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Atrophic Kidney-Like Lesion - Case Report of A Provisional Entity with Brief Review of Literature. 萎缩性肾样病变1例报告并文献复习。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01541
Balamurugan Thirunavukkarasu, Saket Singh, Aditya Prakash Sharma, Amanjit Bal

Atrophic kidney-like lesion is a recently recognized entity, post 2016 World Health Organization Classification of tumors of the urinary system. The behavior of this tumor is not fully known as only a handful of cases with limited follow-up are available. This entity closely mimics thyroid-like follicular carcinoma of the kidney, which has different prognosis. We report a case of incidentally detected atrophic kidney-like lesion in an elderly gentleman who had urothelial carcinoma of the urinary bladder with a brief review of literature. Atrophic kidney-like lesion and urothelial carcinoma of the urinary bladder association has not been reported in the literature.

萎缩性肾样病变是最近被认可的实体,2016年世界卫生组织将泌尿系统肿瘤分类。这种肿瘤的行为尚不完全清楚,因为只有少数病例随访时间有限。这种肿瘤与肾甲状腺样滤泡癌非常相似,但预后不同。我们报告一个偶然发现的萎缩性肾样病变在一个老年绅士谁有尿路上皮癌的膀胱简要回顾文献。萎缩性肾样病变与尿路上皮癌的相关性尚未见文献报道。
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引用次数: 2
Evidence for Diverse Prognosis in High-Grade Serous Ovarian Carcinoma: Solid, Pseudoendometrioid, and Transitional-Like; So-Called "SET Morphology" and Progesterone Receptor Status. 高级别浆液性卵巢癌不同预后的证据:实体癌、假子宫内膜样癌和移行样癌;所谓的“SET形态学”和孕酮受体状态。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2022.01571
Halit Uner, Metin Demir, Dincer Goksuluk, Ayse Kars, Meral Uner, Alp Usubutun

Objective: High-grade serous ovarian carcinoma (HGSC) is one of the major tumors of the gynecological system with a poor survival rate and variable microscopic appearance. It was suggested that SET (solid, pseudo-endometrioid and transitional-like) morphology in ovarian HGSC is predictably associated with BRCA deficiencies. In this study, we investigated the microscopic patterns and some immunohistochemical markers predicting the prognosis of serous carcinoma.

Material and method: We re-evaluated 305 HGSC ovarian resections morphologically and calculated the SET morphology percentages for each case. Morphological and immunohistochemical data correlated with the survival and post-treatment disease progression data.

Results: The median age at diagnosis was 57 years and the median follow-up period was 3.1 years. The median overall survival (OS) of ovarian carcinoma in SET-predominant tumors (n=60) was 81 months, while for tumors with SET non-dominant morphology (n=63) and non-SET morphology (n=182) it was 59.7 and 44.7 months, respectively.

Conclusion: Predominant (more than 50%) SET morphology was significantly associated with increased survival rates of HGSC. Immunohistochemically, p53, ERCC1, ER, and PR antibodies were applied and only PR antibody positivity was found to be associated with borderline statistical significance for increased survival rates. Our results suggest that SET morphology may be a potential predictive and prognostic marker in managing the treatment strategies of HGSC.

目的:高级别浆液性卵巢癌(HGSC)是妇科系统的主要肿瘤之一,生存率低,镜下表现多变。有人认为,卵巢HGSC中的SET(实体、假子宫内膜样和移行样)形态可预测地与BRCA缺陷相关。在这项研究中,我们研究了浆液性癌的显微镜模式和一些免疫组织化学标志物预测预后。材料和方法:我们从形态学角度重新评估了305例HGSC卵巢切除术,并计算了每个病例的SET形态学百分比。形态学和免疫组织化学数据与生存率和治疗后疾病进展数据相关。结果:诊断时的中位年龄为57岁,中位随访期为3.1年。SET占优势的肿瘤(n=60)的卵巢癌的中位总生存期(OS)为81个月,而SET非占优势形态(n=63)和非SET形态(n=182)的肿瘤分别为59.7和44.7个月。结论:占优势(50%以上)的SET形态与HGSC生存率的提高显著相关。免疫组化显示,应用p53、ERCC1、ER和PR抗体,发现只有PR抗体阳性与生存率增加的临界统计学意义相关。我们的研究结果表明,SET形态可能是管理HGSC治疗策略的潜在预测和预后标志物。
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引用次数: 1
The Significance of Histopathologic Assessment in Bone Marrow Disease in Neuroblastoma. 神经母细胞瘤骨髓疾病组织病理学评估的意义。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01556
Sumeyye Ekmekci, Dilek Ince, Nur Olgun, Erdener Ozer

