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Renal Neoplasms with Concurrent Castleman-Like Regional Lymphadenopathy. 并发卡斯特曼样区域淋巴腺病的肾肿瘤
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-30 DOI: 10.1177/10668969241271421
Alcino Gama, Ruoji Zhou, Ivan De La Riva-Morales, Jeffrey Sosman, Bogdan Isaila, Qing C Chen, Xiaoqi Lin, Bonnie Choy, Yi-Hua Chen, Ximing J Yang

Introduction: Renal cell neoplasms are known to be associated with paraneoplastic syndromes, and the association with Castleman-like regional lymphadenopathy has been rarely reported. We aim to characterize the association between renal neoplasms and Castleman-like lymphadenopathy.

Methods: A search for renal neoplasms with concurrent Castleman-like lymphadenopathy in one single medical institution from 2000 to 2023 resulted in 4 specimens. A literature search for "Castleman" and "renal neoplasm" resulted in 8 reports. Patients' demographics, clinical presentation, gross and histologic features, results of ancillary studies, treatment, and follow-up were evaluated.

Results: Our patients included 3 men and 1 woman, with a mean age of 60 years. Four different subtypes of renal neoplasms were diagnosed, including clear cell renal cell carcinoma (RCC), papillary RCC, chromophobe RCC, and mucinous cystadenoma of the renal pelvis. For Castleman-like regional lymphadenopathy, 2 were plasma-cell predominant, and 2 were hyaline-vascular. After a median follow-up of 84 months, all patients were alive with no recurrence or progression of Castleman-like features following nephrectomies.

Conclusion: Castleman-like regional lymphadenopathy should be considered in patients with renal tumors and lymphadenopathy. Although more prevalent in clear cell RCC, it can be also associated with other renal neoplasms. The concurrent lymphadenopathy was remitted following the renal tumor resections.

简介众所周知,肾细胞瘤与副肿瘤综合征有关,而与卡斯特曼样区域淋巴结病有关的报道却很少。我们旨在描述肾肿瘤与卡斯特曼样淋巴结病之间的关联:方法:在一家医疗机构检索 2000 年至 2023 年期间并发 Castleman 样淋巴结病的肾肿瘤,共获得 4 份标本。以 "Castleman "和 "肾肿瘤 "为关键词进行文献检索,共获得 8 篇报道。我们对患者的人口统计学、临床表现、大体和组织学特征、辅助检查结果、治疗和随访进行了评估:我们的患者包括 3 名男性和 1 名女性,平均年龄为 60 岁。诊断出四种不同亚型的肾肿瘤,包括透明细胞肾细胞癌(RCC)、乳头状肾细胞癌(RCC)、嗜色细胞肾细胞癌(RCC)和肾盂粘液性囊腺瘤。在 Castleman 样区域淋巴结病中,2 例以浆细胞为主,2 例为透明血管性。中位随访84个月后,所有患者均存活,肾切除术后无复发或卡斯特曼样特征进展:结论:患有肾肿瘤和淋巴结病的患者应考虑卡斯特曼样区域淋巴结病。结论:患有肾肿瘤和淋巴结病的患者应考虑卡斯特曼样区域淋巴结病,虽然这种病在透明细胞RCC中更为常见,但也可能与其他肾肿瘤有关。肾肿瘤切除术后,并发淋巴结病得到了缓解。
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引用次数: 0
A Lymphoepithelioma-Like Intrahepatic Cholangiocarcinoma With Massive Multinucleated Giant Cell Reaction. 伴有大量多核巨细胞反应的淋巴上皮细胞瘤样肝内胆管癌
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-30 DOI: 10.1177/10668969241268392
Fang Luyao, Zhang Xiangnan, Min Qiaoyun, Zhang Hui, Meng Huanping, Xu Tianwen, Zhao Huanfen

Lymphoepithelioma-like intrahepatic cholangiocarcinoma (LEL-ICC) is a rare liver tumor that appears as a hepatic nodule on imaging with a specific pathological pattern, and the definitive diagnosis relies on its pathological histomorphology, immunophenotype, and Epstein-Barr encoding region test. Radical surgical resection is the primary treatment modality, and immunotherapy is expected to be a new adjuvant treatment option. LEL-ICC with massive multinucleated giant cell infiltration has not been reported so far. In this article, we report a patient with LEL-ICC showing massive multinucleated giant cell infiltration, review the relevant literature, and analyze its clinicopathological features and prognosis to accumulate experience for the accurate diagnosis of LEL-ICC.

