Archives ofDisease in Childhood 1990; 65: 269-274 269 Phaeochromocytoma-investigation and Arch Dis Child: first published as 10.1136/adc.65.3.269 on 1 March 1990. Downloaded from management of 10 cases J E Deal, P S Sever, T M Barratt, M J Dillon Abstract A system for the classification of tumours of Since 1960 we have diagnosed phaeochromo- the autonomic nervous system was proposed by cytoma (paraganglioma) in 10 children. The Glenner and Grimley in 1974 and is now widely cases include a 15 year old girl who over a accepted.3 Under this classification a tumour three year period presented with multiple arising from the adrenal medulla is termed a paragangliomata and an associated malignant phaeochromocytoma and chromaffin tumours carotid body tumour. AR children were hyper- arising outside the adrenals of paraganglionic tensive, eight of 10 presenting with severe origin are termed paragangliomata. Paragang- headaches. Diagnosis was based on finding a liomata are more commonly found at extra- raised urinary vaniliylmandelic acid excretion adrenal sites in children than in adults and can and plasma noradrenaline concentration. In occur anywhere in the autonomic nervous addition six of eight children were hyper- system. Tumours of the carotid body, in as- calcaemic with raised plasma calcitonin con- sociation with phaeochromocytoma, have been centrations; plasma parathyroid hormone frequently reported in adults,4 5 but there is concentrations were high in two of seven and only one report in the literature of an affected four out of eight children had raised plasma child, that of a 12 year old boy.' We report here renin activities on presentation. No child, 10 cases of paragangliomata, including one child however, was found to have a multiple endo- with multiple lesions and a malignant carotid crine neoplasia syndrome. Despite the intro- body tumour, that have been seen at the duction of newer techniques for the detection Hospital for Sick Children since 1960. of catecholamine producing tumours we found that selective arteriography and venous catecholamine sampling were superior for Patients tumour localisation compared with ultrasound REVIEW OF CASES scanning, computed tomography, and meta- Clinical details of all 10 children are summarised iodo-benzyl-guanidine (MIBG) scanning. in table 2; the cases are tabulated in chrono- logical order of presentation. They ranged in age from 3 5 months to 16 years, mean age 10-3 http://adc.bmj.com/ Phaeochromocytoma is a rare but treatable years. Only three children were less than 10 cause of hypertension in childhood.' At the years old at presentation. Hospital for Sick Children, Great Ormond The commonest presentation in the present Street, London, which is a tertiary referral series was severe headaches; varying in duration centre for childhood hypertension, 1-3% of from 11 days to 2-5 years. The youngest child cases referred over a 10 year period, for (case 3) presented with episodes of diarrhoea investigation of high blood pressure, were and sweating and two generalised fits, presumed on October 1, 2021 by guest. Protected copyright. found to have a phaeochromocytoma (table 1). to be due to her hypertension, at the age of In children there is a higher incidence (32%) of 3 months. All children were hypertensive; in multiple tumours and an increased occurrence eight children it was sustained and in only two of the multiple endocrine neoplasia syndromes was the blood pressure labile. One girl (case 10) when compared with that reported in adults.' was diagnosed fortuitously: although asympto- The incidence of malignancy is 2-4%, compared matic she was found to be hypertensive at an Renal Unit, with a malignancy rate of 10% in adults.2 outpatient clinic visit where she was being Hospital for Sick followed up for a stage IV Wilms' tumour that Children and Department seven ofPaediatric Nephrology, Table I Diagnoses of 454 cases of hypertension admitted had been diagnosed and treated years Institute of Child Health, to the renal unit at the Hospital for Sick Children, previously by chemotherapy, radiotherapy, and London 1975-85, including six of the children reported here a right nephrectomy. J E Deal Diagnosis No (%) of children Another girl (case 7) who presented with T M Barratt headaches, pallor, and tachycardia, was initially M J Dillon Coarse renal scarring: Department of Clinical Reflux nephropathy 84 (19) found to have two para-aortic paragangliomata. Pharmacology, Obstructive uropathy 72 (16) After surgical removal of these she presented Glomerular disease 123 (27) St Mary's Hospital, Renovascular disease 38 (8) over the subsequent three years with two London Haemolytic uraemic syndrome 32 (7) further extra-adrenal tumours, bilateral intra- P S Sever Polycystic kidney disease 