Arch Dis Child: first published as 10.1136/adc.53.4.341 on 1 April 1978. Downloaded from

Archives of Disease in Childhood, 1978, 53, 341-348

Short reports

Late functioning adrenocortical On the first occasion she made a complete recovery postoperatively, but after the second episode she carcinoma in a 5-year-old girl unfortunately remained paraplegic. The major part of the tumour was at this time found to be anterior Adrenocortical carcinoma is a rare form of malig- to the vertebral bodies. Further review of the nancy in childhood and its incidence has not been histology confirmed the suspicion of an adreno- accurately recorded. The mode of presentation cortical carcinoma as the primary tumour. There is usually the development of signs of virilisation was still no clinical or radiological evidence of this or Cushing's syndrome. Very rare feminising and no evidence of increased production of oxo- tumours and occasional nonfunctioning ones have or oxogenic steroids. The tumour was at this time been reported (Lewinsky et al., 1974). The tumour enlarging radiologically. may present in association with hemihypertrophy, In April 1977, 10 months after the onset of her urinary tract abnormalities, and other anomalies illness, she rapidly developed plethora, coarsening (Fraumeni and Miller, 1967). We present the case of the skin with acne, trunkal obesity, and progres- of a 5-year-old girl with a late functioning tumour, sive virilisation with pubic and axillary hair. and an unusual presentation. Urinary steroid levels were as shown in the Table, first estimation. The oxosteroid and oxogenic Case report steroid levels rose further over the next week, second estimation. She then developed multiple A 5-year-old girl presented in July 1976 with lung metastases of cannonball type with an asso- paraplegia of acute onset. There was associated ciated pleural effusion and died suddenly. Necropsy retention of urine and a sensory level at D8. She was not permitted. had previously been a healthy child apart from having gluten-sensitive enteropathy which was diagnosed in 1972 and more recently she had dev- Table Results ofbiochemical tests http://adc.bmj.com/ eloped recurrent abdominal pain and occasional First Second nocturnal enuresis. estimation estimation Normal range Plain x-rays of her dorsal spine showed a collapse Urinary 17-oxosteroids of the vertebral body at D7 with a associated large (pmol/24 h) 73 118 0.S-6-9 Urinary 17-oxogenic paravertebral mass. At operation extradural tumour steroids (jsmol/24 h) 13 17 13.9-34-7 was found compressing the spinal cord and a lam- Plasma testosterone (nmol/l) 4.9 4.9 <1.0 inectomy and biopsy were performed. This was Midnight plasma initially reported as having the characteristics of a cortisol (nmol/l) 371 133 50-120 on September 25, 2021 by guest. Protected copyright. neuroblastoma. She was treated with radiotherapy 9.00 a.m. plasma and chemotherapy (vincristine, actinomycin D, and cortisol (nmol/l)] 168 203 248-607 cyclophosphamide), after which she made a full Urinary steroids and plasma testosterone estimated at onset of virilisation and 3 weeks later showing progressive rise in levels. Cor- recovery with return of normal motor function. tisol levels show reverse of diurnal variation. The child came under our care in December Conversion: SI to traditional units-17-oxosteroids and 17-oxogenic steroids: 1 jsmol/24 hsw0-288 mg/24 h. Testosterone: 1 imol/lw 1976. Chemotherapy was continued. The biopsy 1 aswO036 slides were reviewed and it was felt that they were 0.288 ng/ml. Cortisol: nmol/I pg/100 ml. more compatible with a primary tumour of carcin- omatous histology, probably arising in the adrenal Discussion cortex. Blood levels of electrolytes, calcium, and glucose, and urinary catecholamine excretion were Adrenocortical carcinoma is a rare tumour in all normal. Intravenous pyelography and skeletal childhood. In one large review (Hutter and Kayhoe, survey showed no abnormality apart from the verte- 1966) 40 cases presenting under the age of 20 years bral lesion. were reported out of a total of 186. The mean age Over the following 4 months she twice developed was 36 years with a range of 6 months to 72 years an acute paraplegia. On each occasion cisternal and the female to male ratio was 2:1. The majority myelography showed obstruction to the flow of of cases in children manifest with signs and symp- Mydil and operative decompression was attempted. toms of adrenal hypercorticism, and a neurological 341 Arch Dis Child: first published as 10.1136/adc.53.4.341 on 1 April 1978. Downloaded from