淋巴上皮瘤样肝内胆管癌(LEL-ICC)是一种罕见的肝脏肿瘤,在影像学上表现为肝结节,具有特殊的病理形态,明确诊断依赖于其病理组织形态学、免疫表型和 Epstein-Barr 编码区检测。根治性手术切除是主要的治疗方式,免疫疗法有望成为一种新的辅助治疗选择。伴有大量多核巨细胞浸润的 LEL-ICC 迄今为止尚未见报道。本文报告了一名出现大量多核巨细胞浸润的LEL-ICC患者,回顾了相关文献,分析了其临床病理特征和预后,为准确诊断LEL-ICC积累了经验。
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引用次数: 0
Clinicopathologic Insights and Molecular Oncogenesis: Understanding Epstein-Barr Virus-Induced B-cell Lymphoproliferations. 临床病理学见解与分子肿瘤发生:了解 Epstein-Barr 病毒诱导的 B 细胞淋巴增生。
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-21 DOI: 10.1177/10668969241266933
Adam S Bronson, Yuanzhe Zhu, Cullen M Lilley, Genevieve M Crane, Kamran M Mirza

Epstein-Barr virus (EBV) is a highly prevalent virus among adults worldwide. In an immunocompetent individual, EBV infection generally results in lifelong latency of the virus and no sequelae. However, in the setting of immune dysfunction, EBV can induce the development of autoimmune disorders, hyperplastic proliferations, and cancers, including lymphoma. Here, we explore the pathogenic and oncogenic role of EBV in Burkitt lymphoma, diffuse large B-cell lymphoma, Hodgkin lymphoma, plasmablastic lymphoma, lymphomatoid granulomatosis, and post-transplant lymphoproliferative disorders and lymphoproliferative disorders associated with immune deficiency and dysregulation. In addition to describing general mechanisms of EBV-associated oncogenesis, we also discuss EBV-associated oncogenesis in the context of each disorder, as well as their microscopic, phenotypic, and clinical presentations.

Epstein-Barr 病毒(EBV)是一种在全球成年人中高度流行的病毒。在免疫功能正常的个体中,EB 病毒感染通常会导致病毒终生潜伏,不会留下后遗症。然而,在免疫功能失调的情况下,EBV 可诱发自身免疫性疾病、增生性增殖和癌症,包括淋巴瘤。在此,我们探讨了 EBV 在伯基特淋巴瘤、弥漫大 B 细胞淋巴瘤、霍奇金淋巴瘤、浆细胞性淋巴瘤、淋巴瘤样肉芽肿病、移植后淋巴增生性疾病以及与免疫缺陷和失调相关的淋巴增生性疾病中的致病和致癌作用。除了介绍 EBV 相关肿瘤发生的一般机制外,我们还结合每种疾病讨论了 EBV 相关肿瘤发生,以及它们的显微、表型和临床表现。
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引用次数: 0
A Rare Placental Site Nodule Involving the Ovary. 罕见的胎盘部位结节累及卵巢
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-21 DOI: 10.1177/10668969241269974
Saroja Devi Geetha, Sudarshana Roychoudhury, Sunder Sham, Alexander M Truskinovsky

