Testicular tumors in Carney's complex.

PURPOSE: Bilateral sex cord stromal testicular tumors are common in the syndrome of myxoma, spotty pigmentation and endocrine overactivity (Carney's complex). Large cell calcifying Sertoli cell tumor is the particular testicular tumor found in Carney's complex. A clinicopathological review of 26 patients is presented.

MATERIALS AND

METHODS

We report 2 cases of Carney's complex with testicular tumors. An additional 24 patients with Carney's complex and testicular tumors were identified by MEDLINE search and review of the literature.

RESULTS

Bilateral testicular tumors were found in 16 patients (61%) with a familial occurrence in 10 (38%). A testicular mass was the most common presentation. The associated findings of Carney's complex included cardiac myxoma in 16 patients, skin myxoma in 16, skin pigmentation in 15, Cushing's syndrome in 8, acromegaly in 3 and schwannoma in 3. Excisional biopsy, surveillance, bilateral orchiectomy and unilateral orchiectomy were performed in 7, 4, 7 and 8 patients, respectively.

CONCLUSIONS

No local tumor recurrence or metastasis has developed in patients with bilateral and/or multifocal testicular tumors. Excisional biopsy or surveillance only are treatment options for bilateral testicular tumors in Carney's complex.