Edition 53 - 2015, June / Images

Images in Pediatric Endocrinology: PEDIATRIC CUSHING’ SYNDROME SECONDARY TO AN ADRENAL CARCINOMA – Ed. 53

Celeste Mattone y Silvia M Gil

Servicio de Endocrinología. Hospital de Pediatría “Prof. Dr. J. P. Garrahan”, Buenos Aires, Argentina.

Figure 1. Cushing’s syndrome

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Photograph showing the girl`s face three months before and at the moment of clinical evaluation. In the latter, the characteristic plethoric full moon face of the Cushing’s syndrome is shown.

Case Report:

A 1.8 year-old girl was sent to our hospital for the assessment of weight gain and premature pubarche. She was born at term, with normal weight; her personal anamnesis did not show any remarkable diseases. Since the age of eight months, she had presented a progressive weight gain, and a decrease in growth velocity. Pubarche started few months before evaluation.

On physical exam her weight and height were: 16 Kg (+ 3.5 SDS) and 76.5 cm (-2.3 SDS), respectively. She had a plethoric full moon face (Figure 1), hirsutism, acne, fat deposits on the back of the neck and shoulders and obesity.  Pubertal development showed Tanner’s stage PH3,  B1 (absence of estrogenic stimulation), and no clitoral hypertrophy.

Hormonal studies (Table 1) confirmed the hyperandrogenism and the endogenous hypercortisolism. The overnight dexamethasone suppression test revealed non suppressible cortisol and androgen production, which along with low ACTH levels, suggested a primary adrenal pathology.

Table 1

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Tabla 2: Prueba de inhibición con dexametasona

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Abdominal ultrasound and computed tomography showed a tumor mass in the left adrenal gland with calcifications. The left kidney was inferiorly displaced. Left adrenal vein thrombosis was observed. No evidence of distant metastases was detected (Figure 2).

Figure 2. Axial computed tomography of thorax and abdomen: Left adrenal Tumor (red arrow)

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Complete tumor resection was performed. Histological studies evidenced an Adrenal Mass tumor (weight, 93,20 grams); with a solid growth pattern (> al 30 %), rupture of the capsule: (+), eosinophilic cells (> al 30%), necrosis (+), pleomorphism (focal), hyperchromasia and prominent nucleoli (focal).

According to Weiss criteria (2), the lesion was classified as: Adrenal Carcinoma.

Comments

Cushing syndrome (CS) results from the presence of high and sustained circulating concentrations of glucocorticoids (GC). Exogenous CS is the most frequent in pediatric age.

Clinical manifestations of CS are variable, and they are dependent of the severity and duration of hypercortisolism. Growth retardation is the most characteristic sign in pediatric CS and it can be the first sign. Fat accumulation has a characteristic central distribution, fat deposits in the cheeks and temporal fosses induces the characteristic “full moon” round face. Clinically virilizing or mixed tumors produce androgen excess and early onset of pubic hair (pubarche).

The etiology of CS can be grouped in two main categories: ACTH-dependent and non-ACTH dependent CS. Non-ACTH dependent CS can be secondary to corticoadrenal tumors (CAT) or bilateral adrenal hyperplasia

CATs are an infrequent cause of Cushing syndrome, except in certain geographic areas of the world, like southern Brasil (3.4 per million children). The frequency of CATs is 0.4 cases per million persons during the first 4 years of life.

In pediatric age, 90% of CATs are functioning and show clinical manifestation or hormonal hyperproduction. Signs and symptoms of virilization were the most common (90% of patients), accompanied by clinical manifestations of overproduction of glucocorticoids in a third of them. Finally, overproduction of glucocorticoids alone (Cushing syndrome) was evident in only 8% of patients.

Patients with CS who also show signs of excessive androgens, should be studied for CATs.

Surgery with complete tumor resection is the cornerstone of the management of pediatric CAT and is the most important prognostic factor. Mitotane has been used in an adjuvant setting. However, its efficacy in pediatric CAT has not been studied systematically.

Bibliography

  1. Costanzo M, Guercio G, Ciaccio M, Rivarola MA, Belgorosky A. Evaluación de eje suprarrenal- Síndrome de Cushing
  2. Weiss L.M.  Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol 1984, 8(3): 163-169.
  3. Ribeiro R.C., Figueiredo B. Childhood adrenocortical tumors. Eur J Cancer 2004, 40: 1117-1126

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