Placental site nodule (PSN) is a benign lesion representing a nodular aggregate of intermediate trophoblast, embedded in a hyalinized stroma, thought to arise from noninvoluted placental site remaining from a past gestation. Uterus is the most common site of PSN. Occurrence in extrauterine sites is rare, with most examples being reported in the fallopian tubes. Here we report an example of PSN in the ovary. A 35-year-old woman, gravida 4, para 1, with history of adnexal ectopic pregnancy treated with methotrexate, at 39 weeks and 1 day of a subsequent pregnancy, underwent a scheduled C-section. The surgery was successful, and a healthy female infant was delivered. Intraoperative adnexal inspection revealed a small pedunculated mass on the right ovary, which was excised and sent for pathological examination. Gross inspection showed a soft, tan-white tissue fragment measuring 2.0 × 1.0 × 0.2 cm. Microscopic examination showed epithelioid cells with hyperchromatic, mildly atypical nuclei and abundant eosinophilic cytoplasm, embedded in a hyalinized stroma, forming a nodule. A diagnosis of placental site nodule was made. Immunohistochemical stains for keratin AE1/AE3, vimentin, and inhibin were strongly positive in the epithelioid cells, and immunostain for p63 was focally positive, supporting the diagnosis. PSN of the ovary is extremely rare. To our knowledge, there has been only one reported patient in the literature so far. Extrauterine PSNs are thought to arise from ectopic pregnancies. Our patient's ovarian PSN is most likely a consequence of her previous adnexal ectopic pregnancy, which was treated medically.

胎盘部位结节(PSN)是一种良性病变,是中间滋养细胞的结节状聚集,嵌入透明质基质中,被认为是由过去妊娠遗留的非膨胀性胎盘部位产生的。子宫是 PSN 最常见的部位。发生在子宫外部位的病例很少见,大多数病例发生在输卵管。在此,我们报告一例卵巢 PSN。一名 35 岁的女性,孕酮 4,1 级,曾有附件异位妊娠病史,接受过甲氨蝶呤治疗,在妊娠 39 周零 1 天时接受了预定的剖腹产手术。手术很成功,产下一名健康女婴。术中附件检查发现右侧卵巢上有一个小蒂状肿块,切除后送去做病理检查。大体检查显示有一个棕白色的软组织碎片,大小为 2.0 × 1.0 × 0.2 厘米。显微镜检查显示,上皮样细胞核色素沉着,轻度不典型,胞质大量嗜酸性,包埋在透明基质中,形成结节。诊断为胎盘部位结节。上皮样细胞中角蛋白 AE1/AE3、波形蛋白和抑制素的免疫组化染色呈强阳性,p63 的免疫染色呈局灶性阳性,支持诊断。卵巢 PSN 极其罕见。据我们所知,迄今为止文献中仅报道过一名患者。宫外 PSN 被认为是由异位妊娠引起的。我们这位患者的卵巢 PSN 很可能是她之前的附件异位妊娠的后遗症,当时她接受了药物治疗。
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引用次数: 0
AI-Based Computational H&E Staining Enables Spatial Transcriptomic Analysis in Classic Hodgkin Lymphoma. 基于人工智能的 H&E 染色计算可实现经典霍奇金淋巴瘤的空间转录组分析
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-21 DOI: 10.1177/10668969241268410
Michael E Kallen, Laura M Wake, Rima Koka, Elba Vidal, Raymond Kozikowski, Yair Rivenson, Serge Alexanian
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引用次数: 0
Intrinsic Causes of Nonfibrotic Portal Hypertension-A Clinicopathologic Review of 56 Patients. 非纤维化门静脉高压症的内在原因--56 例患者的临床病理学回顾。
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-21 DOI: 10.1177/10668969241268406
Hamza Ashmila, Nazia Khatoon, Andrew Keaveny, Muli Krishna, Raouf Nakhleh

Aim: The differential diagnosis of intrinsic nonfibrotic conditions that may lead to portal hypertension include hepatoportal sclerosis (HPS), nodular regenerative hyperplasia (NRH), and sinusoidal obstruction syndrome (SOS). In this article, we characterize the clinical features and outcome of these lesions when they manifest as portal hypertension. Methods: Data was collected through retrospective patient medical records. Results: Patients (HPS: 28, NRH: 17, SOS: 11) were identified more frequently in recent years. All groups presented with signs and symptoms of portal hypertension. All patients had complex medical histories. An elevated serum alkaline phosphatase occurred in all groups and an elevated bilirubin with SOS. Imaging of the liver with HPS and NRH suggested cirrhosis, which was not seen with SOS. 11%, 12%, and 9% of patients in the HPS, NRH, and SOS respectively, underwent transjugular intrahepatic portosystemic shunt placement to manage the complications of portal hypertension, while 43%, 24%, and 36% of patients respectively, received a liver transplant. Conclusions: Patients with HPS, NRH, and SOS had complex medical histories, likely contributing to the development of these lesions. They are recognized more frequently now. In contrast to HPS and NRH, SOS occurred in liver transplant recipients, was associated with elevated serum bilirubin, and imaging did not suggest the presence of advanced fibrosis/cirrhosis. Liver transplantation appeared to be a viable treatment for complications related to HPS and NRH. Retransplantation for SOS yielded mixed results. HPS, SOS, and NRH should be considered when evaluating liver specimens from patients with unexplained nonfibrotic portal hypertension. Key message: Intrinsic nonfibrotic causes of portal hypertension appear to be increasing in frequency. The differential diagnosis includes NRH, HPS, and SOS. These conditions are associated with complex diseases and possibly due to treatments. Pathologists need to be aware of this differential diagnosis when presented with liver biopsies performed to assess portal hypertension.

