Category: Adrenal Disorders

Monitor: 6

6 - AN OCCULT SOURCE OF ANDROGENS: UNDIAGNOSED CONGENITAL ADRENAL HYPERPLASIA CAUSING INADEQUATE RESPONSE TO ANDROGEN DEPRIVATION THERAPY IN PROSTATE CANCER

Friday, Apr 26
11:30 AM – 12:00 PM

Objective : This case highlights the importance of testosterone level monitoring in prostate cancer patients after medical or surgical castration.


Methods : This is a case report. Please see details under discussion section.


Results :

This is a case report. Please see details under discussion section.


Discussion :

We present the case of a 73-year-old Caucasian male with history of adenocarcinoma of the prostate (pT2CN0M0 with Gleason score of 9) undergoing androgen deprivation therapy. The patient underwent radical prostatectomy upon initial diagnosis and was later started on androgen deprivation therapy following local recurrence of tumor with involvement of pelvic lymph nodes. Despite treatment with leuprolide, he continued to have total testosterone levels as high as 294.4 ng/dL as well as elevated prostate specific antigen (PSA).  During routine surveillance CT scan for cancer restaging, patient was found to have incidental bilateral adrenal nodular hyperplasia. He was referred to our endocrinology clinic for further evaluation of adrenal nodular hyperplasia and failure to suppress his testosterone levels. Given concerns about overproduction of adrenal androgens, the levels of androstenedione and dehydroepiandrosterone were obtained and both were elevated. Consequently, an adrenocorticotropic hormone (ACTH) stimulation test was performed demonstrating significant elevation in 17-hydroxyprogesterone at baseline (19,910 ng/dL) with increase to (>20,000 ng/dL) at 30 and 60 minutes. Cortisol level, however, remained unchanged at ~5 mcg/dL throughout the stimulation test.  Based on these results, genetic testing for congenital adrenal hyperplasia was pursued revealing a common and rare mutation of the CYP21A2 gene. The first mutation was located in intron 2 and is usually associated with simple virilizing or salt wasting phenotypes of classic congenital adrenal hyperplasia. The second common mutation detected was in the I172N sequence and is associated with the simple virilizing phenotype. The patient had no salt-wasting features and was clinically diagnosed with simple virilizing, classic congenital adrenal hyperplasia. The patient was started on dexamethasone and had marked decrease in his adrenal androgens, testosterone, and PSA levels. 21-hydroxylase deficiency comprises nearly 95% of all congenital adrenal hyperplasia cases with an incidence of 1 in 10,000 in Western countries. Males with the simple virilizing subtype are at risk of not being diagnosed because they are phenotypically normal at birth. 


Conclusion : Inadequate responses to androgen deprivation therapy should trigger further evaluation for congenital adrenal hyperplasia, a condition that is often overlooked.

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Mustafa Kinaan

Endocrinology Fellow
University of Central Florida College of Medicine/HCA GME Consortium
Kissimmee, Florida

Dr. Kinaan is a first year endocrinology fellow at the University of Central Florida College of Medicine/HCA GME Consortium program.

Akin Eraslan

Endocrinologist
Division of Endocrinology, Orlando VA Medical Center.

Dr. Akin Eraslan is an endocrinologist at the Orlando VA Medical Center.

Hanford Yau

Endocrinologist
The University of California, San Francisco, Fresno, CA
ORLANDO, Florida

Dr. Hanford Yau is an endocrinologist at the Orlando VA Medical Center.

Kenneth Simon

Oncologist
Division of Hematology and Oncology, Orlando VA Medical Center.

Dr. Kenneth Simon is an oncologist at the Orlando VA Medical Center