INTRODUCTION

Adrenal tumours are rare childhood tumours.1–3 These constituteless than 1% of all paediatric neoplasms,1 with adrenal cortical car-cinoma comprising only 0.002% of all childhood malignancies.2Tumours arising from the adrenal cortex and the medullapresent with a distinct characteristic and often fascinating spectrumof clinical features. A careful biochemical evaluation is essential todiagnose endocrinopathies associated with these tumours. Com-pared to adults, a higher proportion of adrenal tumours in childrenis malignant.4–6 The biological behaviour of these tumours isdifficult to predict and it is often difficult to label a tumour asbenign or malignant on the basis of histological features.7,8

Surgery remains the mainstay of treatment and offers cure inbenign cases, and is the best means of palliation in malignantcases.8,9 We present an audit of 13 cases of adrenal tumours in children treated at Sanjay Gandhi Postgraduate Institute ofMedical Sciences over an 81/2-year period.

METHODS

The medical records of 13 children with adrenal tumoursmanaged at Sanjay Gandhi Postgraduate Institute of MedicalSciences between June 1990 and January 1999 were reviewed.Demographic data, clinical details, investigation findings, opera-tive details, histopathological findings and follow-up detailswere recorded. Diagnostic tests included estimation of basal

serum cortisol, low-dose dexamethasone suppression tests(LDDST) and high-dose dexamethasone suppression tests(HDDST), serum testosterone, serum aldosterone and urinarycatecholamines (free catecholamines or metanephrines).Tumours were localized with abdominal ultrasonography (USG)and/or abdominal computed tomography (CT) scanning. X-rays ofthe chest were done in all cases to check for metastases.

One patient who presented with features of hypercortisolismwith virilization refused to undergo surgical treatment. Theremaining 12 children were subjected to surgery, with a curativeintent. Children with hypercortisolism were given stress doses ofsteroid beginning on the morning of surgery, in the form of intra-venous infusion. The doses were tapered and patients wereswitched to oral prednisolone within 3–5 days, which was continueduntil approximately 6 months postoperatively when recovery ofthe hypothalamus–pituitary–adrenal (H-P-A) axis was demon-strated by a short adrenocorticotropic hormone (ACTH; synac-thenR, Ciba, UK) test. In children with phaeochromocytoma(PCC), α-blockade was achieved preoperatively with α-blockers.

Patients were discharged once the stitches were removed andpostoperative drug (steroid and/or antihypertensives) require-ments were stabilized. The children were followed up 3-monthly twice, 6-monthly during the subsequent 1 year, andannually thereafter. On the follow-up visits biochemical (serumbasal cortisol and/or testosterone, short ACTH test in adrenalcortical tumours; and urinary metanephrines in PCC) andimaging studies (if required) were carried out.

RESULTS

Of the 13 children managed during the study period, 10 childrenhad adrenal cortical tumours while three had PCC. The mean

ANZ J. Surg. (2001) 71, 103–107

ORIGINAL ARTICLE

FUNCTIONING ADRENAL TUMOURS IN CHILDREN AND ADOLESCENTS: AN INSTITUTIONAL EXPERIENCE

ANJALI MISHRA, GAURAV AGARWAL, ANAND KUMAR MISRA, AMIT AGARWAL AND SAROJ K. MISHRA

Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

Background: The purpose of the present paper was to carry out an audit of clinicopathological profile and treatment outcome in 13children with functioning adrenal tumours.Methods: The medical records of 13 children with functioning adrenal tumours who were managed between June 1990 andJanuary 1999 were reviewed. Demographic data, clinical features, biochemical and localization studies, operative details andfollow-up records were studied. Children with neuroblastoma were excluded.Results: The mean age was 7.4 ± 5.3 years. Seven patients had Cushing’s syndrome (CS), two patients had virilizing tumours, threepatients had phaeochromocytoma (PCC) and one patient had Conn’s syndrome. All patients (except one child with CS) weretreated surgically. Two children with adrenocortical carcinoma (ACCa) died during the perioperative period. Histopathological diag-nosis was adrenal cortical adenoma (ACAd) in four patients, ACCa in five patients and PCC in three patients. Two ACCa patients diedof metastases at 12 and 14 months, respectively, while the third is alive and well at 30 months. Children with ACAd are alive and wellat 91, 56, 32 and 27 months postoperatively. Children with PCC are free of disease (normal urinary metanephrines) at 63, 18 and 8months after surgery but require antihypertensive drugs in low doses.Conclusion: The outcome of surgery is good in cases of ACAd and PCC. Although outcome is poor in ACCa, surgery remains themainstay of treatment and offers good palliation.

