The fusion of data features from different modes, such as pathology images and sequence data, has the potential to predict the overall survival (OS) of patients with cervical cancer. This study aims to develop a novel prediction model for overall survival (OS) that incorporates pathology images, clinical data and molecular data. The model underwent training using comprehensive cervical cancer data from The Cancer Genome Atlas (TCGA), which include 119 patients. To independently validate the model, we used a manually collected dataset from Peking Union Medical College Hospital (PUMCH), comprising 53 patients with cervical cancer. LASSO Cox regression analysis was applied to identify relevant features associated with overall survival (OS), resulting in the identification of 484 genes, including RGR, DBN1 and CALCR, as well as numerous image features. Building upon these findings, a multimodal deep learning model was developed to effectively classify the overall survival (OS) of patients with cervical cancer into two categories: short term (ST: ≤ 3 years) and long term (LT: > 3 years) based on the integration of pathology images and clinical features. The developed model achieved reasonably good prediction accuracy in the independent testing dataset from PUMCH, with an area under the curve (AUC) value of 0.783. In conclusion, the combination of pathology images with clinical and molecular data enables the creation of accurate and reliable prediction models for cervical cancer.
Objective To summarize the clinicopathological features,immunohistochemical characteristics,HOX transcript antisense RNA(HOTAIR)in situ hybridization status,treatment,and prognosis of myxopapillary ependymoma(MPE). Methods A total of 17 patients diagnosed with MPE based on pathological evidence in the Department of Pathology of Peking Union Medical College Hospital from November 2006 to July 2023 were selected,and the clinicopathological data of these patients were collected.Immunohistochemical staining for trimethylation at lysine 27 of histone H3 (H3K27me3),glial fibrillary acidic protein(GFAP),and epithelial membrane antigen(EMA)and alcian blue-periodic acid Schiff(AB-PAS)staining were performed in all the patients.Sixteen patients with spinal ependymomas were selected as the control group.Tissue microarrays were prepared from 17 MPE patients and the control group.HOTAIR ISH was performed and semi-quantitatively scored,and the scores of the two groups were compared by the Wilcoxon rank-sum test. Results The 17 MPE patients aged 14-64 years,with the mean age of(37.48±16.10)years and the male-to-female ratio of 0.7∶1.Their clinical manifestations mainly included lumbosacral and lower limb pains.Microscopically,tumor cells were arranged in a papillary pattern around fibrovascular axis,with abundant myxoid materials,and tumor cells were arranged in a loose meshwork in some patients.The immunohistochemical staining results showed that 17(100%),10(58.82%),and 8(47.06%)patients expressed GFAP,EMA,and D2-40,respectively,and 2(11.76%)patients lacked expression of H3K27me3.AB-PAS staining showed blue myxoid materials in all the 17(100%)patients.HOTAIR was expressed in both MPE and control groups,with higher semi-quantitative score in the MPE group than in the control group(P=0.004).Twelve patients were followed up,with a median follow-up period of 65.50 months,during which three patients showed recurrence.Conclusions MPE exhibits typical pathological features,and the combination with immunohistochemical staining for GFAP and EMA as well as AB-PAS staining facilitates diagnosis of this disease.A small number of patients loss the expression of H3K27me3.HOTAIR is highly expressed in MPE but lacks specificity,which limits its auxiliary diagnostic value.The overall prognosis of MPE is favorable,with a few patients experiencing recurrence.