目的:可能导致门静脉高压的内在非纤维化病变的鉴别诊断包括肝门静脉硬化症(HPS)、结节性再生增生(NRH)和窦道阻塞综合征(SOS)。在本文中,我们将描述这些病变表现为门脉高压症时的临床特征和预后。研究方法通过回顾性病历收集数据。结果患者(HPS:28 例;NRH:17 例;SOS:11 例)的发病率近年来有所上升。所有组别均出现门静脉高压的体征和症状。所有患者都有复杂的病史。所有组别患者的血清碱性磷酸酶均升高,SOS患者的胆红素升高。HPS 和 NRH 的肝脏成像提示肝硬化,而 SOS 则未发现肝硬化。在 HPS、NRH 和 SOS 组中,分别有 11%、12% 和 9% 的患者接受了经颈静脉肝内门体分流术,以控制门脉高压并发症,而分别有 43%、24% 和 36% 的患者接受了肝移植。结论HPS、NRH 和 SOS 患者病史复杂,可能是导致这些病变发生的原因。现在,这些病变被更多人所认识。与HPS和NRH不同的是,SOS发生在肝移植受者身上,与血清胆红素升高有关,而且影像学检查并未提示存在晚期纤维化/肝硬化。肝移植似乎是治疗 HPS 和 NRH 相关并发症的一种可行方法。针对 SOS 的再移植结果不一。在评估不明原因的非纤维化门脉高压症患者的肝脏标本时,应考虑到HPS、SOS和NRH。关键信息:非纤维化性门脉高压的内在原因似乎越来越多。鉴别诊断包括 NRH、HPS 和 SOS。这些病症与复杂的疾病相关,也可能是由于治疗所致。病理学家在进行肝活检以评估门静脉高压时,需要注意这种鉴别诊断。
{"title":"Intrinsic Causes of Nonfibrotic Portal Hypertension-A Clinicopathologic Review of 56 Patients.","authors":"Hamza Ashmila, Nazia Khatoon, Andrew Keaveny, Muli Krishna, Raouf Nakhleh","doi":"10.1177/10668969241268406","DOIUrl":"https://doi.org/10.1177/10668969241268406","url":null,"abstract":"<p><p><b>Aim:</b> The differential diagnosis of intrinsic nonfibrotic conditions that may lead to portal hypertension include hepatoportal sclerosis (HPS), nodular regenerative hyperplasia (NRH), and sinusoidal obstruction syndrome (SOS). In this article, we characterize the clinical features and outcome of these lesions when they manifest as portal hypertension. <b>Methods:</b> Data was collected through retrospective patient medical records. <b>Results:</b> Patients (HPS: 28, NRH: 17, SOS: 11) were identified more frequently in recent years. All groups presented with signs and symptoms of portal hypertension. All patients had complex medical histories. An elevated serum alkaline phosphatase occurred in all groups and an elevated bilirubin with SOS. Imaging of the liver with HPS and NRH suggested cirrhosis, which was not seen with SOS. 11%, 12%, and 9% of patients in the HPS, NRH, and SOS respectively, underwent transjugular intrahepatic portosystemic shunt placement to manage the complications of portal hypertension, while 43%, 24%, and 36% of patients respectively, received a liver transplant. <b>Conclusions:</b> Patients with HPS, NRH, and SOS had complex medical histories, likely contributing to the development of these lesions. They are recognized more frequently now. In contrast to HPS and NRH, SOS occurred in liver transplant recipients, was associated with elevated serum bilirubin, and imaging did not suggest the presence of advanced fibrosis/cirrhosis. Liver transplantation appeared to be a viable treatment for complications related to HPS and NRH. Retransplantation for SOS yielded mixed results. HPS, SOS, and NRH should be considered when evaluating liver specimens from patients with unexplained nonfibrotic portal hypertension. <b>Key message:</b> Intrinsic nonfibrotic causes of portal hypertension appear to be increasing in frequency. The differential diagnosis includes NRH, HPS, and SOS. These conditions are associated with complex diseases and possibly due to treatments. Pathologists need to be aware of this differential diagnosis when presented with liver biopsies performed to assess portal hypertension.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142008791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cowper Glands Identified in Prostate and Urethral Specimens: A Comprehensive Immunohistochemical Characterization and Potential Diagnostic Pitfall. 前列腺和尿道标本中发现的 Cowper 腺:全面的免疫组化特征和潜在的诊断陷阱。
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-21 DOI: 10.1177/10668969241268375
Ankur R Sangoi, Khaleel I Al-Obaidy, Mahmut Akgul, Rohit Mehra, Emily Chan, Sean R Williamson

