arap S: Ureteral necrosis in dermatomyositis. J Urol 139: 1275-1277, 1988. 5. Mesrobian HGJ, and Azizkhan RG: Pyeloureterostomy with appendiceal interposition. J Urol142: 1288- 1289.1989. 6. Zantos D, Zhang Y, and Felson D: The overall and temporal association of cancer with polymyositis and dermatomyositis. J Rheumatol 21: 1855-1859, 1994. 7. Callen JP: Dermatomyositis and malignancy. Clin Rheum Dis 8: 369-381, 1982. 8. Airio A, Pukkala E, and lsomaki H: Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol 22: 1300-1303, 1995. 9. Sigurgeirsson B, Lindelof B, Edhag 0, and Allander E: Risk of cancer in patients with dermatomyositis or polymyositis-a population based study. N Engl J Med 326: 363-367, 1992. 10. Koep L, and Zuidema GD: The clinical significance of retroperitoneal fibrosis. Surgery 82: 250-257, 1977. 11. Lepor H, and Walsh PC: Idiopathic retroperitoneal fibrosis. J Urol 122: 1-6, 1979. 12. Spital A, Valvo JA, and Segal AJ: Nondilated obstructive uropathy. Urology 31: 478-482, 1988. 13. Cogan E, and Fastrez R: Azathioprine-an alternative treatment of recurrent idiopathic retroperitoneal fibrosis. Arch Intern Med 145: 753-755. 1985.
137
OBSTRUCTIVE ANURIA A UNIQUE B. G. PARULKAR,
AND DERMATOMYOSITIS: ASSOCIATION J. KEN,
AND
D. TRIFILIO
ABSTRACT
This case is the first reported presentation of obstructive anuria due to extrinsic ureteral compression in a patient with dermatomyositis. Further evaluation revealed disseminated sigmoid colon adenocarcinoma. Conservative treatment of ureteral obstruction by internal double-J stents achieved appropriate palliation. UROLOGY 50: 135-l 37, 1997. 0 1997, Elsevier Science Inc. All rights reserved.
D
ermatomyositis (DM) is an inflammatory muscle disease characterized by symmetric proximal muscle weakness and typical skin lesions, such as heliotrope eruptions and Gottron’s sign.’ Malignancies can occur concurrently with DM, and quite often their course may parallel or complement each other.2 Obstructive anuria due to either idiopathic or secondary retroperitoneal fibrosis is a well-described entity in urologic literature. This paper reports a patient who presented with bilateral ureteral obstruction with features suggestive of retroperitoneal fibrosis (RPF). After thorough evaluation, an underlying disseminated adenocarcinoma of the sigmoid colon was detected and the patient succumbed to her disease a few weeks later. This case illustrates the rarity of the association of urologic disease in patients with DM and strongly emphasizes the importance of a thorough evaluation to rule out underlying secondary pathology in these patients, with special emphasis on the detection of malignancy. CASE REPORT A 64-year-old female retired health worker was diagnosed to have DM in November 1995. Prednisone therapy brought about a rapid improvement in the skin lesions but produced minimal change in fatigue symptoms.
From the Department of Urology, University of Massachusetts and the Department of Urology, St. Vincent’s Hospital, Worcester, Massachusetts Reprint requests: J. Isen, M.D., Department of Urology, FaIlon Clinic, 135 Goldstur Boulevard, Worcester, MA 01606 Submitted: August 1, 1996, accepted (with revisions): February 5, 1997 0 1997, ELSEVIER SCIENCE ALL RIGHTS RESERVED
INC.
