Purpose:To explore the clinical and pathological characters and immunophenotype of 2 cases nasal type extranodal NK/T cell lymphoma primarily derived from adrenal gland.Methods:The clinical and pathological features of the 2 cases were analyzed and the immunostaining for UCHL-1,CD43,CD3,CD56,TIA-1 and LMP1 was performed using EnVision TM . IgH and TCR? gene rearrangement was analyzed by using PCR techniques.Results:Both patients were male and aged 50 and 51. Occupying masses in their right adrenal glands was detected. The two tumors showed similar morphological features, composing of small to middle sized and multi formed cells and growing invasive. Inflammatory background,necrosis and vessel involvement were seen. Positive surface staining of UCHL-1,CD43,CD56 and cytoplasma staining of CD3 was demonstrated. TIA-1 and LMP1 expression was also detected. No clonal rearrangement in both IgH and TCR? genes was detected.Conclusions:Nasal type extranodal NK/T cell lymphoma can occur primarily in adrenal gland. It should be distinguished from other small cell malignant tumor of this site.

BACKGROUNDHemoptysis is a significant clinical entity with high morbidity and potential mortality. Both medical management (in terms of resuscitation and bronchoscopic interventions) and surgery have severe limitations in these patients population. Bronchial artery embolization (BAE) represents the first-line treatment for hemoptysis. This article discusses clinical analysis, embolization approach, outcomes and complications of BAE for the treatment of hemoptysis.

METHODSA retrospective analysis of 344 cases, who underwent bronchial arteriography at Tianjin Haihe Hospital between 2006 and 2013. Several aspects of outcome were analyzed: Demographics, clinical presentation, radiographic studies, results, complications and follow-up of BAE.

RESULTSThree hundred and forty-four consecutive patients underwent bronchial arteriography, 336 of 344 patients (97.7%) performed BAE; there were 1530 coils for 920 arteries embolized; the main responsible sources for bleeding were right bronchial artery (29.7%), left bronchial artery (21.6%), combined right and left bronchial trunk (18.4%), right intercostal arteries (13.3%); 61 patients (17.7%) had recurrent hemoptysis within 1 month after undergoing BAE, 74 patients (21.5%) had recurrent hemoptysis over 1 month after undergoing BAE; The common complications of BAE included subintimal dissection, arterial perforation by a guide wire, fever, chest pain, dyspnea, etc. The follow-up was completed in 248 patients, 28 patients had been dead, 21 patients still bleed, 92 patients had lost to follow-up.

CONCLUSIONSThe technique of BAE is a relatively safe and effective method for controlling hemoptysis . The complications of BAE are rare. Although the long-term outcome in some patients is not good, BAE may be the only life-saving treatment option in patients who are poor surgical candidates.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Bronchial Arteries ; Embolization, Therapeutic ; methods ; Female ; Hemoptysis ; therapy ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

Objective:Charcot-Marie-Tooth disease (CMT) comprises a group of clinically and genetically heterogeneous inherited neuropathies with an estimated prevalence of 1 in 2500. This study aimed to analyze the clinical and mutational characteristics of Chinese CMT patients with MFN2, BSCL2 and LRSAM1 variants.Methods:In this study, genetic analysis was performed in 206 Chinese patients at Chinese PLA General Hospital from December 2012 to March 2020 with clinical diagnosis of CMT, and reported variants of MFN2, BSCL2 and LRSAM1 related to CMT2.Results:We reported ten MFN2 mutations in ten unrelated patients (7 male, 3 female), two of whom had positive family history. Three novel mutations were detected including c.475-2A>G (splicing); c.687dupA (p.E230Rfs*16) and c.558dupT (p.S186fs). We reported three BSCL2 mutations of four unrelated patients, including c.461C>G (p.S154W), c.461C>T(p.S154L), and novel variants of c.1309G>C (p.A437P) and c.845C>T (p.A282V). Furthermore, two novel variants of LRSAM1, including c.1930G>T (p.G644C) and c.1178T>A (p.L393Q) were detected in two unrelated patients.Conclusion:Mutational spectrum of MFN2-, BSCL2-and LRSAM1-related CMT disease is expanded with the identification of novel variants in Chinese patients.

Objective: To investigate androgen receptor(AR)expression in invasive breast carcinoma and the correlation with surrogate molecular breast carcinoma subtypes. Methods: Immunohistochemical staining of AR and other biomarkers was performed in a cohort of 870 cases of primary invasive breast carcinomas collected from August to December, 2016. The association of AR expression with different histological and surrogate molecular subtypes was analyzed. Results: The positive expression rate of AR in the immunohistochemistry-based surrogate subtypes was 96.3%(207/215) for Luminal A, 89.8%(378/421) for Luminal B, 82.4%(75/91) for HER2 overexpression and 37.1%(53/143) for triple negative breast carcinoma, with significant differences among the four groups (P<0.01). AR correlated positively with the expression of ER(P<0.01), PR(P<0.01), HER2(P=0.007), GATA3(P<0.01), GCDFP15(P<0.01)and mammaglobin(P<0.01), while negatively with the expression of Ki-67(P<0.01), CK5/6(P<0.01)and CK14(P<0.01). Conclusions AR exhibits a high expression in invasive breast carcinoma, which is mainly correlated with ER-positive breast carcinoma. Regardless of the relatively low expression rate, AR is a potential therapeutic target in triple negative breast carcinoma.

Objective:To investigate the clinical and electrophysiological characteristics of ANCA-associated vasculitic neuropathy (VN) and analyze the predictors of treatment outcomes.Methods:Retrospective case series. In all, 652 consecutive patients with ANCA-associated vasculitis were admitted to the First Medical Center of the Chinese PLA General Hospital between January 2006 and December 2022. Peripheral neuropathy occurred in 91 patients. Patients were excluded if other known causes of neuropathy were present. Sixty-one patients were eventually enrolled, including 17 with eosinophilic granulomatosis with polyangiitis (EGPA), 11 with granulomatosis polyangiitis (GPA), and 33 with microscopic polyangiitis (MPA). Their clinical data were collected and clinical characteristics, VN manifestations, electrophysiological findings (including interside amplitude ratio [IAR]), and treatment outcomes were compared among the three subsets of AAV. Then, factors influencing the treatment outcomes were analyzed using multivariable logistic regression analysis.Results:Peripheral neuropathy occurred in 62.1%(18/29) of EGPA, 8.3%(15/180) of GPA, and 13.1%(58/443) of MPA patients. The age at onset and examination was higher in patients with MPA than those with EGPA or GPA ( P<0.01). The occurrence of VN was later in patients with GPA than those with EGPA ( P<0.01), and the GPA group had fewer affected nerves than the other two groups ( P<0.016). The abnormal IARs of motor nerves in lower limbs were more detected in the EGPA than the MPA group ( P<0.01). Logistic regression analysis suggested that higher Birmingham vasculitis activity score-version 3 (BVAS-V3) ( OR=6.85, 95% CI 1.33-35.30) was associated with better treatment outcomes of VN. However, central nervous system involvement was a risk factor for poor treatment outcomes ( OR=0.13, 95% CI 0.02-0.89). Conclusions:The clinical and electrophysiological characteristics of VN were slightly different among subsets of AAV. Patients with GPA often presented with polyneuropathy and had fewer nerves affected; mononeuritis multiplex was more common in EGPA than GPA and MPA. Higher BVAS-V3 and central nervous system involvement might predict the treatment outcome of VN.