RECURRENT ADRENOCORTICAL CARCINOMA IN A 4 YEAR OLD GIRL
AHMED H. AL-SALEM AND HUSSAIN A. ABU-SRAIR
Division of Paediatric Surgery and Department of Paediatrics, Qatif Central Hospital, Qatif, Saudi Arabia
Adrenocortical tumours are rare in the paediatric age group. This is a report of a 4 year old girl with recurrent adrenocortical carcinoma emphasizing the role of surgery as the definitive form of therapy even in recurrent cases.
Key words: adrenocortical carcinoma, adrenocortical tumours, precocious puberty, virilization.
INTRODUCTION
Adrenocortical tumours occur frequently in adults, but are very rare in the paediatric age group.1.2 In children these tumours usually function with an increase in the secretion of adrenocortical hormones, especially andro- gens, leading to precocious puberty or virilization.1.3 In some cases these tumours produce Cushing’s syndrome and rarely hyperaldosteronism or feminization.
In this report, a 4 year old girl with virilization and Cushing’s syndrome due to recurrent adrenocortical car- cinoma is described.
CASE REPORT
A 4 year old girl was referred to Qatif Central Hospital with a 3 month history of increasing abdominal mass, pubic and axillary hair and clitoral enlargement. She was a product of a full-term normal vaginal delivery. She was a known case of adrenocortical carcinoma hav- ing had complete surgical excision of a left adrenocortical carcinoma 1 year previously. Twelve months earlier, the patient at another hospital had presented with an abdomi- nal mass, Cushingoid features, pubic and axillary hair and clitoral enlargement. She was investigated by plain abdominal X-ray, abdominal ultrasound, intravenous uro- gram, computerized tomography (CT) scan of the abdo- men and bone scan and was found to have a left suprarenal tumour. Her plasma cortisol and testosterone levels were 82.6 µg/dL (n=4-6) and 15.2 mmol/L (n=0.4-3.9), re- spectively. She underwent excision of the left suprarenal tumour and histopathology revealed adrenocortical car- cinoma. She was progressing well until 3 months prior to her presentation at the present institution when she was noted to have increasing abdominal mass and pubic and axillary hair. Her only brother, a 14 year old, was being treated in another hospital for osteogenic sarcoma and her father died from colorectal carcinoma. Her mother was healthy and well.
Correspondence: Dr A. H. Al-Salem. PO Box 6078, Dammam 31442. Saudi Arabia.
At presentation, her weight was 21.6kg and blood pressure was 120/80 mmHg, with Cushingoid features including moon face, obesity and striae over the abdomen and thigh.
There was fine hair over the upper lip and dark hair in the axilla and pubic region. The clitoris was enlarged measuring 2 × 1 cm (Fig. 1). There was no labial fusion and both vaginal and urethral orifices were separate. Abdominal examination revealed a transverse upper ab- dominal scar with striae. There was a large mass, firm in consistency measuring 12 × 9 cm occupying mainly the left side (Fig. 2).
Investigations revealed a haemoglobin level of 9.2 g/dL, white blood cell count of 13.4 × 109/L, normal electro- lytes, urea and creatinine. Blood cortisol was 34.0 µg/dL (n =4-26), blood testosterone was 16.8 mmol/L (n = 0.4- 3.9), dehydroepiandrosterone sulphate (DHEAS) more than 1000 µg/dL (n=35-430) and 24h urinary 17- ketosteroids was 56 mg/24 h (n =1 mg/24 h). The plain
X-ray of the abdomen showed a left-sided soft tissue density with fine calcification and a skeletal survey was normal. Ultrasonography of the abdomen showed a left- sided suprarenal mass about 12 × 10 cm with hyperechoic areas suggestive of calcification. Another smaller mass superior to the larger one and measuring about 6 × 5 cm was also defined. Both ovaries were normal. The intra- venous urogram demonstrated a left suprarenal mass with calcification, which was displacing the left kidney down- wards. Computerized tomography of the abdomen showed a huge mass filling almost the whole of the left half of the abdominal cavity. Multiple hypodense areas were seen within the mass suggesting necrotic changes. Areas of calcification within the mass were also noted. The left kidney appeared separate from the mass but displaced downwards and posteriorly. There was no evidence of liver secondaries and the right kidney was normal (Fig. 3). The reconstruction film suggested two masses adherent to each other.
The patient was operated on, and two separate masses lying side by side were found. The lower one was anteriorly placed and adherent to the mesentery of the descending colon, while the second mass was superior and posterior lying in contact with the left kidney and pancreas. The two masses were vascular but were com- pletely excised and on sectioning were found to contain a lot of necrotic tissue. At a histological examination, the tumour had a fibrous capsule from which trabeculae infiltrated into the tumour. The tumour was made up of compact cells with eosinophilic granular cytoplasm and clear cells in variable proportion interspersed with dilated thin walled sinusoids. Cells and nuclei were fairly uni- form but occasionally a giant nucleus, which was often hyperchromatic, or a larger cell was seen and there were
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occasional mitotic figures. Areas of haemorrhage and necrosis were present.
Postoperatively, the patient did well and was dis- charged home on the tenth postoperative day. She was readmitted again about 1 month later with a urinary tract infection. On follow up in the outpatient clinic 3 months postoperatively she was doing well and her general appearance had improved markedly. She had lost about 4 kg of weight and her hormonal studies were normal. She was followed up in the outpatient clinic at regular intervals. She was last seen about 18 months postopera- tively. Her Cushingoid and virilization features had dis- appeared except her clitoris which was still slightly enlarged. There was no clinical evidence of recurrence and her hormonal profile was normal.
