gallium-ga-68-dotatate and Cushing-Syndrome

Ectopic adrenocorticotropic hormone (ACTH)-secreting tumors are commonly small, yet they often lead to fulminant forms of Cushing syndrome. High-resolution functional imaging modalities, such as [Ga]-DOTATATE, have been recently introduced in clinical practice for the identification of neuroendocrine tumors. In this review, we focus on the performance of [Ga]-DOTATATE as a tool for localizing primary and metastatic sources of ectopic Cushing syndrome (ECS).. Prompt surgical removal of ectopic ACTH-secreting tumors is the mainstay of therapy in patients with ECS. Detecting such tumors with conventional cross-sectional imaging is often unsuccessful, owing to their small size. [Ga]-DOTATATE has been approved in 2016 by the Federal Drug Administration for imaging well differentiated neuroendocrine tumors. Data regarding the performance of [Ga]-DOTATATE for detecting ectopic ACTH-secreting tumors remain limited, in part owing to the recent introduction of this imaging modality in clinical practice, and in part because of the low prevalence of ECS. Nevertheless, [Ga]-DOTATATE has been reported to be useful in identifying primary and metastatic ectopic ACTH-secreting lesions that were not apparent on other imaging studies, impacting the clinical care of many patients with ECS.. [Ga]-DOTATATE-based imaging, which targets the somatostatin receptors abundantly expressed in neuroendocrine tumors, has generally high, although variable resolution in detecting the source(s) of ECS.

Topics: Cushing Syndrome; Gallium Radioisotopes; Humans; Multimodal Imaging; Neuroendocrine Tumors; Organometallic Compounds; Radionuclide Imaging; Receptors, Somatostatin

Adrenocorticotropic hormone-producing extraadrenal paragangliomas are extremely rare. We present a case of severe hypercortisolemia due to ectopic adrenocorticotropic hormone secretion by a nasal paraganglioma.. A 70-year-old Caucasian woman, was emergently admitted to our department with supraventricular tachycardia, oedema of face and extremities and hypertensive crisis. Initial laboratory evaluation revealed severe hypokalemia and hyperglycemia without ketoacidosis, although no diabetes mellitus was previously known. Computed tomography revealed a large tumor obliterating the left paranasal sinus and a left-sided adrenal mass. After cardiovascular stabilisation, a thorough hormonal assessment was performed revealing marked adrenocorticotropic hormone-dependent hypercortisolism. Due to the presence of a cardiac pacemaker magnetic resonance imaging of the hypophysis was not possible. [68Ga-DOTA]-TATE-Positron-Emission-Tomography was performed, showing somatostatin-receptor expression of the paranasal lesion but not of the adrenal lesion or the hypophysis. The paranasal tumor was resected and found to be an adrenocorticotropic hormone-producing paraganglioma of low-proliferative rate. Postoperatively the patient became normokaliaemic, normoglycemic and normotensive without further need for medication. Genetic testing showed no mutation of the succinatdehydrogenase subunit B- and D genes, thus excluding hereditary paragangliosis.. Detection of the adrenocorticotropic hormone source in Cushing's syndrome can prove extremely challenging, especially when commonly used imaging modalities are unavailable or inconclusive. The present case was further complicated by the simultaneous detection of two tumorous lesions of initially unclear biochemical behaviour. In such cases, novel diagnostic tools - such as somatostatin-receptor imaging - can prove useful in localising hormonally active neuroendocrine tissue. The clinical aspects of the case are discussed and relevant literature is reviewed.

Topics: ACTH Syndrome, Ectopic; Adrenocorticotropic Hormone; Aged; Biomarkers; Cushing Syndrome; Female; Humans; Hydrocortisone; Nose Neoplasms; Organometallic Compounds; Paraganglioma, Extra-Adrenal; Positron-Emission Tomography; Radiopharmaceuticals; Severity of Illness Index; Tomography, X-Ray Computed; Treatment Outcome

Endocrine tumors comprise a range of benign and malignant conditions that produce a spectrum of clinical symptoms and signs depending on the specific hormones they produce. The symptoms and presentations of these tumors are often independent of their size and location. Because of their expression of cell membrane receptors or production of specific types of hormones or peptides, endocrine tumors can be identified with functional radionuclide imaging much more readily compared to standard cross-sectional imaging. In recent years, 18F-fluoro-deoxy- D-glucose positron emission tomography (18F-FDG-PET) has emerged as a useful tool for diagnosing and assessing many tumors. In this review we describe how PET, using 18F-FDG and other radiopharmaceuticals can be useful in the diagnosis and management of a wide range of endocrine tumors.

Topics: Adrenal Gland Neoplasms; Adrenal Medulla; Carcinoma, Neuroendocrine; Catecholamines; Cushing Syndrome; Endocrine Gland Neoplasms; Fluorodeoxyglucose F18; Humans; Organometallic Compounds; Parathyroid Neoplasms; Pituitary Neoplasms; Positron-Emission Tomography; Radiopharmaceuticals; Receptors, Somatostatin; Thyroid Neoplasms; Tomography, X-Ray Computed

Adrenocorticotropic hormone-independent Cushing syndrome due to ectopic adrenocortical adenoma is a very rare entity. We herein present a case of a 57-year-old woman who was referred to our hospital with persistent Cushing syndrome after undergoing unnecessary laparoscopic left adrenalectomy. 68Ga-DOTATATE PET/CT revealed increased uptake in the nodule in the right hilum, which was histologically confirmed to be ectopic adrenocortical adenoma. Removal of the tumor was followed by the disappearance of clinical symptoms of Cushing syndrome. In this case, 68Ga-DOTATATE PET/CT contributed to the diagnosis of ectopic adrenocortical adenoma.

