Scand J Urol Nephrol26: 301-303, 1992
BILATERAL EPIDIDYMAL SARCOIDOSIS Case Report
Naoki Yamamoto, Yoshikazu Hasegawa, Kohji Miyamoto, Tsuneko Ikeda, Manabu Kuriyama and Yukimichi Kawada From the Departments of Urology, Surgery and Pathology, Matsunami General Hospital, and the Department of Urology, Gifu University School of Medicine, Japan
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/02/15 For personal use only.
(Submitted March 27, 1991. Accepted for publication July 21, 1991)
Abstract. A 59-year-old man presented with a fourmonth history of almost painless bilateral scrotal swellings. Histological examination of a biopsy specimen confirmed sarcoidosis. He was well two months later, the mass had not increased, and he was receiving no treatment. This is the nineteenth reported case to our knowledge, and the eighth with bilateral disease. Key words: sarcoidosis, epididymis, bilateral involvement.
Sarcoidosis is a chronic, multisystem disorder of unknown aetiology characterized by non-caseating epithelioid granulomas in the affected organs. Practically any organ can be affected, but the most common are the lungs, eyes, lymph nodes, and skin (3). Sarcoidosis of the genital organs is relatively rare with a reported incidence of less than 5 Oo/ at necropsy (10) and 0.5% clinically (4, 7). We report an additional histologically diagnosed case of bilateral epididymal sarcoidosis. To our knowledge, this is only the eighth reported case of bilateral disease. CASE REPORT A 59-year-old man presented with a four-month history of almost painless bilateral scrotal swelling. He had no previous episodes of either urethritis or tuberculosis. There was a firm mass at the epididymis on both sides of the scrotum, which seemed to involve the entire portion of the bilateral epididymis. Results of serum biochemical investigations, full blood count and urinalysis were within the reference range. The scrotum was explored because the mass had increased in size despite treatment with antibiotics. The testes were normal and surrounded by small hydroceles. There were no adhesions around the testes, and bilateral epididymes were nodular and hard. The
epididymal tumors, the largest of which measured 5 m m in diameter, were biopsied. Histological examination showed non-caseating granuloma, which is compatible to sarcoidosis (Fig. 1 ) . Neither acid fast bacilli nor spermatozoa were found in the specimen. Computed tomography and magnetic resonance imaging of the chest showed enlarged mediastinal lymph nodes, which was compatible with sarcoidosis (Fig. 2). The activity of angiotensin converting enzyme (ACE) was 22.4 IU, which is above the reference range. Culture of urine failed to grow Mycobacterium tuberculosis and the patient did not reach to skin testing with purified protein derivative of old tuberculin. He was not given steroids and the mass was unchanged two months later.
DISCUSSION Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology that most commonly affects young adults. It usually presents with bilateral hilar lymphadenopathy, pulmonary infiltration, and skin or eye lesions (3). Signs of sarcoidosis vary from incidental radiological findings without associated symptoms to severe incapacity and death (3). Sarcoidosis of the urogenital tract is relatively rare with a reported incidence of less than 5 % at necropsy (1). Krauss described the first clinical case with epididymal sarcoidosis in 1958 (8). Since then until 1990, 18 cases has been reported (1-2, 4-18) (Table I). Though some cases of epididymal sarcoidosis could not be detected clinically, there can be massive disease of all the scrotal contents which presents as a large mass with the epididymis indistinguishable from the testis (9). Epididymal lesions are usually unilateral, being bilateral less common and occur in less than a third of the cases (4). In &and J Urol Nephrol26
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/02/15 For personal use only.
302
N . Yamamoto et al.
Fig. 1. Histopathological slide showing multiple granulomatous lesions containing epithelioid cells surrounding Langhans type giant cells and asteroid body (black arrow), but no Schaumann body was seen.
Fig. 2. Horizontal section of magnetic resonance image of the chest showing enlarged mediastinal lymph nodes.
some cases the disease presents as either acute or chronic epididymitis ( 1 7, 18). The main finding to rule out in any case of suspected epididymal sarcoidosis is a malignant tumour (10). Some researchers have recently reported on the ultrasonographic appearance of epididymal sarcoidosis (2, 4), which is of a homogeneous hypoechoic lesion. Other condi-
tions that present as epididymal enlargement with multiple hypoechoic foci include epididymitis, cystic degeneration, multiple cysts, spermatoceles, sperm granuloma, tuberculosis, lymphogranuloma venereum, granuloma inguinale, filarial granuloma, and malignant tumour (2). The sonographic appearance of any mass may therefore be crucial in suggesting the correct
Table I. Summary of 19 reported cases of epididymal sarcoidosis Case no. (ref.)