Background:Current limited evidence suggests that the use of pembrolizumab combined with chemotherapy may be effective for treatment-naïve patients with metastatic non-small cell lung cancer (NSCLC) and negative programmed cell death ligand 1 (PD-L1) expression, but real-world data are relatively scarce. This retrospective cohort study analyzed the efficacy, adverse events, and prognostic factors in these patients treated with chemotherapy with or without pembrolizumab. Methods:This retrospective study analyzed the data of patients with unresectable, locally advanced or metastatic NSCLC without sensitive epidermal growth factor receptor (EGFR) or anaplastic lymphoma kinase (ALK) or proto-oncogene receptor tyrosine kinase (ROS1) alterations who had negative PD-L1 expression and received first-line pembrolizumab plus chemotherapy (the Pembro group) or platinum-based dual chemotherapy with or without bevacizumab (the Chemo group). The efficacy outcomes and safety profiles of the two groups were compared. Results:The study included 246 patients (Pembro group, n=114; Chemo group, n=132). The median follow-up period was 28.3 months. The Pembro group significantly prolonged progression-free survival (PFS) compared with the Chemo group [9.5 months, 95% confidence interval (CI): 7.5-11.5 vs. 7.2 months, 95% CI: 5.7-8.7; hazard ratio (HR) =0.64, 95% CI: 0.46-0.87; P=0.004]. Squamous cell lung cancer (SCC) patients demonstrated substantial PFS benefit (13.8 months, 95% CI: 3.2-24.1 vs. 4.8 months, 95% CI: 3.4-6.2; P<0.001), while non-SCC patients showed comparable PFS (9.3 months, 95% CI: 7.6-11.0 vs. 8.0 months, 95% CI: 6.0-10.0; P=0.56). Overall survival (OS) favored the Pembro group (21.2 months, 95% CI: 16.0-26.4 vs. 20.1 months, 95% CI: 15.5-24.7; HR =0.71, 95% CI: 0.50-1.00; P=0.052). The SCC patients in the Pembro group demonstrated a significant survival benefit with a median OS that was not reached, compared to 14.2 months (95% CI: 6.3-22.1) in the chemo group (HR =0.42, 95% CI: 0.22-0.78; P=0.007). Grade ≥3 non-immune-related adverse events (non-irAEs) occurred more often in the Pembro group (46.8%) than the Chemo group (33.1%, P=0.03). Moreover, 45 (39.5%) patients experienced 63 irAEs, and no grade 5 or new irAEs were observed. Conclusions:Pembrolizumab combined with chemotherapy may prolong survival in patients with PD-L1-negative advanced NSCLC, particularly those with squamous histology.
Eosinophilic otitis media (EOM) is an intractable otitis media characterized by highly viscous secretions containing eosinophils in the middle ear. Clinically, bacterial infection complicates the course of EOM and may accelerate the progression of sensorineural hearing loss. We present a case of a woman in her late 50s, diagnosed with severe EOM, experienced recurrent ear infections despite repeated tympanostomy tube insertions, intratympanic corticosteroid injections, and systemic corticosteroid treatment. Microbiological analysis of middle ear effusion revealed methicillin-resistant Staphylococcus aureus and Candida parapsilosis coinfection. The use of corticosteroid and biologic agents was contraindicated due to the active infectious process. The patient underwent surgical debridement by subtotal petrosectomy, and obtained a successful infection control. Following resolution of the infectious process, the patient with bilateral severe mixed hearing loss and inadequate benefit from conventional hearing aids underwent successful cochlear implantation (CI), achieving satisfactory auditory rehabilitation. In cases of severe EOM complicated by refractory infections, subtotal petrosectomy represents a potentially effective therapeutic strategy for infection control and disease progression mitigation. Subsequent CI may be considered as a viable option for auditory rehabilitation in selected cases.
Access at https://isn-slidearchive.org/?col=ISN&fol=Archive&file=BPA-24-07-CI-180-3.svs and https://isn-slidearchive.org/?col=ISN&fol=Archive&file=BPA-24-07-CI-180-R4.svs. A 16-year-old girl presented to the clinic with a 2-year history of weight gain. In the last week, she had developed blepharoptosis and blurred vision, with headache and vomiting. She did not have a prior history of tumors. Physical examination showed a moon face, buffalo hump, and purpura. Laboratory findings showed elevated 24-hour urinary free cortisol (UFC) and plasma adrenocorticotropic hormone (ACTH). The low-dose dexamethasone suppression test failed to suppress cortisol production, but the high-dose test did. Magnetic resonance imaging (MRI) revealed a 15.9 × 10.1 × 12.8 mm3 sellar mass with heterogeneous T1 signal enhancement (Figure 1). It extended into the bilateral cavernous sinuses and the suprasellar cistern, impacting the optic chiasm. Surgery was pursued for a definitive diagnosis and tumor debulking (Box 1). Histopathological examination