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      Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma

      case-report

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          Summary

          Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature.

          Learning points

          • Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies.

          • Most paraganglioma of the head and neck are not hormonally active.

          • Nasal paraganglioma, especially ACTH producing, is a very rare tumour.

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          Most cited references8

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          Hereditary paraganglioma targets diverse paraganglia.

          B Baysal (2002)
          Paragangliomas are highly vascularised and often heritable tumours derived from paraganglia, a diffuse neuroendocrine system dispersed from skull base to the pelvic floor. The carotid body, a small oxygen sensing organ located at the bifurcation of the carotid artery in the head and neck and the adrenal medulla in the abdomen, are the most common tumour sites. It now appears that mutations in SDHB, SDHC, and SDHD, which encode subunits of mitochondrial complex II (succinate dehydrogenase; succinate-ubiquinone oxidoreductase), are responsible for the majority of familial paragangliomas and also for a significant fraction of non-familial tumours. Germline mutations in complex II genes are associated with the development of paragangliomas in diverse anatomical locations, including phaeochromocytomas, a finding that has important implications for the clinical management of patients and genetic counselling of families. Consequently, patients with a paraganglioma tumour, including phaeochromocytoma, and a complex II germline mutation should be diagnosed with hereditary paraganglioma, regardless of family history, anatomical location, or multiplicity of tumours. This short review attempts to bring together relevant genetic data on paragangliomas with a particular emphasis on head and neck paragangliomas and phaeochromocytomas.
          Article 0 comments Cited 31 times – based on 0 reviews     Review now
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            Ectopic ACTH syndrome

            Andrea Isidori, Andrea Lenzi (2007)
            Ectopic adrenocorticotropic secretion (EAS) is responsible for 12-17% of cases of Cushing's syndrome (CS) and covers a range of tumours, from undetectable benign lesions to widespread metastases. The syndrome is often associated with severe hypercortisolaemia, which aggravates the underlying condition. EAS requires a complete workup that includes the establishment of endogenous CS, diagnosis of adrenocorticotropic hormone (ACTH) dependency, localization of the source of ACTH secretion and rapid biochemical control of hypercortisolaemia. Dynamic endocrine tests should include inferior petrosal sinus sampling with CRH stimulation. Localization studies depend on the availability of reliable high-resolution cross-sectional imaging. This systematic review of the largest published series of patients with EAS (over 380 patients) reveals the common trends in the prevalence and management of this syndrome. The concept of 'occult' EAS has been revisited and the terms 'overt' and 'covert' EAS introduced. In addition to small cell lung carcinoma, the most common causes of ectopic EAS are bronchial carcinoids, thymic tumours, islet cell tumour of the pancreas, medullary thyroid carcinomas, and phaeochromocytomas. Their prevalence and the best localization modalities are presented. Medical and surgical management is discussed on the basis of the extensive experience of major referral centres.
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              Paragangliomas: etiology, presentation, and management.

              Karen Joynt, Javid J Moslehi, Kenneth Baughman (2009)
              Paragangliomas are catecholamine-secreting tumors arising from the chromaffin cells of the sympathetic ganglia, and are known as extra-adrenal pheochromocytomas. These tumors commonly present with episodic hypertension, tachycardia, headache, and diaphoresis, and can be either benign or malignant. Diagnosis is made by serum and urine analysis for catecholamines and metanephrines, and confirmed with imaging studies including computed tomography scanning, magnetic resonance imaging, or 123-I metaiodobenzylguanidine imaging. Although the majority of paragangliomas are sporadic, a growing percentage of cases are found to be part of a familial genetic syndrome. Genetic testing should be offered to patients diagnosed with paraganglioma, particularly in patients who are young, have multiple tumors, or have a family history of malignancy. Management of paraganglioma is predicated on surgical resection, and careful perioperative management with alpha- and beta-adrenergic blockade is imperative for optimal outcomes. The majority of these tumors are benign, but for patients with malignant disease, chemotherapy, and radiation therapy may provide modest benefit. Long-term follow-up is essential, as paragangliomas can recur many years after initial diagnosis. Ongoing research into the genetic underpinnings of this tumor may allow for more targeted molecular therapies in the future.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Endocrinol Diabetes Metab Case Rep
                Journal ID (iso-abbrev): Endocrinol Diabetes Metab Case Rep
                Journal ID (pmc): edm
                Journal ID (publisher-id): EDM Case Reports
                Title: Endocrinology, Diabetes & Metabolism Case Reports
                Publisher: Bioscientifica Ltd (Bristol )
                ISSN (Electronic): 2052-0573
                Publication date (Electronic): 16 September 2013
                Publication date (Print): 2013
                Volume: 2013
                Electronic Location Identifier: 130038
                Affiliations
                [1]Department of Endocrinology Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental LisbonPortugal
                [1 ]Department of Endocrinology Hospital Central do Funchal FunchalPortugal
                [2 ]Department of Neurosurgery Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental LisbonPortugal
                [3 ]Department of Pathology Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental LisbonPortugal
                Author notes
                Correspondence should be addressed to F Serra Email: filipaalvesserra@ 123456hotmail.com
                Article
                Publisher ID: EDM130038
                DOI: 10.1530/EDM-13-0038
                PMC ID: 3922001
                PubMed ID: 24616770
                SO-VID: 35055d62-2ff6-4eab-a5b8-c0f75d269361
                Copyright © © 2013 The authors
                License:

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                Date received : 27 August 2013
                Date accepted : 30 August 2013
                Categories
                Subject: Unique/Unexpected Symptoms or Presentations of a Disease

                Data availability:

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