Clinicopathological and immunohistochemical characteristics of bullous pilomatricoma: a retrospective, single-center study, and comparison with ordinary pilomatricoma

Abstract Background Bullous pilomatricoma is a rare variant of pilomatricoma. As it has been published in sporadic case reports, a limited understanding of its clinicopathological characteristics restricts its effective diagnosis and treatment. Objectives This study aimed to analyze the clinicopathological and immunohistochemical characteristics of bullous pilomatricoma to better understand the bullous transformation of pilomatricoma. Methods The authors conducted a retrospective study of 12 patients with bullous pilomatricoma and compared their clinical, histopathological, and immunohistochemical data with those of patients with ordinary pilomatricoma. Results Bullous pilomatricoma showed no sex preference, with a mean onset age of 31.2 years. The common sites were the upper extremities and trunk. Bullous pilomatricoma had a shorter disease duration, a larger diameter, and a greater tendency to increase in size than those of ordinary pilomatricoma. Histopathologically, bullous pilomatricoma had a shorter duration, lesser calcification, more mitotic figures, and distinct dermal features from those of ordinary pilomatricoma. Immunohistochemically, the expression of Matrix Metalloprotease (MMP)-2, MMP-9, vascular endothelial growth factor receptor-3 (VEGFR-3), and VEGF-C was elevated. Study limitations The study was retrospective, and the sample size was small. Conclusion The distinctive features of bullous pilomatricoma potentially result from dermal changes associated with the release of angiogenic factors and proteolytic enzymes. This comprehensive analysis provides novel insights into the clinical features and pathogenesis of bullous pilomatricoma.

Clinical characteristics of early-onset gastric cancer. A study in a Colombian population

Gastric cancer is a multifactorial disease with important genetic and environmental factors. It is the fifth most common cancer in incidence, and the fourth cause of death secondary to cancer. The incidence of early-onset gastric cancer is increasing worldwide, but clinical information on these patients has not been well established. We analyzed the association between age and clinical, endoscopic, and histopathological characteristics of gastric cancer at the time of diagnosis in a Latin American population. A retrospective and descriptive cross-sectional study was carried out using the database of the Gastroenterology Service of the Clínica Foscal and Clínica Foscal Internacional in Bucaramanga, Colombia. Between January 2016 and December 2019, 259 de novo gastric cancer cases were diagnosed, of which 36 patients (13.9%) were 40 years old or younger. In patients with early-onset gastric, the prevalence of gastric cancer diagnosis was lower in men. A family history of gastric cancer or any other neoplasm was not associated with a higher prevalence of gastric neoplasms. In young patients, vomiting and ascites were more common, the preferred anatomical location was the body of the stomach, and the Borrmann IV classification and the diffuse-type histology were more likely. Our study showed an approximation of the characteristics of early-onset gastric cancer in a Latin American population, where we observed that early-onset gastric cancer has different demographic, anatomical, and histological features than late-onset gastric cancer.

El cáncer gástrico es una enfermedad multifactorial con importantes factores genéticos y ambientales. Es el quinto cáncer más común en incidencia y la cuarta causa de muerte secundaria al cáncer. La incidencia del cáncer gástrico de inicio temprano está aumentando en todo el mundo, pero la información clínica sobre estos pacientes no está bien establecida. Analizamos la asociación entre la edad y las características clínicas, endoscópicas e histopatológicas del cáncer gástrico al momento del diagnóstico en una población latinoamericana. Se realizó un estudio retrospectivo y descriptivo de corte transversal utilizando la base de datos del Servicio de Gastroenterología de la Clínica Foscal y Clínica Foscal Internacional en Bucaramanga, Colombia. Entre enero de 2016 y diciembre de 2019 se diagnosticaron 259 casos de cáncer gástrico de novo, de los cuales 36 pacientes (13,9%) tenían 40 años o menos. En pacientes con enfermedad gástrica de inicio temprano, la prevalencia del diagnóstico de cáncer gástrico fue menor en los hombres. El antecedente familiar de cáncer gástrico o cualquier otra neoplasia no se asoció con una mayor prevalencia de neoplasias gástricas. En pacientes jóvenes fueron más frecuentes los vómitos y la ascitis, la localización anatómica preferida fue el cuerpo del estómago, siendo más probable la clasificación de Borrmann IV y la histología de tipo difuso. Nuestro estudio mostró una aproximación a las características del cáncer gástrico de inicio temprano en una población latinoamericana, donde observamos que el cáncer gástrico de inicio temprano tiene diferentes características demográficas, anatómicas e histológicas que el cáncer gástrico de inicio tardío.