revealed a biphasic tumor with a neuroendocrine and a mesenchymal component. The neuroendocrine component, with well-differentiated solid or glandular structures, showed positive staining for Synaptophysin (Syn), ACTH, and transcription factors T-PIT and INSM-1 but negative for PIT-1 and SF-1 (Figure 2). The mesenchymal component in the stromal background was composed of spindle or irregular cells with large nuclei and abundant eosinophilic cytoplasm. These tumor cells were positive for Desmin, MyoD1, and Myogenin (Figure 2). Unlike the neuroendocrine component, the mesenchymal component exhibited strong nuclear p53 positivity and loss of ATRX expression. Mitoses were frequent in the mesenchymal component (up to 8 per 10 HPF). The mitotic count was in keeping with the Ki67 labeling (the Ki67 index was significantly higher in the mesenchymal components [50%] compared with the epithelial cells [3%]). NGS testing identified several pathological mutations as follows: DICER1 (c.4860dup, p.Cys1621Leufs*31), DICER1 (c.5428G>T, p.Asp1810Tyr), TP53 (c.740A>T, p.N247I), ATRX (c.594+1G>T), and PIK3CA copy-number gain. Corticotrophin tumor/adenoma in association with primary intracranial sarcoma, DICER1-mutant. This is a unique case of a young patient who presented with signs and symptoms of Cushing's disease and was diagnosed post-surgery as "corticotrophin tumor/adenoma associated with primary intracranial sarcoma, DICER1-mutant." Primary intracranial sarcoma, DICER1-mutant (PIS-DICER1) is a rare molecularly defined entity. According to the 2021 World Health Organization (WHO) CNS tumor classification, it is defined as a primary intracranial sarcoma with distinctive morphology, typically showing immunophenotypic myogenic differentiation [1]. DICER1 mutations are definite features, commonly along with TP53 mutations and ATRX inactivation. These tumors usually occur in young patients (median age at diagnosis as 6 years) and appear to be aggressive, notably at risk for DICER1 syndrome. Without appropriate genetic testing, these neoplasms may be easily misclassified as "rhabdomyosarcoma," potentially underestimating their clinical implications. The present tumor was initially suspected to be a corticotrophin tumor/adenoma based on Cushing's manifestations and histological findings, which showed well-differentiated nests of pituitary cells with positive Syn, INSM-1, and T-PIT lineage markers (including T-PIT and ACTH). However, the discovery of rhabdomyoblast-like cells with positive skeletal muscle differentiation markers (Desmin, MyoD1, and Myogenin) in the stromal background, raised the possibility of alternative diagnoses, such as PIS-DICER1 or rhabdomyosarcoma. Consequently, the final diagnosis of "corticotroph tumor/adenoma associated with primary intracranial sarcoma, DICER1-mutant" was made after confirming the presence of DICER1 mutations. Such a case is exceedingly rare. To date, only four cases of sellar rhabdomyosarcoma in association with pituitary adenomas have been previously reported [2, 3]. All these were in adults, ranging in age from 34 to 77 years, and one case had a history of prior radiation exposure. Unlike these cases, PIS-DICER1 is more common in younger patients. For this age group and DICER1 mutation, pituitary blastoma (PitB) should be included as a differential diagnosis. However, morphologic features of PitB, such as undifferentiated blastemal cells and Rathke's pouch epithelium are not seen in this case, thereby not being considered. Another differential diagnosis, DICER1-associated rhabdomyosarcoma, was excluded since no cases of intracranial DICER1-associated rhabdomyosarcoma have been reported. The histogenesis of PIS-DICER1 and its association with corticotrophin tumor/adenoma remain unclear. Further investigation is required to separately assess the mutation profiles of both tumor components, which may provide additional insights into the pathogenesis of such complex tumors. Our patient received chemotherapy after surgery and was kept alive with no evidence of disease after 8 months. Currently, there is no indication of DICER1 tumor predisposition syndrome in this case; however, continued monitoring and follow-up are warranted. Yinbo Xiao wrote the main manuscript text. Can Yin prepared the figures. Junliang Lu performed NGS testing. Shuangni Yu, Zhen Huo, and Zhiyong Liang reviewed and edited the manuscript. All authors approved the final version of the paper. This work was supported by the National High-Level Hospital Clinical Research Funding (grant number 2022-PUMCH-B-063). The authors declare that there is no conflict of interest. The studies were approved by the Ethics Review Committee of the Peking Union Medical College Hospital (Ethics Certificate No. K2750). The data that support the findings of this study are available from the corresponding author upon reasonable request.