Age (yrs)
Side
Size
Treatment
Outcome
41
Right
Orchidectomy
Not applicable
31 21 38 33 41 29
Left Right Right Bilateral Bilateral Bilateral
Steroids None Orchidectom y Steroids Steroids Steroids
Not stated Spontaneous remission Not applicable Epididymitis recurred Improvement Improvement
17 21 35 2
Left Left Right Bilateral
None None Steroids Steroids
Not stated Not stated N o response Remission
20 36 22 56 29 27 20 44
Left Bilateral Left Bilateral Right Bilateral Right Bilateral
Four times normal Not stated Not stated 6 cm long Not stated Not stated Right-Not stated Left 1 . 5 ~ 1 . 5cm 2 cm diameter I x 2 cm Not stated Right 4.5 X 3.5 cm Left 3 ~ 3 . 5cm Not stated 6 x 4 ~ cm 4 (Both) 2 x 2 cm Not stated Not stated 0.5 cm (Largest) 2.5x1.5 cm 2 cm in diameter
Orchidectomy Orchidectom y Orchidectomy Steroids Orchidectomy None None None
Not applicable Not applicable Not applicable Responded Not applicable Not stated Not stated Unchanged
Scand J Urol NephrolZ6
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Bilateral epididymal sarcoidosis management of this disease. Evaluation and treatment of scrota1 mass, although it is rare, should be considered to avoid unnecessary orchidectomy. There are diverse opinions about the effectiveness of steroid treatment. Considering the fact that pulmonary function was normal and there was no ocular involvement in our case, we decided to withhold steroids treatment for the present. The previously reported response to steroid treatment lasted for only 15 months (18). McWilliams et al. claimed that short courses of steroids should be given only when lesions continue to increase in size (10).
REFERENCES 1. Amenta PS, Gonick P, Katz S. Sarcoidosis of
2.
3.
4. 5.
testis and epididymis. Urology 1980; 17: 6 16-61 7. Burke CBJ. Parker MSH. Hopper KD. Pienkos EJ. The ultrasonographic appearance of coexistent epididymal and testicular sarcoidosis. J Clin Ultrasound 1990; 18: 522-526. Crystal RG, Braunwald E. Isselbacher KJ, Petersdorf RG, Wison JD, Martin JB, Fauci AS. Harrison's Principles of Internal Medicine. 1 Ith ed. New York: McGraw-Hill, 1987: 1445-1 450. Forte MD. Brant WE. Ultrasonographic detection of epididymal sarcoidosis. J Clin Ultrasound 1988: 16: 191-194. Gerstenhaber BJ, Green R. Sachs FL. Epididyma1 sarcoidosis: A report of two cases and review of the literature. Yale J Biol Med 1977; 50: 669-61 5.
303
6. Hefferman JC, Blenkinsopp WK. Epididymal sarcoidosis. Br J Urol 1978; 50: 2 I 1. 7. Hines HL. Elgart ML, Mackenzie AR. Sarcoidosis: case presentation and discussion. J Urol 1961; 85: 71-74. 8. Krauss L. Genital sarcoidosis: case report and review of the literature. J Urol 1958; 80: 367-370. 9. McGowan AJ Jr, Smith EH. Urological implications of sarcoidosis. J Urol 1967; 97: 1090-1093. 10. McWilliams WA, Abramowitz L, Tiamson EM. Epididymal sarcoidosis: Case report and review. J Urol 1983; 130: 1201-1203. ' I . Mikheil JR, Mitchell DN, Dyson JL, Williams WJ, Ogunlesi TOO, Van Hein-Wallace SE. Sarcoidosis with genital involvement. Am Rev Res Dis 1972; 106: 465-468. 12. Ritchie AWS. Hindmarsh JR. Bilateral epididyma1 sarcoid. Br J Urol 1979; 51: 240-241. 13. Rudin L. Megalli M. Mesa-Tajada R. Genital sarcoidosis. Urology 1974; 3: 750-754. 14. Singer EP. Hensler NM. Flynn PF. Sarcoidosis: An analysis of forty-five cases in a large military hospital. Am J Med 1959: 26: 364-375. 15. Takagi E, Itoh K, Ohi K, Takeura S, Nagata A, Shimokata K. A case of sarcoidosis with lesions in nasal mucosa and epididymis. Jpn J Thor Dis 1986; 24: 83-86. 16. Torrington KG, Lewis PG, Tellis CJ, Swedarsky RH. Systemic sarcoidosis presenting with a testicular nodule. South Med J 1979; 72: 1 00 1- 1003. 17. Weinberg A. Ginsberg CM. Epididymal sarcoidosis in a prepubertal child. Am J Dis Child 1982; 136: 71-72. 18. Winnacker JL. Becker KL. Katz S, Matthews MJ. Recurrent epididymitis in sarcoidosis report of a patient treated with corticosteroids. Ann Intern Med 1967; 66: 743-748.
