Arch Dis Child: first published as 10.1136/adc.39.204.197 on 1 April 1964. Downloaded from Arch. Dis. Childh., 1964, 39, 197. HYPOGLYCAEMIA WITH WILMS' TUMOUR* BY A. H. LOUTFI, I. MEHREZ, S. SHAHBENDER and F. H. ABDINE From the Paediatric Surgical Department, Kasr el Aini Children's Hospital, Cairo University, U.A.R. (RECEIVED FOR PUBLICATION DECEMBER 16, 1963) Hypoglycaemia occurring in association with of the tumour to hypoglycaemia, only six cases were certain extrapancreatic tumours, mesodermal in type described before 1954 (Seckel, 1939; Arkless, 1942; and mostly malignant, has become a well-established Hines, 1943; Staffieri, Cames and Cid, 1949; Skillern, entity of organic hypoglycaemia. Conn and Seltzer McCormack, Hewlett and Crile, 1954). However, (1955), in a review of spontaneous hypoglycaemia, from 1955 to 1962, 25 more cases were added (our added this group to the well-known causes of organic case is the 33rd). All these tumours, without hypoglycaemia, namely, pancreatic, hepatic, anterior exception, have been described in adults, and ours pituitary, adrenocortical and central nervous system is the first to be recorded in a child. The purpose of lesions (Table). this communication is to introduce this entity to paediatricians and to familiarize all those concerned TABLE with the main aspects of the disease. AETIOLOGICAL CLASSIFICATION OF SPONTANEOUS HYPOGLYCAEMIA (Abbreviated from Conn and Seltzer) Case Report copyright. A 5-year-old boy was admitted to the University I. Organic: Recognizable anatomical lesion (1) Hyperinsulinism: (a) pancreatic islet-cell adenoma Children's Hospital, Mounira, on February 17, 1962, on (b) pancreatic islet-cell carcinoma account of a swelling in the abdomen of two weeks' (c) generalized hypertrophy and hyperplasia of the islets of Langerhans duration. Unusual voracious apetite was noticed for (2) Hepatic disease two weeks before admission and the parents sought (3) Anterior pituitary hypofunction (4) Adrenocortical hypofunction medical advice. They were told that the child had a mass sarcomas (5) Fibromas and in the abdomen, possibly an enlarged spleen, and were http://adc.bmj.com/ (6) Central nervous system lesions: hypothalamus or brain-stem interference with the nervous control of blood sugar referred to the Children's Hospital. There was no pain in the abdomen, no urinary disturbances and no gastro- II. Functional: No recognizable anatomical lesion, explained on basis of unusual somatic function intestinal upsets. However, the child had seemed to be (1) Hyperinsulinism: imbalance of the autonomic nervous system off colour. (2) Alimentary hyperinsulinism: rapid intestinal absorption (3) Hyperinsulinism of infancy: Staub-Traugott phenomenon Examination revealed a quiet co-operative child, (4) Idiopathic spontaneous hypoglycaemia of infancy somewhat pale, with blood pressure 140/100 mm. Hg, (5) Renal glycosuria: severe degrees of low renal threshold for glucose and a normal temperature. Palpation revealed a (6) Lactation mass in the left lumbar and hypochondrial regions, the (7) Severe continuous muscular work size of a grapefruit, firm with a smooth surface, not on September 23, 2021 by guest. Protected 1II. Factitious: Exogenous hyperinsulinism (surreptitious insulin tender and not moving with respiration. The descending administration) colon could be rolled over it. The right kidney and liver were not palpable. The spleen was not enlarged. No other mass could be felt. The preliminary diagnosis was Although 32 cases only have been recorded up to a left Wilms' tumour. June 1962 (de Coster, Payfa, Bellens, Conard and The following day, two hours after intravenous pyelo- Bastenie, 1962), there is more awareness of the graphy, the patient suddenly became unconscious. At condition today, as evidenced by an increasing first, this was thought to be momentary, but it persisted the for six hours. No explanation could be given at the report of cases. Since Doege (1930) reported time, although some advanced the possibility of an first case and established subsequently the relation allergic reaction to Uroselectan. However, 25% glucose, 50 ml., was given on an empirical basis, and to the * A paper read at a meeting of the British Association of Paediatric great surprise of those attending the case, the child Surgeons in London, September 1962. recovered. 197 Arch Dis Child: first published as 10.1136/adc.39.204.197 on 1 April 1964. Downloaded from 198 LOUTFI, MEHREZ, SHAHBENDER AND ABDINE -Ar ..i- ... ..Rl..d. i FIG. 1.-Patient unconscious during a hypoglycaemic attack. FIG. 2.