胰腺囊实性乳头状上皮性肿瘤临床病理特点

目的评价胰腺囊实性乳头状上皮性肿瘤的临床病理特点及其治疗.方法分析我院2003年收治的经手术病理证实的2例胰腺囊实性乳头状上皮性肿瘤的临床病理资料,并复习国内外相关文献.结果该病好发于青年女性,临床表现主要为上腹部肿物,腹痛,上腹不适等,无特异表现,超声、CT、MRI等影像学检查有诊断价值,最终诊断依靠病理学检查.本组2例均行手术切除,疗效满意.结论胰腺囊实性乳头状上皮性肿瘤为低度恶性肿瘤,预后较好,治疗首选手术切除.     

食管炎性纤维性息肉的临床病理特征

目的:探讨食管炎性纤维性息肉(inflammatory fibroid polyp,IFP)的临床病理学特征、诊断、鉴别诊断、治疗方法和预后.方法:报道1例罕见的食管IFP,对其进行光镜观察、免疫组织化学染色,并结合文献探讨该病的临床病理学特征.结果:患者为71岁的男性,因进行性吞咽困难3mo余入院,内镜检查发现食管黏膜距门齿约35cm处可见一大小约2.5cm半球状黏膜隆起,超声内镜见一起源于黏膜层大小约13.2mm×11mm低回声团块,回声欠均匀,其间可见无回声影,边界尚清晰.入院完善相关检查后在全麻下行食管占位切除术,术后病理显示肿瘤位于黏膜下层,向食管管腔内隆起生长,肿瘤主要由3种成分构成:明显增生的梭形细胞、大量的炎症细胞和血管网,最后诊断为炎性纤维性息肉.结论:食管IFP是一种罕见的间叶源性肿瘤,术前诊断困难,确诊依靠术后病理检查.以外科手术或内镜下肿物完整切除为主要治疗方法,预后良好.     

原发性胰腺淋巴瘤诊疗体会

目的 探讨原发性胰腺淋巴瘤（PPL）的临床特点以及诊疗体会。方法回顾性分析5例原发性胰腺淋巴瘤的临床资料。结果本组5例患者中病灶位于胰头部3例,胰体尾2例。临床表现包括上腹痛、黄疸、发热、盗汗,体重减轻等。其中一例CAl9—9增高,其余肿瘤指标均处于正常范围。影像学检查均示胰腺癌可疑,2例行剖腹探查术、组织活检,2例行胰十二指肠切除术。1例行外周浅表淋巴结活检,均诊断为胰腺非霍奇金淋巴瘤。术后5例患者均予以CHOP（环磷酰胺、阿霉素、长春新碱、泼尼松）方案化疗。结论原发性胰腺淋巴瘤临床症状不典型,极易与胰腺癌混淆,影像学对诊断有提示作用,最终的诊断需要依靠病理学检查。治疗手段为手术切除术后加以化疗,无法切除的患者仅行化疗。胰腺原发淋巴瘤的预后明显优于胰腺癌。     

经超声内镜引导下细针穿刺确诊的11例胰腺转移性肿瘤的临床分析

目的分析胰腺转移性肿瘤的临床特征, 评估经超声内镜引导下细针穿刺或活检术(EUS-FNA/B)对其诊断的临床价值。方法回顾性分析2011年1月至2020年12月间海军军医大学第一附属医院经EUS-FNA/B确诊的11例胰腺转移性肿瘤的临床、影像学及病理资料, 记录患者的肿瘤大小、病灶数目、转移病灶距离原发灶诊断的时间间隔、影像学及EUS征象、病理类型等, 分析EUS-FNA/B成功率及诊断率。结果 11例胰腺转移性肿瘤患者年龄43～76岁, 男性7例, 女性4例;8例首发症状为腹痛、纳差, 1例颈部淋巴结肿大, 2例常规体检发现;5例血清肿瘤标志物异常。所有患者均有明确的原发肿瘤病史, 胰腺转移病灶距原发肿瘤确诊的中位时间间隔为24个月(-1～124个月)。EUS显示7例为单发性占位, 4例为多发性结节。8例临床首次诊断为胰腺占位或胰腺肿瘤, 3例考虑为胰腺转移性肿瘤。11例经EUS-FNA/B均获得组织条, 经组织病理学检查均确诊为胰腺转移性肿瘤, 病理类型以肺小细胞神经内分泌癌为主(4例), 其次为肾细胞癌(3例), 食管鳞状细胞癌、肺鳞状细胞癌、恶性黑色素瘤和胃腺癌各1例。结论胰...     

血栓弹力图评估胰腺癌患者凝血功能状态及鉴别诊断的应用

目的:探讨血栓弹力图(TEG)指标在评估胰腺癌患者凝血功能状态及其鉴别诊断的应用价值.方法:选取120例被确诊为胰腺肿瘤患者,根据2019版WHO胰腺肿瘤分类标准分为:胰腺癌患者组为试验组,共57例,其中低分化组21例、中高分化组36例;胰腺良性和低度恶性肿瘤患者组为良性对照组,共63例;另外选取64名健康体检者作为正...     