The non-communicating intracranial-extracranial Rosai-Dorfman disease: a case report / 中华神经科杂志

Rosai-Dorfman disease (RDD) is a benign self-limited disease characterized by lymphadenopathy and phagocytosis of lymphocytes by histiocytes. A case of intracranial-extracranial non communicating RDD was reported in this paper. The patient was admitted to Shiyan Taihe Hospital in May 2020 because of "the left top scalp tumor was found for 4 months, and the right lower limb was numb for more than half a month". The plain scan and enhanced scan of the patient′s head magnetic resonance imaging (MRI) showed that the disease focus of the left parietal bone was slightly uneven enhanced, its internal and external soft tissues were significantly enhanced, and the local internal and external soft tissues were significantly thickened irregularly, with the size of about 3.2 cm× 4.7 cm, and adjacent brain parenchyma was compressed. After resection of left top mass and intracranial mass, pathological results showed spindle cell proliferation with inflammatory reaction, and immunohistochemical staining results supported the diagnosis of RDD. The neurological function of the patient recovered to normal basically 7 months after operation, and no recurrence of the disease was found in the MRI examination of the head. The treatment effect was satisfactory.

Clinicopathological characteristics and prognosis of idiopathic membranous nephropathy with or without C3 deposition / 中华全科医师杂志

Objective:To investigate the clinicopathological characteristics and prognosis of idiopathic membranous nephropathy (IMN) with or without C3 deposition.Methods:Clinical and pathological data of 576 patients with IMN diagnosed in Affiliated Hospital of Qingdao University from January 2017 to January 2021 were retrospectively analyzed. The patients were divided into C3 deposition group and non-C3 deposition group according to the immunofluorescence staining of C3. The clinical and pathological characteristics were compared between the two groups. Kaplan-Meier survival curve was used to compare the prognosis of the two groups.Results:A total of 576 IMN patients (male 364 (63.20%)) were enrolled, including 400 patients (69.44%) with C3 deposition and 176 patients (30.56%) without C3 deposition. Compared with the non-C3 deposition group, the levels of total blood cholesterol ( t=0.94, P=0.002) and the proportion of phospholipase A2 receptor ( χ2=9.99, P=0.002), IgG ( χ2=10.67, P=0.001), IgM ( χ2=7.00, P=0.008), IgA ( χ2=7.87, P=0.005) and C1q ( χ2=8.28, P=0.004) depositions in renal tissues was higher in C3 deposition group, while the levels of serum C3 ( t=2.87, P=0.004), albumin ( t=3.57, P<0.001) and IgG ( Z=3.55, P<0.001) were lower in C3 deposition group. There were no significant differences in other clinicopathological indicators between the two groups. The survival analysis was performed in 460 patients who were followed for>6 months, including 319 cases (69.35%) of C3 deposition and 141 cases (30.65%) of non-C3 deposition. The end point event was defined as an eGFR decline>30% or entry into end stage renal disease (ESRD). There was no statistically significant difference in treatment method between the two groups ( P>0.05). The median follow-up time was 22 (13,32) months, 327 (71.09%) patients achieved remission, and 22 patients had renal end-point events. Compared with the non-C3 deposition group, the proportion of urinary protein remission was lower ( χ2=10.85, P<0.05), the incidence of renal end-point events was higher ( χ2=5.05, P<0.05). Kaplan-Meier survival analysis showed that patients with C3 deposition had a lower cumulative remission rate (Log-rank χ2=6.68, P=0.010), and a lower cumulative renal survival than those without C3 deposition had ( χ2=5.42, P=0.020). Conclusions:Compared with patients without C3 deposition, IMN patients with C3 deposition have more severe clinical and pathological changes, lower renal cumulative remission rate, and are more likely to have poor prognosis.

Clinicopathological features and prognostic analysis of primary duodenal adenocarcinoma / 中华普通外科杂志

Objective:By analyzing the clinical data of patients with primary duodenal adenocarcinoma (PDA), the risk factors affecting the postoperative prognosis of PDA patients were discussed.Methods:The clinical data of 191 patients diagnosed with PDA in Peking University First Hospital from Jan 2009 to Dec 2022 were collected. The survival rate was calculated and the survival curve was plotted by Kaplan-Meier method. Univariate analysis was performed by Log-Rank test, and multivariate analysis was performed by COX proportional hazards regression model to obtain independent risk factors.Results:The median age of onset in patients with PDA is 65 years old, and the most common symptoms are abdominal pain and abdominal distension. Prognostic analysis showed that the survival rates at 1, 3 and 5 years were 73.8%, 44.6%, and 23.0%. The analysis of Cox risk proportional regression model showed that preoperative CA19-9 level, depth of tumor invasion, degree of differentiation, TNM stage, and surgical mode were independent risk factors for the prognosis of PDA (all P<0.01). Conclusion:The overall incidence of PDA is low, but the prognosis is rather poor. Multvariable factors are associated with its prognosis and surgery is still the mainstay for hope of cure.

