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A peculiar uncommon cutaneous neoplasia: Angiosarcoma of the head and neck of the elderly (Wilson Jones Angiosarcoma) 一种罕见的皮肤肿瘤:老年人头颈部血管肉瘤(威尔逊·琼斯血管肉瘤)
Pub Date : 2019-07-19 DOI: 10.5430/CRCP.V6N1P9
A. D'Amuri, Federica Floccari, R. Filotico, M. Filotico
Cutaneous Angiosarcoma of the elderly's face is a rare and peculiar neoplasm with all its own clinical and morphological characteristics. The observation of a classic case in the clinical presentation and morphology has offered the opportunity to revisit this topic.
摘要老年人面部皮肤血管肉瘤是一种罕见而独特的肿瘤,具有独特的临床和形态学特征。在临床表现和形态学的一个经典病例的观察提供了机会,重新审视这个话题。
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引用次数: 0
Eosinophilic peritonitis in a patient with liver cirrhosis: A case report 肝硬化患者嗜酸性腹膜炎1例
Pub Date : 2019-06-04 DOI: 10.5430/CRCP.V6N1P5
M-H Pang, F. Hua, Yixiao Zhi, E. Qin, Y. Tao, Rui Hua
Eosinophilic gastroenteritis (EG) is a gastrointestinal disease characterized by abnormal infiltration of eosinophilic cells in the gastrointestinal tract, excluding known causes of eosinophilia. Eosinophilic peritonitis (EP) is rare and is considered by most scholars to be a systemic or local allergy to exogenous or endogenous allergens. It is a clinical manifestation of eosinophilic gastroenteritis involving the serosal layer. Hereby, we report a case of EP in a patient with liver cirrhosis. A 32-year-old man was admitted to our hospital for intermittent fatigue, abdominal distension and abdominal pain. On account of clinical feature and pathological results of peritoneal puncture biopsy, excluding other causes of peripheral eosinophilia, the diagnosis of EP with hepatic cirrhosis was established. The possibility of EP should be paid great attention to patients with cirrhosis with peritonitis. Gastrointestinal endoscope biopsy, laparoscopy or peritoneal puncture biopsy are conducive to the diagnosis and differential diagnosis. Therefore, once the disease is suspected, gastrointestinal endoscope biopsy should be performed actively, and multiple pathological samples should be taken to contribute to diagnosis and treatment. Laparoscopy or peritoneal puncture biopsy is of vital significance for definitive diagnosis. 
嗜酸性肠胃炎(EG)是一种胃肠道疾病,其特征是胃肠道中嗜酸性细胞异常浸润,不包括已知的嗜酸性粒细胞增多原因。嗜酸性腹膜炎(EP)是罕见的,大多数学者认为是对外源性或内源性过敏原的全身或局部过敏。这是一种涉及浆膜的嗜酸性肠胃炎的临床表现。在此,我们报告一例肝硬化患者的EP。一名32岁的男子因间歇性疲劳、腹胀和腹痛入院。根据腹膜穿刺活检的临床特点和病理结果,排除其他外周嗜酸性粒细胞增多的原因,确定EP合并肝硬化的诊断。肝硬化合并腹膜炎患者应高度重视EP的可能性。胃肠镜活检、腹腔镜或腹膜穿刺活检有利于诊断和鉴别诊断。因此,一旦怀疑疾病,应积极进行胃肠道内窥镜活检,并采集多个病理样本,为诊断和治疗做出贡献。腹腔镜或腹膜穿刺活检对明确诊断具有重要意义。
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引用次数: 0
Acute transaminitis as an atypical presentation of Graft-versus-Host Disease following Hematopoietic Stem Cell Transplantation 急性转氨炎是造血干细胞移植后移植物抗宿主病的不典型表现
Pub Date : 2019-03-25 DOI: 10.5430/CRCP.V6N1P1
R. Ramkissoon, A. Shetty, Adam C Doyle, O. Adeyi, K. Patel
Graft versus Host Disease (GVHD) can present with mucocutaneous, gastrointestinal and hepatic manifestations, specifically a cholestatic transaminitis. Rarely, some cases can present with only a hepatocellular transaminitis. Our patient presented with an acute hepatitis on day +90 post-hematopoietic stem cell transplant, without other overt manifestations of GVHD. The initial work up was negative for a viral etiology or causative drug, and the patient’s transaminases continued to rise. On day +96, an erythematous rash appeared with biopsy indicating lymphocytic and eosinophilic infiltrates concerning for cutaneous GVHD. Subsequently, a liver biopsy was obtained, and showed marked ductopenia with cholestasis, consistent with hepatic GVHD. 