Cowper glands recognition remains one of the key histoanatomic benign mimics of prostatic adenocarcinoma. In most instances, these can be identified based on the dimorphic population of lobulated acini and duct(s). However, in the prostate biopsy setting with incomplete/distorted cores, this may not be immediately apparent and may warrant use of immunohistochemistry to argue against prostatic adenocarcinoma. Although immunohistochemical pitfalls in Cowper glands have been described, to our knowledge a comprehensive evaluation of both traditional and purportedly prostate-specific novel markers in Cowper glands has not been previously performed. Herein, we studied the clinicopathological and immunohistochemical features of 21 male patients (age range 39-81 years; mean = 63 years), including 15 prostate biopsies (7 of which also had prostate cancer in the same specimen set and 2 of which had both prostate cancer and Cowper glands in the same biopsy core). Immunohistochemistry showed the following results in Cowper glands: 100% positive for NKX3.1, 100% positive (basal cells) for both high molecular weight keratin and p63, 57% positive for PSAP, 25% positive for PSMA, 5% positive for AMACR, and 0% positive for PSA. In conclusion, for specimens lacking appreciable dimorphic morphology, caution should be rendered when using prostate-specific markers (PSA, PSAP, PSMA, and NKX3.1) as these can show considerable staining in Cowper glands and be a pitfall. Instead, findings from this cohort indicate relying on basal markers (high molecular weight keratin/p63; either individually or in a "cocktail" approach) and PSA are most useful in distinguishing Cowper glands (retained basal cell markers staining) from prostatic adenocarcinoma.

Cowper腺的识别仍然是前列腺腺癌的主要组织解剖学良性假象之一。在大多数情况下,可以根据分叶状腺体和导管的二形群体来识别这些腺体。然而,在前列腺活检中,由于取芯不完整/扭曲,这种情况可能不会立即显现,因此可能需要使用免疫组化方法来论证前列腺腺癌。虽然已经描述了 Cowper 腺的免疫组化误区,但据我们所知,以前还没有对 Cowper 腺的传统标记物和据称是前列腺特异性的新型标记物进行过全面评估。在此,我们研究了 21 例男性患者(年龄在 39-81 岁之间;平均 = 63 岁)的临床病理和免疫组化特征,包括 15 例前列腺活检样本(其中 7 例在同一标本集中同时存在前列腺癌,2 例在同一活检核心中同时存在前列腺癌和 Cowper 腺)。免疫组化在考伯氏腺体中显示出以下结果:NKX3.1阳性率为100%,高分子量角蛋白和p63阳性率均为100%(基底细胞),PSAP阳性率为57%,PSMA阳性率为25%,AMACR阳性率为5%,PSA阳性率为0%。总之,对于缺乏明显二形形态的标本,在使用前列腺特异性标记物(PSA、PSAP、PSMA 和 NKX3.1)时应谨慎,因为这些标记物可能会在 Cowper 腺体中显示大量染色,从而造成误诊。相反,该队列的研究结果表明,依靠基底标志物(高分子量角蛋白/p63;可单独使用或以 "鸡尾酒 "方法使用)和 PSA 最有助于区分 Cowper 腺(保留基底细胞标志物染色)和前列腺腺癌。
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引用次数: 0
Urothelial Carcinoma with Trophoblastic Differentiation-A Report with Review. 滋养细胞分化的尿路上皮癌--报告与综述
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-19 DOI: 10.1177/10668969241266934
Monica Mishra, Pavithra Ayyanar, Sagar Ranjan Tripathy, Manoj Kumar Das