Key words: Conn’s syndrome, Cushing’s syndrome, phaeochromocytoma, virilizing adrenal tumours.

Correspondence: Dr S. K. Mishra, Department of Endocrine Surgery,Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road,Lucknow 226 014, India. Email: skmishra@sgpgi.ac.in

Accepted for publication 18 September 2000.

age of these children was 7.4 ± 5.3 years. The mean age did notdiffer significantly in cortical tumours and PCC (7.3 ± 5.8 years vs7.6 ± 4.0 years). Because the number of cases is small the sexpredilection cannot be commented upon. The median follow up was28.5 months (range: 8–91 months).

Adrenocortical tumours

The mean duration of illness was 14.2 ± 10.9 months (range:4–36 months). Seven patients predominantly had symptoms andsigns of hypercortisolism (CS), two had purely virilizingtumours, and one had Conn’s syndrome. The clinical features ofthese patients are summarized in Table 1. Biochemical findings andtumour characteristics are summarized in Table 2.

One child, while on follow up for congenital adrenal hyperpla-sia (CAH) developed features of virilization and growth acceler-ation. Ultrasonography revealed a left adrenal tumour alongwith bilateral hyperplastic adrenals. The tumour was found to beincreasing in size on repeat ultrasonographic examination 6months later, and it was resected.

Imaging studies carried out included abdominal USG alone infour cases, CT scanning alone in one case and both in five cases.

As well as localizing the adrenal tumours, CT scanning was able todetect lymph node enlargement in one case, inferior vena cava(IVC) thrombosis in one case, and it documented the presence ofbilateral adrenal hyperplasia with a left-sided adrenal tumour in achild with CAH. The patient with IVC thrombosis (Conn’s syn-drome) also underwent magnetic resonance angiography(MRA), and thrombus was found extending into the bilateraliliac veins and right femoral vein. Head CT scans were reported asnormal in both the cases with convulsions.

The parents of one child with CS (case 2; Table 1) refusedsurgery. An abdominal CT scan in this case showed a large leftadrenal tumour with calcification and necrosis, leading to a high suspicion of malignancy. All other cases were explored through an anterior transperitoneal route. Nine adrenalectomies were performed (five left-sided and four right-sided). Lymph node dis-section was performed in two cases of adrenocortical carcinoma(ACCa). En bloc removal of the left adrenal tumour, left kidney andlymph nodes, along with a thrombectomy from the IVC and bothiliac veins, was performed in the child with Conn’s syndrome.

Two children died during the perioperative period. The childwith Conn’s syndrome needed mechanical ventilation in thepostoperative period and could not be weaned off the ventilator.

104 MISHRA ET AL.

Table 1. Adrenocortical tumours: clinical features

Age Sex Duration of illness Cushingoid features Virilization Hypertension Abdominal lump Proximal myopathy(years) (months)

6 F 6 + + + – +16 M 36 + + + – +6 F 6 + + – – –1 F 4 + + + – +6 M 36 + – – – –3 M 6 + + + – –4 F 6 – + – + –

16 F 12 – – + + –14 M 12 + + – – +

1.6 F 12 + + + – –

Table 2. Adrenocortical tumours: biochemical findings and tumour characteristics

Serum cortisol LDDST* HDDST† Serum testosterone Serum aldosterone Tumour Histopathology(nmol/L) (nmol/L)‡ (pg/mL) characteristics(normal range: (normal range:138–698) 10–160)

Weight Size(g) (cm)

1027.86 Non-suppressed Non-suppressed 17.36 — 32 6.0 Carcinoma987.6 Non-suppressed Non-suppressed — — — — —

1104 Non-suppressed Non-suppressed 19.08 — 35 5.0 Carcinoma916.4 Non-suppressed Non-suppressed 32.8 — 12 3.8 Adenoma— — — 24.73 — 25 3.0 Adenoma491 Non-suppressed Non-suppressed — — 81 8.0 Adenoma17 Suppressed — 440 — 600 13 Carcinoma— Suppressed — 2.2 210 500 20 Carcinoma

2207.2 Non-suppressed Non-suppressed — — 370 14 Carcinoma1379.5 Non-suppressed Non-suppressed — — 56 4.0 Adenoma

LDDST, low-dose dexamethasone suppression test; HDDST, high-dose dexamethasone suppression test.*LDDST: suppressed if plasma cortisol < 0.15 nmol/L.†HDDST: suppressed if plasma cortisol suppressed to < 50% of basal cortisol.‡normal range: prepubertal, 0.3 nmol/L; post-pubertal male, 10–34 nmol/L; post-pubertal female, 0.7–26 nmol/L.

