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Diagnosis and Management of Testicular Sarcoidosis
0022-534 7/86/1352-0380$02.00/0
Vol. 135, February Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1986 by The Williams & Wilkins Co.
DIAGNOSIS AND MANAGEMENT OF TESTICULAR SARCOIDOSIS CHARLES 0. TURK, MARK SCHACHT
AND
LAWRENCE ROSS
From the Department of Urology, Michael Reese Hospital and Medical Center, Chicago, Illinois
ABSTRACT
We report testicular sarcoidosis in a patient who had known systemic involvement with this disease. The literature is reviewed and a protocol is presented to delineate specific indications for biopsy rather than orchiectomy to preserve testicular function. Sarcoidosis of the testis is a rare disease that most commonly affects black men during the peak reproductive years. As is the case with any solid mass infiltrating the testis, orchiectomy is the primary method of diagnosis. We report a case of testicular sarcoidosis and recommend a management protocol to substitute testicular biopsy for orchiectomy in 2 high risk groups.
DISCUSSION
Sarcoidosis most commonly presents with intrathoracic manifestations (84 per cent of the cases). Other organ systems potentially involved include the reticuloendothelial in 40 per cent, ocular in 25 per cent, nervous in 7 per cent, parotid in 6 per cent, osseous in 3 per cent, myocardial and endocrine. 1 The frequency of genitourinary sarcoidosis has been shown to be less than 0.2 per cent in clinically diagnosed cases, 2-4 and 5 per cent in autopsy studies, and it has been shown to be 10 times more frequent in black men. 5 Initial presentation can range from the more common asymptomatic, painless mass in the scrotum to the more unusual presentation of acute epididymoorchitis. All reported cases to date have been associated with hilar adenopathy. The combination of a testicular mass and hilar adenopathy can present a significant diagnostic challenge.6 Tuberculosis, fungal infection and syphilis must be ruled out by serology, skin tests and cultures. Because the average age of patients with genitourinary sarcoidosis is 31, which coincides with the peak occurrence of testicular malignancy, a high index of suspicion for malignancy must be maintained. Ultrasound can help to delineate testicular from extratesticular masses, as well as a solid from a cystic lesion. 7 Serum markers should be drawn preoperatively. Exploration of the scrotal contents, biopsy and orchiectomy may be required to make the diagnosis. Since simultaneous sarcoidosis and malignancy have been reported in the testes an inguinal approach is recommended. 8 Periodic chest x-rays should be performed postoperatively to detect the rare but previously reported coincidence of a pulmonary neoplasm. 3 Because the average patient age at presentation coincides
CASE REPORT
A 24-year-old black man was first seen in 1972 with cutaneous nodules and pulmonary hilar adenopathy. The diagnosis of sarcoidosis was confirmed by cutaneous biopsy. Subsequently, involvement of the larynx and phalangeal joints developed, which was confirmed by biopsy. Since the initial diagnosis the patient had been treated with oral steroids, 10 to 80 mg. prednisone daily. For 8 months before the initial genitourinary evaluation the dosage was decreased to 10 mg. daily. When seen at our urology clinic the patient complained of a painless nodular mass on the left testis and a 20-pound weight loss in 8 months. He denied dysuria, frequency or previous urinary tract infection. Physical examination revealed perioral and perinasal nodules, and decreased flexion of the metacarpal-phalangeal joints. The left testis was enlarged to 6 X 4 X 3 cm., and was firm with a nodular surface along the upper pole. The epididymis was not separately palpable. The right testis and epididymis, and prostate were normal. Rapid plasma reagent, sequential multiple analyzer and computer for serum, electrolytes, calcium, phosphorus, alkaline phosphatase, creatinine, phosphokinase, bilirubin, uric acid, albumin, blood urea nitrogen, creatine and glucose, purified protein derivative, a-fetoprotein and /3-human chorionic gonadotropin were normal. A chest x-ray revealed bilateral hilar adenopathy. Left inguinal exploration was followed by orchiectomy when the parenchyma of the testis was found to be diffusely involved. Convalescence was uneventful. The patient has remained asymptomatic on low doses of prednisone. Pathological examination of the specimen removed at surgery demonstrated a smooth tunica albuginea except for a 1.5 x 1 cm. indurated, raised, plaque-like tan nodule on the anterior surface near the upper pole of the testis, a nodularity of the surface along the lateral border and a similar appearing nodule adjacent to the head of the epididymis. Microscopic study showed discrete and confluent noncaseating epithelioid granulomas of the parenchyma of the testis associated with fibrosis involving the interstitium and extending to the tunica albuginea and epididymis (see figure). Similar granulomas were noted in the tunica vaginalis, as well as the intima of the blood vessels in the spermatic cord. No organisms were seen on acid fast, Gomori silver methenamine or periodic acid, Schiff stains. These findings were consistent with sarcoidosis of the testes and epididymis. Accepted for publication September 27, 1985.