-Recovery after a 45-ml. injection of glucose, 25%. Next day, the possibility ofhypoglycaemia was suggest- reflex; the pulse rate was 112 a minute; blood pressure ed and that of allergy rejected. It was decided to fast 140/90 mm. Hg, temperature 370 C. There were no the child to precipitate a hypoglycaemic attack. Next convulsions. The eyes were almost closed, the pupils morning, the child was comatose. Examination during constricted with no squint. The limbs were flaccid. the crisis (Fig. 1) revealed a completely unconscious child Excessive perspiration was characteristic. A blood with no response to pin prick and absent conjunctival sample was taken for glucose determination. Intraven- CARBOHYDRATE TOLERANCE CURVE 0-40 II I 1 copyright. Laboratory No 4 } I]_ 1 1 1 _ Hospital No. _1_1_ _ 1_ _ Sectlon SAI-4m,1 _ _ _ _ _ _ Name S5if 64u¢. _ _ _ _ _ 0-30 _ _ _ _ _ _ _ _ _ _ _ _ _ _ _ _ _ _ _ Sex AgL. http://adc.bmj.com/ _ _ _ _ _ Ward Bed - _ _ _ _ A Norm I CuIrvel _ _ _ _ Diagnosis: _ _ _ _ _ _ _ _ ' & 020 ia _ _ _ _ 0 _ _ _ _ _ _ _ . required I I 3 Investigation 221l12- _ _ , _ g.7S C. Hours on September 23, 2021 by guest. Protected _ L _ _ r _ _ _ 0-10 _ _ _ _ _ _ _ _ Remarks __ _ r ___ _ _Z _ Sugar _ _r _ _ Acetone _ _ _ _ _ _ _ _ 0 _ > _ t- 589. Date 195 Signature__. :. FIG. 3.-Glucose tolerance test curve; fasting level, 35 mg., peak at FIG. 4.-Intravenous pyelography showing normal right kidney 90 mg. at one hour, then slow fall. function and hydronephrosis on the left side; poor visualization of the lower calyces. Arch Dis Child: first published as 10.1136/adc.39.204.197 on 1 April 1964. Downloaded from H YPOGLYCAEMIA WITH WILMS' TUMOUR 199 ous glucose, 25%, was then administered. After 30 ml. the distal pancreas could be freed from its bed. The the eyes were halfopen, and after 45 ml. the child opened tumour with the involved left kidney, the distal pancreas, his eyes completely, moved in bed and recovered the spleen and the descending colon were all removed in consciousness (Fig. 2). The blood sugar during the one mass. The aorta was carefully cleared of residual episode turned out to be 27 mg./100 ml. Hypoglycaemia lymph nodes. The left suprarenal gland was not was thus confirmed. For further proof a glucose infiltrated, and so was left behind. tolerance test was performed. It showed a low fasting After the operation, the patient received hydrocortisone level (35 mg.), a peak at one hour (90 mg.), then a slow and glucose. Next day, the fasting blood sugar rose to fall (Fig. 3). 180 mg./100 ml. The blood pressure dropped to 105/80 Excretory urography (Fig. 4) showed a good right mm. Hg. Unfortunately, a few days later, the wound kidney function. However, on the left side there was a showed infection and pus was drained. This retarded big opaque shadow that had displaced the intestinal gas convalescence and the use of post-operative deep x-ray shadows to the right; the kidney showed a distorted therapy. Cyclophosphamide ('endoxan') was given, as pyelogram with dilated upper calyces, displaced pelvis, actinomycin D was not available. The child was then and poor visualization of the lower ones. The diagnosis discharged and instructed to attend out-patients' depart- of Wilms' tumour of the left kidney was confirmed. ment once a month for a regular check-up. He had no Other investigations showed: blood urea 23 mg./100 ml.; further attacks of hypoglycaemia, and since the operation Hb 72%; white blood cells 11,000, polymorphs 59%, he has been doing well with no more pain; the latest lymphocytes 39%, monocytes 2%. The urine showed recorded fasting blood sugar was 92 mg./100 ml. No traces of albumin, few pus cells, no casts and no metastases have appeared in the chest so far. Bilharzia ovae; 17-ketosteroids, 0 7 mg./24 hours; blood proteins, 6- 5 g./100 ml. Pathology. The specimen consists of the left kidney While in hospital, the child complained of pain in the with a big tumour mass arising from its antero-lateral abdomen; he felt more comfortable on lying prone, and surface, the spleen, descending colon, tail and part of the he stopped over-eating. He had another attack of body of the pancreas which is attached to the upper pole hypoglycaemia relieved by intravenous glucose. For the of the tumour (Fig. 5). It weighs 820 g. The cut section few days he spent in hospital pending operation, there ofthe kidney and the tumour (Fig. 6) is oval, 17 x 10 cm., was sugar at the bed-side to avert any hypoglycaemic greyish white, homogeneous with small areas of necrosis, attack. We were faced now with the problem of a child and without cystic formation. The tumour has totally having a Wilms' tumour and suffering from hypoglycae- destroyed the central zone of the kidney from which it copyright. mia, which conformed -to the typical Whipple's triad, probably arose, infiltrated the adjacent upper and lower namely, attacks of nervousness or gastro-intestinal zones and, in the main, has grown extrarenally.