Association of microsatellite instability with clinicopathological features and prognosis of colon cancer patients / 中华普通外科杂志

Objective:To investigate the relationship between microsatellite instability (MSI) , and clinicopathological features ,prognosis in patients with stage Ⅱ and Ⅲ colon cancer.Methods:Patients undergoing surgical resection for stage Ⅱ and Ⅲ colonic tumor in the Affiliated Hospital of Qingdao University from Dec 2016 to Nov 2018 were enrolled. All the 292 patients were with stage Ⅱ and Ⅲ colon cancer and MSI status. Propensity score matching method was used to match the two groups of patients according to 1:1. χ 2 analysis, Logistic Regression and COX regression was used to analyse the relationship between MSI status, the clinicopathological features and prognosis. Results:The risk of MSI-H in young patients ( OR=0.340, 95% CI: 0.126~0.921, P=0.034), right-sided colon cancer ( OR=7.985, 95% CI: 3.040-20.973, P<0.001), mucinous adenocarcinoma ( OR=4.285, 95% CI: 1.495-12.284, P=0.007), poorer differentiation ( OR=4.848, 95% CI: 1.597-14.716, P=0.005), N0 staging ( OR=0.235 , 95% CI: 0.077-0.719, P=0.011) increased . The total OS of colon cancer patients in the MSS group (66.7%) and the MSI-H group (86.9%) were statistically different( P=0.003). The MSI status ( HR=0.367, 95% CI: 0.151-0.891, P=0.027) is an independent factor affecting the prognosis of patients. Conclusions:In stage Ⅱ and Ⅲ colon cancer, patients with MSI-H have a better prognosis. MSI status is prognosis relevant factor for colon cancer patients.

Clinicopathological features of cystic neutrophilic granulomatous mastitis / 中华普通外科杂志

Objective:To investigate the etiology, clinical manifestations, clinicopathological features of cystic neutrophil granulomatous mastitis (CNGM).Methods:From Jan 2019 to Dec 2020, 95 CNGM cases diagnosed by biopsy pathology at Chongqing Hospital of Traditional Chinese Medicine and Chongqing Liangping District Hospitol of Traditional Chinese Medicine were reviewed.Results:There were 95 female patients, aged 21 to 50 years, with a median age of 32 years. Laboratory examination showed that 56% (53/95) cases had elevated rheumatoid antibody level, 27 % (26/95) had increased level of serum thyroid antibody, 15% (14/95) had elevated antineutrophil antibody, 35% (33/95) had increased ESR, 38% (36/95) had increased C-reactive protein. The positive rate of Gram-stained bacilli was 82% (78/95). Histology: pyogenic granuloma with lobule of breast as the center, the center of granuloma was cystic vacuole.Immunohistochemistry showed that the inflammatory cells in and around granuloma were mainly CD3 + cells, and CD4 + cells were more than CD8 + cells. Conclusions:The cystic neutrophilie granulo matous mastitis is a rare type of idiopathic granulomatous mastitis. The diagnosis of CNGM is dependent on its specific pathological features.

Expression and significance of programmed death ligand 1 and programmed death 1 in colorectal cancer complicated by schistosomiasis / 中国基层医药

Objective:To investigate the expression and significance of programmed death ligand 1 (PD-L1) and programmed death 1 (PD-1) in colorectal cancer complicated by schistosomiasis.Methods:A total of 134 patients with colorectal cancer who received treatment in Xuancheng People's Hospital during 2014-2021 were included in this study. These patients consisted of 74 patients with colorectal cancer combined with schistosomiasis (patient group) and 60 patients with only colorectal cancer (control group). The expression of PD-L1 and PD-1 in colorectal cancer tissue was detected by an immunohistochemical method. The differences in PD-L1 and PD-1 expression were compared between the two groups. The relationships between PD-L1 and PD-1 expression and clinical pathological characteristics were determined.Results:The positive expression rates of PD-L1 and PD-1 in cancer cells and interstitial lymphocytes were 55.4% and 60.8% respectively in the patient group and they were 35.0% and 40.0% respectively in the control group. The positive expression rates of PD-L1 and PD-1 were significantly higher in the patient group than the control group ( χ2 = 5.55, 5.74, both P < 0.05). The expressions of PD-L1 and PD-1 in the patient group were correlated with lymph node metastasis and high tumor-node-metastasis stage ( P < 0.05). Conclusion:PD-L1 and PD-1 are highly expressed in colorectal cancer complicated by schistosomiasis and are related to their invasive behavior. PD-1/PD-L1 singaling pathway may be involved in the molecular mechanism underlying the occurrence and development of colorectal cancer complicated by schistosomiasis. Blocking PD-1/PD-L1 signaling pathway may be a new strategy for immunotherapy of colorectal cancer complicated by schistosomiasis.