S c a d .I [Jrol Ncphrol 26
BILATERAL EPIDIDYMAL SARCOIDOSIS Case Report
Naoki Yamamoto, Yoshikazu Hasegawa, Kohji Miyamoto, Tsuneko Ikeda, Manabu Kuriyama and Yukimichi Kawada From the Departments of Urology, Surgery and Pathology, Matsunami General Hospital, and the Department of Urology, Gifu University School of Medicine, Japan
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/02/15 For personal use only.
(Submitted March 27, 1991. Accepted for publication July 21, 1991)
Abstract. A 59-year-old man presented with a fourmonth history of almost painless bilateral scrotal swellings. Histological examination of a biopsy specimen confirmed sarcoidosis. He was well two months later, the mass had not increased, and he was receiving no treatment. This is the nineteenth reported case to our knowledge, and the eighth with bilateral disease. Key words: sarcoidosis, epididymis, bilateral involvement.
Sarcoidosis is a chronic, multisystem disorder of unknown aetiology characterized by non-caseating epithelioid granulomas in the affected organs. Practically any organ can be affected, but the most common are the lungs, eyes, lymph nodes, and skin (3). Sarcoidosis of the genital organs is relatively rare with a reported incidence of less than 5 Oo/ at necropsy (10) and 0.5% clinically (4, 7). We report an additional histologically diagnosed case of bilateral epididymal sarcoidosis. To our knowledge, this is only the eighth reported case of bilateral disease. CASE REPORT A 59-year-old man presented with a four-month history of almost painless bilateral scrotal swelling. He had no previous episodes of either urethritis or tuberculosis. There was a firm mass at the epididymis on both sides of the scrotum, which seemed to involve the entire portion of the bilateral epididymis. Results of serum biochemical investigations, full blood count and urinalysis were within the reference range. The scrotum was explored because the mass had increased in size despite treatment with antibiotics. The testes were normal and surrounded by small hydroceles. There were no adhesions around the testes, and bilateral epididymes were nodular and hard. The
epididymal tumors, the largest of which measured 5 m m in diameter, were biopsied. Histological examination showed non-caseating granuloma, which is compatible to sarcoidosis (Fig. 1 ) . Neither acid fast bacilli nor spermatozoa were found in the specimen. Computed tomography and magnetic resonance imaging of the chest showed enlarged mediastinal lymph nodes, which was compatible with sarcoidosis (Fig. 2). The activity of angiotensin converting enzyme (ACE) was 22.4 IU, which is above the reference range. Culture of urine failed to grow Mycobacterium tuberculosis and the patient did not reach to skin testing with purified protein derivative of old tuberculin. He was not given steroids and the mass was unchanged two months later.
DISCUSSION Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology that most commonly affects young adults. It usually presents with bilateral hilar lymphadenopathy, pulmonary infiltration, and skin or eye lesions (3). Signs of sarcoidosis vary from incidental radiological findings without associated symptoms to severe incapacity and death (3). Sarcoidosis of the urogenital tract is relatively rare with a reported incidence of less than 5 % at necropsy (1). Krauss described the first clinical case with epididymal sarcoidosis in 1958 (8). Since then until 1990, 18 cases has been reported (1-2, 4-18) (Table I). Though some cases of epididymal sarcoidosis could not be detected clinically, there can be massive disease of all the scrotal contents which presents as a large mass with the epididymis indistinguishable from the testis (9). Epididymal lesions are usually unilateral, being bilateral less common and occur in less than a third of the cases (4). In &and J Urol Nephrol26
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/02/15 For personal use only.
302
N . Yamamoto et al.
Fig. 1. Histopathological slide showing multiple granulomatous lesions containing epithelioid cells surrounding Langhans type giant cells and asteroid body (black arrow), but no Schaumann body was seen.
Fig. 2. Horizontal section of magnetic resonance image of the chest showing enlarged mediastinal lymph nodes.
some cases the disease presents as either acute or chronic epididymitis ( 1 7, 18). The main finding to rule out in any case of suspected epididymal sarcoidosis is a malignant tumour (10). Some researchers have recently reported on the ultrasonographic appearance of epididymal sarcoidosis (2, 4), which is of a homogeneous hypoechoic lesion. Other condi-
tions that present as epididymal enlargement with multiple hypoechoic foci include epididymitis, cystic degeneration, multiple cysts, spermatoceles, sperm granuloma, tuberculosis, lymphogranuloma venereum, granuloma inguinale, filarial granuloma, and malignant tumour (2). The sonographic appearance of any mass may therefore be crucial in suggesting the correct
Table I. Summary of 19 reported cases of epididymal sarcoidosis Case no. (ref.)