ABSTRACT Objective Tumor‐induced osteomalacia with the culprit tumor located in the knee joint is rare in clinical practice, and previous literature has only been seen in case reports, which pose great challenges to the clinical diagnosis and treatment of such patients. The purpose is to elucidate clinical characteristics and orthopedic surgical treatment experience of tumor‐induced osteomalacia (TIO) with causative tumor located in the knee joint region. Methods Clinical data of all consecutive TIO patients with culprit tumors located in the knee joint region was retrospectively analyzed. All patients were surgically treated by an orthopedic bone and soft tissue tumor sub‐professional team at Peking Union Medical College Hospital from January 2015 to January 2025. The clinical distribution feature and surgical effects were analyzed, and clinical practice experience was presented. Results All nine patients were included in this study. All patients exhibited varying degrees of bone pain and 100% (9/9) of the patients had limited mobility, often accompanied by difficulties in sitting up, walking, and weakness or fatigue. Approximately 44.4% (4/9) of the patients had significantly shorter height after initial symptoms appeared. All patients underwent a total of 10 operations to control the causative tumors in the knee joint region. Culprit tumors were located in the patella (one case), infrapatellar fat pad (three cases), suprapatellar capsule (one case), popliteal fossa (three cases), and the entire knee joint (one case), respectively. There was only one case of skeletal involvement, one case with involvement of bones and soft tissues, and seven cases with soft tissue causative tumors. All the patients had a gradual increase in blood phosphorus levels in the short term after the first orthopedic surgery, after a follow‐up of 12 months to 10 years. During the follow‐up, no patients experienced recurrence. Conclusion The causative tumor for TIO in the knee joint region is hidden and has diverse locations; however, there is no established orthopedic surgical intervention strategy for these rare entities in clinical practice. Due to the unique anatomical location and complex structure of the knee joint, orthopedic surgeons can adopt different surgical approaches to completely remove the causative tumor. For these patients, the prognosis is satisfactory after complete tumor resection, and the condition can be effectively improved. These findings may help to improve the clinical diagnosis and treatment level of orthopedic physicians for this rare entity.
Objective To study the expression of SWI/SNF-related,matrix-associated,actin-dependent regulator of chromatin,subfamily A,member 4(SMARCA4)/Brahma-related gene 1,V-raf murine sarcoma viral oncogene homolog B(BRAF),P53,programmed cell death protein-1(PD-1),and programmed death-ligand 1(PD-L1),and changes in the expression of BRAF and neurotrophic tyrosine receptor kinase(NTRK) in the patients with colorectal cancer in Tibet,thereby providing a basis for targeted therapy and immunotherapy for this disease in Tibet. Methods A total of 64 patients with colorectal cancer resected in the Tibet Autonomous Region People's Hospital from January 2015 to July 2021 were enrolled in this study.The expression of SMARCA4,BRAF,P53,PD-1,and PD-L1 was detected by immunohistochemical staining.The gene fusion involving NTRK1,NTRK2,and NTRK3 was detected by fluorescence in situ hybridization,and the BRAF V600E gene mutation by polymerase chain reaction. Results The 64 patients with colorectal cancer were at a male-to-female ratio of 1.21∶1,with the mean age of (56.59±13.27) years.The tumors were located in the colon in 46(71.88%) patients and in the rectum in 18(28.12%) patients.Sixty(93.75%) patients presented adenocarcinoma,and 4(6.25%) patients presented other types of tumors.The patients in T1/T2 and T3/T4 phases accounted for 17.19%(n=11) and 82.81%(n=53),respectively.Lymph node metastasis occurred in 24(37.50%) patients.The immunohistochemical staining results showed partially down-regulated or absent expression of SMARCA4 in 1(1.56%) patient,positive BRAF expression in 4(6.25%) patients,and mutant expression of P53 in 35(54.69%) patients.The PD-1-expressing tumor associated immune cell was proportion score<10% in 45(70.31%) patients and≥10% in 19(29.69%) patients.The PD-L1 combined positive score was<10 in 52(81.25%) patients and≥10 in 12(18.75%) patients.The gene fusion of NTRK1,NTRK2,and NTRK3 was negative in all the patients,and BRAF V600E gene mutation was positive in 4(6.25%) patients.The SMARCA4 gene alteration was not detected in the patient with partial expression missing of SMARCA4.The PD-L1 combine positive score was correlated with the deficient mismatch repair(dMMR)/microsatellite instability-high (MSI-H) and the PD-1 expression (χ2=10.223,P=0.001;χ2=11.979,P=0.001). Conclusions The down-regulated or absent SMARCA4 expression and NTRK gene fusion are rare in the patients with colorectal cancer in Tibet.A few patients present BRAF V600E gene mutations,and Pan-TRK and BRAF expression can be used for the primary screening of NTRK gene fusion and BRAF gene mutation.The patients with dMMR/MSI-H are prone to high expression of PD-L1 and expected to benefit from immunotherapy.No significant correlation exists between P53 mutation and PD-L1 expression.The high expression of PD-1 is positively correlated with the high expression of PD-L1.
