Management of the ectopic ACTH syndrome due to thoracic carcinoids

Pass, Harvey I.; Doppman, John L.; Nieman, Lynnette K.; Stovroff, Mark C.; Vetto, John T.; Norton, Jeffrey A.; Travis, William D.; Chrousos, George P.; Oldfield, Edward H.; Cutler, Gordon B.

doi:10.1016/0003-4975(90)90085-k

Cited by 87 publications

(52 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Of the papers focusing on lung and mediastinal tumours leading to CS, Pass et al from the NIH reported on 14 cases [14], the paper from Amer et al from the UK on 19 cases [12], Sharger et al from the Massachusetts General Hospital reported on seven cases [13], and Deb et al [11] from Mayo Clinic discussed the clinical and laboratory data of 23 patients.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Ghazi

Dezfooli

Amirbaigloo

et al. 2015

Endokrynologia Polska

View full text Add to dashboard Cite

Introduction: Ectopic Cushing`s syndrome (ECS) secondary to neuroendocrine tumours (NETs) of the lung and mediastinum are rarely encountered. In this study, we present our experience in Iran on 15 patients with ECS secondary to lung and mediastinal tumours over a period of 27 years. Material and methods: Since 1985, 15 patients with ECS secondary to lung and mediastinal tumours have been diagnosed and prospectively followed by the endocrinology team of Taleghani Hospital, Tehran, Iran. The clinical signs and symptoms, laboratory findings, radiological features, immunohistochemical characteristics, management strategies and outcome data are here presented. Results: There were six women and nine men, aged 26-70 years, all presenting with typical features of Cushing's syndrome. Based on histopathologic evaluations, four patients had small cell lung cancer (SCLC) and seven patients had pulmonary NETs, one patient had a thymic NET, and one case was diagnosed as a lung tumourlet. The mean ± SD serum cortisol, 24h urine free cortisol and plasma ACTH were 47.2 ± 20.5 µg/dL, 2,702 ± 5,439 µg/day, and 220 ± 147 pg/mL, respectively. Pulmonary lesions ranged in diameter from 1.1 to 4 cm (mean 1.9 ± 1.1 cm). One patient had a 10 cm mediastinal mass. The duration of follow up in these cases was between one month and seven years (mean 29.9 ± 27.5 months). The four patients with SCLC died within three months of diagnosis. Conclusion: Our data demonstrates the protean clinical and laboratory manifestations of ECS secondary to lung and mediastinal tumours, the problems encountered in diagnosis, and the need for a multidisciplinary approach. This study confirms other series from Western Europe and North America that, unlike the SCLC patients who show a poor outlook, ECS secondary to lung carcinoids has a more favourable prognosis. (Endokrynol Pol 2015; 66 (1): 2-9)

show abstract

Section: Discussionmentioning

confidence: 99%

“…Due to the rarity of the syndrome, most clinical reports in the medical literature present a single case or a limited number of cases, and there are few papers specifically reviewing clinical and laboratory data in a large number of patients suffering from ECS secondary to lung tumours [10][11][12][13][14].…”

Section: Introductionmentioning

confidence: 99%

Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Ghazi

Dezfooli

Amirbaigloo

et al. 2015

Endokrynologia Polska

View full text Add to dashboard Cite

show abstract

“…However with cortisol producing adrenal tumours the high cortisol levels and low ACTH levels are unaffected. 4,5 Octreotide scanning is helpful in locating functionally active neuroendocrine or carcinoid tumours. On administration of octreotide, carcinoid tumours would be seen as an area of increased activity.…”

Section: Discussionmentioning

confidence: 99%

An unusual case of Cushing's syndrome: the importance of a stepwise approach to diagnosis

2009

View full text Add to dashboard Cite

LessonA 58-year-old woman presented with excessive weight gain, abdominal bloating and tiredness. Over the next few months she had excessive facial hair growth, easy bruising and increased pigmentation of skin. Examination showed excess facial hair, moon-shaped face, centripetal obesity, purple striae on abdominal wall, an obvious buffalo hump and some areas of pigmentation in her skin. Systems examination revealed proximal myopathy and a blood pressure of 152/82. Routine blood tests were all normal. 24-hour urinary cortisol was 3,082 nmol/d (0-260). Nine am cortisol was 855 nmol/l with midnight cortisol reaching 349 nmol/l. Thyroid function tests, 24 urinary catecholamines and serum testosterone were all normal. Chest Xray was normal.Clinical appearance and screening tests strongly suggested a diagnosis of Cushing's syndrome. Dexamethasone suppression test showed a lack of suppression of cortisol levels on low dose dexamethasone, confirming a diagnosis of Cushings Syndrome. The high dose dexamethasone suppression (HDDT) test demonstrated no suppression of serum cortisol with partial suppression of urinary cortisol. Magnetic resonance imaging showed normal pituitary and adrenal glands. Corticotrophinreleasing hormone (CRH) stimulation test demonstrated lack of adequate suppression of ACTH and cortisol, implying an ectopic source of ACTH production. Computed tomography chest scan revealed a 3 ϫ 2 cm mass in the anterior mediastinum and an octreotide uptake scan demonstrated increased activity in this location (Fig 1). A diagnosis of ectopic ACTH secretion (EAS) secondary to a neuroendocrine mediastinal tumour was the most likely cause. The patient was initiated on metyrapone in order to reduce cortisol production and improve symptoms. Urinary cortisol levels responded well. The mediastinal mass was removed. Cytology showed benign thymic carcinoid. The tissue was positive for ACTH, chromogranin and synacthophysin, confirming a diagnosis of an ACTH-producing thymic carcinoid. The patient improved well after surgery with gradual disappearance of many symptoms, particularly proximal myopathy. DiscussionEAS accounts for approximately 15% of all cases of Cushing's syndrome, with thymic carcinoid accounting for another 15%. Around 20 to 35% of thymic carcinoid tumours could present as EAS. 1 It is often difficult to diagnose EAS and a combination of endocrine tests and radiological imaging is required. First step is establishing a diagnosis of Cushing's syndrome. Initial screening is by means of overnight dexamethasone suppression test (9 am cortisol level Ͻ50 nmol/l) and a 24-hour urinary cortisol (Ͼ250 nmol/l). The low dose dexamethasone suppression test helps further confirm the diagnosis while the HDDT helps to differentiate pituitary dependent (PD) Cushing's (ie Cushing's disease) from non-PD Cushing's. As this case demonstrates there was a fall in urinary cortisol levels with the HDDT. In fact up to 50% of patients with EAS are believed to show some suppression of cortisol following HDDT while a recent series show...

show abstract

“…The time from presentation to diagnosis is within the limits reported for bronchial carcinoid tumors that cause Cushing's syndrome. 4 On retrospective examination of the patient's CT studies, small lesions were found to be present at least five years before lobectomy. Whether the tumorlets were confined to the right lower lobe remains unanswered, but there was no evidence of other tumorlets or excess corticotropin secretion six months after surgery.…”

Section: Discussionmentioning

confidence: 99%