DISCUSSION
Adrenocortical tumours are rare in children.1.2 Fraumen and Miller collected only 62 cases of adrenocortical tumours from 12 large hospitals over a period of 12-25 years,4 and Nader et al. estimated its frequency as one case in every 1.7 million people.5 Steward et al., over a period of 20 years, reported only five cases of adrenocor- tical carcinoma out of 141 adrenal gland tumours.2
In children, carcinoma of the adrenal cortex occurs most frequently between the ages of 3 and 5 years,” and the left side is affected in 60% of the cases.7 It is more common in females than males with a female/male ratio of 4:1%. The great majority are hormone secreting and they produce androgens, glucocorticoids, a mixture of both or rarely oestrogen or aldosterone.1.3 In prepubertal girls, excess androgen production is associated with rapid growth, facial and pubic hair and clitoral enlargement as in the present case. Telander et al. found that these tumours are accompanied by Cushing’s syndrome in 25% of the cases, and the coexistence of symptoms of virilism and Cushing’s syndrome is highly indicative of malignancy” as in the present patient. These tumours have also been reported to be associated with other abnormalities such as hemihypertrophy, cutaneous le- sions, urinary tract anomalies, the Beckwith-Weideman syndrome and astrocytoma.4.10.11 A high incidence of
malignant tumours in the families and relatives of chil- dren with virilizing adrenal tumours has been reported.12 Two members of the present patient’s family had malig- nant tumours.
The treatment for adrenocortical carcinoma is surgical excision and recent series have shown a better prognosis compared to earlier reports.13.14 In contrast to adults, adrenocortical carcinomas in children are not prone to metastasize early, and there is no close correlation be- tween the pathological appearance and the clinical course of the disease. This is the case with the present patient, as, despite the recurrence, large size of the tumour, and histological features of malignancy, there was no evi- dence of distant metastasis. The patient was treated totally by surgical excision of the tumour only because the value of radiotherpay and chemotherapy in cases of malignant adrenal tumours in children is still debated.15 Hoffman and Mattox justify the use of O,P-DDD only in cases in which surgery does not completely eradicate the adreno- cortical carcinoma, 16 and in cases where it can relieve the symptoms caused by hormonal secretion of the tumours, and there are reports of fatal outcome despite the use of mitotone.17 The present case emphasizes the role of surgery as the definitive form of therapy even in recurrent cases.
The importance of cortisol administration pre- operatively and postoperatively is to be emphasized since the non-affected contralateral gland is frequently sup- pressed by the amount of cortisol produced by the tum- our. These patients should also be followed up regularly and monitored by clinical examination, adrenocortical hormonal assay and when indicated, ultrasound and CT scan evaluation for early detection of recurrence or metastatis.
REFERENCES
1. Telander RL, Zimmerman D, Kaufman BH et al. Paediatric endocrine surgery. Surg. Clin. North Am. 1985; 65: 1560-1.
2. Steward DR, Morris PH. Jollyes A. Carcinoma of the adrenal gland in children. J. Pediatr. Surg. 1974; 9: 59-67.
3. Hayles AB, Hahn HB Jr. Sprague RG et al. Hormone- secreting tumours of the adrenal cortex in children. Paedi- atrics 1966; 37: 19-25.
4. Fraumen JF Jr, Miller RW. Adrenocortical neoplasms with hemihyperthropy, brain tumours and other disorders. J. Pediatr. 1967: 70: 129-38.
5. Nader S, Hickey RC, Sellin RV et al. Adrenal cortical carcinoma. Cancer 1983; 52: 707-11.
6. Hutter AM, Kayhour DE. Adrenal cortical carcinoma: Clinical features of 138 patients. Am. J. Med. 1966; 41: 572-80.
7. Didolkar MS, Besher A, Elais EG et al. Natural history of adrenal cortical carcinoma. Cancer 1981; 47: 2153-61.
8. Richie JP, Gittes RF. Carcinoma of the adrenal cortex. Cancer 1980; 45: 1957-64.
9. Henley DJ, Van Heerden JA, Grant CS et al. Adrenal cortical carcinoma: A continuing challenge. Surgery 1983; 94: 926-31.
10. Haicken B, Normal H, Schulman MD, Keith M, Schneider MD. Adrenocortical carcinoma and congenital hemihyper- trophy. J. Pediatr. 1973; 83: 284-5.
11. Morales L, Rovira J, Rotterman M. Julia V. Adrenocortical tumours in childhood: A report of cases. J. Pediatr. Surg. 1989; 24: 276-81.
12. Kenny FM, Hashida Y, Askari HA et al. Virilizing tumours of the adrenal cortex. Am. J. Dis. Child. 1968; 11: 445-58.
13. Lee PD. Winter RJ, Green OC. Virilizing adrenocortical tumours in childhood: Eight cases and a review of the literature. Pediatrics 1985; 76: 645-50.
14. Zaitoon MM, Mackie GG. Adrenal cortical tumours in children. Urology 1978; 12: 645-50.
15. Cacciari E, Cicognani A, Pirazzoli P et al. Adrenocortical tumours in children: Our experience with nine cases. Acta Endocrinol. 1986; 279: 264-74.
16. Hoffman D. Mattox VR. Treatment of adrenocortical car- cinoma with O, P-DDD. Med. Clin. North Am. 1972; 56: 999-1011.
17. Zaki M, Daoud AS, Mahfouz EH, El-Saleh Q. Virilizing adrenocortical carcinoma in a 4-year old girl with fatal recurrence. Ann. Saudi Med. 1989; 9: 508-10.
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