Hi Mar M.
I found the following post from Carol M. in the archives. It has some very helpful information and sights you can read. I believe the tumors are usually caused by undertreatment of cah and that the first line of treatment is increased steroid therapy.
Very best wishes, Lisa
The following is a post I cut and pasted from Carol M. from the archives:
| 1: J Urol. 1997 Apr;157(4):1460-3. | Related Articles,[unauthorised script deleted] language=JavaScript1.2> Links |
Testis sparing surgery for steroid unresponsive testicular tumors of the adrenogenital syndrome.
Walker BR, Skoog SJ, Winslow BH, Canning DA, Tank ES.
Division of Urology, Oregon Health Sciences University, Portland, USA.
PURPOSE: Surgical management of steroid unresponsive testicular tumors of the adrenogenital syndrome has been orchiectomy. Magnetic resonance imaging (MRI) of these tumors accurately delineates the extent of disease. Testis sparing surgery is an important consideration, since male individuals with congenital adrenal hyperplasia are potentially fertile. We present our results of surgical management of this tumor based on MRI findings. MATERIALS AND METHODS: Four boys with steroid unresponsive testicular tumors of the adrenogenital syndrome were evaluated with MRI, testicular ultrasound and color flow Doppler examinations preoperatively and postoperatively. Three patients had 21-hydroxylase deficiency and 1 had 3-beta-hydroxysteroid dehydrogenase deficiency. Contralateral testicular abnormalities included a vanished testis, testicular atrophy due to trauma and bilateral tumors in 1 boy each. Bilateral orchiectomy and surgical enucleation were performed in 1 and 3 patients, respectively. Followup ranged from 8 to 18 months. RESULTS: Postoperative MRI of the testis in 2 of 3 patients showed no evidence of recurrent tumor. Postoperative testicular sonography revealed no tumor and vascular flow in 2 of 3 patients. All 3 patients who underwent testis sparing surgery have a viable testis in the scrotum without evidence of recurrent disease. CONCLUSIONS: MRI of the testis in patients with testicular tumors of the adrenogenital syndrome accurately defines the extent of disease. Surgical enucleation of this tumor has been performed successfully without recurrent disease. This surgical approach should be considered for any patient with a steroid unresponsive tumor and contralateral abnormalities. We believe that surgical enucleation is the procedure of choice for all patients with this tumor, since it maximizes future fertility potential.
.............Semen analysis could be performed in 11 patients (Table 4
). Azoospermia was found in three patients, all of them had palpable tumors. In four patients, semen analysis revealed oligoasthenoteratozoospermia.........
..............When azoospermia is found in combination with a large testicular tumor on ultrasonography, it is likely to have a mechanical cause, especially when the tumor is located in the mediastinum. At this location, large tumors can easily compress the rete testis and cause obstructive azoospermia (36).
................The preferred method of treatment of testicular adrenal rest tumors and/or impaired spermatogenesis in patients with CAH is intensifying glucocorticoid therapy. This may lead to decrease of tumor size and improvement of testicular function. When the tumor is unresponsive to steroid therapy, surgical treatment should be considered, preferably by a testis-sparing procedure, instead of orchiectomy (5). Also, cryopreservation of the semen can be offered, because fertility prognosis is yet uncertain.
Carol M.