Case of the Week: August 5-August 12, 2010

13 year old with congenital adrenal hyperplasia. Diagnosis please?

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Case Details

Ultrasound images show hypoechoic masses of the mediastinum testes bilaterally with increased flow.

Answer

Diagnosis: TART

Adrenal rest tumors commonly occur in the testes of male patients with congenital adrenal hyperplasia(CAH).   

These tumors rarely occur in the ovaries of female patients with CAH.  

These patients have elevation of ACTH usually due to 21-hydroxylase deficiency.

   Many are aldosterone impaired as well. 

  Adrenal glands develop adjacent to the testes in utero and therefore adrenal tissue may travel with the testis during development and develop in response to high cortisol levels.     

 Neonates have a low incidence of testes adrenal rest tissue(TART)

 Routine screening by ultrasound is recommended by the age of 7 or 8 years. 

     Masses, ranging in size from 2cm-4cm, are found usually in the mediastinum testis and are typically hypoechoic with increased flow.

Masses greater than 2 cm can be palpated.

  MR is equally good at detection but of course is more expensive and may require sedation.

Lesions are variable in signal on T1, low in signal on T2, and do enhance.     

Treatment includes intensifying glucocorticoid therapy, but in not always successful.

TART is the most common cause of infertility in adult males with CAH. 

 Masses can lead to fibrosis and lymphocyte infiltration of the testes.  

Surgery for removal of TART is done in some adult males for relief of pain or even in an attempt to improve fertility, but is not yet being done in children.    

The differential includes neoplasms such as leukemia, lymphoma, germ cell tumor, metastases and Leidig cell tumor. Metastases are rare in children. Most patients with leukemia or lymphoma are known to have that disease.

If lesions are bilateral, other primary neoplasms are much less likely.

 Usually when imaging is consistent in a patient with CAH, biopsy is not necessary and patient can be followed with US.

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