ObjectiveTo summarize the clinical manifestations, pathogenesis, diagnosis and treatment of multiple endocrine neoplasia type 1 (MEN-1) so as to improve the understanding of MEN-1.MethodThe clinical data of 1 case of MEN-1 with new mutated gene in the Yantai Yuhuangding Hospital of Medical College of Qingdao University (our hospital) were analyzed retrospectively.ResultsThe patient was a 73-year-old woman, who was admitted to our hospital because of “abdominal pain, diarrhea” for 4 d. After discussion by a multidisciplinary team, MRI of pancreatic, adrenal and pituitary, and endoscopy examinations were performed to further identify the cause of diarrhea. Meanwhile, MEN-1 gene was detected in the peripheral blood of the patient and her relatives, and the result showed that the proband and his daughter had a cytosine deletion at c.1401 in exon 10 of MEN-1 gene, which resulted in frame shift mutation of p.e468r fs, it was confirmed as MEN-1. After 5 d of conservative treatment of the disease, the patient’s family requested discharge and the patient died half a month after discharge.ConclusionsMEN-1 is a rare autosomal dominant inheritable disease, with diverse clinical manifestations and easy misdiagnosis. Therefore, it is necessary to be alert to abnormal indicators in the glands associated with MEN-1, so as to achieve early diagnosis and treatment.
Thymic neuroendocrine tumors (TNETs) are a series of rare diseases with aggressive biology and poor prognosis. Clinical manifestations of TNETs are atypical, and ectopic secretion of adrenocorticotropic hormone can be found in some cases, resulting in associated endocrine symptoms. Due to the low morbidity and strong heterogeneity, it’s difficult to diagnose, treat and obtain new treatment regimen. Early complete surgical resection is an effective treatment. For advanced cancer, clinical trials of new drugs are expected to improve the survival of patients.
ObjectiveTo summary the treatment of pancreatic neuroendocrine neoplasms (pNENs). MethodsArticles relevant to pNENs at home and abroad were collected and reviewed. ResultsBecause of rare incidence and non-specific clinical syndromes of pNENs, clinician had no enough cognition about it. For pNENs, surgery was still the preferred option, combining other treatments included chemotherapy, somatostatin analogue, α-interferon, molecular targeted therapy, and peptide receptor radionuclide therapy (PRRT). ConclusionSurgery is still considered as the preferred option for controlling the associated biochemical syndromes and curtailing the malignant progression of pNENs.
ObjectiveTo systematically review the efficacy and safety of CDK4/6 inhibitors combined with endocrine therapy for advanced breast cancer.MethodsPubMed, EMBase, The Cochrane Library, CNKI, WanFang Data and VIP databases were electronically searched to collect randomized controlled trials (RCTs) of CDK4/6 inhibitors combined with endocrine therapy for advanced breast cancer from inception to October 13th, 2017. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies, then, meta-analysis was performed by using RevMan 5.3 software.ResultsA total of 4 RCTs involving 2 524 patients were included. The results of meta-analysis showed that: compared with placebo combined with endocrine therapy, CDK4/6 inhibitors combined with endocrine therapy could improve the median progression free survival rate (RR=0.53, 95%CI 0.47 to 0.60, P<0.000 01) and the objective response rate (RR=1.67, 95%CI 1.47 to 1.91,P<0.000 01). While there was no statistical difference in clinical benefit rate (RR=0.59, 95%CI 0.75 to 1.19,P=0.64). In terms of adverse reactions, CDK4/6 inhibitors combined with endocrine therapy had higher rates of neutropenia (RR=49.76, 95%CI 26.85 to 90.21, P<0.000 01), leukopenia (RR=48.69, 95%CI 18.74 to 133.61,P<0.000 01), fatigue (RR=3.11, 95%CI 1.37 to 7.08,P=0.007) and anemia (RR=2.96, 95%CI 1.61 to 5.42, P=0.000 3). There were no significant differences between two groups in nausea, diarrhea and decreased appetite.ConclusionCDK4/6 inhibitors combined with endocrine therapy for the patients with advanced breast cancer can improve median progression free survival and objective response rate, while increase the incidence of adverse events such as neutropenia, leukopenia, fatigue and anemia. Due to limited quality and quantity of the included studies, more high quality studies are needed to verify above conclusion.
Objective To approach the recent advances in diagnosis and surgical treatment of pancreatic endocrine tumors （PETs）. Methods Articles relevant to diagnosis and treatment of PETs were collected and reviewed. Results PETs are characterized by their ability to over-produce peptides and hormones， which cause specific clinical syndromes. Because of rare incidence and complex clinical syndromes， there are still impediments to early diagnosis of these tumors. Monitoring of serum hormones and imaging method allow early tumor detection. PETs have been investigated for the past several decades. With the great knowledge of these tumors in molecular genetic level， clinical managements have been greatly changed. Conclusions Avoiding misdiagnosis is important for treatment of PETs. Surgical approach is still considered as the preferred option for curtailing the malignant progression of PETs and controlling the associated biochemical syndromes.
Objective To systematically review the efficacy and safety of CDK4/6 inhibitors in combination with endocrine therapy for HR+/HER2‒ breast cancer. Methods The PubMed, Cochrane Library, Web of Science, WanFang Data, CNKI, American Society of Clinical Oncology (ASCO), European Society of Medical Oncology (ESMO) and San Antonio Breast Cancer Symposium (SABCS) databases were electronically searched to collect randomized controlled trials on CDK4/6 inhibitors in combination with endocrine therapy for HR+/HER2‒ breast cancer from inception to July 5, 2023. Two reviewers independently screened the literature, extracted data, and assessed the risk of bias of the included studies. Meta-analysis was then performed using RevMan 5.4 software and Stata 14.0 software. Results A total of 8 studies involving 4 580 patients were included. The results of meta-analysis showed that overall survival and progression-free survival were significantly longer in the combination therapy group than those in the endocrine therapy alone group (HR=0.80, 95%CI 0.73 to 0.89, P<0.05; HR=0.54, 95%CI 0.50 to 0.59, P<0.05). The results also showed that patients in the combination therapy group also had significantly higher rates of objective remission and clinical benefit than those in the endocrine therapy group alone (RR=1.47, 95%CI 1.34 to 1.62, P<0.05; RR=1.20, 95%CI 1.11 to 1.30, P<0.05). In addition, the combination treatment group also increased the incidence of haematological toxicity such as neutropenia and leucopenia, but the differences in the incidence of nausea, diarrhoea and headache were not statistically significant between the two groups. Conclusion The combination of CDK4/6 inhibitors with endocrine therapy for HR+/HER2‒ breast cancer patients improve overall survival, progression-free survival, clinical benefit rate and objective remission rate, with significant long-term and near-term efficacy; however, this regimen increased the incidence of several adverse effects, and clinical use should be considered when considering the occurrence of serious adverse effects.