Diagnosis and Treatment > Medication
Lebanese University, Hadath, Lebanon
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Lebanese University, Hadath, Lebanon
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Mount Lebanon Hospital, Beirut, Lebanon
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Mount Lebanon Hospital, Beirut, Lebanon
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Mount Lebanon Hospital, Beirut, Lebanon
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Summary
The objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome. The latter is present in two out of three cases and in the remaining one, primary tumor could not be localized, representing a small fraction of patients with paraneoplastic Cushing. Diagnosis is established in the setting of high clinical suspicion by documenting an elevated cortisol level, ACTH and doing dexamethasone suppression test. Treatment options include management of the primary tumor by surgery and chemotherapy and treating Cushing syndrome. Prognosis is poor in SCLC. We concluded that in front of a high clinical suspicion, ectopic Cushing syndrome diagnosis should be considered, and identification of the primary tumor is essential.
Learning points:
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Learning how to suspect ectopic Cushing syndrome and confirm it among all the causes of excess cortisol.
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Distinguish between occult and severe ectopic Cushing syndrome and etiology.
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Providing the adequate treatment of the primary tumor as well as for the cortisol excess.
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Prognosis depends on the differentiation and type of the primary malignancy.
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Summary
Insulinoma is a rare form of insulin-secreting pancreatic islet cell neuroendocrine (NE) tumor. The medical treatment of the malignant NE disease of the pancreas deeply changed in the last years, thanks to the introduction of new target molecules, as everolimus. Even if the exact mechanism is not actually known, one of the side effects of everolimus, hyperglycemia, has been demonstrated to be useful to contrast the typical hypoglycemia of the insulinoma. We report the case of a patient with a metastatic malignant insulinoma treated with intermittent everolimus, obtaining an important improvement in the quality of life; this suggests the necessity of preclinical studies to analyze the cellular pathways involved in insulin-independent gluconeogenesis.
Learning points
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Effect of somatostatin analogs is long-lasting in the control of functioning NE tumors.
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Persistent everolimus control of hypoglycemia despite serum insulin levels and disease progression.
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Open issue: are disease progression and the increase in serum markers the only valid criteria to reject a treatment?