移植物抗宿主病(GVHD)可表现为粘膜皮肤、胃肠道和肝脏表现,特别是胆汁淤积性转氨酶。极少数情况下,某些病例仅表现为肝细胞转氨酶。我们的患者在造血干细胞移植后第+90天出现急性肝炎,没有其他明显的移植物抗宿主病表现。最初的检查对病毒病因或致病药物呈阴性,患者的转氨酶持续升高。第+96天,出现红斑性皮疹,活检显示淋巴细胞和嗜酸性粒细胞浸润,与皮肤移植物抗宿主病有关。随后,进行了肝活检,结果显示明显的胆管开放伴胆汁淤积,与肝移植物抗宿主病一致。
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引用次数: 0
Clear cell renal cell carcinoma with heterotopic bone formation: A case report and literature review 透明细胞肾细胞癌伴异位成骨1例并文献复习
Pub Date : 2019-01-15 DOI: 10.5430/CRCP.V5N2P16
Estelle Oertling, Cacey Peters, R. Wood, J. Silberstein, Andrew B. Sholl, Nadja K. Falk
The phenomenon of heterotopic bone formation (osseous metaplasia) is defined as an abnormal ossification of non-skeletal tissues and does represent a rare occurrence in the renal cell carcinoma setting. We describe a case of a 40-year old man with bilateral renal cell carcinomas of the histological clear cell subtype, with the right-sided renal cell carcinoma demonstrating heterotopic bone formation, as well as the presence of intratumoral adipose tissue. The etiology of bone formation in a renal cell carcinoma is unclear, but possible explanations include a response to tissue ischemia and the expression of Bone Morphogenetic Protein 2. The detection of these rare morphologic variations is of paramount importance, not to be mistaken as sarcomatoid transformation and renal sinus fat invasion, which would advance the pathologic tumor stage and aggressiveness of the disease. 
异位骨形成(骨性化生)的现象被定义为非骨骼组织的异常骨化,在肾细胞癌中确实罕见发生。我们报告一例40岁男性双侧肾细胞癌组织学透明细胞亚型,右侧肾细胞癌表现为异位骨形成,以及肿瘤内脂肪组织的存在。肾细胞癌骨形成的病因尚不清楚,但可能的解释包括对组织缺血的反应和骨形态发生蛋白2的表达。这些罕见的形态学变化的检测是至关重要的,不要被误认为是肉瘤样转化和肾窦脂肪浸润,这将提前肿瘤的病理分期和疾病的侵袭性。
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引用次数: 0
Angiolymphoid hyperplasia with eosinophilia: An uncommon presentation and novel treatment of a rare disease 血管淋巴样增生伴嗜酸性粒细胞增多:一种罕见疾病的罕见表现和新的治疗方法
Pub Date : 2018-12-30 DOI: 10.5430/CRCP.V5N2P12
D. Surprenant, Amanda Goslawski, Karen C Kagha, D. Griffin, K. Mudaliar, K. Hutchens, D. Opel, R. Tung
Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign vascular proliferation characterized by isolated or grouped red-brown papules or nodules, most often located on the head or neck. Treatment of ALHE is difficult, with high recurrence rates reported across a myriad of different interventions. We report an atypical clinical presentation of ALHE that was successfully treated with a novel dual-therapeutic approach consisting of surgical excision followed by treatment with the pulsed dye laser.