Renal oncocytoma misdiagnosed as renal cell carcinoma: report of two cases and literature review / 中国基层医药

Objective:To investigate the clinical, imaging, and pathological characteristics of renal oncocytoma, and to improve the understanding, diagnosis, and treatment of renal oncocytoma.Methods:The imaging and pathological data of two patients misdiagnosed with renal cell carcinoma in the 970 Hospital of PLA Joint Logistics Support Force from January to March 2021 were retrospectively analyzed. The relevant literature was reviewed and discussed.Results:The tumors were located in the left kidney of two patients, with diameters of 2.7 cm and 3.2 cm respectively. The patients underwent retroperitoneal laparoscopic removal of partial left kidney and retroperitoneal laparoscopic removal of the whole left kidney separately. The pathological results confirmed the diagnosis of renal oncocytoma.Conclusion:Renal oncocytoma is a rare benign renal cell tumor which is difficult to be diagnosed before surgery. Contrast-enhanced CT can provide evidence for the identification of renal oncocytoma. Its final diagnosis depends on pathological results.

Diagnosis and treatment of primary biliary cholangitis: Is pathology necessary? / 临床肝胆病杂志

Primary biliary cholangitis (PBC) is a chronic intrahepatic cholestatic disease. This article summarizes and reviews the histopathological features of PBC and the role of pathological examination in the diagnosis and treatment of PBC, as well as the role of pathology in staging and prognosis, the diagnosis of atypical PBC and overlap syndrome, the analysis of reasons for poor response to ursodeoxycholic acid, and identification of diseases or exclusion of other comorbidities, so as to improve the awareness of the role of pathological examination in PBC among clinicians.

Diagnosis and treatment of autoimmune hepatitis: The indispensable role of pathology / 临床肝胆病杂志

Autoimmune hepatitis has become the main type of non-infectious hepatitis in China. This article summarizes its characteristic manifestations and the current status of diagnosis and treatment and points out that pathological histology plays an indispensable role in the diagnosis and treatment of autoimmune hepatitis.

Analysis of clinicopathological features of 18 cases of hepatic angiosarcoma / 中华肝脏病杂志

Objective: To investigate the clinicopathological features, treatment, and prognosis of hepatic angiosarcoma. Methods: Clinicopathological data and prognostic conditions of 18 cases with hepatic angiosarcoma were collected retrospectively. The recurrence-free survival rate and overall survival rate were calculated by the Kaplan-Meier method. A Cox regression analysis was used to explore the survival-related risk factors. Results: There were 12 male and 6 female patients, with an average age of 57 (37 ~ 70) years. The tumor's average diameter was 8.40 (2.00 ~ 18.00) cm. Seven cases had multiple tumors, while two cases had large vessel tumor thrombuses. Microscopically, the tumor tissues were irregularly anastomosed, with vascular lacunar or solid bundle-like weaving, and the tissue morphology mimicked capillary hemangioma, cavernous hemangioma, or angioepithelioma, while tumor cells were spindle-shaped or epithelioid, lined with hobnails in the lumen, or formed papillary structures in the lumen. The proportion of highly, moderately, and poorly differentiated tumors was 4:8:6, with six cases having clear tumor boundaries, eight having microvascular tumor thrombi, and sixteen having blood lake formation. Different levels of expression of CD31, CD34, erythroblast transformation-specific related genes, and Fli-1 markers were demonstrated in all of the cases. Four cases had a P53 mutation, and six cases had Ki-67 > 10%. During the follow-up period of 0.23-114.20 months, the five-year recurrence-free survival rate and overall survival rate were 16.7% and 37.2%, respectively. Cox regression multivariate analysis showed that preoperative symptoms and multiple tumors were significant risk factors for recurrence-free survival, while preoperative symptoms and Ki-67 > 10% were significant risk factors for overall survival. Conclusion: Hepatic angiosarcoma is a rare hepatic mesenchymal tumor with high malignancy and a poor prognosis. Pathological morphology and immunohistochemical marker combinations are needed for a definite diagnosis. However, the complexity of angiosarcomas' histological and cytological conformations and the overlap of pathological features with benign vascular tumors, sarcomas, and carcinomas pose difficulties in the differential diagnosis. Thus, the only effective ways to prolong survival are early detection and radical surgical resection.