22 (5) adrenal tumours, and a malignant carotid body Correspondence to: Idiopathic (essential) 18 (4) Dr J E Deal, Department of Renal dysplasia 13 (3) tumour, which were all surgically removed. Paediatric Nephrology, Wilms' tumour 9 (2) Two children had a positive family history: Institute of Child Health, Coarctation 7 (<2) 30 Guilford Street, Phaeochromocytoma 6 (<2) one (case 10) had an uncle who had been London WC1N IEH. Renal vein thrombosis 4 (1) Miscellaneous 26 (6) diagnosed some years before her presentation Accepted 9 October 1989 and the other (case 9) had a 17 year old sister 270 Deal, Sever, Barratt, Dillon Table 2 Clinical details of the 10 children with phaeochromocytoma in chronological order of presentation Case 1 Case 2 Case 3 Case 4 Case S Case 6 Age at presentation Arch Dis Child: first published as 10.1136/adc.65.3.269 on 1 March 1990. Downloaded from (years) 13-3 12-6 0 3 7-5 10-1 10-4 Sex Male Female Female Female Male Female Presentating symptoms Headaches, Headaches, Diarrhoea, Headaches Headaches, Headaches, convulsion pallor, sweating convulsion sweating abdominal pain Investigations: Urinary vanillylmandelic acid T T T T T T Plasma noradrenaline (pg/ml)' - - 1371 5130 18 345 27 700 Plasma renin activity - - N N T N Calcitonin - - T T T Parathyroid hormone - - T N - N Plasma calcium (mmol/l) - - 2-7 2-64 2-75 2-64 Preoperative treatment Phentolamine Phenoxybenzamine, Phenoxybenzamine, Phenoxybenzamine, Phenoxybenzamine Labetalol, propranolol propranolol propranolol phenoxybenzamine, propranolol Site and size of tumour (cm) Right adrenal Right adrenal Left adrenal Right adrenal Left adrenal Right adrenal 5x4 7x6 03x03 5x3 5x5 3x2-5 Other features N, normal; 1, concentration raised. 'To convert pg/ml to nmol/l: x0 005988. who was diagnosed at the same time as he was intravenous pyelography, which failed to localise being investigated. There were no positive the tumour, and then proceeded to an explora- family histories of multiple endocrine neoplasia tory laparotomy. syndromes. At presentation all 10 children had raised 24 hour urinary vanillylmandelic acid concentra- MANAGEMENT tions. Plasma noradrenaline concentrations All patients required treatment for their hyper- were also raised in the eight children measured. tension on presentation as shown in table 2. Case 7, the child with multiple paragangliomata, Six children were successfully treated with a had repeatedly normal urinary vanillylmandelic combination of phenoxybenzamine (1-4 mg/kg! acid concentrations after the removal of her first day) and propranolol (1-10 mg/kg/day) alone. two tumours but raised plasma noradrenaline Case 1 was prepared for surgery with phentola- http://adc.bmj.com/ concentrations. mine (100 [tg/kg intravenously). Treatment Plasma renin activity was raised in four out of with labetalol alone in cases 6, 8, and 10 did not eight patients on presentation and six of the produce adequate a blockade and so their eight had raised plasma calcitonin concentrations treatment was changed. Four of the present (normal <0-08 ,tg/l), but these were normal series were treated with nifedipine (0-5-4 mg/ when repeated postoperatively. Associated with kg/day). In one child (case 8) nifedipine was a raised plasma calcitonin concentration, the six added to the regime of propranolol and on October 1, 2021 by guest. Protected copyright. patients were noted to be hypercalcaemic at phenoxybenzamine to achieve adequate cate- presentation (normal <2-6 mmol/l corrected); cholamine blockade. In case 10 nifedipine alone postoperatively they were normocalcaemic. was insufficient to block the patient's symptoms Plasma concentrations of parathyroid hormone and control the blood pressure, this was only were high in two ofseven children at presentation achieved when phenoxybenzamine and labetalol but fell to normal after removal of their phaeo- were introduced. In two patients (cases 7 and 9) chromocytoma. adequate preoperative control of blood pressure Table 3 shows the localisation techniques that was achieved with nifedipine alone. were used in our patients. Case 1, who presented All 10 children were successfully treated by in 1960 for investigation, had a phaeochromo- surgical removal of their paragangliomata. cytoma clinically diagnosed and underwent There were no operative complications and no Table 3 Radiological techniques used to localise catecholamine secreting tumours in the 10 patients Case No 1 2 3 4 5 6 7 8 9 10 Intravenous pyelography - + Ultrasound -- - + - +* Computed tomography + + '231-MIBG scan +* + ± + Selective arteriography + + + + + + + + Serial catecholamine sampling + + + + + + +, Technique helped localise the tumour; -, technique failed to localise the tumour; +, equivocal result; * MIBG scan localised the tumours