She developed pneumonitis leading to septicaemia, dissemi-nated intravascular coagulation and multiorgan system failureand died on the 9th postoperative day. A male child withCushing’s syndrome (case 9; Tables 1,2) carcinoma succumbed torespiratory failure. His family did not opt for artificial ventilationdue to financial reasons. In addition, one patient each sufferedminor wound collection and superficial wound gaping.

On histological examination four tumours were reported asadrenal cortical adenoma (ACAd) and five as ACCa. One case ofadenoma had evidence of focal vascular invasion but no capsularinvasion. The diagnosis of ACCa was based on the presence ofmetastatic lymph nodes in two cases, liver metastases in onecase and the presence of major vascular and capsular invasion in two cases. The mean tumour weight of cortical tumours was 190 ± 233 g (range: 12–600 g) and was higher for malignant ascompared to benign tumours (490 ± 115.3 vs 40 ± 24.6 g). Meantumour size was 8.5 ± 5.8 cm (range: 3–20 cm) and was con-siderably more for malignant as compared to benign tumours(11.6 ± 6.2 vs 4.7 ± 2.2 cm).

Surgical treatment resulted in a good clinical and biochemicalresponse in all patients. Two patients with ACCa died 12 and 14months after operation, respectively. The first death was due to liverand lung metastases while the second death was due to livermetastases. One patient with virilizing ACCa was alive 30months after operation without any clinical, biochemical or radi-ological evidence of metastatic or recurrent disease. All fourcases of ACAd are alive without any evidence of disease at 91, 56,32 and 27 months of follow up.

Phaeochromocytoma

During the study period five children with PCC were managed atSanjay Gandhi Postgraduate Institute of Medical Sciences. Thetwo children with extra-adrenal PCC were excluded from thepresent study of adrenal tumours. The median duration ofillness was 14 months (range: 3–36 months). The classical clini-cal triad of headache, palpitations and sweating was present in onlyone case (case 3). The nature of hypertension was sustainedwith paroxysmal rise in all three children. There was no familyhistory of PCC or other familial syndromes associated withPCC in these cases. The clinical findings in these cases aresummarized in Table 3. Abdominal CT scanning was done inall three cases for localization. In addition, USG was done intwo cases. Head CT scanning was done in two cases with neuro-logical complications. The child who presented with hemiplegiahad evidence of right basal ganglion and external capsulehaemorrhage, while the child with convulsions had postictaloedema alone. Investigation findings and tumour characteristicsare summarized in Table 4.

The children were prepared with α-blockers (phenoxybenzamine

in the first child, prazosin in the other two), which were given for10–22 days prior to surgery. The dosages of prazosin required toachieve blockade were 15 and 13 mg, respectively. Beta-blocker(propanolol, 40 mg) was required in case 3 only. All three casesrequired calcium channel blockers and angiotensin convertingenzyme inhibitor (ACE inhibitor) in addition to α-blockersbecause blood pressure control was not adequate despite highdoses of α-blockers. All cases were explored through an ante-rior transperitoneal route. Two left-sided and one right-sidedadrenalectomy were performed. The postoperative period wasuneventful. The contralateral adrenal gland and ectopic siteswere found to be free of tumours on exploration in all.

On histological examination all three tumours were reported asbenign adrenal medullary tumours, consistent with the diagnosis ofPCC. There was no evidence of malignancy. Case 3 had exudativeascitis, although no malignant cells were found on cytologicalanalysis of the peritoneal fluid. Thus this case needs careful fol-low up to exclude the possibility of malignancy.

The child with hemiplegia improved with conservative treat-ment and had grade 4–5 muscle powers in various muscle groups atthe time of discharge. All three children had normal urinarymetanephrines at their last follow-up visit (63, 18 and 8 months,respectively), but required antihypertensive treatment for control ofpersistent blood pressure problems, although in reduced doses.