Objective: Neuroblastoma (NB) is the most common extracranial solid tumor in children and is responsible for 12% of cancer-related deaths. The status of metastatic disease in the bone marrow (BM) is a predictor of poor outcome. The purpose of this study was to investigate the predictive significance of histopathological examination of BM in NB.

Material and method: The study included 61 cases with archival bone marrow biopsy tissues. The cases were evaluated regarding the percentage of metastatic tissue and its differentiation. Primary tumor slides were also reviewed to perform the Shimada classification based on the differentiation status and mitosis-karyorrhexis index. The patients' age, gender, NMYC amplification, clinical risk group, and disease outcome were also noted.

Results: Of the 61 cases, 17 had BM involvement. Of those, eight cases (47.1%) were refractory NB showing disease relapse. Based on BM examination, five cases (29.4%) were categorized as complete response, seven (41.2%) as progressive disease, three (17.6%) as minimal disease, and two (11.8%) as stable disease. The progressive disease category was significantly related with refractory disease and NMYC amplification along with the high-risk category (p =0.002 and p= 0.003 respectively). Undifferentiated histology and presence of more than 20% of tumor tissue in the BM biopsy at diagnosis were significantly associated with the progressive disease category (p=0.01 and p < 0.001, respectively).

Conclusion: We conclude that evaluating the percentage of metastatic tumor tissue and tumor differentiation in BM biopsies is of clinical importance in the management of neuroblastoma patients.

目的:神经母细胞瘤(NB)是儿童最常见的颅外实体瘤,占癌症相关死亡的12%。骨髓转移性疾病(BM)的状态是预后不良的预测指标。本研究的目的是探讨NB组织病理学检查对脑脊髓炎的预测意义。材料与方法:研究对象为61例档案骨髓活检组织。对这些病例的转移组织及其分化的百分比进行评估。我们还回顾了原发肿瘤的切片,根据分化状态和有丝分裂-核裂指数进行岛田分类。还记录了患者的年龄、性别、NMYC扩增、临床风险组和疾病结局。结果:61例患者中17例颅脑受累。其中顽固性NB 8例(47.1%)复发。根据BM检查,完全缓解5例(29.4%),进展性疾病7例(41.2%),轻度疾病3例(17.6%),稳定性疾病2例(11.8%)。进行性疾病类别与难治性疾病、NMYC扩增及高危类别显著相关(p =0.002、p= 0.003)。未分化组织学和诊断时BM活检中超过20%的肿瘤组织与进展性疾病类别显著相关(分别为p=0.01和p < 0.001)。结论:我们得出结论,评估BM活检中转移瘤组织的百分比和肿瘤分化对神经母细胞瘤患者的治疗具有临床意义。
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引用次数: 0
PD-L1 Expression in Medullary Thyroid Carcinoma and Its Association with Clinicopathological Findings. PD-L1在甲状腺髓样癌中的表达及其与临床病理的关系。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01558
Yasemin Kemal, Sultan Çalişkan, Seda Gun, Mehmet Kefeli