Noncaseating epithelioid granuloma of testis with giant cells. Normal seminiferous tubules and interstitium are adjacent.
380
381
TESTICULAR SARCOIDOSIS
with the reproductive years, preservation of testicular function is important. The effect of genitourinary sarcoidosis on fertility has not been studied but it is reasonable to assume that the fibrosis and occlusion of the ductus epididymis seen with this disease could cause azoospermia. Testicular involvement in a patient with a single testis may cause decreased spermatogenesis, the degree of which would be related directly to the amount of displaced parenchyma. If orchiectomy is done on 1 side and the patient has subsequent involvement of the contralateral side, fertility could be jeopardized. Clinical response to steroids has been reported in genitourinary sarcoidosis 9 • 10 but steroid treatment is not suggested for involvement of the testes alone. Seaworth and associates discourage the use of steroids as a diagnostic modality because suppression of an inflammatory response around a neoplasm may suggest the resolution of a disease process and delay the diagnosis of a malignancy. 6 We believe that there is enough known about sarcoidosis of the testis to suggest a treatment plan to facilitate diagnosis and to preserve testicular function. Any patient who is known to have systemic sarcoidosis and, subsequently, has a testicular mass deserves an inguinal approach for a planned open biopsy rather than orchiectomy if the lesion is small and completely contained in the specimen. Orchiectomy is reserved for when the testicle is diffusely involved. Recognizing the fact that this disease predominantly affects black men and that testicular neoplasms are much more commonly seen in white men,u we recommend the aforementioned approach' for any black male subject presenting with a testicular mass and pulmonary hilar adenopathy. A vascular occlusion clamp should be used to occlude the spermatic cord until results of the frozen section are known. Steroid therapy should be reserved for treatment of systemic disease unless the patient has a single testicle, or has
bilateral testicular or epididymal involvement. The importance of preservation of fertility must be weighed against the risk of long-term systemic steroids. REFERENCES 1. Singer, E. P., Hensler, N. M. and Flynn, P. F.: Sarcoidosis: an
2.
3. 4.
5. 6. 7. 8. 9. 10. 11.
analysis of forty-five cases in a large military hospital, Amer. J. Med., 26: 364, 1959. Longcope, W. T.: Sarcoidosis or Besnier-Boeck-Schaumann disease. J.A.M.A., 117: 1321, 1941. Brinker, H. and Wilbek, E.: The incidence of malignant tumours in patients with respiratory sarcoidosis. Brit. J. Cancer, 29: 247, 1974. Mayock, R. L., Bertrand, P., Morrison, C. E. and Scott, J. H.: Manifestations of sarcoidosis. Analysis of 145 patients, with a review of nine series selected from the literature. Amer. J. Med., 35: 67, 1963. Ricker, W. and Clark, M.: Sarcoidosis: a clinicopathologic review of three hundred cases, including twenty-two autopsies. Amer. J. Clin. Path., 19: 725, 1949. Seaworth, J. F., Davis, S. J. and Donovan, W. N.: Aggressive diagnostic approach indicated in testicular sarcoidosis. Urology, 21: 396, 1983. Giyanani, V. L., Hennigan, D. B., Fowler, M. and Sanders, T. J.: Sonographic findings in leiomyoma of postorchiectomy scrotum. Urology, 25: 204, 1985. Geller, R. A., Kuremsky, D. A., Copeland, J, S. and Stept, R.: Sarcoidosis and testicular neoplasm: an unusual association. J. Urol., 118: 487, 1977. Chowdhury, S. D. and Higgins, P. M.: Sarcoid of the testis. Brit. J. Urol., 45: 218, 1973. Opal, S. M., Pittman, D. L. and Hofeldt, F. E.: Testicular sarcoidosis. Amer. J. Med., 67: 147, 1979. Skinner, D. G. and deKernion, J. B.: Genitourinary Cancer. Philadelphia: W. B. Saunders Co., p. 448, 1978.
