ESPE Abstracts (2018) 89 P-P3-027

Adrenocortical Tumours in Children - A Case Series

Si Hua Chan & Rashida Farhad Vasanwala

KK Women’s & Children’s Hospital, Singapore, Singapore.

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 1Patient 2Patient 3Normal values
Adrenocortical hormones
17alpha-OH progesterone (nmol/L)>36.634.2 4.450.6–5.5 <3.3 (<1 year old)
Aldosterone (pmol/L)284.8120.8ND97.3–834.0
8am Cortisol (nmol/L)228207ND101–536
DHEA-S (micromol/L)40.471.62.91.1–11.8
Androstenedione (nmol/L)60.767.6ND0.1–2.8
Total Testosterone (nmol/L)9.933.222.50.4–2.0
Estradiol (pmol/L)79968377–2382
Short Synacthen testFailedPassedNot done
Table 2 Surgical and histopathological findings with reference to proposed criteria by Wieneke
Patient 1Patient 2Patient 3
Tumour weight (g)67371Well below 500
Tumour size (cm)7.5×4.5×3.510.5×9.5×6.53.0×2.5×2.3
Periadrenal extensionNoYesNo
Invasion into vena cavaNoNoNo
Venous invasionNoNoNo
Capsular invasionFocal invasion seenNoNo
Lymph node biopsyNegative for malignancyNot doneNot done
Presence of tumour necrosisYesYesNo
MitosesUp to 23 per 50 HPF*, atypical mitoses seenUp to 8 per 50 HPF, atypical mitoses seen6 per 50 HPF
Immunohisto- chemistryp53 positive cellsStrongly positive Patchy positivitypositive
Ki67 index (%)4–5010–255–20
Tumour stagingIIIII

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.

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