DISCUSSION

Adrenal cortical tumours

Adrenal tumours are rare in childhood. Most of the adrenal corti-cal tumours in children present with signs of virilization with orwithout hypercortisolism.10,11 In our experience all children withcortical tumours, except one with hyperaldosteronism, had virilizingfeatures. Seven children had features of hypercortisolism along withvirilization while two had pure virilizing tumours. Pluri-hor-monal manifestations predominate in other series as well.12 Themean age in our series was higher as compared to other previouslypublished series and probably reflects a delay in diagnosis and man-agement in our socio-economically poor population.12,13 Afemale preponderance as observed in the present series has beenreported by others as well.5,13,14

Due to wider application of increasingly sensitive radiologicalinvestigation techniques adrenal tumours are being detectedmore frequently in children with untreated congenital adrenalhyperplasia. There have been numerous such reports, including onefrom our own centre.15–17 In one study the incidence of adrenalmasses was found to be as high as 82% in homozygous and 45% inheterozygous patients with CAH.15 In contrast, virilizing adrenaltumour in female children can be confused with CAH.16

Because CAH is a more frequently occurring disorder, it is

FUNCTIONING ADRENAL TUMOURS IN CHILDREN 105

Table 3. Phaeochromocytoma: clinical features

Age Sex Duration Headache Sweating Palpitation Hypertension Abdominal pain Others(years) (months)

3 M 14 + + – Sustained + Constipation10 F 3 + + – Sustained+ + Convulsion

Paroxysm10 M 36 + + + Sustained+ + Convulsion

Paroxysm Hemiplegia

recommended that CAH should be ruled out in cases of incidentallydetected adrenal masses.14 The presence of a hyperplastic con-tralateral adrenal gland is a useful diagnostic sign in a CAHpatient with complicating adrenal tumour. Although virilization andCS are common, aldosterone-secreting tumours are extremelyrare in children.18

Once diagnosis of CS, virilization or hyperaldosteronism hasbeen substantiated by biochemical tests, abdominal imaging byUSG and CT plays an important role in the localization andanatomic delineation of these tumours, and helps in strategyplanning for surgical intervention.10,18,19 Abdominal CT is superiorin this regard.20 Tumours as small as 0.5 cm have been detected byCT. It can readily detect local invasion, intravenous extension oftumour, lymph node involvement and liver metastases. In astudy of seven children, large tumour size (> 6 cm diameter)and a complex echo pattern were considered to provide a usefulindication of malignancy.19 Although MRI holds considerablepromise, its role is still under scrutiny. The most useful applicationof MRI is in the detection and delineation of venous exten-sion.21

Most of the childhood adrenal tumours are thought to bemalignant.4–6 Definitive diagnosis, however, is not possiblebecause there are no absolute histological criteria to distinguishbenign from malignant tumours. Some patients whose tumoursexhibited benign features have had late metastasis or local recur-rence,22 while others having a microscopic appearance of malig-nancy have survived for years.23,24 Paediatric benign tumourshave been found to have frequent mitosis, necrosis, moderate-to-severe pleomorphism and broad fibrous bands when comparedwith adult tumours and these features are seen in both benign andmalignant tumours.7,8,23 Considering all these facts, some investi-gators have concluded that paediatric tumours are more likely to bebenign than previously thought.7 In the literature the main criteriafor distinguishing benign from malignant tumours are highertumour weight and mitotic activity.23,24 In the present series we alsofound that the mean tumour weight of malignant tumours wasconsiderably higher as compared to benign tumours.

Adrenocortical carcinoma has a poor prognosis. Complete surgi-cal resection is the only effective and potentially curative treat-ment.8,9,24–26 Even for recurrent and metastatic disease, surgeryprovides the only hope for effective palliation or cure.25 Achemotherapeutic regimen using mitotane, and combinationchemotherapy with dacarbazine and other drugs has been used bymany, but its role is not yet established.8,24–26 Considering thehigh cost and toxicity of chemotherapy and the minimal benefit inimproving quality of life, we did not use chemotherapy to treatACCa.

