CASE REFORTS

GENITOURINARY INVOLVEMENT OF SYSTEMIC

SARCOIDOSIS CONFINED TO TESTICLE *

LCDR. ADAM J. SINGER, MC (USNR)

CAPT. GEORGE J. GAVRELL, MC (USN)

LCDR. RAYMOND B. LEIDICH, MC (USN)

LCDR. ANTHONY D. QUINN, MC (USN)

From the Department of Urology, Naval Hospital, Oakland, California

ABSTRACT-We report on a patient with systemic sarcoidosis who presented with a lesion of the genitourinary tract confined to the testicle. Clinically evident sarcoidosis of the testicle has been identified in few instances. Several recommendations in evaluating such lesions have been offered. A consensus on treatment is not yet defined. The therapeutic options are presented and the litera­ture is reviewed.

Sarcoidosis is a chronic, noncaseating granu­lomatous disease of uncertain etiology. Clinical reports of testicular involvement are infre­quent.1.2 Therefore, a consensus on treatment is not yet defined. Treatment modalities include intraoperative limited biopsy and frozen sec­tion,1.3-5 radical orchiectomy when one testicle is affected, 6 unilateral orchiectomy in the pres­ence of bilateral disease,2.7 and empiric steroid therapy.8 The treatment alternatives are pre­sented and the current literature is reviewed.

Case Report

A twenty-nine-year-old black man with a three-month history of intermittent right testic­ular pain was seen by the urology service. A 3.5 cm X 2 cm mass in the inferior pole of the right testicle was observed the day of admission. No history of trauma or other previous geni­tourinary symptoms were observed.

'The work reported herein was performed under the United States Navv Clinical Investigation Program, case report number 86-1970-62. The opinions or assertions expressed herein are those of tbe authors and are not to be construed as official or as neces­sarily reflecting the views of the Department of the Navy or of the Naval service at large.

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The patient had a two-year history of sar­coidosis. Multisystemic involvement was evi­dent. He had been treated with prednisone for bilateral keratoconjunctivitis, iritis, and uveitis which subsequently became quiescent. Chest x-ray film demonstrated bilateral hilar ade­nopathy, paratracheal adenopathy with promi­nent nodular densities, and a stranding intersti­tial pattern in all lung fields. Swelling of the hands and feet were significant. Radiography confirmed sarcoid dactylitis. Generalized adenopathy had prompted a right supracla­vicular node biopsy when he originally sought medical attention. The histology was consistent with sarcoidosis.

His physical examination was supportive of multisystemic sarcoidosis. Except for the right testicular mass, the genitourinary examination was normal. Urinalysis was normal. Ultra­sound of scrotal contents revealed a normal left testicle and epididymis with a solid mass in the inferior pole of the right testicle. A complete blood count demonstrated normochromic, nor­mocytic anemia. Preoperative beta-human cho­rionic gonadotropin and alphal-fetoprotein were normal.

The patient underwent right radical orchiec­tomy. Pathologic inspection disclosed a 3.5 em

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x 2 cm intraparenchymal nodule without cap­sular invasion, with a normal epididymis and spermatic cord. Multiple histologic sections confirmed noncaseating granulomas centered around seminiferous tubules with diffuse oblit­eration of tubular lumina and marked desmo­plastic response. Stains for acid-fast bacilli and fungi were negative (Fig. 1). There were no postoperative complications. The patient has remained stable.

Comment

Prior to this report, there were seven ac­counts of clinically evident sarcoidosis involving the testicle. However, epididymal involvement was noted in five. In two instances, the method of sampling was incisional biopsy alone and no evaluation of the epididymis was documented. Other studies enumerate findings at autopsy. Sarcoidosis of the testicular tunics has also been described. 1,2

The peak incidence of sarcoidosis and testicu­lar neoplasia coincide at twenty to forty years of age. Statistically, sarcoidosis occurs more com­monly in blacks than in whites by 20: 1. Al-

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FIGURE 1. (A) Right radical orchiectomy; speci­men with mass at inferior pole. (B) Granulomas and adjacent uninvolved testis at lower power (original magnification x 10). (C) Noncaseating granulomas at medium power (original magnification x 40).

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ternatively, testicular tumors are uncommon in blacks, accounting for less than 3 percent of re­ported cases. The incidence of neoplasia of the testicle in the general population is approxi­mately 2 per 100,000.2

Importantly, systemic sarcoidosis can occur synchronously with testicular tumors. Granu­lomatous reactions have been associated with seminoma, teratoma, teratocarcinoma, and embryonal carcinoma. 6 ,9-13 Epithelioid granu­lomas may be extensive in some seminomas, simulating granulomatous orchitis. 3

Partial biopsy and steroid therapy would not appear to be appropriate. Obvious disadvan­tages to these methods include misdiagnosis by frozen section, sampling errors by incisional biopsy or unilateral orchiectomy in the presence of bilateral disease, and delay in diagnosis when steroids are used empirically.

We recommend preoperative tumor markers and standard radical orchiectomy in a patient with sarcoidosis presenting with a testicular mass. Bilateral orchiectomy is controversial; however, it should be considered for tumors presenting in both testicles.

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Summary

To our knowledge this is the first reported case of clinically evident, histologically proved, svstemic sarcoidosis confined to the testis. Vie believe the minimum diagnostic and thera­peutic modality includes radical orchiectomy.

Clinical Investigation Department Naval Hospital

Oakland, California 94627 (DR. SINGER)

References

1. Seaworth JF, Davis 5J, and Donovan W:-.I: Aggressive diagnostic approach indicated in testicular sarcoidosis, Urology 21: 396 (1983).

2. Haas GP, Badalament R, Wonnell OM, and Miles BJ: Testic­ular sarcoidosis: a case report and review of the literature, J Urol 135: 1254 (1986).

3. Chowdhury SO, and Higgins PM: Sarcoid of the testis, Br J

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Urol 45: 218 (1973). 4. Wees 5J: Testicular sarcoidosis, South Med J 74: 255 (1981). 5. Turk CO, Schacht M, and Ross L: Diagnosis and manage.

ment of testicular sarcoidosis, J Urol 135: 380 (1986). 6. Blacher EJ, and Maynard JF: Seminoma and sarcoidosis: an

unusual association, J Urol 26: 288 (1985). 7. Amenta PS, Gonick P, and Katz SM: Sarcoidosis of the testis

and epididymis, Urology 17: 616 (1981). 8. Opal SM, Pittman DL, and Hofeldt FD: Testicular sar­

coidosis, Am J Med 67: 147 (1979). 9. Allison RE, and Urbanski SJ: More about association of

seminoma and sarcoidosis, Urology 27: 86 (1986). 10. Colebunders R, Bultinck J, Servais J, and Denis L: A pa­

tient with testis seminoma, sarcoidosis, and neutropenic enteroco­litis, Hum Pathol15: 394 (1984).

11. Geller RA, Kuremsky DA, Copeland JS, and Stept R: Sar­coidosis and testicular neoplasm: an unusual association, J Urol lI8: 487 (1977).

12. Fossa SO, et al: Sarcoid reaction of hilar and paratracheal lymph nodes in patients treated for testicular cancer, Cancer 56: 2212 (1985).

13. Trump DL, Ettinger OS, Feldman MJ, and Dragon LH: Sarcoidosis and sarcoid-like lesions. Their occurrence after cvto­toxic and radiation therapy of testis cancer, Arch Intern Med 141: 37 (1981).

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