血管淋巴增生伴嗜酸性粒细胞增多症(ALHE)是一种罕见的良性血管增生,其特征是孤立或聚集的红棕色丘疹或结节,最常见于头部或颈部。ALHE的治疗很困难,在各种不同的干预措施中,复发率很高。我们报告了ALHE的非典型临床表现,该表现通过一种新的双重治疗方法成功治疗,该方法包括手术切除和脉冲染料激光治疗。
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引用次数: 1
SDHB mutated paraganglioma imitating thyroid tumor: A case report and review of literature SDHB突变的模仿甲状腺肿瘤的副神经节瘤1例报告及文献复习
Pub Date : 2018-08-28 DOI: 10.5430/CRCP.V5N2P5
Vygantė Maskoliūnaitė, G. Makunaite, A. Garnelytė, A. Abraitienė, V. Beisa
Paragangliomas of the head and neck are uncommon tumors arising from parasympathetic ganglia. Paragangliomas are mostly asymptomatic and may manifest as palpable mass of neck. The morphologic features are non-specific and comparable to the other neuroendocrine tumors. Most of hereditary cases are associated with alterations in genes of succinate dehydrogenase (SDH). SDHA and SDHB immunohistochemistry is considered as reliable screening method to detect tumors with genetical alterations. Of note, SDHB mutated paragangliomas have the highest risk of local recurrence, distant metastasis and the development of other tumor phenotypes, which are associated with mutation. Therefore, active surveillance of patients and early surgical treatment are essential. In contrast, SDHB mutated head and neck paragangliomas was considered as completely benign tumors, although, the latest literature describes more controversial cases, which may increase awareness. Here, we present a rare case of 21 years old female with asymptomatic neck paraganglioma, which was unexpectedly diagnosed after pathological and immunohistochemical testing of removed thyroid gland and showed unusual immunohistochemical variation for SDH mutation.
头颈部副神经节瘤是由副交感神经节引起的罕见肿瘤。副神经节瘤大多无症状,可表现为颈部可触及的肿块。其形态学特征是非特异性的,可与其他神经内分泌肿瘤相比较。大多数遗传性病例与琥珀酸脱氢酶(SDH)基因的改变有关。SDHA和SDHB免疫组织化学被认为是检测遗传改变肿瘤的可靠筛查方法。值得注意的是,SDHB突变的副神经节瘤具有最高的局部复发、远处转移和其他肿瘤表型发展的风险,这些表型与突变有关。因此,对患者进行积极监测和早期手术治疗至关重要。相比之下,SDHB突变的头颈部副神经节瘤被认为是完全良性的肿瘤,尽管最新文献描述了更多有争议的病例,这可能会提高人们的认识。在此,我们报告了一例罕见的21岁女性无症状颈部副神经节瘤病例,该病例在切除甲状腺的病理和免疫组织化学检测后意外诊断,并显示SDH突变的异常免疫组织化学变化。
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引用次数: 1
Cellular schwannoma of the breast: A case report and review of literature 乳腺细胞神经鞘瘤1例报告及文献复习
Pub Date : 2018-07-09 DOI: 10.5430/CRCP.V5N2P1
Yan Zhang, Hongyi Gao, An-qin Zhang, Jiang-yu Zhang, Kun-he Wu, Yi Wang
Schwannoma of breast is a relatively uncommon disease characterized by painless and slow growing. We report a case of a 28-year-old female who present a mass of the right breast. Ultrasonography revealed a well-circumscribed and inhomogeneous hypoechoic mass under the skin of the breast. Then a biopsy and immunohistochemical analysis were performed. The results were suggestive for cellular schwannoma of breast. The tumour was removed successfully and the patient has been followed-up for six months with no evidence of recurrence. Cellular schwannoma is a rare benign neoplasm without metastasis. Distinction cellular schwannoma from other malignant tumours is very important. It is necessary to combine strict criteria on histological, immunohistochemical analysis, biological behavior, image examination and clinical features for the final diagnosis.