< strong > Objective: < /strong > Medullary thyroid carcinoma (MTC) is a rare tumor originating from parafollicular C cells. It has more aggressive biologic behavior than differentiated thyroid carcinomas, and it is insensitive to treatment with radioactive iodine. Vandetanib and cabozantinib are the newly approved tyrosine kinase inhibitors in advanced stages, but novel effective systemic therapeutics could be crucial and needed for the clinical management of these patients. We aimed to evaluate the Programmed death-ligand 1 (PD-L1) expression, which is a novel immunotherapy target, in our MTC cohort, and determine whether it has an association with clinical and pathological features. < strong > Material and Method: < /strong > This retrospective study involved 41 cases of MTC with a median follow-up of 54 months. PD-L1 monoclonal antibody (SP263 clone) was investigated immunohistochemically. Complete and/or partial membranous staining pattern in more than 1% of tumor cells was considered positive. The correlations of PD-L1 expression with clinicopathologic and prognostic features were analyzed. < strong > Results: < /strong > PD-L1 positivity was detected in 5 (12.2%) of 41 tumors. The extent of PD-L1 staining was low ( < 5%) for all tumors. There was no clinicopathologic and prognostic relevance regarding PD-L1 expression in our MTC patients. < strong > Conclusion: < /strong > Although PD-L1 expression could be a potential biomarker to predict the prognosis of various cancers and response to checkpoint inhibitors, we did not find any significant correlation between PD-L1 expression and clinicopathologic features in our cases. Studies with larger patient numbers are still required to perform a more comprehensive analysis.

目的:< /strong >甲状腺髓样癌(MTC)是一种起源于滤泡旁C细胞的罕见肿瘤。它比分化型甲状腺癌具有更强的侵袭性生物学行为,并且对放射性碘治疗不敏感。Vandetanib和cabozantinib是新批准的晚期酪氨酸激酶抑制剂,但新的有效的全身治疗可能是至关重要的,并且需要对这些患者的临床管理。我们的目的是评估程序性死亡配体1 (PD-L1)的表达,这是一个新的免疫治疗靶点,在我们的MTC队列中,确定它是否与临床和病理特征有关。< strong >材料与方法:< /strong >本回顾性研究纳入41例MTC,中位随访54个月。免疫组织化学方法检测PD-L1单克隆抗体(SP263克隆)。超过1%的肿瘤细胞呈完全和/或部分膜性染色模式为阳性。分析PD-L1表达与临床病理及预后的相关性。结果:41例肿瘤中有5例(12.2%)PD-L1阳性。所有肿瘤的PD-L1染色程度均较低(< 5%)。在我们的MTC患者中,PD-L1表达没有临床病理和预后相关性。结论:< /strong >尽管PD-L1表达可能是预测各种癌症预后和对检查点抑制剂反应的潜在生物标志物,但在我们的病例中,我们未发现PD-L1表达与临床病理特征之间有任何显著相关性。患者数量较多的研究仍需要进行更全面的分析。
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引用次数: 2
Clinico-Histological Features of Thrombotic Microangiopathy in Renal Biopsies: A Retrospective Study. 肾活检中血栓性微血管病变的临床组织学特征:回顾性研究。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01536
Niraimathi Manickam, Vinita Agrawal, Pallavi Prasad, Manoj Jain, Narayan Prasad

Objective: Thrombotic microangiopathy (TMA) is often first detected on a renal biopsy performed for renal manifestations. Apart from hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura, there are various secondary conditions associated with TMA. This study analyzes the clinico-pathological spectrum, etiological factors and renal outcome of TMA diagnosed on renal biopsy.

Material and method: A retrospective evaluation of renal biopsies for TMA over 5.5 years was performed. Clinical and laboratory data was collected from patient records.

Results: A total of 40 biopsies from 39 patients showed TMA comprising 33 native and 7 transplant biopsies. Malignant hypertension (n=13) was the most common etiology in native biopsies followed by postpartum TMA (n=7), atypical HUS (aHUS) (n=7), and lupus nephritis (n=6). TMA in transplant biopsies was due to acute rejection (n=4) and CNI toxicity (n=3). Serum creatinine was high in most patients (mean 5.6 + 2.5 mg/ dl). aHUS showed the highest mean LDH levels and the lowest average platelet counts. Renal biopsies in malignant hypertension and postpartum TMA showed isolated arterial changes while aHUS and lupus nephritis showed both glomerular and arterial involvement. Postpartum TMA and aHUS had poor renal outcome requiring renal replacement therapy.

