Conventional and Nuclear Medicine Imaging in Ectopic Cushing's Syndrome: A Systematic Review

Isidori, Andrea M.; Sbardella, Emilia; Mantovani, Giovanna; Albiger, Nora; Ambrogio, Alberto Giacinto; Arnaldi, Giorgio; Arvat, Emanuela; Berardelli, Rita; Boscaro, Marco; Boschetti, Mara; Cannavò, Salvatore; Cavagnini, Francesco; Colao, Annamaria; Corsello, Salvatore Maria; Cozzolino, Alessia; Leo, Monica De; Somma, Chiara; Esposito, Katherine; Ferone, Diego; Foresta, Carlo; Gatto, Federico; Giordano, Carla; Giugliano, Dario; Graziadio, Chiara; Isidori, Andrea M.; Loli, Paola; Manetti, Luca; Mannelli, Massimo; Marzullo, Paolo; Mantero, Franco; Minuto, Francesco; Paragliola, Rosa Maria; Giraldi, Francesca Pecori; Pivonello, Rosario; Reimondo, Giuseppe; Scaroni, Carla; Scillitani, Alfredo; Simeoli, Chiara; Stigliano, Antonio; Terzolo, Massimo; Tortora, Fabio; Trementino, Laura; Vitale, Giovanni; Zatelli, Maria Chiara

doi:10.1210/jc.2015-1589

Cited by 125 publications

(152 citation statements)

References 143 publications

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“…Neuroendocrine tumors (NETs), mainly bronchial carcinoids (BC) (3-54.8%), are the most frequent causes of ectopic ACTH secretion in more recent series (3,4,5,6,7,8), whereas small-cell lung carcinoma (SCLC) represented the most common tumor associated with ECS in early series (3.3-50%) (9). Other less-frequent causes are thymic carcinoids (TC) (5-42%), pancreatic (p) NETs (7.5-25%), pheochromocytomas (2.5-25%) and medullary thyroid carcinomas (MTC) (2-8%) (3,4,5,6,7,8). Unknown primary tumors account for .5% of all causes of ectopic ACTH production (3).…”

Section: Introductionmentioning

confidence: 99%

Prognostic factors in ectopic Cushing’s syndrome due to neuroendocrine tumors: a multicenter study

Davì

Cosaro

Piacentini

et al. 2017

European Journal of Endocrinology

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Objective: Evidence is limited regarding outcome of patients with ectopic Cushing's syndrome (ECS) due to neuroendocrine tumors (NETs). Design: We assessed the prognostic factors affecting the survival of patients with NETs and ECS. Methods: Retrospective analysis of clinicopathological features, severity of hormonal syndrome, treatments from a large cohort of patients with NETs and ECS collected from 17 Italian centers. Results: Our series included 110 patients, 58.2% female, with mean (±s.d.) age at diagnosis of 49.5 ± 15.9 years. The main sources of ectopic ACTH were bronchial carcinoids (BC) (40.9%), occult tumors (22.7%) and pancreatic (p)NETs (15.5%). Curative surgery was performed in 56.7% (70.2% of BC, 11% of pNETs). Overall survival was significantly higher in BC compared with pNETs and occult tumors (P = 0.033) and in G1-NETs compared with G2 and G3 (P = 0.007). Negative predictive factors for survival were severity of hypercortisolism (P < 0.02), hypokalemia (P = 0.001), diabetes mellitus (P = 0.0146) and distant metastases (P < 0.001). Improved survival was observed in patients who underwent NET removal (P < 0.001). Adrenalectomy improved short-term survival. Clinical Study

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Section: Introductionmentioning

confidence: 99%

Prognostic factors in ectopic Cushing’s syndrome due to neuroendocrine tumors: a multicenter study

Davì

Cosaro

Piacentini

et al. 2017

European Journal of Endocrinology

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“…As a relatively aggressive tumour, several case reports have demonstrated 18 FDG uptake in thymic NENs, an uncommon finding in most other NENs [47][48][49] . In the latest study which reviewed the reports of patients with thymic carcinoids associated with EAS, the sensitivity of conventional SRS, 18 FDG-PET, and 68 Ga-DOTATOC PET/CT in thymic carcinoids was 12/14, 5/8, and 2/4, respectively, with positivity on CT scanning in 85% [50] . Both conventional SRS and 68 Ga-DOTATOC PET/CT are helpful in surveillance of thymic NENs [51, 52] , but 68 Ga-DOTATOC PET/CT might be the best imaging modality to identify bone metastases in positive patients [53] .…”

Section: Nuclear Medicinementioning

confidence: 99%

Thymic Neuroendocrine Neoplasms: Biological Behaviour and Therapy

Jia

Šulentić

et al. 2017

Neuroendocrinology

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Thymic neuroendocrine neoplasms are rare tumours, but their management can often be highly problematic. While previously assumed to be essentially variants of bronchopulmonary (lung) carcinoids, they are generally more aggressive and more difficult to treat. Some 25% are associated with multiple endocrine neoplasia-1, while a higher proportion are associated with the ectopic ACTH syndrome, and occasionally both. We discuss the classification of these tumours, their biology as far as is known, and their clinical, biochemical and imaging features. We also review possible management options and suggest stratagems to optimise their treatment, which even today is far from optimal.

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“…Many of these tumours may be small and be elusive to imaging modalities, rendering localisation studies a diagnostic challenge. Recent studies have shown a superiority of somatostatin analogue-based PET imaging over CT and MRI scans for localisation studies of these NETs 1 71 72. For example, scintigraphy with 68 Gallium-SSTR-PET/CT was reported to have 100% sensitivity in detecting NET with ACTH or CRH production in a recent study 72.…”

Section: Investigations For Localisationmentioning

confidence: 99%

Cushing's syndrome: a practical approach to diagnosis and differential diagnoses

et al. 2017

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Diagnosis of Cushing's syndrome (CS) and identification of the aetiology of hypercortisolism can be challenging. The Endocrine Society clinical practice guidelines recommends one of the four tests for initial screening of CS, namely, urinary-free cortisol, late night salivary cortisol, overnight dexamethasone suppression test or a longer low-dose dexamethasone suppression test, for 48 hours. Confirmation and localisation of CS requires additional biochemical and radiological tests. Radiological evaluation involves different imaging modalities including MRI with or without different radio-nuclear imaging techniques. Invasive testing such as bilateral inferior petrosal sinus sampling may be necessary in some patients for accurate localisation of the cause for hypercortisolism. This best practice review discusses a practical approach for the diagnostic evaluation of CS with a brief discussion on differential diagnoses, and cyclical CS, to enhance the skills of clinicians and laboratory personnel.

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Conventional and Nuclear Medicine Imaging in Ectopic Cushing's Syndrome: A Systematic Review

Abstract: Nuclear medicine improves the sensitivity of conventional radiology when tumor site identification is problematic. OCT offers a good availability/reliability ratio, and FDG-PET was proven useful. 68Gallium-SSTR-PET/CT use was infrequent, despite offering the highest sensitivity.

Cited by 125 publications

References 143 publications

Prognostic factors in ectopic Cushing’s syndrome due to neuroendocrine tumors: a multicenter study

Prognostic factors in ectopic Cushing’s syndrome due to neuroendocrine tumors: a multicenter study

Thymic Neuroendocrine Neoplasms: Biological Behaviour and Therapy

Cushing's syndrome: a practical approach to diagnosis and differential diagnoses

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