In the first week of March 1996, she presented to the emergency department with a 2-week history of malaise, nausea, and bloating and a 24hour history of nonfecal vomiting, marked decrease in urine output, and puffiness of face. Physical examination was normal, except for significant gaseous distention coupled with minimal ascites. Rectal and pelvic examination was normal. Her laboratory evaluation revealed a normal white blood count of 6800/mm3 (normal 3800 to 10,100) and a hematocrit of 36.6% (normal 39.5 to 47.7). She was in acute renal failure, as evident from a creatinine of 11.4 mg/dL (normal 0.5 to 1.4) and a blood-urea nitrogen (BUN) of 76 mg/ dL (normal 5 to 25). Her serum KC level at presentation was 8.1 mmoVL (normal 3.5 to 5.3). Real-time ultrasound scanning of the abdomen and pelvis showed a moderate degree of bilateral hydroureteronephrosis, with well-preserved renal parenchyma and minimal ascites. Cystoscopy and bilateral retrograde ureteropyelogram was performed. This revealed bilateral concentric narrowing and slight medial deviation of the segment of ureters (4 cm long and 1 mm wide) overlapping the regions of the fifth lumbar vertebra and the sacrum (Fig. 1). The distal pelvic ureters were normal in course and caliber. Proximal ureters and both pelvicaliceal systems showed moderate to severe dilation (right greater than left). The tentative diagnosis of RPF of unknown etiology was arrived at. Floppy-tip guidewires (0.038 in.) were advanced in the renal pelves bilaterally under fluoroscopy. In spite of some difficulty with their passage, 6F double-J stents were positioned bilaterally over the guidewires. A significant postobstructive diuresis ensued, and she had a urine output of greater than 0090-4295/97/$17.00 PII SOO90-4295(97)00200-S
135
After appropriate discussion with the patient, a palliative loop transverse colostomy was performed. Minilaparotomy during colostomy showed abdominal carcinomatosis with diffuse secondary lesions. Since the diagnosis of disseminated malignancy was confirmed and the prognosis was evidently poor, further tests such as CTguided retroperitoneal needle biopsies were declined by the patient. She was placed on palliative cycled chemotherapy with 5-FU and appropriate pain medications. She died within 2 months. COMMENT
FIGURE 1. Bilateral retrograde pyelogram showing medial displacement and narrowing of the sacral segment of both ureters (arrows) and proximal dilation of both pelvicaliceal systems.
3.5 L over the first 24 hours. Her serum creatinine improved to 1.0 mg/dL within the first 48 hours, and serum K+ decreased to 4.5 mmol/L. The urine culture was sterile. A noncontrast computed tomography (CT) scan of the abdomen and the pelvis showed streaking effect and an area of thickening in the retroperitoneal region between the fifth lumbar and the proximal sacral vertebrae. These findings conformed with the diagnosis of RPF. One of the treatment options, consistent with the therapy of RPF, considered at this time was ureterolysis and omental wrap. We pursued further evaluation of the ascites. An ultrasound-guided ascitic tap drained approximately 500 cc of straw-colored exudate. Ascitic fluid cytology was positive for a moderately differentiated adenocarcinoma. A transvaginal ultrasound study was normal. Colonoscopy showed a partially obstructing mass in the sigmoid colon 25 cm from the anal verge. Biopsy of the mass revealed adenocarcinoma. Laboratory studies showed very high levels of gastrointestinal malignancy tumor markers, including carcinoembryonic antigen (CEA): 817 ng/mL (normal 0.7 to 5.0); carbohydrate antigen type 125: 302 U/mL (normal 8.5 to 35); carbohydrate antigen 19-9: 52.1 U/mL (normal less than 40). 136
