Cushing's syndrome due to adrenocorticotropic hormone-secreting metastatic neuroendocrine tumor of unknown primary origin: a case report and literature review.
Hormones (Athens)
; 21(1): 147-154, 2022 Mar.
Article
in English
| MEDLINE | ID: covidwho-1392070
ABSTRACT
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 68Ga-SSTR PET/CT and 18FDG-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.Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
ACTH Syndrome, Ectopic
/
Neoplasms, Unknown Primary
/
Carcinoid Tumor
/
Neuroendocrine Tumors
/
Cushing Syndrome
/
Lung Neoplasms
Type of study:
Case report
/
Diagnostic study
/
Prognostic study
/
Reviews
Topics:
Long Covid
Limits:
Adult
/
Humans
/
Male
Language:
English
Journal:
Hormones (Athens)
Journal subject:
Endocrinology
Year:
2022
Document Type:
Article
Affiliation country:
S42000-021-00316-z
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