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Primary Appendiceal Gastrointestinal Stromal Tumor 原发性阑尾胃肠道间质瘤
Pub Date : 2021-12-21 DOI: 10.1097/PCR.0000000000000410
Joseph S. Kim, A. Jhala, Kristen M. Stashek, R. Tondon
Abstract Primary appendiceal gastrointestinal stromal tumor is extremely rare. To our knowledge, only 17 cases have been reported so far. Presented is a case of an 84-year-old woman who underwent elective laparoscopic right partial colectomy for an ileocecal mass found during colonoscopy. On gross examination, a 0.8-cm well-defined tan-white nodule was present in the distal portion of the appendix. Histology of the nodule showed proliferation of irregular fascicles of bland spindle cells, which stained positive for DOG1 (Ano1) and CD117 (KIT) and negative for S-100 and SMA. We report the incidental primary gastrointestinal stromal tumor of the appendix and discuss its biological behavior.
摘要原发性阑尾胃肠道间质瘤极为罕见。据我们所知,到目前为止只报告了17例。本文报告一位84岁妇女,因结肠镜检查发现回盲肿块,接受选择性腹腔镜右结肠部分切除术。大体检查,在阑尾远端可见一0.8 cm清晰的棕白色结节。结节的组织学表现为不规则的淡梭形细胞束的增生,DOG1 (Ano1)和CD117 (KIT)染色呈阳性,S-100和SMA呈阴性。我们报告偶发的原发性胃肠道间质肿瘤的阑尾和讨论其生物学行为。
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引用次数: 0
Too Young for Clear Cell Sarcoma of the Kidney? A Case Report With Review of Differential Considerations 过早得肾透明细胞肉瘤?一个病例报告,回顾不同的考虑
Pub Date : 2021-11-01 DOI: 10.1097/PCR.0000000000000408
D. Russell
Abstract Clear cell sarcoma of the kidney (CCSK) comprises 3% of all childhood renal cancers. Accurate diagnosis is vital for appropriate therapy, which results in a 70% to 90% overall survival rate in this previously lethal tumor. Renowned for its ability to mimic and be mimicked by every other pediatric renal tumor, and even some extrarenal retroperitoneal tumors, CCSK has a unique metastatic pattern and molecular aberrations, as well as a generally consistent clinical presentation which is of great utility in the differential. A case of CCSK in a 4-month-old boy is presented, only the fifth case reported in a patient younger than 6 months.
肾透明细胞肉瘤(CCSK)占所有儿童肾癌的3%。准确的诊断对于适当的治疗至关重要,这使得这种以前致命的肿瘤的总生存率达到70%至90%。CCSK以其模仿和被模仿的能力而闻名于世,它具有独特的转移模式和分子畸变,以及普遍一致的临床表现,这在鉴别中非常有用。一例CCSK在一个4个月大的男孩提出,只有第五例报告的患者年龄小于6个月。
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引用次数: 0
Mucinous Cystic Neoplasm of the Liver With Associated Neuroendocrine Carcinoma: A Case Report 肝脏粘液囊性肿瘤伴神经内分泌癌1例报告
Pub Date : 2021-11-01 DOI: 10.1097/PCR.0000000000000470
Ane Kongsgaard, K. Brudvik, T. Syversveen, H. Reims
Abstract Mucinous cystic neoplasms (MCNs) of the liver are relatively rare tumors that constitute less than 5% of liver cysts and are subcategorized into low/intermediate- and high-grade lesions based on the degree of epithelial dysplasia. An associated invasive carcinoma component occurs in 6% of cases, usually as adenocarcinoma with tubular growth. The liver is a frequent metastatic site for neuroendocrine carcinomas (NECs), whereas primary hepatic NECs are extremely rare. We present the case of a 63-year-old woman with a history of choledochal cyst, who now presented with abdominal pain. Computed tomography showed a multilocular cystic lesion in the liver. The cyst was surgically enucleated, and the histological examination revealed an MCN with low-grade dysplasia, with an associated invasive large cell NEC. To our knowledge, this is the first report in the literature of a biliary MCN with associated NEC.