Urology literature is bereft of any report of the association of DM with extrinsic bilateral ureteral obstruction. Sporadic instances of ureteral obstruction arising from the association of DM with ureteral diverticulosis3 and ureteral necrosis in juvenile DM4,5 have been reported. DM has been temporally associated with malignancy. ’ The relative risk of cancer among DM cases is very high (standardized incidence ratio 26) in the first year after diagnosis of DM. Callen’ suggested that of the cancers associated with DM, about one third occur before, one third concurrent with, and one third after the diagnosis of DM. The risk is further enhanced in patients aged 50 years or older8 and in female patients.’ The types of neoplasms found in association with DM parallel those observed in the general population.2 Although no definite proof has been offered, it is believed that the myositis may be a generalized allergic phenomenon induced by the “antigens” on the malignant tumors or an immune complex-mediated vascu1opathy.l RPF has been broadly classified into two varieties: idiopathic (with no predisposing disease) and secondary (to a disease such as malignancy). Although the disease may be diffusely present in the retroperitoneum, it is mainly centered around the region from the fourth lumbar to the first sacral vertebra. Retroperitoneal malignancy was reported as the cause of RPF in 7.9% of 491 patients.” The malignancy-induced RPF may result from periureteral metastasis with fibrosis, primary retroperitoneal tumor such as lymphoma and sarcoma, or serotonin-producing carcinoid tum0rs.l’ Therapy for obstructive anuria due to disseminated malignancy-induced RPF is palliative. Conservative therapy and bypass of the obstructed system lead to a dramatic improvement in renal function.12 By contrast, ureteral obstruction secondary to idiopathic RPF may respond well to corticosteroids and azathioprine and to surgical ureterolysis.13 The patient in our study was on prednisone therapy for 5 months before the onset of obstructive anuria. Thus, a de facto trial of steroids for UROLOGY
50 (I), 1997
medical management of RPF had already failed at diagnosis. This case is uniquely different from other case reports of DM in urologic literature in that the urologic involvement was secondary to a bilateral extrinsic obstruction of the ureters, raising the possibility of association with RPF. The ureters were compressed to a cross section of about 1 mm over a 4-cm length. The urologist should be diligent in pursuing all causes of ureteral obstruction, including malignancy, in patients with bilateral extrinsic retroperitoneal ureteral obstruction leading to renal failure. DM is known to be associated with higher risk of malignancy, especially in patients over the age of 50 years. The heightened suspicion that led to the diagnosis of disseminated colonic malignancy spared this patient extensive laparotomy for ureterolysis. REFERENCES 1. Stonecipher MR, Callen JP, and Jorizzo JL: The red face: dermatomyositis. Clin Dermatol 11: 261-273, 1993.
UROLOGY
50 (11, 1997
2. Bernard P, and Bonnetblanc JM: Dermatomyositis and malignancy. J Invest Dermatol 100: 128S-132S, 1993. 3. Bernath AS, Garcias VA, Son YO, Fernandez R, Schutte H, and Nagamatsu G: Ureteral diverticulosis in a case of dermatomyositis. J Urol 124: 921-922, 1980. 4. Borrelli M, Prado MJ, Cordeiro P, Wroclawski ER, Junior JM, Kiss MHB, Saldanha LB, and Arap S: Ureteral necrosis in dermatomyositis. J Urol 139: 1275-1277, 1988. 5. Mesrobian HGJ, and Azizkhan RG: Pyeloureterostomy with appendiceal interposition. J Urol142: 1288- 1289.1989. 6. Zantos D, Zhang Y, and Felson D: The overall and temporal association of cancer with polymyositis and dermatomyositis. J Rheumatol 21: 1855-1859, 1994. 7. Callen JP: Dermatomyositis and malignancy. Clin Rheum Dis 8: 369-381, 1982. 8. Airio A, Pukkala E, and lsomaki H: Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol 22: 1300-1303, 1995. 9. Sigurgeirsson B, Lindelof B, Edhag 0, and Allander E: Risk of cancer in patients with dermatomyositis or polymyositis-a population based study. N Engl J Med 326: 363-367, 1992. 10. Koep L, and Zuidema GD: The clinical significance of retroperitoneal fibrosis. Surgery 82: 250-257, 1977. 11. Lepor H, and Walsh PC: Idiopathic retroperitoneal fibrosis. J Urol 122: 1-6, 1979. 12. Spital A, Valvo JA, and Segal AJ: Nondilated obstructive uropathy. Urology 31: 478-482, 1988. 13. Cogan E, and Fastrez R: Azathioprine-an alternative treatment of recurrent idiopathic retroperitoneal fibrosis. Arch Intern Med 145: 753-755. 1985.
137