Objective To explore the clinicopathologic features,diagnosis,treatment and prognosis of ACTH-independent subclinical Cushing syndrome caused by cortisol-secreting and hypo-androgen black adrenal cortical adenomas with lipomatous metaplasis.Methods The clinical and pathologic features of 1 patient presented with hypo-androgen and atypical signs and symptoms of Cushing syndrome were reported.The patient's 24 h UFC (235.62 μg) was higher than normal range,his plasma ACTH concentrations (＜1.1 pmol/L,was lower than normal range and his serum cortisol concentrations lacked diurnal rhythm.There was absence of serum cortisol suppression in overnight dexamethasone suppression tests.Serum levels of androgens (1.2 nmol/L) were lower than normal range.24 h urine catecholamine was normal,NE was 160.5 nmol/24 h (5-591 nmol/24 h),E was 23.1 nmol/24 h (0-82 nmol/24 h),DA was 1496nmol/24 h (424-2612 nmol/24 h).Aldosterone lying and standing test:ALD1 138-415 pmol/L,AT-Ⅱ1 64.54 ng/L,PRA1 0.80 μg · L-1 · h-1 Abdomen CT showed right adrenal mass (3.1 cm).131Ⅰ-MIBG scanning was normal.Adrenocortical carcinoma was considered as a possible diagnosis based on cortisol hypersecretion,hypo-androgen and the imaging characteristics.This patient accepted laparoscopic adrenal adenoma resection.Results The adrenal tumor size was 3.5 cm.Immunohistochemical (IHC) staining showed Melan-A,CgA,α-inhibin and Syn positive and Ki-67 ＜ 1％.Histopathologic results showed black adrenal cortical adenomas with myelolipomatous change.After surgery,patient's serum androgen was normal,and the signs and symptoms of Cushing syndrome and oligo-androgen disappeared.There was recurrence after 1.5 years follow-up.Conclusions Although most patients with corticotropin-independent Cushing syndrome associated with abnormal androgen proved to have adrenocortical carcinoma,the clinician should be aware of the possibility of benign,black adrenal adenoma.

Objective To evaluate the safety and feasibility of transurethral resection of paragangliomas in urinary bladder.Methods Clinical data of 11 patients (5 males and 6 females) with paragangliomas in urinary bladder who underwent transurethral resection in Peking Union Medical College Hospital from June 2008 to February 2014 were analyzed retrospectively.The age ranged from 30 to 76 years (mean 54± 14 years).All cases were diagnosed as single primary tumor.The preoperative CT or MRI showed the tumors located in bladder wall.The diameter of tumors ranged from 0.9-3.0 cm (mean 1.9±0.8).Nine cases presented with hypertension after micturition and 2 cases presented with sustained hypertension.The tumors were localized by B ultrasound,enhanced CT or MRI.The diagnosis was confirmed by detection of 24 hours urinary catecholamine,Octreotide scanning or 131I-MIBG scanning.Transurethral resection of tumors was performed after pharmachological preperation.Eight cases underwent conventional transurethral electroresection and 3 cases underwent transurethral resection with 2 μm thulium laser.All cases were followed up every 3 to 6 months and 24 hours urinary catecholamine and CT scan were performed.Results All tumors were successfully resected without open conversion.The operative time was 20 to 45 min (mean 34±8 min).The estimated blood loss ranged from 10 to 100 ml (mean 27±26 ml).Fluctuation of blood pressure during operation occurred in 9 cases.The maximum systolic blood pressure during operation rised to 220 mmHg (1 mmHg =0.133 kPa) and sodium Nitroprusside was administed to control blood pressure.Blood pressure of all cases returned to normal after operation.No perioperative complications occurred.No recurrence was found during follow-up (3-58 months,mean 26 months).Conclusions For small localized paraganglioma (the diameter less than 3 cm) in urinary bladder,transurethral resection could be a safe and feasible choice of treatment.And 2 μm thulium laser resection has an advantage over conventional electroresection for tumors located in lateral bladder wall to avoid obturator nerve reflex.

Objective: To review and discuss the experience of diagnosis and treatment of adrenal ganglioneuroma. Methods: Clinical data of 80 cases of adrenal ganglioneuroma undergoing surgery during January 1982 to May 2017 at Peking Union Medical College Hospital were retrospectively analyzed. There were 36 male and 44 female patients. Age ranged from 8 to 69 years old (mean 37.7 years old). The tumor diameter were 1.5 to 18.0 cm (mean 4.3 cm). There were 61 cases of adrenal ganglioneuroma diagnosed by imaging examination. All patients underwent adrenalectomy. Results: All procedures were performed successfully without serious complication. Including 15 open surgery and 64 retroperitoneal laparoscopic surgery. One laparoscopic case conversion to open surgery. The pathological diagnosis was adrenal ganglioneuroma. Fifteen cases were mixed with other tumor components. No recurrence or metastasis was observed during the follow-up of 2 months to 35 years. Conclusions The diagnostic techniques for adrenal ganglioneuroma are helpful to judge the tumor property before operation. The laparoscopic surgery has become the current method for resecting tumors. It shows advantages of small trauma and speedy recovery. The relationship between tumor and surrounding tissues should be understand clearly before operation. Full preparation must be made before operation if adrenal ganglioneuroma mixed with other tumor components.