342 Short reports presentation is very rare. In Hutter and Kayhoe's Lipsett, M. B., Hertz, R., and Ross, G. T. (1963). Clinical series there were only 9 nonfunctioning tumours. and pathophysiologic aspects of adrenocortical carcinoma. American Journal of Medicine, 35, 374-383. Dumb-bell tumours due to neuroblastoma can Rapaport, E., Goldberg, M. B., Gordan, G. S., and Hinman, cause an acute onset paraplegia, but they are not F., Jr., (1952). Mortality in surgically treated adrenocortical usually associated with any vertebral collapse. tumors. Review of cases reported for 20 year period 1930- The bony lesions seen in disseminated neuroblastoma 1949, inclusive. Postgraduate Medicine, 11, 325-353. Stewart, D. R., Jones, P. H. M., and Jolleys, A. (1974). are rarely single and are more frequent in the limb Carcinoma of the adrenal gland in children. Journal of bones and calvarium. Metastatic- adrenocortical Pediatric Surgery, 9 59-67. carcinoma in the spinal extradural space has rarely been described (Lipsett et al., 1963). Vertebral H. B. MARSDEN, P. MORRIS JONES, P. D. LEES, and metastases are also very rare, occurring in only 7 of I. M. HANN Hutter and Kayhoe's 138 patients. Metastases Royal Manchester Children's Hospital, Pendlebury, usually manifest the same hormonal pattern as the Manchester M27 primary tumour but a few cases have been reported JHA. in which functioning tumours recurred as non- Correspondence to Dr P. Morris Jones functioning types (Rapaport et al., 1952). The absence of any clinical or radiological evidence of primary adrenocortical tumour is very unusual, but in the absence of a necropsy we were unable to rule out a small tumour which was not Unusual case of adrenal cortical causing renal displacement. Aortography, reno- graphy (Craig et al., 1975), and adrenal scintigraphy carcinoma in a female infant (Jorgensen et al., 1975) might have elucidated this problem but rapid deterioration in the patient's A 44-month-old girl remains well 39 months after clinical condition did not allow their performance. removal of a left adrenal cortical carcinoma. The The response of this tumour to radiation and clinical presentation of this tumour differed from chemotherapy is much better than in adult tumours those previously reported. of the same type and we have previously reported survival of metastatic cases (Stewart et al., 1974). Case report Initial response to radiotherapy and chemotherapy was demonstrated here. A girl weighing 2480 g was born at term to a 27- http://adc.bmj.com/ year-old gravida 2 primiparous mother. Growth Summary progressed at the 3rd centile. At 3 months transient bloody stools resolved after change A 5-year-old with adrenocortical carcinoma pre- of milk formula. At 4i months a left abdominal sented with acute paraplegia. The tumour was mass was noted after a 2-week history of weight loss, initially nonfunctioning but finally showed rapid irritability, and increasing abdominal girth. Positive dissemination and the patient then developed physical findings included blood pressure 190/140 on September 25, 2021 by guest. Protected copyright. Cushingoid features and virilisation. mmHg and a tender left abdominal mass filling the entire side of the abdomen. Congenital anomalies, hirsutism, Cushing's syndrome, "and inappropriate References feminisation, the most commonly reported presenta- Craig, W. P., Jr.'-Hawkins, I. F., Jr., Ind Kaude, J. V. tions of this tumour in this age group and during (1975). Selective adrenal venography in an infant with childhood, were absent (Reidel, 1952; Knight et al., virilizing adrenocortical carcinoma. Pediatric Radiology, 1960; Bacon and Lowrey, 1965; Cooper et al., 1967; 3, 53-54. Fraumeni and Miller, 1967; Kenny et al., 1968; Fraumeni, J. F., and Miller, R. W. (1967). Adreno cortical neoplasms with hemihypertrophy, brain tumors and Gilbert and Cleveland, 1970; Stewart et al., 1974). other disorders. Journal ofPediatrics, 70, 129-138. Initial clinical impression was either neuroblastoma Hutter, A. M., Jr., and Kayhoe, D. E. (1966). Adrenal or nephroblastoma. cortical carcinoma. Clinical features of 138 patients. could not be done American Journal of Medicine, 38, 572-580. Preoperative investigation due Jorgensen, H., Norman, N., and Sundsfjord, J. A. (1975). to the sudden onset of respiratory distress secondary Scintigraphy with I 131-19-iodocholesterol in adrenal to rapid, progressive expansion of the abdominal disease. Acta Medica Scandinavica, 197, 345-351. mass. At surgery, a 10 x 18 x 10 cm haemorrhagic Lewinsky, B. S., Grigor, K. M., and Symington, T. (1974). mass the entire left The clinical and pathological features of 'non-hormonal' occupied retroperitoneum, en- adrenocortical tumors. Report of twenty new cases and casing the left kidney and invaded by direct extension review of the literature. Cancer, 33, 778-790. the mesocolon at the splenic flexure. Microscopic