Conclusion: Most postpartum TMA and aHUS had systemic features of TMA while malignant hypertension and lupus nephritis showed 'isolated renal TMA'. This emphasizes the importance of careful evaluation of renal biopsies even in the absence of systemic features of TMA.

目的:血栓性微血管病变(TMA)通常首先在肾脏活检中发现。除了溶血性尿毒症综合征(HUS)和血栓性血小板减少性紫癜外,还有各种与TMA相关的继发性疾病。本研究分析经肾活检诊断的TMA的临床病理谱、病因及肾脏转归。材料和方法:对5.5年来TMA的肾活检进行回顾性评价。临床和实验室数据从患者记录中收集。结果:39例患者40例活检显示TMA,其中本地活检33例,移植活检7例。恶性高血压(n=13)是最常见的病因,其次是产后TMA (n=7)、非典型溶血性尿毒综合征(aHUS) (n=7)和狼疮性肾炎(n=6)。移植活检中的TMA是由于急性排斥反应(n=4)和CNI毒性(n=3)。大多数患者血清肌酐偏高(平均5.6 + 2.5 mg/ dl)。aHUS患者LDH平均水平最高,血小板平均计数最低。恶性高血压和产后TMA的肾脏活检显示孤立的动脉改变,而aHUS和狼疮性肾炎显示肾小球和动脉均受累。产后TMA和aHUS的肾脏预后较差,需要肾脏替代治疗。结论:产后TMA和aHUS多表现为全身性TMA,而恶性高血压和狼疮性肾炎表现为“孤立性肾性TMA”。这强调了仔细评估肾活检的重要性,即使没有TMA的全身性特征。
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引用次数: 2
Mesenchymal Tumors Involving the Pancreas: A Clinicopathologic Analysis and Review of the Literature. 累及胰腺的间充质肿瘤:临床病理分析及文献回顾。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2022.01567
Gokce Askan, Olca Basturk

Objective: Most pancreatic tumors are epithelial, and, among these, more than 90% are of ductal origin. However, a variety of mesenchymal tumors may involve the pancreas and may manifest different clinicopathological characteristics. The literature on mesenchymal tumors in the pancreas is largely limited to individual case reports or analyses of small series, predominantly focusing on radiologic features.

Material and method: Authors' institutional and consultation databases were reviewed to identify the mesenchymal tumors involving the pancreas.

Results: Forty cases were identified; twenty-five (63%) tumors were benign/borderline, and the remaining fifteen (37%) were malignant. Of the benign/borderline tumors; 9 were solitary fibrous tumors, 6 gastrointestinal stromal tumors (GISTs), 4 schwannomas, 2 desmoid type fibromatosis, 1 lymphangioma, 1 ganglioneuroma, 1 inflammatory myofibroblastic tumor, and 1 low grade mesenchymal neoplasm. Malignant tumors included 6 cases of leiomyosarcomas, 4 liposarcomas, 2 rhabdomyosarcomas, 1 epithelioid angiosarcoma, 1 malignant peripheral nerve sheet tumor, and 1 undifferentiated pleomorphic sarcoma. Four cases (multicystic schwannoma, desmoid fibromatosis, lymphangioma and inflammatory myofibroblastic tumor) were preoperatively misdiagnosed as a primary epithelial tumor of the pancreas.

Conclusion: Mesenchymal tumors rarely involve the pancreas. They are usually benign/borderline neoplasms but may be diagnostically challenging, especially clinically/radiologically, as they may form cystic and/or large lesions in the pancreas. Mesenchymal tumors should be considered in both the clinical/radiological and pathological differential diagnosis of pancreatic lesions.