肝脏粘液囊性肿瘤(mcn)是一种相对罕见的肿瘤,占肝囊肿的不到5%,根据上皮发育不良的程度又分为低/中、高级别病变。6%的病例伴有浸润性癌,通常为腺癌伴管状生长。肝脏是神经内分泌癌(NECs)的常见转移部位,而原发性肝脏NECs极为罕见。我们提出的情况下,一个63岁的妇女胆总管囊肿的历史,谁现在提出腹痛。计算机断层扫描显示肝脏多房囊性病变。手术切除囊肿,组织学检查显示MCN伴低级别发育不良,伴浸润性大细胞NEC。据我们所知,这是文献中首次报道胆道MCN合并NEC。
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引用次数: 0
Uterine Sarcoma With a Novel WWOX-NTRK2 Fusion in a Postmenopausal Woman With Li-Fraumeni–Like Syndrome: A Case That Expands the Spectrum of NTRK-Rearranged Uterine Tumors 绝经后li - fraumeni样综合征女性子宫肉瘤伴新型WWOX-NTRK2融合:一例扩展了ntrk重排子宫肿瘤谱系的病例
Pub Date : 2021-11-01 DOI: 10.1097/PCR.0000000000000476
Michelle Moh, Colin M. Johnson, Jennifer L. Geurts, E. Bishop
Abstract NTRK-rearranged tumors are being increasingly recognized and targeted with TRK inhibitor therapies. A novel NTRK2 fusion–positive uterine sarcoma arising in a patient with Li-Fraumeni–like syndrome is described in this article.
ntrk重排肿瘤正越来越多地被TRK抑制剂治疗所识别和靶向。一种新的NTRK2融合阳性子宫肉瘤出现在患者与li - fraumeni样综合征在这篇文章中进行了描述。
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引用次数: 3
Primary Peritoneal Ependymoma: A Case Report and Literature Review 原发性腹膜室管膜瘤1例报告及文献复习
Pub Date : 2021-11-01 DOI: 10.1097/PCR.0000000000000462
J. Vazzano, B. Swanson, P. Wakely
Abstract Extra-axial ependymoma is a rare neoplasm. Reported anatomic sites include the ovary, para-ovarian tissues, pelvis, paracervical soft tissue, lung, omentum, small bowel, and posterior mediastinum. Primary peritoneal ependymomas are extremely rare. We report such an example in a 58-year-old woman who presented with abdominal pain, bloating, and history of a 70-lb weight loss over the prior year. Computed tomography scan showed a 20-cm pelvic mass extending into the abdomen and a 3.5-cm peritoneal-based right hepatic lobe/diaphragm mass. Histologic examination of the pelvic mass revealed an ovarian mucinous cystadenoma. The peritoneal mass showed a highly cellular solid neoplasm containing perivascular pseudorosettes and true ependymal rosettes. Tumor cell nuclei were rounded, oval, and elongated with coarse, granular chromatin and occasional small nucleoli. Mitoses were rare. Immunophenotype showed positive staining with bcl-2 and GFAP and scattered staining with keratins MNF116, CAM5.2, and cytokeratin AE1/AE3, along with chromogranin and EMA, but negative staining with inhibin, HMB45, SOX10, TLE-1, CD10, synaptophysin, Olig2, CD34, CD117, STAT-6, smooth muscle actin, and S-100. Conventional cytogenetic analysis showed normal chromosomes. Foundation One molecular profiling showed no actionable mutations. Ependymoma is an entity that rarely exists outside the central neuraxis having no connection with its central nervous system counterpart. Pathologic diagnosis is based primarily on presence of unequivocal ependymal differentiation.
