Sarcoidosis presenting as intrascrotal mass: case report and review.

Abstract: Sarcoidosis is a multisystemic disease that usually involves the lungs and lymph nodes, but almost any organ can be involved. Genitourinary involvement with sarcoidosis is extremely rare. We report the case of a 30-year-old African-American male who presented with a right-sided intrascrotal mass and diffuse lymphadenopathy. On further workup, he was found to have sarcoidosis. Two months of corticosteroid treatment resulted in the disappearance of his intrascrotal mass.

Key Words: sarcoidosis, noncaseating granulomas, genitourinary sarcoidosis, testicular sarcoidosis, epididymal sarcoidosis

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Sarcoidosis is a systemic granulomatous disease of uncertain etiology characterized by the formation of noncaseating granulomas in multiple organs. It rarely involves the genitourinary tract; however, because of concerns regarding possible testicular malignancy, one third of male patients with this type of involvement may receive unnecessary orchiectomies. (1)

A 30-year-old African-American male presented to our hospital with a 5-month history of painless right-sided intrascrotal swelling. He denied any history of fever, weight loss, night sweats, dysuria or trauma. He was a nonsmoker and his review of systems was unremarkable. Examination revealed bilateral inguinal, axillary, and cervical lymphadenopathy. There was mild splenomegaly. Cardiac and lung examinations were unremarkable. The genitourinary system examination revealed both testes to be nontender and of normal size. There was a nontender right epididymal mass measuring 3 to 4 cm in size. There was no skin rash, clubbing, pedal edema or jugular venous distension. Scrotal ultrasound revealed multiple hypoechoic areas scattered throughout both testicles. There were no calcifications or gross edema. The right epididymis was enlarged. Lab work showed complete blood count, electrolytes, renal function, and liver function tests to be within normal limits. [beta]-human chorionic gonadotropin and a-fetoprotein levels were normal. His chest x-ray revealed bilateral mediastinal and hilar lymphadenopathy with reticulonodular shadows more prominent in the upper zones. CT scan of the chest (Fig. 1) showed mediastinal and hilar lymphadenopathy with bilateral peribronchiovascular nodularity, more prominent in the upper lobes. The patient's HIV (human immunodeficiency virus), PPD (purified protein derivative) and sputum for AFB (acid-fast bacilli) tests were all negative. His serum ACE (angiotensin-converting enzyme) level was 198 U/L (normal range of 9-67 U/L), and FNAC (fine needle aspiration cytology) of the right inguinal lymph node showed noncaseating granulomatous inflammation. His right cervical lymph node biopsy (Fig. 2) also showed compact noncaseating granulomas consistent with sarcoidosis. We started the patient on prednisone 40 mg/d and after two months of treatment, his scrotal swelling disappeared.

Discussion

Sarcoidosis is a multisystem disorder of unknown cause(s). (2) In 84% of cases, the disease is localized to the chest. (3) Genitourinary involvement with sarcoidosis is very uncommon, occurring in 0.2% of the clinically diagnosed cases and 5% of the cases found at autopsy. (4,5) Genitourinary manifestations of sarcoidosis include renal disease with nephrocalcinosis, painless testicular masses, and acute epididymo-orchitis with testicular swelling. Genitourinary sarcoidosis has been shown to be ten times more frequent in black men. (5)

[FIGURE 1 OMITTED]

Testicular involvement of sarcoidosis is very rare. Schaumann described the first case of testicular sarcoidosis in 1936. (6) It occurs most commonly in black males between 20 and 40 years of age. (7) Sarcoid granulomas have been found in order of decreasing frequency in the epididymis, testis and prostate gland. Involvement of the spermatic cord, scrotum and penis is rare. The clinical presentation is usually a scrotal mass. Epididymal involvement by sarcoidosis is usually unilateral, nodular and painless. Bilateral lesions are less common and reported in less than a third of patients with epididymal sarcoidosis. (8)

Other lesions to consider in the differential diagnosis of a scrotal mass include testicular tumor, tuberculosis, sperm granulomas, cysts and less commonly, lymphogranuloma inguinale, granuloma inguinale, filarial granulomas and other fungal diseases. If a lesion can be localized confidently to the epididymis on physical examination and the patient has other evidence of sarcoidosis or a history of sarcoid, then surgical exploration is not mandatory. (9) However, if the lesion continues to enlarge on corticosteroid therapy, then a surgical biopsy should be performed. (9) Lesions involving the testis alone or epididymis and testis should be explored, even if the patient has evidence of sarcoidosis in other organ systems. (9)