Phaeochromocytoma

Phaeochromocytoma is a rare cause of hypertension in child-hood, accounting for less than 1% of all hypertensive chil-dren.3,27,28 Phaeochromocytomas are more likely to be familial,bilateral, multifocal and extra-adrenal in children. In our shortseries of five children with PCC, two children (40%) had PCC atextra-adrenal sites. No bilaterality or multifocality, however,was present in the three cases of adrenal PCC. All the three childrenwith adrenal PCC in the present series had sustained hypertension,a finding that is consistent with other series. 131Iodine-metaiodobenzyl guanidine (131I-MIBG) scanning is very helpful inlocalization of adrenal, extra-adrenal, metastatic and ectopicsites of these tumours and in total removal of this potentiallycurable cause of hypertension.29 This radiopharmaceutical wasnot routinely available at Sanjay Gandhi Postgraduate Institute ofMedical Sciences until recently, and so 131I-MIBG scanningcould not be performed in the present study.

Complete surgical excision of adrenal and/or extra-adrenalPCC results in cure of hypertension in most children.3,27 Persistentpostoperative hypertension may result due to incompleteremoval of the tumour in the primary site, unidentified multifocaldisease at adrenal or extra-adrenal sites, and local residualmalignant or metastatic PCC. In all these conditions the urinary cat-echolamines and metanephrines would remain high, unlike that ofour three PCC patients, who remained hypertensive postoperatively.Causes for persistent postoperative hypertension without cate-cholamine hypersecretion include reno-vascular hypertension,as was the case in one of our patients, irreversible vascularchanges caused by prolonged hypertension due to delay inseeking medical attention, as could be in the remaining twocases, and high set baro-receptors. If the urinary metanephrine levelis normal than antihypertensives are used for control of bloodpressure. In inoperable and metastatic cases, besides pharmaco-logical treatment, 131I-MIBG therapy can offer some palliation.29

As for most endocrine tumours, conventional histological fea-tures are inaccurate in predicting the biological behaviour of thePCC. The presence of metastatic disease at non-chromaffinsites, such as the lymph nodes, liver etc. or a major vascular andcapsular invasion and tumour thrombi can be considered asbeing diagnostic only of a malignant nature. These cases require acareful lifelong follow up to identify and manage a recurrentmalignant or metachronous multifocal disease, which can manifestlong after the primary management. If a patient is found to haveraised catecholamines or metanephrines during follow up,appropriate imaging by CT and 131I-MIBG is useful in identifyingrecurrent or metastatic disease.3

106 MISHRA ET AL.

Table 4. Phaeochromocytoma: investigation findings and tumour characteristics

Fundus Renal scan Catecholamines* Metanephrines* Tumour Histopathology(ug/dL) (mg/24 h) characteristics

(normal range: 31–61) (normal range: 0.05–1.2)Weight Size

(g) (cm)

Papilloedema — 72.75 — PCCGrade 4 Left renal artery

retinopathy stenosis — 4.6 34 PCCNormal — — 12.1 80 PCC

*Urinary levels.PCC, phaeochromocytoma.

ACKNOWLEDGEMENTS

The authors wish to acknowledge the contribution of Dr Vijay-alakhsmi Bhatia (Additional Professor, Department of MedicalEndocrinology, Sanjay Gandhi Postgraduate Institute ofMedical Sciences, Lucknow) who was the treating paediatricendocrinologist for all the subjects in the present study.

REFERENCES1. Morales L, Rovira J, Rotterman M, Julia V. Adrenocortical

tumours in childhood: A report of four cases. J. Pediatr. Surg.1989; 24: 276–81.

2. Young JL, Miller RW. Incidence of malignant tumours in US chil-dren. J. Pediatr. 1975; 86: 254–8.

3. Caty MG, Coran AG, Geagen M, Thompson NW. Current diag-nosis and management of pheochromocytoma in children. Arch.Surg. 1990; 125: 978–81.

4. Zaitoon MM. Adrenal cortical tumours in children. Urology1978; 12: 645–9.

5. Chudler RM, Kay R. Adrenocortical carcinoma in children.Urol. Clin. North Am. 1989; 16: 469–79.

6. Hayles AB, Hahn HB, Sprague RG, Bahn RC, Priestly JT.Hormone-secreting tumours of adrenal cortex in children. Pedi-atrics 1966; 37: 19–25.

7. Cagle PT, Hough AJ, Pysher TJ et al. Comparision of adrenal cor-tical tumours in children and adults. Cancer 1986; 57: 2235–7.