Background: Urothelial carcinoma with trophoblastic differentiation is a rare type of urothelial carcinoma that poses a diagnostic challenge.

Case details: A 50-year-old man presented with hematuria for 4 months duration. Ultrasonography examination showed polypoidal lesions along the right lateral wall and near the right vesicoureteric junction of the urinary bladder. The transurethral resection of the bladder tumor (TURBT) specimen showed marked necrosis and urothelial tumor cells arranged in nests, sheets, and papillae, admixed with multinucleated large cells. Deep muscle and extensive lymphovascular invasion were noted. The tumor cells were diffuse immunopositive for GATA3 and focal positive for p63 and SALL4. Large multinucleated tumor cells were immunopositive for β-hCG, GATA3, inhibin-α, and PLAP, focally positive for SALL4 while negative for p63. The patient denied further treatment and succumbed to the disease after 8 months of the TURBT procedure.

Conclusion: We report a rare invasive urothelial carcinoma with trophoblastic differentiation and discuss the differential diagnosis and literature review.

背景:具有滋养细胞分化的尿路上皮癌是一种罕见的尿路上皮癌,给诊断带来了挑战:病例详情:一名 50 岁男子出现血尿 4 个月。超声波检查显示,膀胱右侧壁和右侧膀胱输尿管交界处附近有息肉样病变。经尿道膀胱肿瘤切除术(TURBT)标本显示肿瘤明显坏死,尿路肿瘤细胞呈巢状、片状和乳头状排列,并混有多核大细胞。深部肌肉和淋巴管广泛受侵。肿瘤细胞的 GATA3 呈弥漫性免疫阳性,p63 和 SALL4 呈局灶性阳性。大的多核肿瘤细胞β-hCG、GATA3、抑制素-α和PLAP免疫阳性,SALL4局灶阳性,p63阴性。患者拒绝进一步治疗,在接受 TURBT 手术 8 个月后死亡:我们报告了一例罕见的伴滋养细胞分化的浸润性尿路上皮癌,并讨论了鉴别诊断和文献综述。
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引用次数: 0
Isolated Monoclonal T-Cell Receptor Gene Rearrangement in a Lung Adenocarcinoma Harboring MET Exon 14 Skipping: Diagnostic Pitfall. 肺腺癌中分离出的单克隆 T 细胞受体基因重排(携带 MET 第 14 号外显子缺失):诊断陷阱。
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-18 DOI: 10.1177/10668969241266927
Wangpan Jackson Shi, Joshua Nguyen, Wei Song, Huan-You Wang, Grace Y Lin, Oluwole Fadare, Li Lei

In the diagnostic workup of poorly differentiated tumors, T-cell receptor (TCR) clonality has long been considered as evidence of T-cell lymphoma. MET exon 14 skipping (METex14) is a mutation typically seen in lung adenocarcinoma. Herein, we present the first report of METex14 lung adenocarcinoma with isolated monoclonal TCRγ gene rearrangement. A 69-year-old woman presented to an outside hospital with pleural effusions. A pleural decortication demonstrated malignant cells positive for CD30 and CD138 but negative for BerEP4, KRT5, and EMA. An equivocal HHV8 staining was interpreted as positive, leading to the erroneous outside diagnosis of primary effusion lymphoma. Additional workup at our institution revealed a lack of HHV8 and T-cell markers but the presence of TCRγ clonality, pankeratin, and TTF1 expression. Repeat TCRγ testing on the in-house biopsy was negative for clonality. Next-generation sequencing detected METex14, confirming the diagnosis of lung adenocarcinoma. The potential diagnostic pitfall and prognostic/predictive implications are discussed.