Therapy for genitourinary sarcoidosis is controversial. Spontaneous resolution has been shown to occur. (10) The symptoms of pain and swelling are usually relieved by corticosteroid treatment. However, in most instances of symptomatic epididymal sarcoidosis, excisional biopsy is necessary for the cessation of pain. The effect of genitourinary sarcoidosis on fertility has not been studied but it can be assumed that fibrosis and occlusion of the ductus epididymis by sarcoidosis could cause azoospermia. (4) Recently, a patient with testicular sarcoidosis has shown improvement of azoospermia and hypogonadism after treatment with high dose corticosteroids. (11) Svetec and colleagues (12) have reported a patient with systemic and epididymal sarcoidosis whose sperm counts fluctuated in response to steroid courses administered for control of pulmonary disease activity. The improvements in sperm count observed in their patient suggested that ductal obstruction could respond to steroid therapy, although spontaneous resolution could not be excluded.

[FIGURE 2 OMITTED]

In conclusion, when intrascrotal masses are found in patients with confirmed sarcoidosis, the possibility of sarcoidosis should always be considered because it can prevent unnecessary orchiectomies.

References

1. Carmody JP, Sharma OP. Intrascrotal sarcoidosis: case reports and review. Sarcoidosis Vasc Diffuse Lung Dis 1996;13:129-134.

2. Yamamoto M, Sharma OP, Hosoda Y. Special report: the 1991 descriptive definition of sarcoidosis. Sarcoidosis 1992;9:33-34.

3. Roos N, Bick U, Vassallo P, et al. Thorakale sarkoidose. Radiologe 1990;30: 581-590.

4. Turk CO, Schacht M, Ross L. Diagnosis and management of testicular sarcoidosis. J Urol 1986;135:380-381.

5. Ricker W Clark M. Sarcoidosis: a clinical pathologic review of 300 cases including 22 autopsies. Am J Clin Pathol 1949;19:725.

6. Schaumann J. Lymphogranulomatosis benigna in the light of prolonged clinical observations and autopsy findings. Br J Dermatol 1936;48:399.

7. Singer AJ, Gavrell GJ, Leidich RB, et al. Genitourinary involvement of systemic sarcoidosis confined to testicle. Urology 1990;35:442-444.

8. Gerstenhaber BJ, Green R, Sachs FL. Epididymal sarcoidosis: a report of two cases and a review of the literature. Yale J Biol Med 1977;50:669-675.

9. McWilliams WA, Abramowitz L, Tiamson EM. Epididymal sarcoidosis: case report and review. J Urol 1983;130:1201-1203.

10. Ryan DM, Lesser BA, Crumley LA, et al. Epididymal sarcoidosis. J Urol 1993;149:134-136.

11. Rees DA, Dodds AL, Rathbone N, et al. Azoospermia in testicular sarcoidosis is an indication for corticosteroid therapy. Fertil Steril 2004;82:1672-1674.

12. Svetec DA, Waguespack RL, Sabanegh ES. Intermittent azoospermia associated with epididymal sarcoidosis. Fertil Steril 1998;70:777-779.

Time is the coin of your life. It is the only coin you have, and only
you can determine how it will be spent. Be careful lest you let other
people spend it for you
--Carl Sandburg

Tajender S. Vasu, MD, Robert S. Lai, MD FRCSC, Ioana G. Amzuta, MD, Michel R. Nasr, MD, and Robert J. Lenox, MD, FCCP

From the Department of Medicine and the Division of Pulmonary and Critical Care Medicine, State University of New York Upstate Medical University, Syracuse, NY.

Reprint requests to Robert J. Lenox, MD, FCCP, Associate Professor of Medicine, Department of Medicine, Division of Pulmonary and Critical Care Medicine, State University of New York Upstate Medical University, 750 East Adams Street, Syracuse, NY 13201. Email: [e-mail]

Accepted March 2, 2006.

RELATED ARTICLE: Key Points

* Sarcoidosis is a systemic granulomatous disease of unknown etiology.

* It rarely involves the genitourinary system.

* Spontaneous resolution of genitourinary sarcoidosis has been known to occur.

* One third of male patients with testicular sarcoidosis may receive unnecessary orchiectomies.