8. Moore L, Barker AP, Byard RW, Bourne AJ, Ford WD.Adrenal cortical tumours in childhood: Clinicopathological featuresof 6 cases. Pathology 1991; 23: 94–7.

9. Ahalman H, Jansson S, Wangberg B. Adrenal cortical carci-noma: Diagnostic and therapeutic implications. Eur. J. Surg.1993; 159: 149–58.

10. Sabbaga CC, Avilla SG, Schulz C, Garbers J, Blucher D.Adrenocortical carcinoma in children: Clinical aspects andprognosis. J. Pediatr. Surg. 1993; 28: 841–3.

11. Latronico AC, Chrousos GP. Extensive personal experience:Adrenocortical tumours. J. Clin. Endocrinol. Metab. 1997; 82:1318–24.

12. Perilongo G, Rigon F, Muriga A. Oncologic causes of preco-cious puberty. Pediatr. Hematol. Oncol. 1990; 37: 1313–32.

13. Upadhye PS, Desai MP, Colaco MP, Desai PB, Deshpande RK,Mokal RA. A clinicopathological profile of adrenocorticaltumours. Indian Pediatr. 1997; 34: 481–90.

14. Neblett WW, Frexes-Steed M, Scot HW. Experience withadrenocortical neoplasm in childhood. Am. Surg. 1987; 53:117–25.

15. Jaresch S, Kornely E, Kley H-K, Schlaghecke R. Adrenal inci-dentaloma and patients with homozygous or heterozygous con-genital adrenal hyperplasia. J. Clin. Endocrinol. Metab. 1992;74: 685–9.

16. Bauman A, Bauman CG. Virilizing adrenocortical carcinoma:Development in a patient with salt-losing congenital adrenalhyperplasia. JAMA 1982; 248: 3140–1.

17. Bhatia V, Shukla S, Mishra SK, Gupta RK. Adrenal tumourcomplicating untreated 21-hydroxylase deficiency in a 51/2 year oldboy. Am. J. Dis. Child. 1993; 147: 1321–3.

18. Li JT, Shu SG, Chi CS. Aldosterone secreting adrenal corticaladenoma in an 11-year old child and collective review of theliterature. Eur. J. Pediatr. 1994; 153: 715–17.

19. Boothroyd AE, Dicks-Mireaux C, Malone M. Adrenal corticaltumours in children. Eur. J. Radiol. 1994; 8: 199–204.

20. Abrams HL, Siegelman SS, Adams DF, Sanders R, Finberg HJ,Hessel SJ. Computed tomography versus ultrasound of adrenalgland. Radiology 1982; 143: 121–8.

21. Smith SM, Patel SK, Turner DA, Matalon TA. Magnetic resonanceimaging of adrenal cortical carcinoma. Urol. Radiol. 1989; 11: 1–6.

22. Tang CK, Gray GF. Adrenocortical neoplasm. Prognosis andmorphology. Urology 1975; 5: 691–5.

23. Slooten HV, Schaberg A, Smeenk D, Moolenaar AJ. Morphologiccharacteristics of benign and malignant adrenocortical tumours.Cancer 1985; 55: 766–73.

24. Pommier RF, Brennan MF. An eleven-year experience withadrenocortical carcinoma. Am. J. Surg. 1992; 112: 963–71.

25. Wooten MD, King DK. Adrenal cortical carcinoma: Epidemiologyand treatment with mitotane and a review of the literature.Cancer 1993; 72: 3145–55.

26. Teinturier C, Brugieres L, Lemerle J, Chaussain JL, Bougneres PF.Adrenal cortical carcinoma in children: Retrospective study of 54cases. Arch. Pediatr. 1996; 3: 235–40.

27. Bloom DA, Fonkalsrud EW. Surgical management of pheochro-mocytoma in children. J. Pediatr. Surg. 1974; 9: 179–84.

28. Ein SH, Pullerits J, Creighton R, Balfe JW. Pediatric pheochro-mocytoma: A 36-year review. Pediatr. Surg. Int. 1997; 12:595–8.

29. Khafagi FA, Shapiro B, Fischer M, Sisson JC, Hutchinson R,Beierwaltes WH. Phaeochromocytoma and functioning para-ganglionoma in childhood and adolescence: Role of iodine131metaiodobenzylguanidine. Eur. J. Nucl. Med. 1991; 18: 191–8.

FUNCTIONING ADRENAL TUMOURS IN CHILDREN 107