Myelodysplastic syndrome(MDS)is a malignant clonal disease with high heterogeneity in cytogenetics and molecular genetics,and the risk of progression to acute myeloid leukemia(AML)is extremely high.Transforming growth factor-β(TGF-β)is closely related to the pathogenesis of MDS.It is a key executor of immune homeostasis and immune tolerance,and can inhibit the expansion and function of many components of immune system.TGF-β signaling pathway can regulate hematopoietic cells and immune cells in microenvironment,inhibit their normal biological functions,and thus accelerate the progression of MDS.This study reviews regulatory effects of TGF-β signaling pathway on MDS blood cells,T lymphocytes,natural killer cells and macrophages in recent years,and provides a new perspective for the pathogenesis and treatment of MDS.

Skull deformity is common ailment of infants,and maybe cause developmental deformity,with high incidence in China.In this article,we reviewed the related factors,prevention and treatment for skull deformity,aimed at providing references for its further application.

Objective:To investigate the clinical features and long-term prognosis of patients co-existing with ovarian endometrioma (OMA) and deep infiltrating endometriosis (DIE).Methods:Totally 358 OMA patients were retrospectively analyzed, who had a minimum of 8 years follow-up after laparoscopic cystectomy, which was performed by one professional endometriosis surgery team at Peking Union Medical College Hospital from January 2009 to April 2013. All women were divided into DIE group and non-DIE group, and analysis was performed in preoperative characteristics, surgical findings and postoperative outcomes during follow-up.Results:A total of 358 OMA patients were included, of which 190 patients (53.1%, 190/358) were in the DIE group, while other 168 patients (46.9%, 168/358) in the non-DIE group. The average ages between the two groups were (33.7±5.4), (32.5±5.3) years ( P=0.047), the average parity was (0.4±0.6) times vs (0.3±0.5) times ( P=0.079). There were significant differences in the proportions of moderate to severe dysmenorrhea [67.4% (128/190) vs 56.5% (95/168)], chronic pelvic pain [24.2% (46/190) vs 7.7% (13/168)], and the increase in CA 125 [79.9% (139/190) vs 65.2% (101/168)] between the two groups (all P<0.05). The average operation time in the DIE and non-DIE groups was (75±21) vs (39±36) minutes ( P<0.01). There was a significant difference in adenomyosis presence between the two groups [41.6% (79/190) vs 22.0% (37/168); P=0.001]. All patients were followed up for at least 8 years. At the end of the follow-up, though the DIE group was with higher total rate of disease relapse, yet no significant difference was found between the two groups in statistical comparison [21.6% (41/190) vs 16.1% (27/168); P=0.185]. A total of 41 cases in the DIE group recurred, the recurrence rate of pain was 15.8% (30/190), and the recurrence rate of cyst was 8.4% (16/190); 27 cases had recurrence after operation in the non-DIE group, the recurrence rate of pain was 8.9% (15/168), and the recurrence rate of cyst was 10.7% (18/168). There were no significant differences in the pain recurrence rate ( P=0.067) and cyst recurrence rate ( P=0.460) between the two groups. As for the successfully pregnant patients, live birth rates were 100.0% (65/65) vs 94.4% (68/72) between DIE group and non-DIE groups ( P=0.120). Conclusions:Compared with the non-DIE group, OMA patients with concurrent DIE might have severe pain symptoms, higher probability of abnormal CA 125 levels and more severe pelvic adhesions. Although there are no significant differences in the total recurrence rate and the recurrence rate of various types between the two groups, the proportion of pain recurrence in the DIE group is higher than that in the non-DIE group. In terms of fertility outcomes, patients in the DIE group are with lower likelihood of pregnancy after surgery during the long-time follow-up. DIE has no significant influence on the fertility outcome.

Objective: To investigate the impact factors for central neck lymph node metastases(CLNM) of papillary thyroid carcinoma(PTC). Methods: A total of 498 patients with PTC who underwent total or hemi-thyroidectomy plus central neck lymph node dissection between January 2014 and July 2016 were included. Univariate and multivariate analyses were performed to identify clinicopathological characteristics, thyroid function parameters and US findings that associated with CLNM of PTC. A nomogram was developed to predict the probability of CLNM. The receiver operating characteristic curve(ROC) was used to estimate the efficiency of the nomogram. Results: Among 498 patients, 284 patients were affected by CNLM. The sensitivity and specificity of US in predicting PTC metastasis in the central neck were 31.3% and 88.3%, respectively. Univariate and multivariate analyses showed that gender, age, number and size of suspicious malignant nodules in thyroid, and suspicious lymph node metastasis detected by ultrasonography were independently correlated with CLNM. The ROC showed that the AUC was 0.748, with sensitivity of 80.8%, and specificity of 59.8%. Conclusions Gender, age, number and size of suspicious malignant nodules in thyroid, suspicious lymph node metastasis were predictive factors for CLNM in patients with PTC. The nomogram developed based on related factors with CLNM is more sensitive than sonographic central neck lymph node features in predicting the probability of CLNM.

Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.