摘要乳腺神经鞘瘤是一种较少见的疾病,其特点是无痛、生长缓慢。我们报告一例28岁女性右乳房肿块。超声检查显示乳腺皮肤下有一界限清楚、不均匀的低回声肿块。然后进行活检和免疫组织化学分析。提示乳腺细胞性神经鞘瘤。肿瘤成功切除,患者随访6个月,无复发迹象。细胞神经鞘瘤是一种罕见的无转移的良性肿瘤。将细胞神经鞘瘤与其他恶性肿瘤区分开来是非常重要的。需要结合严格的组织学、免疫组化分析、生物学行为、影像学检查和临床特征进行最终诊断。
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引用次数: 1
Hemosiderotic fibrolipomatous tumor: A poorly known entity 含铁血黄素纤维脂肪瘤:一个鲜为人知的实体
Pub Date : 2018-06-28 DOI: 10.5430/crcp.v5n1p23
S. Chaieb, M. Njima, N. B. Abdeljelil, A. Bellalah, S. Chouchane, A. Zakhama, A. Moussa
Hemosiderotic fibrohistiocytic lipomatous tumor (HFLT) is a rare and supposedly benign fibrolipomatous entity. Clinical and imaging features are not specific. So, histologic, immunohistochemical and molecular analyses are required for diagnosis. The clinic and pathologic aspects of this lesion are diversely reported. Moreover, its reactive or neoplastic origin is still under debate. This lesion should be treated by complete excision. It is mandatory to be aware of its tendency to harbor higher-grade lesions with aggressive outcome. We report a case of HFLT affecting the dorsum of the third right finger in a 52-year-old women, in order to describe histologic patterns of this tumor and to discuss the current literature regarding the etiopathogeny and the cytogenetic features of this rare entity.
含铁血黄素的纤维组织细胞脂肪瘤(HFLT)是一种罕见的良性纤维脂肪瘤。临床和影像学特征并不具体。因此,诊断需要组织学、免疫组织化学和分子分析。这种病变的临床和病理方面有不同的报道。此外,其反应性或肿瘤起源仍在争论中。这种病变应该通过完全切除来治疗。必须意识到其倾向于携带具有侵袭性结果的更高级别病变。我们报告了一例影响52岁女性右手第三指背的HFLT病例,以描述该肿瘤的组织学模式,并讨论有关该罕见实体的病因和细胞遗传学特征的现有文献。
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引用次数: 0
Case report of acquired hemophagocytic lymphohistiocytosis associated with progression of monoclonal gammopathy of unknown significance (MGUS) 获得性噬血细胞性淋巴组织细胞增多症伴不明意义单克隆γ病进展1例报告
Pub Date : 2018-06-21 DOI: 10.5430/CRCP.V5N1P19
Li Liu, L. Contis, O. Palmer
Background: Acquired hemophagocytic lymphohistiocytosis (HLH) is an aggressive hyperinflammatory syndrome. Without prompt diagnosis and proper treatment, it can be life-threatening. HLH is commonly present in the setting of other autoimmune disorders, infection, organ transplantation, and malignancy.  However, to our knowledge, HLH associated with monoclonal gammopathy of undetermined significance (MGUS) has not been reported.Case presentation: A 67-year-old woman with an extensive history of MGUS and renal transplant presented with progressive fatigue, weight loss, intermittent fevers, splenomegaly, and pancytopenia. Serum protein electrophoresis with immunofixation identified a new monoclonal protein IgG lambda and a rapidly increasing pre-existing free kappa light chain. A bone marrow biopsy revealed focal clusters of kappa restricted plasma cells which comprised less than 10% of marrow cellularity. Skeletal survey was negative for osteolytic lesions. She was also free of any end-organ damage.  Histological examination showed a prominent increase in histiocytes and macrophages, many of which show erythrophagocytosis and lymphophagocytosis. Together with her clinical presentation along with a hyperferritinemia, a diagnosis of HLH was confirmed utilizing the criteria of the Histiocyte Society. The patient underwent a splenectomy. Prompt treatment with etoposide and high dose dexamethasone eventually stabilized the patient and resulted in a full recovery, which coincided with the disappearance of the serum monoclonal IgG lambda.Conclusions: This report reveals a novel association of HLH with the progression of MGUS. Familiarity with this syndrome and its association with other conditions is indicated to ensure prompt diagnosis and appropriate patient treatment.