目的:大多数胰腺肿瘤是上皮性的,其中90%以上是起源于导管。然而,多种间充质肿瘤可能累及胰腺,并可能表现出不同的临床病理特征。关于胰腺间充质肿瘤的文献大多局限于个别病例报告或小系列分析,主要集中在放射学特征上。材料和方法:回顾作者的机构和咨询数据库,以确定累及胰腺的间充质肿瘤。结果:共确诊40例;25例(63%)为良性/交界性肿瘤,其余15例(37%)为恶性肿瘤。良性/交界性肿瘤;孤立性纤维性肿瘤9例,胃肠道间质瘤(gist) 6例,神经鞘瘤4例,纤维瘤样纤维瘤2例,淋巴管瘤1例,神经节神经瘤1例,炎性肌纤维母细胞瘤1例,低级别间质肿瘤1例。恶性肿瘤包括平滑肌肉瘤6例、脂肪肉瘤4例、横纹肌肉瘤2例、上皮样血管肉瘤1例、恶性周围神经片瘤1例、未分化多形性肉瘤1例。4例(多囊神经鞘瘤、硬纤维瘤、淋巴管瘤和炎性肌纤维母细胞瘤)术前被误诊为胰腺原发上皮肿瘤。结论:间充质肿瘤很少累及胰腺。它们通常为良性/交界性肿瘤,但诊断上具有挑战性,尤其是临床/影像学上,因为它们可能在胰腺形成囊性和/或大的病变。胰腺病变的临床/影像学和病理鉴别诊断均应考虑间充质肿瘤。
{"title":"Mesenchymal Tumors Involving the Pancreas: A Clinicopathologic Analysis and Review of the Literature.","authors":"Gokce Askan,&nbsp;Olca Basturk","doi":"10.5146/tjpath.2022.01567","DOIUrl":"https://doi.org/10.5146/tjpath.2022.01567","url":null,"abstract":"<p><strong>Objective: </strong>Most pancreatic tumors are epithelial, and, among these, more than 90% are of ductal origin. However, a variety of mesenchymal tumors may involve the pancreas and may manifest different clinicopathological characteristics. The literature on mesenchymal tumors in the pancreas is largely limited to individual case reports or analyses of small series, predominantly focusing on radiologic features.</p><p><strong>Material and method: </strong>Authors' institutional and consultation databases were reviewed to identify the mesenchymal tumors involving the pancreas.</p><p><strong>Results: </strong>Forty cases were identified; twenty-five (63%) tumors were benign/borderline, and the remaining fifteen (37%) were malignant. Of the benign/borderline tumors; 9 were solitary fibrous tumors, 6 gastrointestinal stromal tumors (GISTs), 4 schwannomas, 2 desmoid type fibromatosis, 1 lymphangioma, 1 ganglioneuroma, 1 inflammatory myofibroblastic tumor, and 1 low grade mesenchymal neoplasm. Malignant tumors included 6 cases of leiomyosarcomas, 4 liposarcomas, 2 rhabdomyosarcomas, 1 epithelioid angiosarcoma, 1 malignant peripheral nerve sheet tumor, and 1 undifferentiated pleomorphic sarcoma. Four cases (multicystic schwannoma, desmoid fibromatosis, lymphangioma and inflammatory myofibroblastic tumor) were preoperatively misdiagnosed as a primary epithelial tumor of the pancreas.</p><p><strong>Conclusion: </strong>Mesenchymal tumors rarely involve the pancreas. They are usually benign/borderline neoplasms but may be diagnostically challenging, especially clinically/radiologically, as they may form cystic and/or large lesions in the pancreas. Mesenchymal tumors should be considered in both the clinical/radiological and pathological differential diagnosis of pancreatic lesions.</p>","PeriodicalId":45415,"journal":{"name":"Turkish Journal of Pathology","volume":"38 1","pages":"46-53"},"PeriodicalIF":1.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9999697/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9349530","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Eosinophilic Solid and Cystic Renal Cell Carcinoma: From Unclassified to Classified, A Case Report. 嗜酸性实性和囊性肾细胞癌:从未分类到分类1例报告。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01531
Rashim Sharma, Balamurugan Thirunavukkarasu, Poonam Elhence, Mahaveer Singh Rodha, Binit Sureka

