Adrenocortical Tumours in Children - A Case Series

Chan Si Hua; Vasanwala Rashida Farhad

Background: Paediatric adrenocortical tumours (ACT) are rare and typically present with virilising symptoms and signs which can be similar to other virilising conditions such as congenital adrenal hyperplasia.

Case description: We describe three cases of ACT diagnosed and managed at our institution over the past 10 years. The three girls presented with symptoms of virilisation. The mean interval between first symptoms and diagnosis was 19.6 months (ranging from 12 to 31 months). Age of presentation ranged from birth to 12 years. One patient was misdiagnosed and treated for congenital adrenal hyperplasia for 7 months. Diagnosis of ACT was confirmed by laboratory, diagnostic imaging and histopathology (Tables 1 and 2). All three patients underwent complete resection of tumour and affected adrenal gland. Hormone levels returned to normal after surgical resection. Two patients had recurrence – one had right lung metastasis while the other had local tumour recurrence. Both patients achieved complete remission since completion of chemotherapy.

Table 1 Laboratory findings
	Patient 1	Patient 2	Patient 3	Normal values
Adrenocortical hormones
17alpha-OH progesterone (nmol/L)	>36.6	34.2	4.45	0.6–5.5 <3.3 (<1 year old)
Aldosterone (pmol/L)	284.8	120.8	ND	97.3–834.0
8am Cortisol (nmol/L)	228	207	ND	101–536
DHEA-S (micromol/L)	40.4	71.6	2.9	1.1–11.8
Androstenedione (nmol/L)	60.7	67.6	ND	0.1–2.8
Total Testosterone (nmol/L)	9.9	33.2	22.5	0.4–2.0
Estradiol (pmol/L)	79	96	83	77–2382
Short Synacthen test	Failed	Passed	Not done

Table 2 Surgical and histopathological findings with reference to proposed criteria by Wieneke
	Patient 1	Patient 2	Patient 3
Tumour weight (g)	67	371	Well below 500
Tumour size (cm)	7.5×4.5×3.5	10.5×9.5×6.5	3.0×2.5×2.3
Periadrenal extension	No	Yes	No
Invasion into vena cava	No	No	No
Venous invasion	No	No	No
Capsular invasion	Focal invasion seen	No	No
Lymph node biopsy	Negative for malignancy	Not done	Not done
Presence of tumour necrosis	Yes	Yes	No
Mitoses	Up to 23 per 50 HPF*, atypical mitoses seen	Up to 8 per 50 HPF, atypical mitoses seen	6 per 50 HPF
Immunohisto- chemistry	p53 positive cells	Strongly positive	Patchy positivity	positive
Ki67 index (%)	4–50	10–25	5–20
Tumour staging	II	II	I

Conclusion: This descriptive analysis of our cases concurs with many findings in the literature. Based on our experience, we propose that it may be prudent to perform ultrasound abdomen (with emphasis on the adrenal glands) for all girls who present with virilising features.

ESPE Abstracts

P3-P027

Adrenocortical Tumours in Children – A Case Series

Si Hua Chan & Rashida Farhad Vasanwala

Volume 89

57th Annual ESPE

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