Clinicopathologic characteristics and prognostic analysis of testicular diffuse large B-cell lymphoma / 中华血液学杂志

Objective: To analyze the clinicopathologic characteristics and prognosis of testicular diffuse large B-cell lymphoma (DLBCL) . Methods: A retrospective analysis was performed on 68 patients with testicular DLBCL admitted to Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine from October 2001 to April 2020. The gene mutation profile was evaluated by targeted sequencing (55 lymphoma-related genes) , and prognostic factors were analyzed. Results: A total of 68 patients were included, of whom 45 (66.2% ) had primary testicular DLBCL and 23 (33.8% ) had secondary testicular DLBCL. The proportion of secondary testicular DLBCL patients with Ann Arbor stage Ⅲ-Ⅳ (P<0.001) , elevated LDH (P<0.001) , ECOG score ≥ 2 points (P=0.005) , and IPI score 3-5 points (P<0.001) is higher than that of primary testicular DLBCL patients. Sixty-two (91% ) patients received rituximab in combination with cyclophosphamide, adriamycin, vincristine, and prednisone (R-CHOP) -based first-line regimen, whereas 54 cases (79% ) underwent orchiectomy prior to chemotherapy. Patients with secondary testicular DLBCL had a lower estimated 5-year progression-free survival (PFS) rate (16.5% vs 68.1% , P<0.001) and 5-year overall survival (OS) rate (63.4% vs 74.9% , P=0.008) than those with primary testicular DLBCL, and their complete remission rate (57% vs 91% , P=0.003) was also lower than that of primary testicular DLBCL. The ECOG scores of ≥2 (PFS: P=0.018; OS: P<0.001) , Ann Arbor stages Ⅲ-Ⅳ (PFS: P<0.001; OS: P=0.018) , increased LDH levels (PFS: P=0.015; OS: P=0.006) , and multiple extra-nodal involvements (PFS: P<0.001; OS: P=0.013) were poor prognostic factors in testicular DLBCL. Targeted sequencing data in 20 patients with testicular DLBCL showed that the mutation frequencies of ≥20% were PIM1 (12 cases, 60% ) , MYD88 (11 cases, 55% ) , CD79B (9 cases, 45% ) , CREBBP (5 cases, 25% ) , KMT2D (5 cases, 25% ) , ATM (4 cases, 20% ) , and BTG2 (4 cases, 20% ) . The frequency of mutations in KMT2D in patients with secondary testicular DLBCL was higher than that in patients with primary testicular DLBCL (66.7% vs 7.1% , P=0.014) and was associated with a lower 5-year PFS rate in patients with testicular DLBCL (P=0.019) . Conclusion: Patients with secondary testicular DLBCL had worse PFS and OS than those with primary testicular DLBCL. The ECOG scores of ≥2, Ann Arbor stages Ⅲ-Ⅳ, increased LDH levels, and multiple extra-nodal involvements were poor prognostic factors in testicular DLBCL. PIM1, MYD88, CD79B, CREBBP, KMT2D, ATM, and BTG2 were commonly mutated genes in testicular DLBCL, and the prognosis of patients with KMT2D mutations was poor.

