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Placental Chorangiocarcinoma: Case Report with Literature Review of a Rare Entity. 胎盘绒毛管癌:一例罕见实体的病例报告及文献复习。
IF 1 Q3 Medicine Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01548
Nishant Sagar, Parul Tanwar, Nita Khurana, Poonam Kashyap

Chorangiocarcinoma is an extremely rare tumor seen in the placenta, with only six cases reported in the literature so far. Its morphological characteristics, criteria for diagnosis, and the pathophysiology remain controversial to date. Although it was predominantly considered a benign entity, a solitary case of distant metastasis has been reported in the literature. We present a case of this unusual tumor in the preterm placenta of a 29-year-old female. Grossly seen as a grey white nodule, microscopic examination revealed nests of atypical trophoblastic proliferation surrounded by vascularized stroma. No evidence of basement membrane invasion was noted. On immunohistochemistry, the trophoblastic component expressed pancytokeratin, Beta HCG, and Placental Alkaline Phosphatase with high Ki-67 labelling index. The present case highlights this exceedingly rare entity with emphasis on its morpho-immunohistochemical features along with a review of literature.

绒毛管癌是一种极为罕见的胎盘肿瘤,迄今为止文献中仅报道了6例。其形态学特征、诊断标准和病理生理学至今仍有争议。尽管它主要被认为是一种良性实体,但文献中报道了一例远处转移的孤立病例。我们报告了一例29岁女性早产胎盘中的这种不寻常肿瘤。肉眼可见灰白色结节,显微镜检查显示非典型滋养层增生巢被血管化基质包围。没有发现基底膜侵犯的证据。在免疫组织化学上,滋养层成分表达具有高Ki-67标记指数的泛细胞角蛋白、β-HCG和胎盘碱性磷酸酶。本病例强调了这种极其罕见的实体,重点介绍了其形态免疫组织化学特征以及文献综述。
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引用次数: 1
Multifocal Pseudomyogenic Hemangioendothelioma Involving the Scalp and Nose, Misdiagnosed as A Sarcoma: A Rare Case Report. 多灶性假性血管内皮瘤累及头皮及鼻子,误诊为肉瘤1例。
IF 1 Q3 Medicine Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01539
Neha Mittal, Bharat Rekhi, Priyamvada Singhal, Munita Bal, Swapnil Rane, Asawari Patil, Shivakumar Thiagarajan

This case report aims to present clinicopathological features of an extremely rare case of multifocal pseudomyogenic hemangioendothelioma (PMHE) in the scalp. A 21-year-old male developed multiple, focally ulcerated, nodules over the root of his nose and scalp. One of the skin lesions was sampled at another dermatology clinic, where this was diagnosed as a sarcoma. A review of biopsy sections showed well-circumscribed dermal lesions, comprising plump spindle and epithelioid cells, mimicking rhabdomyoblasts. Immunohistochemically, tumor cells were positive for AE1/AE3, CD31, FLI-1 and ERG. INI-1 was retained. A diagnosis of PMHE was offered. Subsequently, the patient underwent wide excision and has been asymptomatic for 8 months, post-surgery. PMHE is rarely reported in the head and neck region, where it can constitute a diagnostic pitfall. Awareness of this tumor and appropriate immunohistochemical stains are necessary for its timely diagnosis, in order to avoid radical treatments. A review of similar, previously documented cases is presented.

本病例报告旨在报告一个极为罕见的头皮多灶性假肌原性血管内皮瘤(PMHE)的临床病理特征。一名21岁男性在其鼻根和头皮上出现多发局灶性溃疡结节。其中一个皮肤病变是在另一个皮肤科诊所取样的,在那里被诊断为肉瘤。活检切片复查显示界限分明的真皮病变,包括丰满的梭形细胞和上皮样细胞,模仿横纹肌母细胞。免疫组化结果显示,肿瘤细胞AE1/AE3、CD31、fl -1、ERG均呈阳性。i -1被保留。诊断为PMHE。随后,患者接受了大范围切除,术后8个月无症状。PMHE很少报告在头颈部,在那里它可以构成一个诊断陷阱。认识这种肿瘤和适当的免疫组织化学染色是必要的,以及时诊断,以避免根治治疗。对以前记录的类似病例进行了审查。
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引用次数: 0
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 Q3 Medicine 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
HHV-8 and EBV Positive Lymphoproliferative Disease: A Challenging Case. HHV-8和EBV阳性淋巴增生性疾病:一个具有挑战性的病例。
IF 1 Q3 Medicine Pub Date : 2022-01-01 DOI: 10.5146/tjpath.2021.01540
Göksenil Bülbül, Gülen Gül, Mehmet Ali Özcan, Sermin Özkal