Vol. 135, February Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1986 by The Williams & Wilkins Co.
DIAGNOSIS AND MANAGEMENT OF TESTICULAR SARCOIDOSIS CHARLES 0. TURK, MARK SCHACHT
AND
LAWRENCE ROSS
From the Department of Urology, Michael Reese Hospital and Medical Center, Chicago, Illinois
ABSTRACT
We report testicular sarcoidosis in a patient who had known systemic involvement with this disease. The literature is reviewed and a protocol is presented to delineate specific indications for biopsy rather than orchiectomy to preserve testicular function. Sarcoidosis of the testis is a rare disease that most commonly affects black men during the peak reproductive years. As is the case with any solid mass infiltrating the testis, orchiectomy is the primary method of diagnosis. We report a case of testicular sarcoidosis and recommend a management protocol to substitute testicular biopsy for orchiectomy in 2 high risk groups.
DISCUSSION
Sarcoidosis most commonly presents with intrathoracic manifestations (84 per cent of the cases). Other organ systems potentially involved include the reticuloendothelial in 40 per cent, ocular in 25 per cent, nervous in 7 per cent, parotid in 6 per cent, osseous in 3 per cent, myocardial and endocrine. 1 The frequency of genitourinary sarcoidosis has been shown to be less than 0.2 per cent in clinically diagnosed cases, 2-4 and 5 per cent in autopsy studies, and it has been shown to be 10 times more frequent in black men. 5 Initial presentation can range from the more common asymptomatic, painless mass in the scrotum to the more unusual presentation of acute epididymoorchitis. All reported cases to date have been associated with hilar adenopathy. The combination of a testicular mass and hilar adenopathy can present a significant diagnostic challenge.6 Tuberculosis, fungal infection and syphilis must be ruled out by serology, skin tests and cultures. Because the average age of patients with genitourinary sarcoidosis is 31, which coincides with the peak occurrence of testicular malignancy, a high index of suspicion for malignancy must be maintained. Ultrasound can help to delineate testicular from extratesticular masses, as well as a solid from a cystic lesion. 7 Serum markers should be drawn preoperatively. Exploration of the scrotal contents, biopsy and orchiectomy may be required to make the diagnosis. Since simultaneous sarcoidosis and malignancy have been reported in the testes an inguinal approach is recommended. 8 Periodic chest x-rays should be performed postoperatively to detect the rare but previously reported coincidence of a pulmonary neoplasm. 3 Because the average patient age at presentation coincides
CASE REPORT
A 24-year-old black man was first seen in 1972 with cutaneous nodules and pulmonary hilar adenopathy. The diagnosis of sarcoidosis was confirmed by cutaneous biopsy. Subsequently, involvement of the larynx and phalangeal joints developed, which was confirmed by biopsy. Since the initial diagnosis the patient had been treated with oral steroids, 10 to 80 mg. prednisone daily. For 8 months before the initial genitourinary evaluation the dosage was decreased to 10 mg. daily. When seen at our urology clinic the patient complained of a painless nodular mass on the left testis and a 20-pound weight loss in 8 months. He denied dysuria, frequency or previous urinary tract infection. Physical examination revealed perioral and perinasal nodules, and decreased flexion of the metacarpal-phalangeal joints. The left testis was enlarged to 6 X 4 X 3 cm., and was firm with a nodular surface along the upper pole. The epididymis was not separately palpable. The right testis and epididymis, and prostate were normal. Rapid plasma reagent, sequential multiple analyzer and computer for serum, electrolytes, calcium, phosphorus, alkaline phosphatase, creatinine, phosphokinase, bilirubin, uric acid, albumin, blood urea nitrogen, creatine and glucose, purified protein derivative, a-fetoprotein and /3-human chorionic gonadotropin were normal. A chest x-ray revealed bilateral hilar adenopathy. Left inguinal exploration was followed by orchiectomy when the parenchyma of the testis was found to be diffusely involved. Convalescence was uneventful. The patient has remained asymptomatic on low doses of prednisone. Pathological examination of the specimen removed at surgery demonstrated a smooth tunica albuginea except for a 1.5 x 1 cm. indurated, raised, plaque-like tan nodule on the anterior surface near the upper pole of the testis, a nodularity of the surface along the lateral border and a similar appearing nodule adjacent to the head of the epididymis. Microscopic study showed discrete and confluent noncaseating epithelioid granulomas of the parenchyma of the testis associated with fibrosis involving the interstitium and extending to the tunica albuginea and epididymis (see figure). Similar granulomas were noted in the tunica vaginalis, as well as the intima of the blood vessels in the spermatic cord. No organisms were seen on acid fast, Gomori silver methenamine or periodic acid, Schiff stains. These findings were consistent with sarcoidosis of the testes and epididymis. Accepted for publication September 27, 1985.