背景:获得性噬血细胞性淋巴组织细胞增多症(HLH)是一种侵袭性高炎症综合征。如果没有及时的诊断和适当的治疗,它可能会危及生命。HLH常见于其他自身免疫性疾病、感染、器官移植和恶性肿瘤。然而,据我们所知,尚未报道HLH与意义不明的单克隆gammopathy(MGUS)相关。病例介绍:一名67岁女性,有广泛的MGUS和肾移植病史,表现为进行性疲劳、体重减轻、间歇性发烧、脾肿大和全血细胞减少。免疫固定的血清蛋白电泳鉴定了一种新的单克隆蛋白IgGλ和一种快速增加的预先存在的游离κ轻链。骨髓活检显示局部聚集的κ-限制性浆细胞,其占骨髓细胞数的比例不到10%。骨骼调查显示溶骨性病变为阴性。她也没有任何末端器官损伤。组织学检查显示组织细胞和巨噬细胞显著增加,其中许多表现为红吞噬细胞和淋巴吞噬细胞。结合她的临床表现和高铁蛋白血症,利用组织细胞学会的标准确认了HLH的诊断。病人接受了脾切除术。依托泊苷和大剂量地塞米松的及时治疗最终使患者稳定下来,并导致完全康复,这与血清单克隆IgGλ的消失相吻合。结论:本报告揭示了HLH与MGUS进展的新联系。熟悉该综合征及其与其他疾病的关联,以确保及时诊断和适当的患者治疗。
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引用次数: 0
Urachal mucinous neoplasms--to be or not to be: Two cases and a review of current diagnostic criteria (WHO 2016) 脐尿管粘液性肿瘤——是或不是:两例病例和当前诊断标准的回顾(世界卫生组织2016)
Pub Date : 2018-05-28 DOI: 10.5430/CRCP.V5N1P12
J. Cuadra, L. Pérez, I. García, M. Álvarez, Bernardo Herrera, I. Hierro, L. Vicioso
The urachus is an embryological remnant that extends from the bladder to the umbilicus. Urachal neoplasms represent less than 1% of malignant bladder tumors. The new 2016 edition of disease classifications from the WHO has updated the classification of epithelial neoplasms of urachal origin, distinguishing between glandular and non-glandular neoplasms and between cystic and non-cystic adenocarcinomas. In this article, we present two cases with similar clinical presentations but differing histology. We address their morphological, immunohistochemical, and molecular characterization; review the classification and diagnostic criteria of glandular urachal neoplasms included in the most recent literature with the aim of clarifying their differential diagnosis as well as their prognostic implication; and, ultimately, better understand these infrequent biological entities.
脐尿管是从膀胱延伸到脐部的胚胎残体。脐尿管肿瘤在膀胱恶性肿瘤中所占比例不到1%。世界卫生组织2016年新版疾病分类更新了脐尿管源性上皮肿瘤的分类,区分了腺性和非腺性肿瘤以及囊性和非囊性腺癌。在这篇文章中,我们介绍了两个临床表现相似但组织学不同的病例。我们讨论了它们的形态学、免疫组织化学和分子特征;回顾最新文献中腺脐尿管肿瘤的分类和诊断标准,以阐明其鉴别诊断及其预后意义;最终,更好地理解这些罕见的生物实体。
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引用次数: 0
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Case reports in clinical pathology
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