Occult ectopic adrenocorticotropic hormone syndrome

Abstract

We do not understand why in the case reported by J R Cuthbertson and colleagues (Feb 10, p 419)1Cuthbertson JR Vinjamuri S Bowen-Jones D Clinical picture: occult ectopic ACTH syndrome.Lancet. 2001; 357: 419Summary Full Text Full Text PDF PubMed Scopus (4) Google Scholar the patient was treated by bilateral adrenalectomy and not by an excision of the carcinoid tumour detected by indium-111 labelled octreotide scanning. We would like to know why this chance of successful treatment was lost—did the localisation of the tumour or the patient's general condition not allow for a radical tumour excision?We agree with Cuthbertson and colleagues that 111In-octreotide scanning might have a useful role in occult ectopic adrenocorticotropic hormone (ACTH) syndrome. In 1998 we reported two patients with ectopic ACTH syndrome caused by a bronchial carcinoid, treated successfully by tumour excision.2Kasperlik-Zaluska AA Makowska AM Pietraszek A Carcinoid tumour.Lancet. 1998; 352: 1554Summary Full Text Full Text PDF PubMed Scopus (1) Google Scholar In one patient, multiple round lesions were seen on pulmonary radiography. The scintigraphy with labelled somatostatin, however, showed only one bronchial carcinoid tumour. During the operation, the other round lesions appeared to be flat pleural lipomas (an extraordinary finding in Cushing's syndrome), which, according to chest radiography, were initially interpreted as metastases.In 1999, we also saw a man aged 21 years with fulminant Cushing's syndrome. The laboratory findings (ACTH: 350 ng/L, severe hypokalaemia, no response to dexamethasone in classic Liddle's test) suggested ectopic ACTH syndrome. No source of ectopic corticotropin secretion was detected on chest radiography, but spiral computed tomography showed a round lesion in the sixth segment of the right lung. Octreotide scanning confirmed the presence of increased tracer uptake in this location (figure). The tumour excision resulted in a rapid improvement in Cushing's features. Improvements in medical procedures enable the diagnosis and successful treatment of occult ectopic ACTH syndrome and it is the role of specialistic teams to choose the best method of treatment. We do not understand why in the case reported by J R Cuthbertson and colleagues (Feb 10, p 419)1Cuthbertson JR Vinjamuri S Bowen-Jones D Clinical picture: occult ectopic ACTH syndrome.Lancet. 2001; 357: 419Summary Full Text Full Text PDF PubMed Scopus (4) Google Scholar the patient was treated by bilateral adrenalectomy and not by an excision of the carcinoid tumour detected by indium-111 labelled octreotide scanning. We would like to know why this chance of successful treatment was lost—did the localisation of the tumour or the patient's general condition not allow for a radical tumour excision? We agree with Cuthbertson and colleagues that 111In-octreotide scanning might have a useful role in occult ectopic adrenocorticotropic hormone (ACTH) syndrome. In 1998 we reported two patients with ectopic ACTH syndrome caused by a bronchial carcinoid, treated successfully by tumour excision.2Kasperlik-Zaluska AA Makowska AM Pietraszek A Carcinoid tumour.Lancet. 1998; 352: 1554Summary Full Text Full Text PDF PubMed Scopus (1) Google Scholar In one patient, multiple round lesions were seen on pulmonary radiography. The scintigraphy with labelled somatostatin, however, showed only one bronchial carcinoid tumour. During the operation, the other round lesions appeared to be flat pleural lipomas (an extraordinary finding in Cushing's syndrome), which, according to chest radiography, were initially interpreted as metastases. In 1999, we also saw a man aged 21 years with fulminant Cushing's syndrome. The laboratory findings (ACTH: 350 ng/L, severe hypokalaemia, no response to dexamethasone in classic Liddle's test) suggested ectopic ACTH syndrome. No source of ectopic corticotropin secretion was detected on chest radiography, but spiral computed tomography showed a round lesion in the sixth segment of the right lung. Octreotide scanning confirmed the presence of increased tracer uptake in this location (figure). The tumour excision resulted in a rapid improvement in Cushing's features. Improvements in medical procedures enable the diagnosis and successful treatment of occult ectopic ACTH syndrome and it is the role of specialistic teams to choose the best method of treatment. Occult ectopic adrenocorticotropic hormone syndromeAuthors' reply Full-Text PDF