Gastric adenocarcinoma of the fundic gland type (GA-FG) is a rare gastric neoplasm. We present a unique case of multiple GA-FG that coexisted with the well-differentiated neuroendocrine tumors in a patient with autoimmune gastritis. To our knowledge, this is the first documented instance of such a co-occurrence and the molecular mechanism of their origin has been reviewed systematically. A 47-year-old male presented to our hospital with abdominal distension for over 10 years. Gastroscopy revealed multiple gastric eminence lesions (0.2–1.5 cm). After endoscopic mucosal resection, the pathological morphology showed mixed tumor components infiltrating into the submucosa with puzzling similarity. One with uniform-sized tumor cells arranged in nests or tubes and the other a well-differentiated tubular adenocarcinoma with irregular branching and visible gland fusion. Immunohistochemistry findings revealed the first component expressed typical markers of neuroendocrine tumor, whereas the second component expressed pepsinogen and mucin-6, indicating the presence of oxyntic gland adenocarcinoma. Due to the tumors’ proximity to the surgical margins, the patient underwent laparoscopic subtotal gastrectomy three months after the diagnosis without any tumor residue and showed no recurrence or metastasis occurred in the following regular checkups.
目的 研究西藏地区手结直肠癌中SMARCA4、BRAF、p53、程序性死亡受体 1(programmed death-1,PD-1)及其配体 1 (programmed death-ligand 1,PD-L1)免疫组化表达和BRAF、神经营养性酪氨酸激酶受体(neurotrophin tyrosine kinase receptor,NTRK)基因改变情况,为西藏地区结直肠癌患者靶向治疗及免疫治疗提供依据。方法 收集2015年1月至2021年7月期间西藏自治区人民医院经手术切除病理确诊为结直肠癌病例64例,全部病例均进行SMARCA4、BRAF、p53、PD-1、PD-L1免疫组化染色和NTRK1、NTRK2、NTRK3融合基因荧光原位杂交(fluorescence in situ hybridization ,FISH)检测及BRAF V600E基因突变聚合酶链反应(polymerase chain reaction,PCR)检测。结果 64例结直肠癌病例,男女比例1.21:1,平均年龄(56.59±13.27)岁。46例(46/64,71.88%)位于结肠,18例(18/64,28.12%)位于直肠。60例(60/64,93.75%)为腺癌, 4例(4/64,6.25%)为其他类型。11例(11/64,17.19%)为T1或T2期, 53例(53/64,82.81%)为T3或T4期; 24例(24/64,37.50%)出现淋巴结转移。免疫组化方面,1例(1/64,1.56%)SMARCA4部分肿瘤细胞表达减弱或缺失,4例(4/64,6.25%)BRAF肿瘤细胞阳性表达,35例(35/64,54.69%)p53为突变型表达。45例(45/64,70.31%)肿瘤背景免疫细胞PD-1阳性表达占比<10%,19例(19/64,29.69%)肿瘤背景免疫细胞PD-1阳性表达占比≥10%。52例(52/64,81.25%)PD-L1占比评分(CPS)<10,12例(12/54,18.75%)≥10。64例NTRK1、NTRK1、NTRK1融合基因检测均为阴性;4例(4/64,6.25%)检测到BRAF V600E基因突变;1例SMARCA4表达缺失病例未检测到SMARCA4基因改变。PD-L1的表达高低与有无错配修复缺陷/高度微卫星不稳定和PD-1的表达高低之间的差异有统计学意义(P=0.001)。结论 西藏地区结直肠癌中罕见出现SMARCA4表达减弱或缺失及NTRK融合基因改变,少数病例有BRAF V600E基因突变,Pan-TRK和BRAF免疫组化可作为NTRK融合基因及BRAF基因突变的初筛方法。错配修复缺陷/高度微卫星不稳定的病例中更容易出现PD-L1蛋白高表达,这部分患者有望获益于免疫治疗。PD-1的高表达和PD-L1的高表达呈正相关。
西藏自治区人民医院病理科已有50余年历史,是西藏自治区病理诊断质控中心的依托单位,医疗"组团式"援藏以来,科室以做强病理诊断中心建设为抓手,强化规划引领作用,在医疗管理和教学科研方面同步推进.本研究总结近几年西藏自治区人民医院病理科学科建设状况,旨在健全西藏自治区病理学科建设体系,促进全区病理学科进一步发展.