Eosinophilic solid and cystic renal cell carcinoma (ESC RCC) is a novel tumour with unique morphological and immunohistochemical features. It is a recently described entity after the 2016 World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs and is characterised by a solid cystic tumour composed of polygonal cells with voluminous eosinophilic cytoplasm and CK20 positivity. This tumour has uncertain malignant potential and also has an association with tuberous sclerosis complex (TSC). Sarcomatoid differentiation has not been reported in ESC RCC till now. ESC RCC poses a diagnostic challenge as many eosinophilic/oncocytic renal tumours are included in the differentials. We present a case of ESC RCC with sarcomatoid differentiation in an elderly female without any clinical features of TSC and discuss the differential diagnosis of oncocytic renal tumours.

嗜酸性实体囊性肾细胞癌(ESC RCC)是一种具有独特形态和免疫组织化学特征的新型肿瘤。它是继2016年世界卫生组织泌尿系统和男性生殖器官肿瘤分类之后最近被描述的一个实体,其特征是由多边形细胞组成的实体囊性肿瘤,具有大量嗜酸性细胞质和CK20阳性。该肿瘤具有不确定的恶性潜能,也与结节性硬化症(TSC)有关。到目前为止,在ESC RCC中尚未见肉瘤样分化的报道。ESC RCC提出了一个诊断挑战,因为许多嗜酸性/嗜酸细胞性肾肿瘤包括在鉴别中。我们报告一例无任何TSC临床特征的老年女性ESC RCC伴肉瘤样分化,并讨论嗜瘤细胞性肾肿瘤的鉴别诊断。
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引用次数: 1
Low-Grade Oncocytic Tumor: Report of Two Cases of An Emerging Entity in the Spectrum of Oncocytic Renal Neoplasms. 低级别嗜酸细胞肿瘤:肾嗜酸细胞肿瘤谱中一个新出现的实体的两例报告。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01549
Divakar Sharma, Trupti Pai, Gagan Prakash, Sangeeta Desai, Santosh Menon

Low-grade Oncocytic Tumor (LOT) of kidney is an emerging neoplasm that forms an important differential diagnosis in a spectrum of entities with oncocytic morphology. It has overlapping features with renal oncocytoma and eosinophilic variant of chromophobe renal cell carcinoma, but with distinct clinical, histomorphological and immunohistochemical features. LOT exhibits characteristic low grade oncocytic morphology with a CD117 negative/CK7 positive immunophenotype. Herein, we describe two cases of this emerging entity, LOT, with emphasis on the diagnostic aspects, including the histomorphology, immunoprofile and discussion on the close differentials.

肾低级别嗜瘤细胞肿瘤(LOT)是一种新兴的肿瘤,在一系列嗜瘤细胞形态的实体中形成重要的鉴别诊断。它与肾嗜酸性细胞瘤和嗜酸性细胞癌有重叠特征,但具有不同的临床、组织形态学和免疫组织化学特征。LOT表现出典型的低级别癌细胞形态,CD117阴性/CK7阳性免疫表型。在这里,我们描述了两个病例,这种新兴的实体,LOT,重点是诊断方面,包括组织形态学,免疫特征和讨论密切的差异。
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引用次数: 4
Mixed Epithelial and Stromal Tumor Family of Kidney (Adult Cystic Nephroma, Mixed Epithelial and Stromal Tumor): Retrospective Clinicopathological Evaluation. 肾脏混合上皮和间质肿瘤家族(成人囊性肾病、混合上皮和基质肿瘤):回顾性临床病理评价。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2022.01575
Hale Demir, Zehra Sahin, Oktay Ozman, Muhammed Demirbilek, Sami Berk Ozden, Iclal Gurses, Haydar Durak, Nesrin Uygun, Bulent Onal

Objective: Tumors belonging to the mixed epithelial stromal tumor family (MESTF) are rare; thus clinicopathological experience about them are limited. Each epithelial and stromal component shows different patterns in these tumors.

