OBJECTIVES: Paraneoplastic Cushing syndrome is a rare condition, representing a small fraction of the adrenocorticotropic hormone (ACTH)-dependent cases of Cushing syndrome Methods: Four case descriptions and literature review, highlighting the diagnostic challenges and treatment options are presented. RESULTS: Different tumor types can be associated with ectopic ACTH secretion. The most common types are bronchial carcinoids and small cell lung carcinoma (SCLC). However, in approximately 10 to 20% of the cases, no overt tumor (occult tumor) can be found. The diagnosis is made in a multistep process. Firstly, hypercortisolemia and adrenocorticotropin hormone dependency have to be confirmed. Distinction between a pituitary or ectopic cause can be cumbersome. MRI of the pituitary gland, a corticotropin releasing hormone stimulation test and a sinus petrosus sampling can be used. Treatment options consist of tumor management, somatostatin analogs, steroidogenesis inhibitors, and bilateral adrenalectomy. CONCLUSION: Clinicians should consider the diagnosis, and opt for specific treatment, especially in patients with a history of neuroendocrine tumors.
OBJECTIVES:Paraneoplastic Cushing syndrome is a rare condition, representing a small fraction of the adrenocorticotropic hormone (ACTH)-dependent cases of Cushing syndrome Methods: Four case descriptions and literature review, highlighting the diagnostic challenges and treatment options are presented. RESULTS: Different tumor types can be associated with ectopic ACTH secretion. The most common types are bronchial carcinoids and small cell lung carcinoma (SCLC). However, in approximately 10 to 20% of the cases, no overt tumor (occult tumor) can be found. The diagnosis is made in a multistep process. Firstly, hypercortisolemia and adrenocorticotropin hormone dependency have to be confirmed. Distinction between a pituitary or ectopic cause can be cumbersome. MRI of the pituitary gland, a corticotropin releasing hormone stimulation test and a sinus petrosus sampling can be used. Treatment options consist of tumor management, somatostatin analogs, steroidogenesis inhibitors, and bilateral adrenalectomy. CONCLUSION: Clinicians should consider the diagnosis, and opt for specific treatment, especially in patients with a history of neuroendocrine tumors.
Authors: Sofia Castro Oliveira; João Sérgio Neves; Pedro Souteiro; Sandra Belo; Ana Isabel Oliveira; Helena Moreira; Paulo Mergulhão Gomes; Lígia Coelho; Cristina Sarmento; Elsa Fonseca; Celestino Neves; Paula Freitas; Davide Carvalho Journal: Case Rep Endocrinol Date: 2019-10-15
Authors: Valentina Di Ruscio; Giada Del Baldo; Maria Debora De Pasquale; Rita De Vito; Evelina Miele; Giovanna Stefania Colafati; Annalisa Deodati; Maria Antonietta De Ioris; Assunta Tornesello; Giuseppe Maria Milano; Angela Mastronuzzi Journal: Front Oncol Date: 2020-04-28 Impact factor: 6.244