摘要轴外室管膜瘤是一种罕见的肿瘤。已报道的解剖部位包括卵巢、卵巢旁组织、骨盆、宫颈旁软组织、肺、大网膜、小肠和后纵隔。原发性腹膜室管膜瘤极为罕见。我们报告一位58岁的女性患者,她表现为腹痛、腹胀,并且在过去一年中体重减轻了70磅。计算机断层扫描显示一个20厘米的盆腔肿块延伸到腹部,一个3.5厘米的基于腹膜的右肝叶/膈肿块。盆腔肿块的组织学检查显示为卵巢粘液囊腺瘤。腹膜肿块表现为高度细胞性的实体肿瘤,包含血管周围假玫瑰花和真室管膜玫瑰花。肿瘤细胞核呈圆形、卵圆形、细长状,染色质粗粒状,偶见小核仁。有丝分裂很少见。免疫表型显示bcl-2、GFAP阳性,角蛋白MNF116、CAM5.2、细胞角蛋白AE1/AE3、色素粒蛋白和EMA分散染色,抑制素、HMB45、SOX10、TLE-1、CD10、synaptophysin、Olig2、CD34、CD117、STAT-6、平滑肌肌动蛋白、S-100阴性。常规细胞遗传学分析显示染色体正常。基础1分子分析显示没有可操作的突变。室管膜瘤是一种很少存在于中枢神经轴外的实体,与中枢神经系统没有联系。病理诊断主要是基于存在明确的室管膜分化。
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引用次数: 0
Composite Renal Cell Carcinoma and Angiomyolipoma: A Rare Case and Possible Diagnostic Pitfalls 复合肾细胞癌和血管平滑肌脂肪瘤:一个罕见的病例和可能的诊断缺陷
Pub Date : 2021-11-01 DOI: 10.1097/PCR.0000000000000461
C. Dehner, Jennifer K Sehn
Abstract Angiomyolipomas are benign renal neoplasms commonly associated with tuberous sclerosis complex (Semin Diagn Pathol 1998;15(1):21–40). However, angiomyolipoma with concurrent renal cell neoplasia is very uncommon, with fewer than 50 reported cases. Even less common is direct admixture of renal cell carcinoma (RCC) and angiomyolipoma within 1 tumor mass, with only rare possible cases reported in the literature. A multi-institutional study of 36 cases of synchronous renal cell neoplasm and angiomyolipoma found that clear cell RCC was the most common concurrent cancer in patients with sporadic or tuberous sclerosis-associated angiomyolipoma; angiomyolipoma in these cases was incidental (Mod Pathol 2001;14(3):157–163). Here, we report the exceptionally uncommon occurrence of a composite RCC and angiomyolipoma, occurring as an intimate admixture within a single tumor mass.
血管平滑肌脂肪瘤是一种良性肾脏肿瘤,通常与结节性硬化症(Semin Diagn Pathol 1998;15(1): 21-40)有关。然而,血管平滑肌脂肪瘤并发肾细胞瘤是非常罕见的,报道的病例不到50例。在一个肿瘤块内直接合并肾细胞癌(RCC)和血管平滑肌脂肪瘤的情况就更少见了,文献中仅有罕见的可能病例报道。一项对36例同步肾细胞肿瘤和血管平滑肌脂肪瘤的多机构研究发现,透明细胞肾细胞癌是散发性或结节性硬化症相关血管平滑肌脂肪瘤患者中最常见的并发癌症;血管平滑肌脂肪瘤在这些病例中是偶然的(现代病理学杂志2001;14(3):157-163)。在这里,我们报告罕见的复合RCC和血管平滑肌脂肪瘤,发生在一个肿瘤肿块内的亲密混合物。
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引用次数: 0
Sinonasal Ameloblastoma: A Case Report and a Review of Gnathic Lesions That Occur in the Sinonasal Tract 鼻窦成釉细胞瘤:一例报告并回顾发生在鼻窦道的颌骨病变
Pub Date : 2021-07-01 DOI: 10.1097/PCR.0000000000000455
Rachel M. Whitehair, E. B. Stelow
Abstract Primary sinonasal ameloblastomas are rare, benign odontogenic lesions that are locally aggressive. We report a case of a primary sinonasal ameloblastoma in a 65-year-old man who presented with sinonasal obstruction and rhinorrhea. The clinicopathologic features of this entity are discussed, along with the differential diagnosis and other gnathic lesions that present as sinonasal lesions.
摘要原发性鼻窦成釉细胞瘤是一种罕见的、局部侵袭性的良性牙源性病变。我们报告一例原发性鼻窦成釉细胞瘤在一个65岁的男人谁提出鼻窦阻塞和鼻漏。本文讨论了该实体的临床病理特征,以及鉴别诊断和其他表现为鼻窦病变的颌骨病变。
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引用次数: 0
Human Papillomavirus–Related Multiphenotypic Sinonasal Carcinoma 人乳头瘤病毒相关的多表型鼻窦癌
Pub Date : 2021-07-01 DOI: 10.1097/PCR.0000000000000465
M. P. Crawford, E. Stelow
Abstract Human papillomavirus–related mulitphenotypic sinonasal carcinoma is a recently described high-grade malignancy demonstrating significant morphologic overlap with adenoid cystic carcinoma and other salivary-type malignancies. We present a prototypical case of human papillomavirus–related mulitphenotypic sinonasal carcinoma in a 51-year-old woman and discuss the diagnosis, as well as the differential diagnosis.