Material and method: Clinicopathological features of 11 MESTF cases that were diagnosed between 2000 and 2021 at a single center were evaluated.

Results: Ten of the 11 patients were female (F:M = 10:1). The mean age of the females was 47 (31-63) years; the male patient was 45 years old. The mean tumor diameter was 6.7 (3.5-19) cm. All tumors had varying proportions of cystic and solid components. Eight cases were well circumscribed, and the others had distinct but irregular borders. Two of the tumors with irregular borders were bulging into the renal sinus. The epithelial component was dominant in most cases. In the epithelial component, macrocyst, microcyst, and tubules were the most common patterns and the most common types of lining epithelium were flat, cuboidal and hobnail. The stromal component was variable in most cases and included hypocellular (mostly collagenous) and cellular areas. In most cases, the cellular stroma had an ovarian-like appearance. Among the other features observed, hyalinization and dystrophic calcification were common. The positivity for estrogen and progesterone receptor in the stromal component was observed in almost all female cases.

Conclusion: MESTF, which has distinctive features, should be considered in the differential diagnosis of cystic kidney tumors.

目的:属于混合上皮间质瘤家族(MESTF)的肿瘤是罕见的;因此,关于它们的临床病理经验是有限的。在这些肿瘤中,每个上皮和基质成分都显示出不同的模式。材料和方法:评估2000年至2021年间在一个中心诊断的11例MESTF病例的临床病理特征。结果:11例患者中10例为女性(F:M=10:1)。女性的平均年龄为47岁(31-63岁);男,45岁。平均肿瘤直径为6.7(3.5-19)cm。所有肿瘤都有不同比例的囊性和实体性成分。八个案件的边界很好,其他案件的边界明显但不规则。其中两个边界不规则的肿瘤隆起进入肾窦。上皮成分在大多数情况下占主导地位。在上皮成分中,大囊肿、小囊肿和小管是最常见的模式,最常见的衬里上皮类型为扁平、立方体和钉状。基质成分在大多数情况下是可变的,包括细胞下(主要是胶原)和细胞区域。在大多数情况下,细胞间质具有卵巢样外观。在观察到的其他特征中,透明化和营养不良钙化是常见的。在几乎所有女性病例中都观察到基质成分中雌激素和孕激素受体的阳性。结论:肾囊性肿瘤的鉴别诊断应考虑MESTF的特点。
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引用次数: 1
Methylation Profiling of Specific Genes in Ependymomas. 上皮细胞瘤中特定基因的甲基化谱。
IF 1 Q4 PATHOLOGY Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01565
Naz Kanit, Pelin Yalcin, Serhat Erbayraktar, Erdener Ozer

Objective: Ependymomas are neuroepithelial tumors of the central nervous system with heterogeneous biology and clinical course. The aim of the present study is to investigate the relationship between the methylation status and clinicopathological parameters in ependymomas.

Material and method: DNA methylation status of CDKN2A, RASSF1A, KLF4 and ZIC2 genes were quantitatively analyzed with pyrosequencing in 44 ependymoma tumor tissues. The relationship of methylation profiles with tumor subtype, histological grade and patient age was statistically analyzed.

Results: DNA methylation analyses for CDKN2A revealed no difference in methylation levels. Of the 31 included samples for optimal ZIC2 methylation analysis, 10 were hypermethylated (32.3%) and this change was significantly found in the adult spinal ependymomas (p=0.01). KLF4 hypermethylation was observed in 6 of the overall included 35 samples (17.1%); however, there was no statistically significant relation of the methylation status with tumor subtype, histological grade or age group. RASSF1A hypermethylation was observed in overall 40 included samples with varying methylation levels. Higher levels of hypermethylation were significantly related to the grade 3 histology (p=0.01) and spinal ependymomas (p=0.006). The pediatric cases with grade 3 ependymomas and ependymomas of adulthood showed significantly increased RASSF1A hypermethylation levels (p < 0.001 and p=0.001, respectively).

