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Abdomen » Retroperitoneum
Virilizing Adrenocortical Adenoma
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Presentation A two-year old female child presented with signs of virilization. Laboratory tests confirmed the presence of increased levels of testosterone, dehydroepiandrosterone (DHEAS) and 17-hydroxy progesterone. The child was confirmed to be genetically a female, therefore her symptoms could be described as heterosexual pseudoprecocious puberty. An abdominal ultrasound was performed in search of a cause of this syndrome.
Caption: Transverse scan of the right upper quadrant
Description: A mass appears above the upper pole of the right kidney, abutting the external wall of the inferior vena cava. The mass is hypoechoic, slightly inhomogeneous
Caption: Sagittal scan of the right kidney
Description: The mass is inhomogeneous, with a regular shape and it measures 7cm X 5.5cm There is a clear separation between the tumor and the right kidney.
Caption: Transverse scan of the right upper quadrant – color Doppler
Description: The mass presents poor vascularization.
Differential Diagnosis The main differential diagnosis in the case of a testosterone-secreting adrenal mass in childhood is between an adrenal adenoma and carcinoma.
Final Diagnosis Adrenal Adenoma
Discussion     The most common causes of virilisation in children are the adrenarche (the normal onset of androgen secretion from the adrenal gland), congenital adrenal hyperplasia (CAH) and adrenocortical tumors [1]. These tumors are quite rare, with a frequency of 0.3-0.38 cases per one million children a year [2]. They may occur in several syndromes, such as hemyhypertrophy, Beckwith-Wiedemann or Li-Fraumeni syndrome, but they may also be isolated [3]. Unlike other childhood tumors, the frequency does not increase with age. The peak incidence is between 0 and 4 years, after which it decreases rapidly. This pattern of age distribution is common for tumors of embryonic origin [3, 4].
    It is common for an adrenal tumor to be an incidental discovery in adults. However, children usually present with clinical manifestations due to the increased level of one or more of the following hormones: androgens, cortisol, aldosterone or estrogen. 90% of adrenocortical tumors produce one or more of these hormones and non-secreting tumors are much more rare than in the adult population [4]. In most cases, adrenocortical tumors produce an excess of testosterone, which causes virilisation in girls and isosexual pseudoprecocious puberty in boys. It seems that functional tumors have a better prognosis than non-secreting ones [5].
    The clinical signs and symptoms of a virilising tumor include hypertrophy of the clitoris or penis, acne, pubic hair, gynaecomastia, deepening of the voice, accelerated growth and, in a few cases, a palpable abdominal tumour [2].
Ultrasound is suitable as a first examination in children who present with virilising features [6].Because adrenal tumors in children may have extensive areas of necrosis and hemorrhage, they are very friable. Therefore needle biopsies should be avoided [4].
    Even by combining ultrasound, CT and MRI the differential diagnosis between adrenal adenoma and carcinoma is very difficult in children.  The large size of the tumor in this case did raise the possibility of it being an adrenal carcinoma, although there were no enlarged abdominal lymph nodes or extension towards the inferior vena cava.
    The treatment of adrenocortical tumors in children is always surgical, as there is always a risk for the tumor to be malignant, even if its imaging aspect is benign [4].
Case References
  1. Street ME et al Girls with virilisation in childhood: a diagnostic protocol for investigation, J Clin Pathol 1997; 50:379-383
  2. Bonfig W, Bittmann I, Bechtold S Virilising adrenocortical tumours in children, Eur J Pediatr 2003; 162:623–628
  3. Ribeiro RC et al Adrenocortical tumors in children, Braz J Med Biol Res 2000; 33(10):1225-1234
  4. Ribeiro RC, Figueiredo B Childhood adrenocortical tumours, European Journal of Cancer 2004; 40:1117–1126
  5. Patil KK, Ransley PG , McCullagh M, Malone M and Spitz L Functioning adrenocortical neoplasms in children, BJU International 2002; 89:562–565
  6. Westra SJ, Zaninovic AJ, Hall TR, Kangarloo H, Boechat I Imaging of the adrenal gland in children, RadioGraphics 1994; 14:1323-1340
Follow Up The mass in the right adrenal gland was removed surgically and was determined to be an adrenal adenoma.
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