人乳头瘤病毒相关的多表型鼻窦癌是最近发现的一种与腺样囊性癌和其他唾液型恶性肿瘤在形态学上有明显重叠的高级别恶性肿瘤。我们报告一例51岁女性人乳头瘤病毒相关多表型鼻窦癌的典型病例,并讨论其诊断及鉴别诊断。
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引用次数: 1
Sinonasal Lymphoma: Extranodal Natural Killer/T-Cell Lymphoma and Its Differential Diagnosis 鼻窦淋巴瘤:结外自然杀伤/ t细胞淋巴瘤及其鉴别诊断
Pub Date : 2021-07-01 DOI: 10.1097/PCR.0000000000000458
M. Girton, N. Aguilera, Henry R Bateman
Abstract Lymphoma is the second most common malignancy in the nasal tract and paranasal sinuses after squamous cell carcinoma (Acta Oncol 1997;36:45–50). Sinonasal lymphoma is the most common hematolymphoid lesion at the site, accounting for approximately 1% of all lymphomas (Virchows Arch A Pathol Anat Histopathol 1989;414:399–405). These neoplasms are diverse, including B cell, T cell, and natural killer cell differentiation. This group of lymphomas includes chronic and aggressive entities, some with clearly defined etiologies and associations including Epstein-Barr virus, HIV, and human T-cell leukemia virus infections, with variable differentiation and distinctive growth patterns. In this report, we will review the most prevalent entities with a focus on extranodal natural killer/T-cell lymphoma. A sinonasal lymphoma differential diagnosis is provided with review of the epidemiologic, histomorphologic, and immunophenotypic characteristics and discuss diagnostic pitfalls. The discussion is accompanied by representative micrographs, with an explanation of the particular features of each diagnosis. Lastly, we will briefly discuss important clinical characteristics.
淋巴瘤是鼻道和鼻窦中仅次于鳞状细胞癌的第二常见的恶性肿瘤(Acta Oncol 1997; 36:45-50)。鼻窦淋巴瘤是该部位最常见的血淋巴样病变,约占所有淋巴瘤的1% (Virchows Arch A Pathol Anat Histopathol 1989; 414:399-405)。这些肿瘤是多种多样的,包括B细胞、T细胞和自然杀伤细胞分化。这组淋巴瘤包括慢性和侵袭性实体,其中一些具有明确的病因和关联,包括爱泼斯坦-巴尔病毒、HIV和人类t细胞白血病病毒感染,具有不同的分化和独特的生长模式。在本报告中,我们将回顾最普遍的实体,重点是结外自然杀伤/ t细胞淋巴瘤。一个鼻窦淋巴瘤的鉴别诊断提供了回顾流行病学,组织形态学和免疫表型的特点,并讨论诊断陷阱。讨论附有代表性的显微照片,并解释了每种诊断的特定特征。最后,我们将简要讨论重要的临床特征。
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引用次数: 0
Low-Grade Biphenotypic Sinonasal Sarcoma: Case Report, Current Understanding, and Differential Diagnosis 低级别双表型鼻窦肉瘤:病例报告,目前的认识和鉴别诊断
Pub Date : 2021-07-01 DOI: 10.1097/PCR.0000000000000453
J. Coppock, E. Stelow
Abstract Low-grade biphenotypic sinonasal sarcoma (BSS) is a rare, locally aggressive mesenchymal neoplasm of the sinonasal tract defined by dual expression of neural and myogenic antigens and recurrent PAX3 (paired box gene 3) gene rearrangements. We herein present the case of a 53-year-old woman with an incidentally identified lesion on surveillance magnetic resonance imaging for a pituitary microadenoma, ultimately leading to biopsy and resection of a low-grade BSS. Because of its relatively indolent behavior and extensive differential diagnosis, recognition and appropriate diagnostic workup of this entity are of utmost importance. The current understanding of low-grade BSS, its diagnosis, and differential diagnosis in the sinonasal tract are discussed.
摘要低级别双表型鼻窦肉瘤(BSS)是一种罕见的局部侵袭性鼻窦间充质肿瘤,其特征是神经和肌源性抗原的双重表达以及PAX3(配对盒基因3)基因重排的复发性。我们在此报告一位53岁女性的病例,她在监测磁共振成像中偶然发现了垂体微腺瘤的病变,最终导致了低级别BSS的活检和切除。由于其相对惰性的行为和广泛的鉴别诊断,认识和适当的诊断工作是至关重要的。本文讨论了目前对低级别BSS的认识、诊断和鼻窦的鉴别诊断。
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引用次数: 1
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AJSP: reviews & reports
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