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Testis Sparing Surgery for Steroid Unresponsive Testicular Tumors of the Adrenogenital Syndrome

1997; Lippincott Williams & Wilkins; Volume: 157; Issue: 4 Linguagem: Inglês

10.1016/s0022-5347(01)65023-7

1527-3792

Brady R. Walker, Steven J. Skoog, Boyd H. Winslow, Douglas A. Canning, Edward S. Tank,

Adrenal and Paraganglionic Tumors

No AccessJournal of UrologyPediatric Urology: Editorial1 Apr 1997Testis Sparing Surgery for Steroid Unresponsive Testicular Tumors of the Adrenogenital Syndrome Brady R. Walker, Steven J. Skoog, Boyd H. Winslow, Douglas A. Canning, and Edward S. Tank Brady R. WalkerBrady R. Walker , Steven J. SkoogSteven J. Skoog , Boyd H. WinslowBoyd H. Winslow , Douglas A. CanningDouglas A. Canning , and Edward S. TankEdward S. Tank View All Author Informationhttps://doi.org/10.1016/S0022-5347(01)65023-7AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract 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 abnormalaties. We believe that surgical enucleation is the procedure of choice for all patients with this tumor, since it maximizes future fertility potential. References 1 : Macrogenitosomia precox associated with hyperplasia of the adrogenic tissue of the adrenal and death from corticoadrenal insufficiency. Case report. Endocrinology1940; 26: 385. Google Scholar 2 : Die cortisondauerbehandlung des Kongenitalen Adrenogenitalen Syndroms. Helv. Paed. Acta1953; 8: 386. Google Scholar 3 : Testicular tumors in congenital adrenal hyperplasia: steroid measurements from adrenal and spermatic veins. J. Clin. Endocr. Metab.1991; 73: 1129. 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Google Scholar From the Divisions of Urology, Oregon Health Sciences University, Portland, Oregon, and Medical College of Virginia, Norfolk, Virginia, and Pediatric Urology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania© 1997 by American Urological Association, Inc.FiguresReferencesRelatedDetailsCited byAshley R, McGee S, Isotaolo P, Kramer S and Cheville J (2018) Clinical and Pathological Features Associated With the Testicular Tumor of the Adrenogenital SyndromeJournal of Urology, VOL. 177, NO. 2, (546-549), Online publication date: 1-Feb-2007.POHL H, SHUKLA A, METCALF P, CILENTO B, RETIK A, BAGLI D, HUFF D and RUSHTON H (2018) PREPUBERTAL TESTIS TUMORS: ACTUAL PREVALENCE RATE OF HISTOLOGICAL TYPESJournal of Urology, VOL. 172, NO. 6 Part 1, (2370-2372), Online publication date: 1-Dec-2004. Volume 157Issue 4April 1997Page: 1460-1463 Advertisement Copyright & Permissions© 1997 by American Urological Association, Inc.MetricsAuthor Information Brady R. Walker More articles by this author Steven J. Skoog More articles by this author Boyd H. Winslow More articles by this author Douglas A. Canning More articles by this author Edward S. Tank More articles by this author Expand All Advertisement PDF downloadLoading ...