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Occult renal cell carcinoma in a patient with polymyositis
CASE REPORT
OCCULT RENAL CELL CARCINOMA IN A PATIENT WITH POLYMYOSITIS ADAM P. KLAUSNER, MICHAEL C. OST, ROBERT L. WATERHOUSE, JR,
AND
STEPHEN J. SAVAGE
ABSTRACT Polymyositis (PM) is associated with an increased risk of malignancy. We report a case in which a 45-year-old patient with PM underwent computed tomography to search for an occult malignant process. The evaluation revealed a left renal mass that was treated successfully by laparoscopic radical nephrectomy. Pathologic evaluation revealed a clear cell renal cell carcinoma. The patient remained free of disease at 2 years postoperatively and required decreasing amounts of medication to manage his PM. This suggests that renal malignancies may contribute to the PM disease process in a paraneoplastic fashion and that screening for occult malignancy in the presence of PM is warranted. UROLOGY 59: 773xiii–773xiv, 2002. © 2002, Elsevier Science Inc.
P
olymyositis (PM) is associated with an increased risk of malignancy. We report a case in which the evaluation of a patient with PM revealed an occult renal malignancy successfully treated by laparoscopic radical nephrectomy. CASE REPORT A 45-year-old man presented with a 2-year history of diffuse muscle pain and weakness and was diagnosed with PM on the basis of clinical criteria. Medical therapy was started with methotrexate, which resulted in symptom improvement and reduction of serum creatinine phosphokinase. The patient was subsequently evaluated for the presence of an occult malignancy. The evaluation included a negative stool guaiac, normal carcinoembryonic antigen (1.1 ng/mL), normal prostatespecific antigen (0.19 ng/mL), normal prostate examination, negative esophagogastroduodenoscopy, and normal chest x-ray. However, contrastenhanced computed tomography of the abdomen and pelvis revealed a 4.5-cm solid mass in the midportion of the left kidney (Fig. 1). No associated hematuria was present. The bone scan was normal,
From the Department of Urology, Mount Sinai Medical Center, New York, New York; and Department of Urology, Bronx Veterans Affairs Medical Center, Bronx, New York Address for correspondence: Stephen J. Savage, M.D., Department of Urology, Box 1272, Mount Sinai Medical Center, One Gustave Levy Place, New York, NY 10029 Submitted: October 30, 2001, accepted (with revisions): January 2, 2002 © 2002, ELSEVIER SCIENCE INC. ALL RIGHTS RESERVED
FIGURE 1. Contrast-enhanced computed tomography scan demonstrating a solid 4.5-cm mass (arrow) arising from the mid-portion of the left kidney.
as was the serum creatinine (0.9 mg/dL). A fourport transperitoneal laparoscopic left radical nephrectomy was performed with intact specimen extraction. The operative time was 4 hours, 51 minutes. Pathologic examination revealed a 3.5-cm renal cell carcinoma (RCC), clear cell type, Fuhrman grade 2, extending through the renal capsule but entirely contained within Gerota’s fascia (pT3a), with negative surgical margins. Postoperatively, the patient had a flare of myositis associated with exquisite tenderness at the dependent right flank region and dramatic elevation of his creatinine phosphokinase levels. This re0090-4295/02/$22.00 PII S0090-4295(02)01533-9 773xiii
solved with high-dose steroids and pain control. The patient was discharged on postoperative day 6. Follow-up computed tomography at 1 and 2 years demonstrated no evidence of local recurrence or metastases. The patient no longer required methotrexate but continued with drastically reduced levels of oral steroids compared with the preoperative period.
symptoms improve with treatment, unless recurrence occurs.4 Additionally, 2 cases of PM after immunotherapy have been reported in patients with metastatic RCC.7,8 Although the lack of prospective data precludes definitive universal recommendations, the investigation for occult malignancy allowed for earlier detection of a pT3a RCC in this 45-year-old man with PM.