Topics: Adrenocortical Adenoma; Cushing Syndrome; Female; Humans; Middle Aged; Positron Emission Tomography Computed Tomography

Topics: Cushing Syndrome; Fluorodeoxyglucose F18; Humans; Pituitary Neoplasms; Positron Emission Tomography Computed Tomography; Positron-Emission Tomography

Approximately 5% to 15% cases of endogenous Cushing syndrome are due to ectopic adrenocorticotrophic hormone (ACTH)-producing neuroendocrine tumors, which are commonly located in bronchopulmonary system, thymus, and gastrointestinal tract including pancreas. Although carcinoid tumors of the appendix are revealed in 0.3% of patients undergoing routine appendectomy, ACTH-secreting appendicular carcinoid is a rare entity. We present a case of a 22-year-old woman with ectopic ACTH-dependent Cushing syndrome due to an appendicular carcinoid, which was localized with the help of Ga-DOTATATE PET/CT scan.

Topics: Appendiceal Neoplasms; Cushing Syndrome; Female; Humans; Organometallic Compounds; Positron Emission Tomography Computed Tomography; Young Adult

Ectopic adrenocorticotropic hormone (ACTH) secretion is a less common cause of Cushing syndrome and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care center in Southern India.. The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. The clinical features, clinical history, biochemical values, imaging features, including radiologic findings and positron emission tomography scans, management, details of follow-up, and outcomes, were documented. We compared the biochemical findings in these patients with 20 consecutive patients with Cushing disease (Cushing syndrome of pituitary origin).. A total of 21 patients were studied. The median age at presentation was 34 years (range, 19 to 55 years). Seven patients had thymic carcinoid, 7 had bronchial carcinoid, 3 had lung malignancies, 2 had medullary carcinoma thyroid, 1 patient had a pancreatic neuroendocrine tumor, and 1 patient had an occult source of ACTH. The most common clinical features at presentation were muscle weakness (95%), hyperpigmentation (90%), facial puffiness (76%), easy bruising (61%), edema (57%), and striae (52%). Extensive acne was seen in a large number of patients (43%). Only 3 patients (14%) had central obesity. The median 8 am cortisol was 55.5 μg/dL (range, 3.8 to 131 μg/dL), median 8 am ACTH was 207 pg/mL (range, 31.1 to 703 pg/mL), and the median 24-hour urinary free cortisol was 2,484 μg (range, 248 to 25,438 μg). Basal cortisol and ACTH, as well as midnight cortisol and ACTH level, were markedly higher in patients with ectopic Cushing syndrome as compared to patients with Cushing disease. Twelve of 21 patients had developed life-threatening infections by follow-up. Nine patients had undergone surgical intervention to address the primary tumor. However, only 1 patient exhibited a complete cure on follow-up.. In our series, ectopic Cushing syndrome was most commonly seen in association with intrathoracic tumors such as bronchial or thymic carcinoid. Hyperpigmentation and proximal myopathy were frequent, while central obesity was uncommon. Early and rapid control of hypercortisolemia was important in order to prevent life-threatening infections and metabolic complications.. ACTH = adrenocorticotropic hormone CT = computed tomography DOTATATE =

Topics: Acne Vulgaris; ACTH Syndrome, Ectopic; Adult; Bronchial Neoplasms; Carcinoid Tumor; Carcinoma, Neuroendocrine; Cushing Syndrome; Edema; Female; Humans; Hydrocortisone; Hyperpigmentation; India; Lung Neoplasms; Male; Middle Aged; Muscle Weakness; Neoplasms; Neuroendocrine Tumors; Obesity, Abdominal; Organometallic Compounds; Pancreatic Neoplasms; Positron-Emission Tomography; Radiopharmaceuticals; Retrospective Studies; Tertiary Care Centers; Thymus Neoplasms; Thyroid Neoplasms; Young Adult

Diagnosis of ectopic adrenocorticotropic hormone (ACTH) and corticotropin-releasing hormone (CRH) co-secreting tumors causing Cushing syndrome (CS) is challenging because these tumors are rare and their diagnosis is frequently confused with Cushing disease (CD), caused by the effect of CRH on the pituitary. We report a case of a 21-year-old male patient who was referred to our institution with persistent hypercortisolemia and CS after undergoing unnecessary transsphenoidal surgery (TSS). ⁶⁸Ga-DOTA-TATE PET/CT revealed increased tracer uptake in the thymus, which was histologically proven to be a neuroendocrine tumor (NET) that stained positive for ACTH and CRH. Imaging with ¹⁸F-FDG PET/CT was not diagnostic.

Topics: Adrenocorticotropic Hormone; Corticotropin-Releasing Hormone; Cushing Syndrome; Fluorodeoxyglucose F18; Humans; Male; Multimodal Imaging; Neuroendocrine Tumors; Organometallic Compounds; Positron-Emission Tomography; Radiopharmaceuticals; Thymus Neoplasms; Tomography, X-Ray Computed; Young Adult