Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s Syndrome
BackgroundEctopic adrenocorticotropic syndrome (EAS) is a rare cause of endogenous ACTH-dependent Cushing’s syndrome, usually associated with severe hypercortisolism as well as comorbidities. Tumor detection is still a challenge and often requires several imaging procedures. In this report, we descr...
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Frontiers Media S.A.
2021-07-01
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| Series: | Frontiers in Endocrinology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fendo.2021.687539/full |
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| author | Andreea Liliana Serban Lorenzo Rosso Lorenzo Rosso Paolo Mendogni Arianna Cremaschi Arianna Cremaschi Rita Indirli Rita Indirli Beatrice Mantovani Beatrice Mantovani Mariagrazia Rumi Mariagrazia Rumi Massimo Castellani Arturo Chiti Arturo Chiti Giorgio Alberto Croci Giorgio Alberto Croci Giovanna Mantovani Giovanna Mantovani Mario Nosotti Mario Nosotti Emanuele Ferrante Maura Arosio Maura Arosio |
| author_facet | Andreea Liliana Serban Lorenzo Rosso Lorenzo Rosso Paolo Mendogni Arianna Cremaschi Arianna Cremaschi Rita Indirli Rita Indirli Beatrice Mantovani Beatrice Mantovani Mariagrazia Rumi Mariagrazia Rumi Massimo Castellani Arturo Chiti Arturo Chiti Giorgio Alberto Croci Giorgio Alberto Croci Giovanna Mantovani Giovanna Mantovani Mario Nosotti Mario Nosotti Emanuele Ferrante Maura Arosio Maura Arosio |
| author_sort | Andreea Liliana Serban |
| collection | DOAJ |
| description | BackgroundEctopic adrenocorticotropic syndrome (EAS) is a rare cause of endogenous ACTH-dependent Cushing’s syndrome, usually associated with severe hypercortisolism as well as comorbidities. Tumor detection is still a challenge and often requires several imaging procedures. In this report, we describe a case of an ectopic ACTH secretion with a misleading localization of the responsible tumor due to a concomitant rectal carcinoma.Case presentationA 49-year-old man was referred to our Endocrinology Unit due to suspicion of Cushing’s syndrome. His medical history included metastatic rectal adenocarcinoma, diagnosed 5 years ago and treated with adjuvant chemotherapy, radiotherapy and surgical resection. During follow-up, a thoracic computed tomography scan revealed two pulmonary nodules located in the superior and middle lobes of the right lung with a diameter of 5 and 10 mm, respectively. However, these nodules remained radiologically stable thereafter and were not considered relevant. All biochemical tests were suggestive of EAS (basal ACTH levels: 88.2 ng/L, nv 0–46; basal cortisol levels: 44.2 µg/dl, nv 4.8–19.5; negative response to CRH test and high dose dexamethasone suppression test) and radiological localization of the ectopic ACTH-secreting tumor was scheduled. The CT scan revealed a dimensional increase of the right superior lung nodule (from 5 to 12 mm). [68Ga]-DOTA-TOC PET/CT scan was negative, while [18F]-FDG-PET/CT showed a tracer accumulation in the superior nodule. After a multidisciplinary consultation, the patient underwent thoracic surgery that started with two atypical wedge resections of nodules. Frozen section analyses showed a neuroendocrine tumor on the right middle lobe nodule and a metastatic colorectal adenocarcinoma on the superior lesion. Then, a right superior nodulectomy and a right middle lobectomy with mediastinal lymphadenectomy were performed. The final histopathological examination confirmed a typical carcinoid tumor, strongly positive for ACTH. A post-surgical follow-up showed a persistent remission of Cushing’s syndrome.ConclusionsThe present report describes a case of severe hypercortisolism due to EAS not detected by functional imaging methods, in which the localization of ACTH ectopic origin was puzzled by a concomitant metastatic rectal carcinoma. The multidisciplinary approach was crucial for the management of this rare disease. |
| first_indexed | 2024-12-22T13:30:33Z |
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| language | English |
| last_indexed | 2024-12-22T13:30:33Z |
| publishDate | 2021-07-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Endocrinology |
| spelling | doaj.art-5e0f75d476f24b9c96e5a28ffcab31b92022-12-21T18:24:12ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922021-07-011210.3389/fendo.2021.687539687539Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s SyndromeAndreea Liliana Serban0Lorenzo Rosso1Lorenzo Rosso2Paolo Mendogni3Arianna Cremaschi4Arianna Cremaschi5Rita Indirli6Rita Indirli7Beatrice Mantovani8Beatrice Mantovani9Mariagrazia Rumi10Mariagrazia Rumi11Massimo Castellani12Arturo Chiti13Arturo Chiti14Giorgio Alberto Croci15Giorgio Alberto Croci16Giovanna Mantovani17Giovanna Mantovani18Mario Nosotti19Mario Nosotti20Emanuele Ferrante21Maura Arosio22Maura Arosio23Endocrinology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, ItalyThoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyDepartment of Pathophysiology and Transplantation, University of Milan, Milan, ItalyThoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyEndocrinology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, ItalyDepartment of Clinical Sciences and Community Health, University of