Cushing's syndrome due to adrenocorticotropic hormone-secreting metastatic neuroendocrine tumor of unknown primary origin: a case report and literature review.

Autor: Bostan H; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey. drhayribostan@gmail.com., Duger H; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey., Akhanli P; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey., Calapkulu M; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey., Turkmenoglu TT; Department of Pathology, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey., Erdol AK; Department of Chest Diseases, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey., Duru SA; Department of Chest Diseases, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey., Sencar ME; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey., Kizilgul M; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey., Ucan B; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey., Ozbek M; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey., Cakal E; Department of Endocrinology and Metabolism, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ziraat Mahallesi, Şht. Ömer Halisdemir Blv. No. 1, 6110, Ankara, Turkey.
Jazyk: angličtina
Zdroj: Hormones (Athens, Greece) [Hormones (Athens)] 2022 Mar; Vol. 21 (1), pp. 147-154. Date of Electronic Publication: 2021 Sep 03.
DOI: 10.1007/s42000-021-00316-z
Abstrakt: Background: In this article, we present a case of neuroendocrine neoplasm of unknown primary origin (UPO NEN), which is a rare cause of ectopic Cushing's syndrome (ECS) presenting numerous challenges, together with a literature review.
Case Report: A 43-year-old male patient presented with clinical features consistent with Cushing's syndrome (CS) and adrenocorticotropic hormone (ACTH)-dependent hypercortisolemia. Despite a suspicious lesion on pituitary MRI, the high-dose dexamethasone suppression test and bilateral inferior petrosal sinus sampling results were not compatible with Cushing's disease. Bilateral non-homogeneous opacities were observed in the thorax CT of the patient, who also had a history of COVID-19 infection, but no tumoral lesion was detected. When 68 Ga-SSTR PET/CT and 18 FDG-PET/CT were performed, multiple metastatic foci were detected in mediastinal and hilar lymph nodes and the axial skeleton. Paratracheal-subcarinal lymph nodes were excised mediastinoscopically, and the diagnosis of NEN was made. Histopathological findings indicated that the possible origin was an atypical pulmonary carcinoid with a low Ki-67 labeling index. After controlling hypercortisolemia, a regimen of somatostatin analogs and capecitabine plus temozolomide was decided upon as treatment by a multidisciplinary council.
Conclusion: This is a challenging case of UPO NEN presenting with ECS and confounding factors, such as previous infection and incidental lesions, during the diagnosis process. The case in question highlighted the fact that atypical pulmonary carcinoid with a low proliferation index may cause visible metastases even when radiologically undetectable.
(© 2021. Hellenic Endocrine Society.)
Databáze: MEDLINE
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