Secreting Intrathoracic Ganglioneuroma

Abstract

Intractable diarrhoea with failure to thrive for eighteen months. No respiratory symptoms, but large opacity in right hemithorax discovered on X-ray. Transferred for investigation and removal of tumour, aged 2 years 7 months. On examination: Fretful, wasted child with marked gaseous abdominal distension. Profuse sweating and considerable thirst. Brassy cough. No stridor. Fullness of right upper chest with impaired movement, dull percussion note and absent breath sounds anteriorly and posteriorly. Mediastinum displaced to left. Persistent hypertension with blood pressure 150/110 to 260/150. No abnormal neurological signs. Investigations: Urine: routine normal. X-rays of chest: uniformly dense opacity right hemithorax (Fig 1). Deformity right 3rd rib. Abdomen: multiple fluid levels in distended gutcorrected

Watery diarrhoea and ganglioneuroma with secretion of vasoactive intestinal peptide.

“…While some authors have considered the possible mechanisms whereby catecholamines cause diarrhoea (Hamilton, Radde, and Johnson, 1968;Stickler et al, 1962) others have rejected the idea of a causal relation (Smellie and Sandler, 1961). We have not confirmed Sandler, Karim, and Williams's (1968) prostaglandin hypothesis and we have shown that the diarrhoea was not due to an excess of serotonin.…”

“…VIP is known to provoke vasodilatation, raise blood glucose levels, inhibit histamine-stimulated gastric acid secretion, and stimulate the flow of small intestinal juices. In children with ganglioneuroma and watery diarrhoea facial flushing (Hawfield and Daisley, 1952;Rosenstein and Engelman, 1)63) and low gastric acidity (Hamilton et al, 1968) have been described but the fasting blood glucose measured in one case was normal (Smellie and Sandler, 1961). In our case the fasting blood glucose, calcium, and phosphate were normal and there was no evidence of facial flushing.…”

Catecholamine-Secreting Tumours

“…In our experience and that of others (Smellie & Sandler 1961, Sandler & Ruthven 1961b, Voorhess & Gardner 1962, Voorhess 1966, Sourkes et al 1963, von Studnitz et al 1963, Brett et al 1964, Kaser et al 1964, Ruthven & Sandler 1964) those tumours in the neuroblastoma-ganglioneuroma group producing catecholamines are invariable dopamine secretors, with a high output of HVA, although the very high urinary levels of ,-hydroxylated metabolites also attest to a great overproduction of the more pharmacologically active noradrenaline. Paradoxically, only a minority of subjects with secreting neuroblastoma have a marked elevation in blood pressure (Sourkes et al 1963, von Studnitz et al 1963 whereas phwochromocytoma, with a smaller output of noradrenaline metabolites, gives rise to a severe degree of hypertension.…”

The Management of Cases of Phæochromocytoma

“…A chest X-ray is an essential preliminary; the heart size can be estimated and secondary deposits from a malignant pheochromocytoma may be demonstrated. In one of our patients, a large intrathoracic ganglioneuroma containing noradrenaline and producing severe hypertension was demonstrated (previously reported by Smellie & Sandler 1961). The great majority of phaochromocytomas lie in the retroperitoneal tissues of the abdomen and their presence was suspected in 7 of our patients when the plain film of the abdomen and the intravenous urogram showed downward displacement of a kidney.…”