Objective To study the pathological types,expression of mismatch repair protein,human epidermal growth factor receptor 2(HER2),and Pan-TRK,and Epstein-Barr virus(EBV)infection in patients with colorectal cancer resected in Tibet. Methods A total of 79 patients with colorectal cancer resected in Tibet Autonomous Region People's Hospital from December 2013 to July 2021 were enrolled in this study.The clinical and pathological data of the patients were collected.The expression of mismatch repair protein,HER2,and Pan-TRK was detected by immunohistochemical(IHC)staining,and detection of HER2 gene by fluorescence in situ hybridization(FISH)in the patients with HER2 IHC results of 2+ or above.EBV was detected by in situ hybridization with EBV-encoded small RNA. Results A total of 79 colorectal cancer patients were included in this study,with the male-to-female ratio of 1.26:1 and the mean age of(57.06±12.74)years(24-83 years).Among them,4 patients received preoperative neoadjuvant therapy.Colonic cancer and rectal cancer occurred in 57(57/79,72.15%,including 31 and 26 in the right colon and left colon,respectively)and 22(22/79,27.85%)patients,respectively.The maximum diameter of tumor varied within the range of 1-20 cm,with the mean of(6.61±3.33)cm.Among the 79 colorectal cancer patients,75(75/79,94.94%)patients showed adenocarcinoma.Lymph node metastasis occurred in 12(12/21,57.14%)out of the 21 patients with severe tumor budding,13(13/23,56.52%)out of the 23 patients with moderate tumor budding,and 2(2/31,6.45%)out of the 31 patients with mild tumor budding,respectively.The lymph node metastasis rate showed differences between the patients with severe/moderate tumor budding and the patients with mild tumor budding(all P<0.001).The IHC staining showed that mismatch repair protein was negative in 10(10/65,15.38%)patients,including 5 patients with both MSH2 and MSH6 negative,4 patients with both MLH1 and PMS2 negative,and 1 patient with MSH6 negative.Pan-TRK was negative in 65 patients.The IHC results of HER2 showed 0 or 1+ in 60 patients and 2+ in 5 patients.FISH showed no positive signal in the 5 patients with HER2 IHC results of 2+.The detection with EBV-encoded small RNA showed positive result in 1(1/65,1.54%)patient. Conclusions Non-specific adenocarcinoma of the right colon is the most common in the patients with colorectal cancer resected in Tibet,and 15% of the patients showed mismatch repair protein defects.EBV-associated colorectal carcer is rare,Pan-TRK expression and HER2 gene amplification are seldom.The colorectal cancer patients with moderate and severe tumor budding are more likely to have lymph node metastasis.
Objective To analyze the disease spectrum and clinicopathological characteristics of central nervous system(CNS)diseases diagnosed based on pathological findings in Tibet. Methods We collected the data of all the cases with CNS lesions in Tibet Autonomous Region People's Hospital from January 2013 to December 2020.The clinicopathological features were analyzed via light microscopy,immunohistochemical staining,and special staining. Results A total of 383 CNS cases confirmed by pathological diagnosis were enrolled in this study,with a male-to-female ratio of 188∶195 and an average age of(40.03±17.39)years(0-74 years).Among them,127(33.2%)cases had non-neoplastic diseases,with a male-to-female ratio of 82∶45 and an average age of(31.99±19.29)years;256(66.8%)cases had neoplastic diseases,with a male-to-female ratio of 106∶150 and an average age of(44.01±14.87)years.The main non-neoplastic diseases were nervous system infectious diseases,cerebral vascular diseases,meningocele,cerebral cyst,and brain trauma.Among the infectious diseases,brain abscess,granulomatous inflammation,cysticercosis,and hydatidosis were common.The main neoplastic diseases included meningioma,pituitary adenoma,neuroepithelial tumor,schwannoma,metastatic tumor,and hemangioblastoma.The meningioma cases consisted of 95.4%(103/108)cases of grade Ⅰ,3.7%(4/108)cases of grade Ⅱ,and only 1(1/108,0.9%)case of grade Ⅲ.Among the neuroepithelial tumor cases,the top three were glioblastoma,grade Ⅲ diffuse glioma,and ependymoma. Conclusions There are diverse CNS diseases confirmed by pathological diagnosis in Tibet,among which non-neoplastic diseases account for 1/3 of all the cases.Infectious and vascular diseases are the most common non-neoplastic diseases in Tibet,and tuberculosis and parasitic infections are relatively common.The types and proportion of brain tumors in Tibet are different from those in other regions of China,and meningioma is the most common in Tibet,with higher proportion than neuroepithelial tumor.