Human herpes virus-8 (HHV-8) is linked to four lymphoproliferative diseases: primary effusion lymphoma, HHV-8 positive multicentric Castleman disease (MCD), HHV-8 positive diffuse large B cell lymphoma and HHV-8 positive germinotropic lymphoproliferative disorder (GLPD). The diagnosis of HHV-8 associated lymphoproliferative diseases is quite challenging because each entity is rare and has a wide morphological spectrum. Our aim is to emphasize the overlapping histopathological features of MCD and GLPD as well as to underline the importance of clinicopathological correlation in case these two entities cannot be distinguished by pathological examination. We present here a case of an 82-year-old male patient who was examined for weight loss and multiple lymphadenopathy. Histopathological examination of the axillary lymph node revealed lymphoid follicle structures of varying shapes and sizes, including some atrophic germinal centers. Plasmablast-like cells were notable in some of these areas. HHV-8 and Epstein Barr Virus (EBV) positivity were noted in some of these cells and in a small number of cells in the mantle zone. Based on these findings; a diagnosis of "HHV-8 and EBV positive lymphoproliferative disease" was established. Since HHV-8 positive MCD and GLPD have similar histopathological features, it may not be possible to distinguish these two entities by histopathological examination only. At this point, the importance of clinicopathological correlation becomes more evident, especially in the determination of the treatment protocol to be applied to the patient.

人类疱疹病毒-8 (HHV-8)与四种淋巴增生性疾病有关:原发性积液性淋巴瘤、HHV-8阳性多中心Castleman病(MCD)、HHV-8阳性弥漫性大B细胞淋巴瘤和HHV-8阳性生发性淋巴增生性疾病(GLPD)。HHV-8相关淋巴细胞增生性疾病的诊断是相当具有挑战性的,因为每个实体是罕见的,具有广泛的形态谱。我们的目的是强调MCD和GLPD的重叠组织病理特征,以及强调临床病理相关性的重要性,以防这两种实体无法通过病理检查区分。我们在这里提出一个82岁的男性病人谁检查体重减轻和多发性淋巴结病。腋窝淋巴结的组织病理学检查显示不同形状和大小的淋巴滤泡结构,包括一些萎缩的生发中心。其中一些区域可见浆母细胞样细胞。其中部分细胞和少数地幔区细胞呈HHV-8和eb病毒(EBV)阳性。基于这些发现;诊断为“HHV-8和EBV阳性淋巴增生性疾病”。由于HHV-8阳性MCD和GLPD具有相似的组织病理学特征,因此仅通过组织病理学检查可能无法区分这两种实体。在这一点上,临床病理相关性的重要性变得更加明显,特别是在确定适用于患者的治疗方案时。
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引用次数: 1
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 Q3 Medicine 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
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 Q3 Medicine 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。
{"title":"Eosinophilic Solid and Cystic Renal Cell Carcinoma: From Unclassified to Classified, A Case Report.","authors":"Rashim Sharma,&nbsp;Balamurugan Thirunavukkarasu,&nbsp;Poonam Elhence,&nbsp;Mahaveer Singh Rodha,&nbsp;Binit Sureka","doi":"10.5146/tjpath.2021.01531","DOIUrl":"https://doi.org/10.5146/tjpath.2021.01531","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":45415,"journal":{"name":"Turkish Journal of Pathology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9999703/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9325320","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: 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 Q3 Medicine 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
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 Q3 Medicine 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。
{"title":"Clinico-Histological Features of Thrombotic Microangiopathy in Renal Biopsies: A Retrospective Study.","authors":"Niraimathi Manickam,&nbsp;Vinita Agrawal,&nbsp;Pallavi Prasad,&nbsp;Manoj Jain,&nbsp;Narayan Prasad","doi":"10.5146/tjpath.2021.01536","DOIUrl":"https://doi.org/10.5146/tjpath.2021.01536","url":null,"abstract":"<p><strong>Objective: </strong>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.</p><p><strong>Material and method: </strong>A retrospective evaluation of renal biopsies for TMA over 5.5 years was performed. Clinical and laboratory data was collected from patient records.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":45415,"journal":{"name":"Turkish Journal of Pathology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9999687/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9332266","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
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 Q3 Medicine 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。
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引用次数: 2
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 Q3 Medicine 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。
{"title":"The Significance of Histopathologic Assessment in Bone Marrow Disease in Neuroblastoma.","authors":"Sumeyye Ekmekci,&nbsp;Dilek Ince,&nbsp;Nur Olgun,&nbsp;Erdener Ozer","doi":"10.5146/tjpath.2021.01556","DOIUrl":"https://doi.org/10.5146/tjpath.2021.01556","url":null,"abstract":"<p><strong>Objective: </strong>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.</p><p><strong>Material and method: </strong>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.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":45415,"journal":{"name":"Turkish Journal of Pathology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9999680/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9687416","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
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Turkish Journal of Pathology
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