Clinicopathologic analysis of micro and mini parotid gland tumors / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinicopathological characteristics of micro and mini parotid gland tumors and to provide reference for their clinical diagnosis and treatment.@*METHODS@#Patients with parotid gland tumors treated in the Department of Oral and Maxillofacial Surgery, Peking University School and Hospital of Stomatology from December 2012 to April 2020 were selected. Relevant clinical data of the patients with tumor diameter ≤20 mm detected by preoperative CT were collected to analyze the clinicopathological characteristics and prognosis of micro and mini parotid gland tumors. And the collected data were divided into two groups with diameter 11-20 mm and diameter ≤10 mm according to tumor diameter measured by preoperative CT. The clinicopathological differences between the two groups were statistically analyzed.@*RESULTS@#A total of 2 067 patients with primary epithelial parotid gland tumors were collected, and 685 patients with tumor diameter ≤20 mm were examined by CT, accounting for 33.1%. The ratio of male to female patients with micro and mini parotid gland tumors was 1 ∶1.93, the average age was (45.3±13.8) years (12-83 years), and the median course of disease was 12 months (1 week to 30 years). Among them, 635 cases (92.7%) were benign tumors, 50 cases (7.3%) were malignant tumors, and the ratio of benign to malignant was 12.7 ∶1. The most common benign tumor was pleomorphic adenoma, and the most common malignant tumor was mucoepidermoid carcinoma. The micro and mini parotid gland tumors were divided into 11-20 mm group (n=611) and ≤10 mm group (n=74), the clinical characteristics comparison of the two groups of gender ratio, average age, course of di-sease had no statistical difference (P>0.05). In the 11-20 mm diameter group, the percentage of benign and malignant tumor was 92.8% (567/611) and 7.2% (44/611) respectively, and the ratio of benign to malignant tumors was 12.9 ∶1. In the ≤10 mm diameter group, the percentage of benign and malignant tumor was 91.9% (68/74) and 8.1% (6/74) respectively, and the ratio of benign to malignant tumors was 11.3 ∶1. There was no significant difference between the two groups (P>0.05). Fifty patients with malignant tumor were followed up for the median follow-up period of 39.5 months (1-91 months). Local recurrence occurred in 2 patients with one death. The overall 2-year survival rate was 93.7% and the 5-year survival rate was 89.3%.@*CONCLUSION@#The majority of micro and mini parotid gland tumors was benign lesion. There was a good prognosis for micro and mini parotid gland carcinoma. Early surgical treatment was recommended for micro and mini parotid gland tumors.

Clinicopathological analysis of 105 patients with fibrous dysplasia of cranio-maxillofacial region / 北京大学学报(医学版)

OBJECTIVE@#To compare the clinicopathologic features and prognosis of the different types of fibrous dysplasia (FD) of cranio-maxillofacial region, so as to provide a new reference for clinicians to treat these patients and make prognostic judgement.@*METHODS@#Clinical records, radiographic data and pathological information of 105 patients diagnosed with FD or McCune-Albright syndrome (MAS) at the Department of Oral Pathology, Peking University Hospital of Stomatology from January 2013 to December 2020 were collected. The patients were divided into 4 groups: monostotic FDs, polyostotic FDs, MAS and a specific type called craniofacial fibrous dysplasia (CFD) limited in the craniofacial region. The clinicopathological characteristics, treatment and follow-up data of each type were analyzed.@*RESULTS@#Of all the 105 patients, 46 were males and 59 were females, with a male-to-female ratio of 1 ∶1.3. The onset age ranged from 0 to 56 years and the median age was 12 years. On the basis of different involvement conditions, 4 types were divided. The most common type was monostotic FDs (43 cases, 40.95%), including maxilla (29 cases), mandibular (12 cases) and zygoma (2 cases). 32 cases (30.48%) were diagnosed with polyostotic FDs, 7 cases (6.67%) were MAS, and 23 cases (21.90%) were CFDs confirmed by computed tomography (CT) analysis. CFD was clearly distinct from other types of FD, such as the patient gender and the serum alkaline phosphatase level in peripheral blood before operative surgery. The pathologic findings of various types FD were quite similar, whilst the predominant fibrous tissue hyperplasia could be observed in polyostotic FDs and MAS types.@*CONCLUSION@#The clinicopathologic features of FD in the cranio-maxillofacial region are different from the FD lesions in other parts of the body. The clinicopathological features of CFD are significantly different from those of monostotic and polyostotic FDs in the cranio-maxillofacial region. Therefore, the clinicians should pay attention to distinguish CFD in clinic, imaging and pathology aspects, so as to further clarify its features in clinic management and prognosis.

Efficacy and thoughts of endoscopic submucosal dissection for rectal neuroendocrine tumor / 中华消化内镜杂志

To evaluate the efficacy and safety of endoscopic submucosal dissection (ESD) for rectal neuroendocrine tumor (RNET). Data of 71 patients (43 males and 28 females, aged 46.66±10.15 years) with histologically diagnosed RNET ≤15 mm in diameter who underwent ESD in Department One of Gastroenterology, Digestive Diseases Hospital, Heilongjiang Provincial Hospital from December 2012 to January 2021 were retrospectively analyzed. Epidemiological characteristics, endoscopic characteristics, the operation time, complications, pathology results, the consistency of endoscopic ultrasound (EUS) and pathology and prognosis were analyzed. Sixty-nine patients had single lesion and 2 patients had multiple lesions (two of each). Seventy lesions (70/73, 95.89%) were?located?in?the?middle-lower?rectal?segment, and 3 lesions (3/73, 4.11%) in the upper segment. The maximum diameter was 8.54±3.12 mm. The en bloc resection rate was 100%. No adverse event was observed. The R0 resection rate was 87.67% (64/73), with 8 had undetermined vertical resection margin and 1 had lymphovascular invasion. EUS showed no case involving the muscularis propria with pathologic coincidence rate of 100.0%. The follow-up period was 4.52±3.85 years (1-9 years). No local recurrence or distant metastasis was found. ESD is safe and effective in RNET ≤15 mm in diameter. For?tumors of G1 without metastasis in preoperative evaluation, close?follow-up?seems?to?be?a?feasible?option with lymphovascular invasion or undetermined margin after?ESD.