目的 探讨西藏地区藏族人群非yon Hippel-Lindau(VHL)病相关的中枢神经系统血管母细胞瘤(HB)的临床病理学特征及免疫组化表型.方法 回顾性分析2010-01-2020-10西藏自治区人民医院病理科存档的全部中枢神经系统HB病例,收集患者临床及病理资料,其中7例为非VHL病相关的中枢神经系统HB.全部7例病例均经GFAP、CD31、CD34、S-100、α-inhibin、NSE、D2-40、TFE3等免疫组化染色.结果 7例HB病例,年龄23~56岁,男女比例5∶2,临床表现主要为头痛、头晕和恶心.影像学显示5例为小脑占位,1例为左侧枕叶占位,1例为椎管内占位.显微镜下,4例为网状型,3例为细胞型,1例肿瘤细胞有明显异型性.7例均未见坏死及病理性核分裂象.免疫组化方面,7例HB血管内皮细胞均表达CD31和CD34;间质细胞不同程度表达vimentin、S-100、α-inhibin、NSE、EGFR、CD56和D2-40;3例周边肿瘤组织中可见GFAP阳性的胶质细胞成分;TFE3、Olig-2、CD10、EMA、CgA和Syn均为阴性.5例患者有随访资料,术后随访12~ 46个月,均存活.结论 西藏地区中枢神经系统HB占该地区病理送检中枢神经系统肿瘤的3.9%,其中70%为非VHL病相关的病例.经典的影像学表现、组织形态学表现及免疫组化可以帮助诊断及鉴别诊断.免疫组化TFE3没有表达,HB的发生与TFE3基因可能关系不大.经手术切除治疗后患者预后良好.
目的 探讨Ber-EP4免疫组化染色在皮肤基底细胞癌中的表达及在鉴别诊断中的意义.方法 收集本院近3年来病理确诊皮肤基底细胞癌患者20例作为研究组,并收集同时期病理确诊的其他皮肤病变35例作为对照组,全部病例均行常规苏木精-伊红染色和免疫组化Ber-EP4染色,并观察组织病理表现及免疫组化表达结果.结果 研究组均可见肿瘤细胞与表皮基底部相连,均成团、巢或条索排列,细胞大小较一致、核质比增高,周边细胞呈栅栏状排列,可见核分裂象;免疫组化方面:研究组18例(90.0%)Ber-EP4呈阳性表达,其中2例为复发病例;对照组中7例(20%)Ber-EP4呈阳性表达,其中鳞癌和脂溢性角化病全部阴性.两组差异有统计学意义(P<0.01).结论 Ber-EP4对于皮肤基底细胞癌的诊断有一定价值,有助于基底细胞癌与皮肤鳞癌、脂溢性角化病的鉴别诊断.
目的 探讨西藏高原地区改良苏木精-伊红(Hematoxylin-eosin,HE)病理染色技术在病理诊断中的应用和效果.方法 选取我院2014年5月~2015年4月期间病理诊断的切片500例为常规组,选取2019年2月~2020年1月期间病理诊断的切片500例为改良组,全部病理切片均进行HE染色.常规组采用常规HE病理染色技术,改良组采用改良HE病理染色技术,在光镜下观察两种病理染色技术的染色效果并进行评估效果.结果 改良组染色合格率为94%,常规组为70%,两组差异具有统计学意义(p<0.05);改良组切片合格率为92.00%,常规组62.67%,两组差异具有统计学意义(p<0.05).结论 改良组HE病理染色技术提高了高原地区病理切片染色合格率,更加有助于病理诊断,值得推广和应用.
肺硬化性肺细胞瘤是临床上罕见的肺肿瘤,其组织形态学表现复杂,发病率低,大多数患者无症状[1],术前活检及冰冻诊断存在很大的误诊率,难与腺癌等进行鉴别[2].笔者医院曾接诊1例初诊误诊为肺腺癌的肺硬化性肺细胞瘤患者.其病理特点进行报道,旨在提高临床病理医师对本病的认识.
