Clinical_Rounds_in_Endocrinology_Volume_II_-_Pediatric_Endocrinology

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31. How to evaluate a child with ACTH - independent Cushing ’ s syndrome?

Adrenal CT is the fi rst-line investigation in children with ACTH-independent

Cushing’s syndrome. Presence of a heterogeneous unilateral mass >4 cm with

irregular margin, necrosis, hemorrhage or calcifi cation, attenuation value >10

HU, and delayed contrast washout (<50 % at 10 min) favors a diagnosis of

adrenocortical carcinoma (ACC, Fig. 4.11 ). In contrast, adrenal adenomas are

small (<4 cm), homogenous, and have regular margins, with attenuation value

<10 HU and have early contrast washout (>50 % at 10 min). Children with

PPNAD usually have normal adrenals on imaging; however, occasionally

“beads on a string appearance” may be seen on adrenal CT.

Further Readings

1. Chan L, Storr H, Grossman A, Savage M. Pediatric Cushing’s syndrome: clinical features,
   diagnosis, and treatment. Arq Bras Endocrinol Metab. 2007;51:1261–71.


2. DeGroot L, Jameson J. Endocrinology. Philadelphia: Saunders/Elsevier; 2010.


3. Inder WJ, Hunt PJ. Glucocorticoid replacement in pituitary surgery: guidelines for periopera-
   tive assessment and management. J Clin Endocrinol Metab. 2002;87:2745–50.


4. Melmed S, Williams R. Williams textbook of endocrinology. Philadelphia: Elsevier/Saunders; 2011.


5. Nieman LK, Biller BM, Findling JW, Murad MH, Newell-Price J, Savage MO, Tabarin
   A. Treatment of Cushing’s syndrome: an endocrine society clinical practice guideline. J Clin
   Endocrinol Metab. 2015;100:2807–31.


6. Nieman L, Biller B, Findling J, Newell-Price J, Savage M, Stewart P, et al. The diagnosis of
   Cushing’s syndrome: an endocrine society clinical practice guideline. J Clin Endocrinol Metab.
   2008;93:1526–40.


7. Savage MO, Storr HL. Pediatric Cushing’s disease: management issues. Indian J Endocrinol
   Metab. 2012;16:S171–5.


8. Storr H, Chan L, Grossman A, Savage M. Paediatric Cushing’s syndrome: epidemiology,
   investigation and therapeutic advances. Trends Endocrinol Metab. 2007;18:167–74.


9. Stratakis C. Cushing syndrome in pediatrics. Endocrinol Metab Clin North Am.
   2012;41:793–803.

Fig. 4.11 Axial CT upper
abdomen showing
heterogeneous mass in the
left adrenal region abutting
the pancreatic tail
suggestive of
adrenocortical carcinoma
( red arrow )

4 Childhood Cushing’s Syndrome