Noncaseating epithelioid granuloma of testis with giant cells. Normal seminiferous tubules and interstitium are adjacent.
380
381
TESTICULAR SARCOIDOSIS
with the reproductive years, preservation of testicular function is important. The effect of genitourinary sarcoidosis on fertility has not been studied but it is reasonable to assume that the fibrosis and occlusion of the ductus epididymis seen with this disease could cause azoospermia. Testicular involvement in a patient with a single testis may cause decreased spermatogenesis, the degree of which would be related directly to the amount of displaced parenchyma. If orchiectomy is done on 1 side and the patient has subsequent involvement of the contralateral side, fertility could be jeopardized. Clinical response to steroids has been reported in genitourinary sarcoidosis 9 • 10 but steroid treatment is not suggested for involvement of the testes alone. Seaworth and associates discourage the use of steroids as a diagnostic modality because suppression of an inflammatory response around a neoplasm may suggest the resolution of a disease process and delay the diagnosis of a malignancy. 6 We believe that there is enough known about sarcoidosis of the testis to suggest a treatment plan to facilitate diagnosis and to preserve testicular function. Any patient who is known to have systemic sarcoidosis and, subsequently, has a testicular mass deserves an inguinal approach for a planned open biopsy rather than orchiectomy if the lesion is small and completely contained in the specimen. Orchiectomy is reserved for when the testicle is diffusely involved. Recognizing the fact that this disease predominantly affects black men and that testicular neoplasms are much more commonly seen in white men,u we recommend the aforementioned approach' for any black male subject presenting with a testicular mass and pulmonary hilar adenopathy. A vascular occlusion clamp should be used to occlude the spermatic cord until results of the frozen section are known. Steroid therapy should be reserved for treatment of systemic disease unless the patient has a single testicle, or has
bilateral testicular or epididymal involvement. The importance of preservation of fertility must be weighed against the risk of long-term systemic steroids. REFERENCES 1. Singer, E. P., Hensler, N. M. and Flynn, P. F.: Sarcoidosis: an
2.
3. 4.
5. 6. 7. 8. 9. 10. 11.
analysis of forty-five cases in a large military hospital, Amer. J. Med., 26: 364, 1959. Longcope, W. T.: Sarcoidosis or Besnier-Boeck-Schaumann disease. J.A.M.A., 117: 1321, 1941. Brinker, H. and Wilbek, E.: The incidence of malignant tumours in patients with respiratory sarcoidosis. Brit. J. Cancer, 29: 247, 1974. Mayock, R. L., Bertrand, P., Morrison, C. E. and Scott, J. H.: Manifestations of sarcoidosis. Analysis of 145 patients, with a review of nine series selected from the literature. Amer. J. Med., 35: 67, 1963. Ricker, W. and Clark, M.: Sarcoidosis: a clinicopathologic review of three hundred cases, including twenty-two autopsies. Amer. J. Clin. Path., 19: 725, 1949. Seaworth, J. F., Davis, S. J. and Donovan, W. N.: Aggressive diagnostic approach indicated in testicular sarcoidosis. Urology, 21: 396, 1983. Giyanani, V. L., Hennigan, D. B., Fowler, M. and Sanders, T. J.: Sonographic findings in leiomyoma of postorchiectomy scrotum. Urology, 25: 204, 1985. Geller, R. A., Kuremsky, D. A., Copeland, J, S. and Stept, R.: Sarcoidosis and testicular neoplasm: an unusual association. J. Urol., 118: 487, 1977. Chowdhury, S. D. and Higgins, P. M.: Sarcoid of the testis. Brit. J. Urol., 45: 218, 1973. Opal, S. M., Pittman, D. L. and Hofeldt, F. E.: Testicular sarcoidosis. Amer. J. Med., 67: 147, 1979. Skinner, D. G. and deKernion, J. B.: Genitourinary Cancer. Philadelphia: W. B. Saunders Co., p. 448, 1978.