1 病例简介 患者女性,藏族,56岁.主因"无明显诱因出现左侧大腿包块2年,加重1月".患者于2年前发现左侧大腿包块,局部发硬,疼痛不明显,给予口服药对症治疗,无明显好转.1月前因包块增大于当地医院住院.患者既往无明确外伤史.入院查体发现左下肢局部肿块,大小15x10cm,质硬,无移动,周围皮肤无发红,触痛及压痛不明显,左侧膝关节及远端活动及感觉良好.左侧胫骨CT示;左侧大腿中远端软组织内高密度团块影,考虑为晚期骨化性肌炎可能.病理切片:成骨及成软骨病变,部分区域纤维组织增生,局灶细胞增生活跃,不除外皮质旁骨肉瘤可能.入院影像学检查显示左侧股骨下段周围见团块致密影,大小约17.1×10.0cm,境界不清,与左股骨下段分界不清,邻近骨皮质增厚,髓腔内见斑片密度增高影(图1).行左侧大腿包块切除术手术,术中所见:皮下深筋膜见肿块,无明显包膜,肿块表面大部区域较光滑,大小约16×17cm,质地较硬,肿块两端质地相对较软,边界不清;包块与股骨远端皮质相连,部分骨皮质变薄.因肿块无法完整切除,手术切除肿块三分之二送病理检查.病理诊断:病变符合皮质旁骨肉瘤.
Objective To summarize the clinical manifestations and pathological features of alveolar echinococcosis (AE) in Tibet, China. Methods Complete pathological data of all patients with surgical resection of AE in the Tibet Autonomous Region People's Hospital from September 2013 to April 2021 were retrospectively studied. Clinical and imaging findings, pathological results, treatment methods, and prognosis information were extracted through the electronic case system. The pathological sections were re-read by microscope to observe the pathological changes. Results A total of 44 AE patients were included. There were 17 males and 27 females; the average age was (36.2±12.3) years; 32 cases were farmers or herdsmen. The most common clinical manifestations were gastrointestinal symptoms (54.5%), followed by respiratory symptoms (13.6%), and 29.5% of patients had no obvious clinical symptoms. CT examination showed that 23 cases had a single lesion and 21 cases had multiple lesions (15 cases with multiple intrahepatic lesions and 6 cases with multiple organ involvement); 33 cases were diagnosed as AE by CT. The specimens for histopathological examination were mainly masses, with a diameter of 1.0-23.0 cm, and the sections were honeycombed. Microscopically, there were small vesicles of different sizes and shapes in the lesions, and the outer wall of the vesicles was a thin layer of pink stratum corneum. The proliferation of vesicles was mainly exogenous. In the early stage, a large number of eosinophils infiltrated around necrotic vesicles with the formation of granuloma; dust-like calcification and massive necrosis were seen in late old lesions. Early granulomatous lesions and late old lesions were mixed in all 44 cases, of which 5 cases were accompanied by abscess formation. A total of 31 cases underwent radical surgery, and 13 cases underwent palliative surgery and drug treatment. After a follow-up of 2-82 months, 3 cases died and 41 cases survived. Conclusions AE patients in China's Tibet are mainly young farmers and herdsmen, mostly women. The liver is the main organ involved, and a few cases can involve the lung or multiple organs. The clinical manifestations are related to the affected organs, and symptoms of the digestive system are more common. Imaging examination has a good reference value for the auxiliary diagnosis of typical cases, but atypical cases or cases complicated with abscess must be confirmed by pathology. Pathological examination shows that the body of alveolar echinococcus with a spherical vesicular structure, and the lesions of different periods often exist together. Most patients have a good prognosis by radical surgical treatment.
中肾样腺癌为已出版的WHO女性生殖系统新的组织学类型[1],其中卵巢中肾样腺癌报道病例数不足20例[2-8].在此,笔者详细报告1例卵巢中肾样腺癌病例,分析其临床资料、组织学特征、免疫表型并复习相关文献,以提高对该类疾病的认识.
目的 探讨宫颈神经内分泌癌的临床病理分析.方法 回顾性分析我院近10年病理存档的全部宫颈神经内分泌癌4例,将患者的临床病理资料,病理切片经光镜下观察并进行免疫组化染色,结合相关文献进行分析.结果 4例宫颈神经内分泌癌病例,患者年龄33~56岁(平均年龄41岁),临床表现为阴道出血(4/4).组织学均为单纯型小细胞癌,肿瘤细胞呈弥漫浸润性生长,细胞体积小,大小一致,呈圆形或短梭型,胞质少、核深染、核分裂像多见(70~90/10个高倍视野),均可见明显坏死.免疫组化表型:4例病例均同时表达CgA、CD56或Syn 3种神经内分泌标记物中的2种或以上.结论 宫颈神经内分泌癌是一种少见的高度恶性肿瘤,以小细胞癌为主,预后极差.诊断依靠其独特的形态学特征和免疫组化特点,应与宫颈转移性小细胞癌、小细胞型鳞癌及非霍奇金淋巴瘤等疾病进行鉴别诊断.