Clinicopathologic characteristics of lupus nephritis with positive antineutrophil cytoplasmic antibody / 中华肾脏病杂志

Objective:To explore the clinical and histopathologic features of lupus nephritis (LN) patients with positive antineutrophil cytoplasmic antibody (ANCA), so as to provide more theoretical basis to recognize and treat this disease.Methods:Clinical data of biopsy-proven LN patients with ANCA test in the First Affiliated Hospital of Sun Yat-sen University from November 1, 2002 to September 11, 2020 were collected and analyzed retrospectively. The difference of clinical data, laboratory examination, and pathological examination of renal biopsy between ANCA-positive group and ANCA-negative group. The clinicopathological characteristics between different ANCA serotypes were compared.Results:A total of 1 304 patients with LN confirmed by renal biopsy and ANCA test results were enrolled. Eighty ANCA-positive patients from 1 304 LN patients were screened. There are 55(68.8%) ANCA-positive LN patients with positive anti-myeloperoxidase antibodies (MPO). There were 14(17.5%) ANCA-positive LN patients with positive anti-proteinase 3 antibodies (PR3), and 11(13.8%) ANCA-positive patients with double positive antibodies of MPO and PR3. ANCA-positive LN patients had significantly higher serum creatinine [135.5(68.0, 361.8) μmol/L vs 88.0(64.0, 165.0) μmol/L, P=0.004] and blood urea nitrogen [12.35(6.35, 21.18) mmol/L vs 8.60 (5.50, 15.70) mmol/L, P=0.026] as well as lower estimated glomerular filtration rate [45.70(13.83, 84.10) ml·min -1·(1.73 m 2) -1 vs 66.75(38.43, 96.22) ml·min -1·(1.73 m 2) -1, P=0.001] than ANCA-negative patients (stratified sampling of 160 patients). ANCA-positive LN patients had higher chronicity index than ANCA-negative LN patients [3(2, 7) vs 2(0, 5), P=0.006]. There were statistically significant difference in hemoglobin, serum creatinine and estimated glomerular filtration rate among ANCA-positive group, ANCA-negative group, and MPO-ANCA and PR3-ANCA double positive group. MPO-ANCA and PR3-ANCA double positive LN patients had the lowest hemoglobin and estimated glomerular filtration rate, and highest serum creatinine among the three groups (all P<0.05). Conclusions:ANCA-positive LN patients have worse renal function and higher renal histological chronicity index than ANCA-negative LN patients, especially for patients with double positive MPO-ANCA and PR3-ANCA. More stringent monitoring and therapy may be needed in this subgroup of LN patients.

Clinical analysis of antineutrophil cytoplasmic antibody-associated vasculitis in 13 children / 中华肾脏病杂志

Objective:To investigate the clinical manifestations, pathological characteristics, treatment and prognosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) in 13 children.Methods:The clinical and pathological data of 13 cases of AAV in children′s Hospital of Nanjing Medical University from June 2000 to December 2021 were retrospectively analyzed.Results:Among the 13 cases, 12 cases were diagnosed with microscopic polyangiitis (MPA) and 1 case was granulomatosis with polyangiitis (GPA), including 10 females and 3 males. The onset age ranged from 3 years and 11 months to 13 years and 10 months. The most frequently involved organ was the kidney (12 cases, 92.3%), followed by respiratory system (7 cases, 53.8%), skin (5 cases, 38.5%), digestive system (4 cases, 30.8%), nervous system (4 cases, 30.8%) and cardiovascular system (3 cases, 23.1%). There were 10 cases with orthotic anemia, 7 cases with positive antinuclear antibody, and 3 cases with mildly decreased complement C3. Among the 12 children with renal impairment, 9 cases were accompanied by abnormal renal function at the beginning of the disease. Renal biopsy was classified according to the Berden as follows: sclerotic in 5 cases, crescentic 3 cases, focal in 2 cases and mixed in 2 cases. All children were treated with glucocorticoid combined with immunosuppressant. During the follow-up time from 8 months to 128 months, 4 cases acquired complete remission, 8 cases achieved partial remission and 1 case recurred after complete remission, and 7 cases progressed to chronic kidney disease stage 5. Three children with complete remission underwent repeated renal biopsy, including 2 cases of mixed type and 1 case of crescent type initially, and all changed to focal type.Conclusions:AAV in children occurs mainly in school-age female, and most of AAV in children is MPA. The clinical manifestations are various. Most of them have renal damage and anemia, and lung damage is also common. Patients with skin purpura onset may be misdiagnosed as Henoch-Schonlein purpura, and AAV with ANA positive or complement reduction should exclude systemic lupus erythematosus. Once the renal function is abnormal in AAV, especially estimated glomerular filtration rate<60 ml·min -1·(1.73 m 2) -1 and the pathological classification is sclerotic type or crescent type, it is difficult to reverse even after active treatment. Early diagnosis and treatment are very important for AAV.