Conclusion: DNA methylation changes are likely to have biological importance in ependymomas. Both ZIC2 and RASSF1A methylation status may be useful parameters in the subclassification of these tumors.

目的:室管膜瘤是中枢神经系统的神经上皮性肿瘤,具有异质性的生物学和临床过程。本研究的目的是探讨室管膜瘤的甲基化状态与临床病理参数之间的关系。材料和方法:应用焦磷酸测序技术对44例室管膜瘤组织中CDKN2A、RASSF1A、KLF4和ZIC2基因的DNA甲基化状况进行定量分析。对甲基化谱与肿瘤亚型、组织学分级和患者年龄的关系进行统计学分析。结果:CDKN2A的DNA甲基化分析显示甲基化水平没有差异。在用于最佳ZIC2甲基化分析的31个纳入样本中,10个样本(32.3%)是高甲基化的,这种变化在成人脊髓室管膜瘤中显著发现(p=0.01)。在总共纳入的35个样本中,有6个样本(17.1%)观察到KLF4高甲基化;然而,甲基化状态与肿瘤亚型、组织学分级或年龄组之间没有统计学上的显著关系。在总共40个甲基化水平不同的纳入样本中观察到RASSF1A超甲基化。较高水平的高甲基化与3级组织学(p=0.01)和脊髓室管膜瘤(p=0.006)显著相关。3级室管膜和成年室管膜的儿科病例显示RASSF1A高甲基化水平显著升高(分别为p<0.001和p=0.001)。结论:DNA甲基化变化可能在室管膜癌中具有生物学意义。ZIC2和RASSF1A甲基化状态可能是这些肿瘤亚类化的有用参数。
{"title":"Methylation Profiling of Specific Genes in Ependymomas.","authors":"Naz Kanit,&nbsp;Pelin Yalcin,&nbsp;Serhat Erbayraktar,&nbsp;Erdener Ozer","doi":"10.5146/tjpath.2021.01565","DOIUrl":"10.5146/tjpath.2021.01565","url":null,"abstract":"<p><strong>Objective: </strong>Ependymomas are neuroepithelial tumors of the central nervous system with heterogeneous biology and clinical course. The aim of the present study is to investigate the relationship between the methylation status and clinicopathological parameters in ependymomas.</p><p><strong>Material and method: </strong>DNA methylation status of CDKN2A, RASSF1A, KLF4 and ZIC2 genes were quantitatively analyzed with pyrosequencing in 44 ependymoma tumor tissues. The relationship of methylation profiles with tumor subtype, histological grade and patient age was statistically analyzed.</p><p><strong>Results: </strong>DNA methylation analyses for CDKN2A revealed no difference in methylation levels. Of the 31 included samples for optimal ZIC2 methylation analysis, 10 were hypermethylated (32.3%) and this change was significantly found in the adult spinal ependymomas (p=0.01). KLF4 hypermethylation was observed in 6 of the overall included 35 samples (17.1%); however, there was no statistically significant relation of the methylation status with tumor subtype, histological grade or age group. RASSF1A hypermethylation was observed in overall 40 included samples with varying methylation levels. Higher levels of hypermethylation were significantly related to the grade 3 histology (p=0.01) and spinal ependymomas (p=0.006). The pediatric cases with grade 3 ependymomas and ependymomas of adulthood showed significantly increased RASSF1A hypermethylation levels (p < 0.001 and p=0.001, respectively).</p><p><strong>Conclusion: </strong>DNA methylation changes are likely to have biological importance in ependymomas. Both ZIC2 and RASSF1A methylation status may be useful parameters in the subclassification of these tumors.</p>","PeriodicalId":45415,"journal":{"name":"Turkish Journal of Pathology","volume":"38 3","pages":"213-218"},"PeriodicalIF":1.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10508420/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39773284","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Turkish Journal of Pathology
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