Age (yrs)
Side
Size
Treatment
Outcome
41
Right
Orchidectomy
Not applicable
31 21 38 33 41 29
Left Right Right Bilateral Bilateral Bilateral
Steroids None Orchidectom y Steroids Steroids Steroids
Not stated Spontaneous remission Not applicable Epididymitis recurred Improvement Improvement
17 21 35 2
Left Left Right Bilateral
None None Steroids Steroids
Not stated Not stated N o response Remission
20 36 22 56 29 27 20 44
Left Bilateral Left Bilateral Right Bilateral Right Bilateral
Four times normal Not stated Not stated 6 cm long Not stated Not stated Right-Not stated Left 1 . 5 ~ 1 . 5cm 2 cm diameter I x 2 cm Not stated Right 4.5 X 3.5 cm Left 3 ~ 3 . 5cm Not stated 6 x 4 ~ cm 4 (Both) 2 x 2 cm Not stated Not stated 0.5 cm (Largest) 2.5x1.5 cm 2 cm in diameter
Orchidectomy Orchidectom y Orchidectomy Steroids Orchidectomy None None None
Not applicable Not applicable Not applicable Responded Not applicable Not stated Not stated Unchanged
Scand J Urol NephrolZ6
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/02/15 For personal use only.
Bilateral epididymal sarcoidosis management of this disease. Evaluation and treatment of scrota1 mass, although it is rare, should be considered to avoid unnecessary orchidectomy. There are diverse opinions about the effectiveness of steroid treatment. Considering the fact that pulmonary function was normal and there was no ocular involvement in our case, we decided to withhold steroids treatment for the present. The previously reported response to steroid treatment lasted for only 15 months (18). McWilliams et al. claimed that short courses of steroids should be given only when lesions continue to increase in size (10).
REFERENCES 1. Amenta PS, Gonick P, Katz S. Sarcoidosis of
2.
3.
4. 5.
testis and epididymis. Urology 1980; 17: 6 16-61 7. Burke CBJ. Parker MSH. Hopper KD. Pienkos EJ. The ultrasonographic appearance of coexistent epididymal and testicular sarcoidosis. J Clin Ultrasound 1990; 18: 522-526. Crystal RG, Braunwald E. Isselbacher KJ, Petersdorf RG, Wison JD, Martin JB, Fauci AS. Harrison's Principles of Internal Medicine. 1 Ith ed. New York: McGraw-Hill, 1987: 1445-1 450. Forte MD. Brant WE. Ultrasonographic detection of epididymal sarcoidosis. J Clin Ultrasound 1988: 16: 191-194. Gerstenhaber BJ, Green R. Sachs FL. Epididyma1 sarcoidosis: A report of two cases and review of the literature. Yale J Biol Med 1977; 50: 669-61 5.
303
6. Hefferman JC, Blenkinsopp WK. Epididymal sarcoidosis. Br J Urol 1978; 50: 2 I 1. 7. Hines HL. Elgart ML, Mackenzie AR. Sarcoidosis: case presentation and discussion. J Urol 1961; 85: 71-74. 8. Krauss L. Genital sarcoidosis: case report and review of the literature. J Urol 1958; 80: 367-370. 9. McGowan AJ Jr, Smith EH. Urological implications of sarcoidosis. J Urol 1967; 97: 1090-1093. 10. McWilliams WA, Abramowitz L, Tiamson EM. Epididymal sarcoidosis: Case report and review. J Urol 1983; 130: 1201-1203. ' I . Mikheil JR, Mitchell DN, Dyson JL, Williams WJ, Ogunlesi TOO, Van Hein-Wallace SE. Sarcoidosis with genital involvement. Am Rev Res Dis 1972; 106: 465-468. 12. Ritchie AWS. Hindmarsh JR. Bilateral epididyma1 sarcoid. Br J Urol 1979; 51: 240-241. 13. Rudin L. Megalli M. Mesa-Tajada R. Genital sarcoidosis. Urology 1974; 3: 750-754. 14. Singer EP. Hensler NM. Flynn PF. Sarcoidosis: An analysis of forty-five cases in a large military hospital. Am J Med 1959: 26: 364-375. 15. Takagi E, Itoh K, Ohi K, Takeura S, Nagata A, Shimokata K. A case of sarcoidosis with lesions in nasal mucosa and epididymis. Jpn J Thor Dis 1986; 24: 83-86. 16. Torrington KG, Lewis PG, Tellis CJ, Swedarsky RH. Systemic sarcoidosis presenting with a testicular nodule. South Med J 1979; 72: 1 00 1- 1003. 17. Weinberg A. Ginsberg CM. Epididymal sarcoidosis in a prepubertal child. Am J Dis Child 1982; 136: 71-72. 18. Winnacker JL. Becker KL. Katz S, Matthews MJ. Recurrent epididymitis in sarcoidosis report of a patient treated with corticosteroids. Ann Intern Med 1967; 66: 743-748.
S c a d .I [Jrol Ncphrol 26