COMMENT An elevated risk of malignancy in patients with PM has been suspected for many years. A population-based study in Sweden identified 392 patients with PM during a 20-year period. Malignancy was diagnosed in 37 patients (9%), with a relative risk of 1.8 in males and 1.6 in females.1 Furthermore, a meta-analysis of case-control and cohort studies examining the relationship of PM to malignancy included 565 patients with PM. This study revealed an odds ratio of 2.1% for the association of cancer with PM.2 Because of this increased risk, clinicians often search for occult malignant processes in patients diagnosed with PM. Many different cancer types have been reported in association with PM. However, researchers have suggested that gynecologic malignancies may be particularly prevalent.3 The Bohan classification of PM-dermatomyositis groups the disease into five distinct entities of which group III includes PM as a possible paraneoplastic process.3 PM presenting as a paraneoplastic manifestation of RCC has been previously reported.4 – 6 Typically,
773xiv
REFERENCES 1. Sigurgeirsson B, Lindelof B, Edhag O, et al: Risk of cancer in patients with dermatomyositis or polymyositis: a population-based study. N Engl J Med 326: 363–367, 1992. 2. Zantos D, Zhang Y, and Felson D: The overall and temporal association of cancer with polymyositis and dermatomyositis. J Rheumatol 21: 1855–1859, 1994. 3. Callen JP: Relationship of cancer to inflammatory muscle diseases: dermatomyositis, polymyositis, and inclusion body myositis. Clin Rheumatic Dis 20: 943–953, 1994. 4. Wurzer H, Brandstatter G, Harnoncourt K, et al: Paraneoplastic polymyositis associated with a renal carcinoma. J Intern Med 234: 521–524, 1993. 5. Kumasaka K, Nomura K, Satoh S, et al: A case of renal cell carcinoma with polymyositis. Hinyokika Kiyo 36: 1317– 1320, 1990. 6. Solon AA, Gilbert CS, and Meyer C: Myopathy as a paraneoplastic manifestation of renal cell carcinoma. Am J Med 97: 491– 492, 1994. 7. Esteva-Lorenzo FJ, Janik JE, Fenton RG, et al: Myositis associated with interleukin-2 therapy in a patient with metastatic renal cell carcinoma. Cancer 76: 1219 –1223, 1995. 8. Matsuya M, Abe T, Tosaka M, et al: The first case of polymyositis associated with interferon therapy. Intern Med 33: 806 – 808, 1994.
UROLOGY 59 (5), 2002
OCCULT RENAL CELL CARCINOMA IN A PATIENT WITH POLYMYOSITIS ADAM P. KLAUSNER, MICHAEL C. OST, ROBERT L. WATERHOUSE, JR,
AND
STEPHEN J. SAVAGE
ABSTRACT Polymyositis (PM) is associated with an increased risk of malignancy. We report a case in which a 45-year-old patient with PM underwent computed tomography to search for an occult malignant process. The evaluation revealed a left renal mass that was treated successfully by laparoscopic radical nephrectomy. Pathologic evaluation revealed a clear cell renal cell carcinoma. The patient remained free of disease at 2 years postoperatively and required decreasing amounts of medication to manage his PM. This suggests that renal malignancies may contribute to the PM disease process in a paraneoplastic fashion and that screening for occult malignancy in the presence of PM is warranted. UROLOGY 59: 773xiii–773xiv, 2002. © 2002, Elsevier Science Inc.
P
olymyositis (PM) is associated with an increased risk of malignancy. We report a case in which the evaluation of a patient with PM revealed an occult renal malignancy successfully treated by laparoscopic radical nephrectomy. CASE REPORT A 45-year-old man presented with a 2-year history of diffuse muscle pain and weakness and was diagnosed with PM on the basis of clinical criteria. Medical therapy was started with methotrexate, which resulted in symptom improvement and reduction of serum creatinine phosphokinase. The patient was subsequently evaluated for the presence of an occult malignancy. The evaluation included a negative stool guaiac, normal carcinoembryonic antigen (1.1 ng/mL), normal prostatespecific antigen (0.19 ng/mL), normal prostate examination, negative esophagogastroduodenoscopy, and normal chest x-ray. However, contrastenhanced computed tomography of the abdomen and pelvis revealed a 4.5-cm solid mass in the midportion of the left kidney (Fig. 1). No associated hematuria was present. The bone scan was normal,
From the Department of Urology, Mount Sinai Medical Center, New York, New York; and Department of Urology, Bronx Veterans Affairs Medical Center, Bronx, New York Address for correspondence: Stephen J. Savage, M.D., Department of Urology, Box 1272, Mount Sinai Medical Center, One Gustave Levy Place, New York, NY 10029 Submitted: October 30, 2001, accepted (with revisions): January 2, 2002 © 2002, ELSEVIER SCIENCE INC. ALL RIGHTS RESERVED
FIGURE 1. Contrast-enhanced computed tomography scan demonstrating a solid 4.5-cm mass (arrow) arising from the mid-portion of the left kidney.