Primary central nervous system T-cell lymphoma: a case report / 中华神经科杂志

Primary central nervous system T-cell lymphomas (PCNSTL) are rare, the clinical symptoms and radiographic imaging of which are unspecific, and the pathological morphology is antypical, leading to misdiagnosis and delays in treatment. A 45-year-old male patient with diplopia accompanied by numbness and dysarthria was reported in this paper, which was considered as "lymphoma or lymphoproliferative lesions" on magnetic resonance imaging (MRI) while no typical tumor cells in brain biopsy. The clinical symptoms worsened one month later and the reexamined MRI showed that the scope of the lesion was enlarged and the enhancement was more obvious than before, which was still considered as lymphoma or lymphoproliferative lesion. The second biopsy was performed and still no typical tumor lymphocytes were seen. Finally, gene rearrangement was carried out and showed the β and γ chains both present positive mutations in T cell receptor (TCR) gene rearrangement. Combined with cell morphology, immunophenotype and TCR gene rearrangement results, the patient was finally diagnosed as PCNSTL. This article reviewed the clinical symptoms, imaging features, laboratory examinations, pathological characteristics, diagnosis and differential diagnosis of PCNSTL, so as to improve the understanding of this rare disease.

Clinicopathological features and myeloid differentiation factor 88 L265P gene mutation in patients with diffuse large B-cell lymphoma of central nervous system / 中华神经科杂志

Objective:To analyze the clinical features and mutation of myeloid differentiation factor 88 (MYD88) L265P in patients with diffuse large B-cell lymphoma (DLBCL) of central nervous system (CNS).Methods:The clinicopathological materials of 45 cases of DLBCL of CNS were retrospectively collected in Xuanwu Hospital, Capital Medical University from September 2014 to February 2017. The clinicopathological data were retrospectively analyzed, combined with immunohistochemistry, EB virus in situ hybridization, imaging and medical history. The mutation of MYD88 L265P gene was detected by pyrosequencing and its clinical significance was analyzed. Results:The age of the patients ranged from 42 to 82 years [(57.6±8.8) years], including 24 males and 21 females. Totally 93.3% (42/45) of the patients had supratentorial tumours, which were single or multiple. The cerebral hemisphere (31/45, 68.9%) was the most common involved site, and 21 cases (21/45, 46.7%) had multiple lesions. Histologically, DLBCL in the CNS showed diffuse infiltration of tumor tissue, some of which grew around blood vessels in a "sleeve" arrangement. CD 20 and CD 79a were diffusely and strongly positive. Thirty-nine cases (39/45, 86.7%) were non-germinal center B cell (non-GCB) subtype and 6 cases (6/45, 13.3%) were germinal center B cell (GCB) subtype. MYD88 L265P mutation was found in 64.4% (29/45) patients. There was statistically significant difference between non-GCB type (71.8%, 28/39) and GCB type DLBCL (1/6, P=0.017). Compared with the operation/biopsy group without chemotherapy, operation+chemotherapy, biopsy+chemotherapy, operation/biopsy+chemotherapy+stem cell transplantation can improve the survival and prognosis ( HR=0.05, 95% CI 0.01-0.33 , P=0.002; HR=0.04, 95% CI 0.01-0.36 , P=0.004; HR=0.01, 95% CI 0.00-0.17 , P=0.001; respectively). Conclusions:DLBCL of the CNS is aggressive tumor with poor prognosis, the clinical manifestations are complex and diverse, and the diagnosis is challenging. MYD88 L265P is a common and specific gene mutation in primary CNS lymphoma(PCNSL), which is of great significance in the diagnosis and treatment of lymphoma. The MYD88 L265P mutation was more frequently detected in non-GCB than GCB subtype. Chemotherapy can improve the survival rate of PCNSL patients. If chemotherapy achieves complete remission and autologous hematopoietic stem cell transplantation is performed, there may be a chance of long-term survival.