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A 5 cm round well encapsulated Rt. adrenocortical solid mass was removed. Histology showed benign adenoma, but as always with endocrine adenomas, malignancy can not be ruled out completely, and only time will tell. Post-operative testosterone is 1.2 nmol/L. The prolonged suppression of the HPA axis, by very high testosterone, will take some time to recover, as well as for her ovarian function to resume and periods to start again.

She did have clitoromegaly according to our urologist colleague, now this may not shrink back to normal.
 
BW
 
Mohammad
 
Dr. M A Al-Jubouri, MB ChB, MSc, FRCP Edin, FRCPath
Consultant Chemical Pathologist


----- Original Message ----
From: Mohammad Al-Jubouri <[log in to unmask]>
To: Mohammad Al-Jubouri <[log in to unmask]>; [log in to unmask]
Sent: Wednesday, 12 March, 2008 3:12:47 PM
Subject: Re: Another exciting fresh case

Thanks for the 4 colleagues who responded so far, exhibiting various valid points as follows:

 

1. Try putting “testosterone-secreting ovarian tumor”into Google Scholar.

 

2. Was ‘she’ hypertensive?  I am puzzled. Looks like a girl brought up as a boy.

How about 17hydroxlase deficiency?

Steroid profiling would be the next step and of course karyotyping.

 

3. I wonder if this is a case of 5 alpha reductase deficiency if not ?on progestorone pills.

 

4. Measure DHT? alpha reducatse activity ?Androgen insensitivity? Taken testo injection? iatrogenic? Other androgens - dheas, a4, 17oh prog , ovarian/liver scans?

Gender assignment? Androgen secreting tumour?

 

Further info and final diagnosis:

 

Karyotyping: 46XX
Not taking any exogenous testosterone.
Examination reveals signs of hirsuitism that the patient conceals well as she works in a beauty saloon. She also has a deep voice that she tries to conceal.

Further tests showed:

DHEAS H >27.1 umol/L (0.0-12.0)
Androstenedione H >35.0 nmol/L(3.0-10.0)
17-OH-Progesterone 9.7 nmol/L (< 12)
Cortisol 243 nmol/L

MRI of adrenal showed a well encapsulated 5 cm lesion in the right adrenal.

This is likely to be a benign androgen secreting adrenal adenoma, and patient should be cured by its removal.

The very high testosterone is a bit unusual for an adrenal adenoma, even when using extraction step, the testosterone level was quite high at 18.5 nmol/L. The direct assay probably showed a lot of interference from DHEAS and androstenedione. However, the patient has done well to conceal her virilsation features. I also found it unusual to come across two aetiologically different cases of females with male level tsetsosterone in such a short period of time. It all adds up to our continuous learning, I hope.

 

regards

 

Mohammad

----- Original Message ----
From: Mohammad Al-Jubouri <[log in to unmask]>
To: [log in to unmask]
Sent: Tuesday, 11 March, 2008 4:14:59 PM
Subject: Another exciting fresh case

This is same sort of case to the one I presented earlier of a female with testosterone at the high end of male range, see below email.
 
This time,  a GP referred 17-year-old female for secondary amenorrhoea investigations, hormonal tests were as follows:

Oestradiol         211pmol/L
Progesterone    20.9 nmol/L
FSH                 <0.1 IU/L
LH                    <0.1 IU/L
Testosterone       36.2 nmol/L ( 0.5-2.6 )
Testosterone by extraction method  18.5 nmol/L

Despite the phenomenally high testosterone, the GP confirmed that there were no obvious virilisation features and the patient looked phenotypically female, adrenarch started at 8-9, menarch at 15 with rapid cessation afterward.

Can you suggest further tests/diagnosis?
 
Regards
 
Mohammad
Dr. M A Al-Jubouri, MB ChB, MSc, FRCP Edin, FRCPath
Consultant Chemical Pathologist


----- Forwarded Message ----
From: Mohammad Al-Jubouri <[log in to unmask]>
To: ACB MAILBASE <[log in to unmask]>
Sent: Friday, 22 June, 2007 2:36:52 PM
Subject: Exciting fresh case

It is nothing more exciting than picking up a fresh case from GP land, that no body has diagnosed before.
 
The case is of 21-year-old lady presented to her GP with primary amenorrhoea. Routine hormonal profile revealed:
 
FSH             2.1 U/L
LH               21.6 U/L
Oestradiol    112 pmol/L
Prolactin      335 mU/L
 
This should be enough to make a final diagnosis.
 
Any taker?
 
regards
 
Mohammad


Dr. M A Al-Jubouri
Consultant Chemical Pathologist


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A Smarter Email. ------ACB discussion List Information-------- This is an open discussion list for the academic and clinical community working in clinical biochemistry. Please note, archived messages are public and can be viewed via the internet. Views expressed are those of the individual and they are responsible for all message content. ACB Web Site http://www.acb.org.uk List Archives http://www.jiscmail.ac.uk/lists/ACB-CLIN-CHEM-GEN.html List Instructions (How to leave etc.) http://www.jiscmail.ac.uk/