as was the serum creatinine (0.9 mg/dL). A fourport transperitoneal laparoscopic left radical nephrectomy was performed with intact specimen extraction. The operative time was 4 hours, 51 minutes. Pathologic examination revealed a 3.5-cm renal cell carcinoma (RCC), clear cell type, Fuhrman grade 2, extending through the renal capsule but entirely contained within Gerota’s fascia (pT3a), with negative surgical margins. Postoperatively, the patient had a flare of myositis associated with exquisite tenderness at the dependent right flank region and dramatic elevation of his creatinine phosphokinase levels. This re0090-4295/02/$22.00 PII S0090-4295(02)01533-9 773xiii
solved with high-dose steroids and pain control. The patient was discharged on postoperative day 6. Follow-up computed tomography at 1 and 2 years demonstrated no evidence of local recurrence or metastases. The patient no longer required methotrexate but continued with drastically reduced levels of oral steroids compared with the preoperative period.
symptoms improve with treatment, unless recurrence occurs.4 Additionally, 2 cases of PM after immunotherapy have been reported in patients with metastatic RCC.7,8 Although the lack of prospective data precludes definitive universal recommendations, the investigation for occult malignancy allowed for earlier detection of a pT3a RCC in this 45-year-old man with PM.
COMMENT An elevated risk of malignancy in patients with PM has been suspected for many years. A population-based study in Sweden identified 392 patients with PM during a 20-year period. Malignancy was diagnosed in 37 patients (9%), with a relative risk of 1.8 in males and 1.6 in females.1 Furthermore, a meta-analysis of case-control and cohort studies examining the relationship of PM to malignancy included 565 patients with PM. This study revealed an odds ratio of 2.1% for the association of cancer with PM.2 Because of this increased risk, clinicians often search for occult malignant processes in patients diagnosed with PM. Many different cancer types have been reported in association with PM. However, researchers have suggested that gynecologic malignancies may be particularly prevalent.3 The Bohan classification of PM-dermatomyositis groups the disease into five distinct entities of which group III includes PM as a possible paraneoplastic process.3 PM presenting as a paraneoplastic manifestation of RCC has been previously reported.4 – 6 Typically,
773xiv
REFERENCES 1. Sigurgeirsson B, Lindelof B, Edhag O, et al: Risk of cancer in patients with dermatomyositis or polymyositis: a population-based study. N Engl J Med 326: 363–367, 1992. 2. Zantos D, Zhang Y, and Felson D: The overall and temporal association of cancer with polymyositis and dermatomyositis. J Rheumatol 21: 1855–1859, 1994. 3. Callen JP: Relationship of cancer to inflammatory muscle diseases: dermatomyositis, polymyositis, and inclusion body myositis. Clin Rheumatic Dis 20: 943–953, 1994. 4. Wurzer H, Brandstatter G, Harnoncourt K, et al: Paraneoplastic polymyositis associated with a renal carcinoma. J Intern Med 234: 521–524, 1993. 5. Kumasaka K, Nomura K, Satoh S, et al: A case of renal cell carcinoma with polymyositis. Hinyokika Kiyo 36: 1317– 1320, 1990. 6. Solon AA, Gilbert CS, and Meyer C: Myopathy as a paraneoplastic manifestation of renal cell carcinoma. Am J Med 97: 491– 492, 1994. 7. Esteva-Lorenzo FJ, Janik JE, Fenton RG, et al: Myositis associated with interleukin-2 therapy in a patient with metastatic renal cell carcinoma. Cancer 76: 1219 –1223, 1995. 8. Matsuya M, Abe T, Tosaka M, et al: The first case of polymyositis associated with interferon therapy. Intern Med 33: 806 – 808, 1994.
UROLOGY 59 (5), 2002