在分化不良肿瘤的诊断工作中,T 细胞受体(TCR)克隆性一直被认为是 T 细胞淋巴瘤的证据。MET外显子14缺失(METex14)是肺腺癌中常见的一种突变。在此,我们首次报告了 METex14 肺腺癌伴孤立的单克隆 TCRγ 基因重排。一名 69 岁的妇女因胸腔积液到外院就诊。胸膜剥离术显示恶性细胞 CD30 和 CD138 阳性,但 BerEP4、KRT5 和 EMA 阴性。HHV8 染色结果不明确,被解释为阳性,因此被误诊为原发性积液淋巴瘤。在本院进行的进一步检查发现,患者体内缺乏 HHV8 和 T 细胞标记物,但存在 TCRγ 克隆、pankeratin 和 TTF1 表达。对内部活检进行的重复TCRγ检测结果显示克隆性阴性。下一代测序检测出 METex14,确诊为肺腺癌。本文讨论了潜在的诊断误区和预后/预测意义。
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引用次数: 0
Variations in Epithelial Lining of Duplication Cysts Cannot be Explained by Known Theories. 复制囊肿上皮内膜的变异无法用已知理论解释
IF 0.9 4区 医学 Q4 PATHOLOGY Pub Date : 2024-08-16 DOI: 10.1177/10668969241265041
Servet Melike Akıncı, Özlem Boybeyi, Diclehan Orhan, Tutku Soyer

Objectives. Duplication cysts are found in any part of the gastrointestinal tract from the oropharynx to anus. Although duplication cysts usually have similar epithelium with the adjacent organ, respiratory epithelium in the enteric duplication cysts is rarely reported. This study was performed to evaluate the variations in the epithelial lining of duplication cysts and its clinical implications. Methods. Patients diagnosed with duplication cysts between 2012 and 2022 were retrospectively reviewed to assess their histopathological results, clinical aspects, treatment options, and demographic characteristics. Results. Twenty-five patients were included. The mean age was 4 years, male-to-female ratio was 15:10. The localizations of duplication cysts were ileum (n = 12), duodenum(n = 4), stomach(n = 3), jejunum(n = 2), colon(n = 2), thoracoabdominal(n = 2), and rectum(n = 1). Most common presentation was abdominal pain (36%). Intestinal (48%) and gastric (40%) epithelia were the most common finding. Four patients (16%) had respiratory epithelium: two in foregut duplication cysts, one each in ileal, and rectal duplications. Twenty-four percentage of patients had associated anomalies. Surgical interventions were performed on 22 patients. Conclusion. Duplication cysts show a wide range of epithelial linings. Respiratory epithelium can be found in not only foregut duplication cysts but also midgut and hindgut duplication cysts. Although the presence of respiratory epithelium did not have any impact on clinical findings, none of the previous theories explain the presence of respiratory epithelium different from foregut duplications.

目的。从口咽到肛门,消化道的任何部位都有重复囊肿。虽然重复囊肿的上皮通常与邻近器官相似,但肠重复囊肿的呼吸道上皮却鲜有报道。本研究旨在评估重复囊肿上皮内膜的变化及其临床意义。研究方法回顾性研究2012年至2022年期间确诊的重复囊肿患者,评估其组织病理学结果、临床表现、治疗方案和人口统计学特征。结果共纳入 25 例患者。平均年龄为4岁,男女比例为15:10。重复囊肿的部位包括回肠(12 例)、十二指肠(4 例)、胃(3 例)、空肠(2 例)、结肠(2 例)、胸腹(2 例)和直肠(1 例)。最常见的表现是腹痛(36%)。最常见的发现是肠上皮(48%)和胃上皮(40%)。四名患者(16%)有呼吸道上皮:前肠重复囊肿两名,回肠和直肠重复囊肿各一名。24%的患者伴有异常。22 名患者接受了手术治疗。结论重复囊肿的上皮衬里种类繁多。呼吸道上皮不仅可见于前肠重复囊肿,也可见于中肠和后肠重复囊肿。虽然呼吸道上皮的存在对临床结果没有任何影响,但之前的理论都无法解释呼吸道上皮的存在为何不同于前肠重复囊肿。
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International Journal of Surgical Pathology
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