Milan, Milan, ItalyEndocrinology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, ItalyDepartment of Clinical Sciences and Community Health, University of Milan, Milan, ItalyEndocrinology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, ItalyDepartment of Clinical Sciences and Community Health, University of Milan, Milan, ItalyDepartment of Clinical Sciences and Community Health, University of Milan, Milan, ItalyHepatology Unit, Ospedale San Giuseppe Multimedica Milan, Milan, ItalyDepartment of Nuclear Medicine, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Milan, ItalyDepartment of Biomedical Sciences, IRCCS Humanitas Research Hospital, Milan, ItalyDepartment of Pathophysiology and Transplantation, University of Milan, Milan, ItalyDivision of Pathology, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyEndocrinology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, ItalyDepartment of Clinical Sciences and Community Health, University of Milan, Milan, ItalyThoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyDepartment of Pathophysiology and Transplantation, University of Milan, Milan, ItalyEndocrinology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, ItalyEndocrinology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, ItalyDepartment of Clinical Sciences and Community Health, University of Milan, Milan, ItalyBackgroundEctopic adrenocorticotropic syndrome (EAS) is a rare cause of endogenous ACTH-dependent Cushing’s syndrome, usually associated with severe hypercortisolism as well as comorbidities. Tumor detection is still a challenge and often requires several imaging procedures. In this report, we describe a case of an ectopic ACTH secretion with a misleading localization of the responsible tumor due to a concomitant rectal carcinoma.Case presentationA 49-year-old man was referred to our Endocrinology Unit due to suspicion of Cushing’s syndrome. His medical history included metastatic rectal adenocarcinoma, diagnosed 5 years ago and treated with adjuvant chemotherapy, radiotherapy and surgical resection. During follow-up, a thoracic computed tomography scan revealed two pulmonary nodules located in the superior and middle lobes of the right lung with a diameter of 5 and 10 mm, respectively. However, these nodules remained radiologically stable thereafter and were not considered relevant. All biochemical tests were suggestive of EAS (basal ACTH levels: 88.2 ng/L, nv 0–46; basal cortisol levels: 44.2 µg/dl, nv 4.8–19.5; negative response to CRH test and high dose dexamethasone suppression test) and radiological localization of the ectopic ACTH-secreting tumor was scheduled. The CT scan revealed a dimensional increase of the right superior lung nodule (from 5 to 12 mm). [68Ga]-DOTA-TOC PET/CT scan was negative, while [18F]-FDG-PET/CT showed a tracer accumulation in the superior nodule. After a multidisciplinary consultation, the patient underwent thoracic surgery that started with two atypical wedge resections of nodules. Frozen section analyses showed a neuroendocrine tumor on the right middle lobe nodule and a metastatic colorectal adenocarcinoma on the superior lesion. Then, a right superior nodulectomy and a right middle lobectomy with mediastinal lymphadenectomy were performed. The final histopathological examination confirmed a typical carcinoid tumor, strongly positive for ACTH. A post-surgical follow-up showed a persistent remission of Cushing’s syndrome.ConclusionsThe present report describes a case of severe hypercortisolism due to EAS not detected by functional imaging methods, in which the localization of ACTH ectopic origin was puzzled by a concomitant metastatic rectal carcinoma. The multidisciplinary approach was crucial for the management of this rare disease.https://www.frontiersin.org/articles/10.3389/fendo.2021.687539/fullCushing’s syndromeectopic ACTH syndromepulmonary carcinoidrectal carcinomahypercortisolism |
| spellingShingle | Andreea Liliana Serban Lorenzo Rosso Lorenzo Rosso Paolo Mendogni Arianna Cremaschi Arianna Cremaschi Rita Indirli Rita Indirli Beatrice Mantovani Beatrice Mantovani Mariagrazia Rumi Mariagrazia Rumi Massimo Castellani Arturo Chiti Arturo Chiti Giorgio Alberto Croci Giorgio Alberto Croci Giovanna Mantovani Giovanna Mantovani Mario Nosotti Mario Nosotti Emanuele Ferrante Maura Arosio Maura Arosio Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s Syndrome Frontiers in Endocrinology Cushing’s syndrome ectopic ACTH syndrome pulmonary carcinoid rectal carcinoma hypercortisolism |
| title | Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s Syndrome |
| title_full | Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s Syndrome |
| title_fullStr | Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s Syndrome |
| title_full_unstemmed | Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s Syndrome |
| title_short | Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s Syndrome |
| title_sort | case report a challenging localization of a pulmonary ectopic acth secreting tumor in a patient with severe cushing s syndrome |
| topic | Cushing’s syndrome ectopic ACTH syndrome pulmonary carcinoid rectal carcinoma hypercortisolism |
| url | https://www.frontiersin.org/articles/10.3389/fendo.2021.687539/full |
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