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Objective: POEMS syndrome is a rare multisystemic disease, with a wide spectrum of clinical endocrinopathies. Patients with POEMS syndrome may present with one or more hormone disorders during the disease course, but such phenomenon has usually been underestimated. In this report, we analyzed the prevalence and clinical characteristics of endocrine abnormalities in a large Chinese cohort with POEMS syndrome., Methods: This retrospective review was performed in patients with a definite diagnosis of POEMS syndrome who were hospitalized in our hospital between January 2000 and January 2020. The clinical data about endocrine abnormalities were extracted from their medical records and analyzed., Results: This study comprised 136 patients (95 male, 41 female) with a median age of 48(40-56) years old. Endocrine abnormalities were frequent (127 cases, 93.38%) in patients with POEMS syndrome. The prevalence of single endocrinopathy and multiple endocrinopathies were 12.60% (16/127 cases) and 87.40% (111/127), respectively. The most frequent endocrinopathy was hypogonadism (98/136, 72.06%), followed by hypothyroidism (83/136, 61.03%), hypocalcemia (50/136, 36.76%), hyperprolactinemia (47/136, 34.56%), abnormal glucose metabolism (41/136, 30.15%) and adrenal insufficiency (41/136, 30.15%). In patients with multiple endocrinopathies, the prevalence of 2, 3, 4, 5, and 6 kinds of endocrine axes involved were 29.92% (38/127), 30.71% (39/127), 17.32% (22/127), 7.09% (9/127) and 2.36% (3/127), respectively. Such hormone disorders cause complex clinical presentations, including overt or subclinical situations., Conclusion: Endocrinopathy manifestations in POEMS syndrome are more frequent, and its clinical complicacy should be emphasized in differential diagnosis. For patients with a definite diagnosis of POEMS syndrome, an early and thorough endocrine evaluation should be performed.

Introduction: The cortisol/ACTH ratio was supposed to be helpful in the screening of subclinical hypercortisolism (SHC) in the published literatures. This study assessed the cortisol/ACTH ratio in patients with adrenal incidentaloma (AI) confirmed to have SHC and investigated its role for screening SCS in patients with AI., Methods: This descriptive retrospective study included 183 patients with AIs [45 with SHC and 138 with non-functional adenoma (NFA)]. Cortisol and ACTH levels were measured at 8:00, 16:00, and 0:00, and the cortisol/ACTH ratio was calculated. ROC curve was used to evaluate efficacy of the cortisol/ACTH ratio, explore the best cut-off value and its corresponding sensitivity and specificity., Results: The cortisol/ACTH ratios at all time points were significantly higher in the SHC group than the NFA group (P<0.05) and were significantly correlated with serum cortisol levels after the 1-mg dexamethasone suppression test (DST). Area Under the Curve (AUC) of the cortisol/ACTH ratio at 0:00 and midnight serum cortisol levels were 0.893 (0.843-0.943) and 0.831 (0.765-0.806), respectively. A cortisol/ACTH ratio at 0:00 cut-off of 32.18 nM/pM showed a sensitivity of 100% and specificity of 39.1%. The optimal cut-off was 68.83 nM/pM (sensitivity 86.7%, specificity 75.4%)., Conclusions: Patients with SHC have a higher cortisol/ACTH ratio than those with NFAs. The cortisol/ACTH ratio is significantly correlated with serum cortisol after the 1-mg DST. The diagnostic performance of the cortisol/ACTH ratio at 0:00 is superior to midnight serum cortisol. Therefore, the cortisol/ACTH ratio at 0:00 may be a reliable parameter for SHC screening in patient with AI., Competing Interests: The authors have no conflict of interest., (© Georg Thieme Verlag KG Stuttgart · New York.)

In the present study, the case of a female patient with pseudo-hermaphrodism caused by an androgen-producing adrenocortical tumor is presented, and the possible mechanism is investigated. The expression of the luteinizing hormone/human chorionic gonadotrophin (LH/hCG) receptor in tumor tissues and normal adrenal tissues was analyzed using immunohistochemistry. Furthermore, the activities of 3β-hydroxysteroid dehydrogenase 2 (HSD2), cytochrome P450 17α-hydroxylase (CYP17) and 17β-hydroxysteroid dehydrogenase 3 (HSD3) enzymes were measured using enzyme-linked immunosorbent assay, and the expression levels of 3β-HSD2, 17β-HSD3, CYP17 and LH/hCG receptor mRNA were determined by quantitative polymerase chain reaction (qPCR). Immunohistochemical staining for the LH/hCG receptor was negative in the tumor tissue and positive in the normal adrenal tissue. The activities of 3β-HSD2 and CYP17 in the tumor tissue were higher than those in the normal tissue (P<0.01), whereas the activity of 17β-HSD3 was lower (P<0.01). The mRNA levels of 3β-HSD2 and CYP17 were higher (P<0.01) and the levels of 17β-HSD3 and LH/hCG receptor were lower (P<0.01) in the tumor tissue compared with those of the normal tissue. In conclusion, in the present study, a rare case of virilization by an androgen-producing adrenocortical tumor is present. The results indicate that it may be associated with increased activities of 3β-HSD2 and CYP17 but not with the expression of the LH/hCG receptor.

Vascular endothelial growth factor (VEGF) plays an important role in many disease states, including ischemia, chronic and acute inflammation, and pathologies associated with angiogenesis such as tumors and wounds. A number of factors regulate VEGF promoter activity and VEGF expression such as four and a half LIM domains 1 (FHL1) and Smad4. FHL1 belongs to a family of LIM-only proteins that regulate gene transcription, cell proliferation, differentiation and apoptosis. Smad4 is a tumor suppressor gene, initially identified as deleted in pancreatic carcinoma locus 4 (DPC4). The aim of this study was to determine whether FHL1 and Smad4 inhibited VEGF signaling. HepG2 cells were transfected with the VEGF-Luc reporter, Smad4 and FHL1 or Smad4 and FHL1 siRNA. Results showed that the overexpression of FHL1 and Smad4 synergistically inhibited the promoter activity, mRNA expression and secretion of VEGF, whereas knockdown of endogenous Smad4 and FHL1 had opposite effects. Moreover, the reduction of endogenous Smad4 eliminated FHL1-mediated inhibition of the VEGF promoter activity. In conclusion, a cooperative regulation of VEGF signaling by FHL1 and Smad4 was evidenced, which may provide a novel regulation mechanism underlying cancer development and progression.