胰腺囊实性乳头状上皮性肿瘤临床病理特点

目的 评价胰腺囊实性乳头状上皮性肿瘤的临床病理特点及其治疗。方法 分析我院2003年收治的经手术病理证实的2例胰腺囊实性乳头状上皮性肿瘤的临床病理资料，并复习国内外相关文献。结果 该病好发于青年女性，临床表现主要为上腹部肿物，腹痛，上腹不适等，无特异表现，超声、CT、MRI等影像学检查有诊断价值，最终诊断依靠病理学检查。本组2例均行手术切除，疗效满意。结论 胰腺囊实性乳头状上皮性肿瘤为低度恶性肿瘤，预后较好，治疗首选手术切除。     

99mTc-HYNIC-TOC生长抑素受体显像诊断胰腺神经内分泌肿瘤的临床应用

传统的影像学检查方法(如CT、MRI)在胰腺神经内分泌肿瘤定位和分期中的价值有限.神经内分泌肿瘤表达生长抑素受体,因此应用放射性核素标记的生长抑素类似物能进行肿瘤显像.目的:评价99mTc-HYNIC-TOC生长抑素受体显像诊断和定位胰腺神经内分泌肿瘤的临床价值.方法:37例临床确诊或怀疑胰腺神经内分泌肿瘤患者行99mTc-HYNIC-TOC生长抑素受体显像,其中胰岛素瘤18例,胃泌素瘤4例,胰高血糖素瘤4例,未分类的胰腺神经内分泌肿瘤ll例.静脉注射99mTc-HYNIC-TOC 350～400 MBq后1、4 h行全身显像,部分阳性区域行局部显像或单光子发射计算体层摄影(SPECT)/CT.结果:37例患者中99mTc-HYNIC-TOC显像阳性27例,其中胰岛素瘤9例,胃泌素瘤4例,胰高血糖素瘤3例,未分类的胰腺神经内分泌肿瘤11例.99mTc-HYNIC-TOC显像诊断胰腺神经内分泌肿瘤的检出率为73.0%.99mTc-HYNIC-TOC显像改变了6例患者的肿瘤分期,并证实了7例患者的治疗效果.结论:99mTc-HYNIC-TOC显像可有效帮助诊断,定位胰腺神经内分泌肿瘤,指导分期,并辅助临床评价各种治疗方法的疗效.     

肝肉瘤样癌的临床病理特征

目的:探讨肝肉瘤样癌患者的临床病理学特征、诊断、鉴别诊断、治疗方法和预后.方法:以我院收治的1例肝肉瘤样癌患者为对象,分析其临床表现及诊疗经过,并进行随访,同时对送检标本进行病理学检查,结合有关文献探讨该病的临床病理学特点.结果:肿瘤位于肝脏右后叶,呈浸润性生长.组织学上可见上皮源性肿瘤成分与梭形细胞肉瘤成分同时存在并伴有移行现象;肿瘤中见大片凝固性坏死.免疫组织化学显示肿瘤细胞呈CK(Pan)和EMA强阳性,AFP和Hepar-1呈弱阳性表达,免疫组织化学阴性为:CK7,CK8,CK18,CK(H),CD34,CD117,Dog-1,Actin,SMA, Caldesmon,Desmin,CD10,CD21,CD23,CD35,CD1a,CD2,CD3,CD20,CD45, ALK,CD68,CD163,CD30,CD15,HMB45,S-100.病理诊断:肝肉瘤样癌.结论:肝肉瘤样癌是一种罕见的高度恶性肿瘤,临床与病理上需要与肝癌肉瘤、炎症性滤泡树突细胞肉瘤、炎性肌纤维母细胞肉瘤等相鉴别,预后与肿瘤分期有关.     

胰腺结核八例诊治分析

胰腺结核（pancreatic tuberculosis）临床少见,缺乏特异性临床表现,极易误诊为胰腺癌、胰腺囊性肿瘤、淋巴瘤、腹膜后肿瘤、胰腺炎、胰腺假性囊肿等。本研究回顾性分析胰腺结核患者的临床资料,并结合相关文献,探讨胰腺结核的诊断及治疗,旨在加深对胰腺结核的认识,提高其诊断水平。     

不同MR快扫序列对胰腺肿瘤的诊断价值

目的明确各种MR快扫序列对胰腺肿瘤的诊断价值,确定最佳扫描序列组合.方法对40名临床怀疑胰腺占位患者行MR检查,扫描序列包括:FS FLASH T1WI、TSE T2WI、True-FISPT2WI、MRCP及多时相动态增强3-D FLASH T1WI.测量正常胰腺与病灶的信号强度,计算胰腺-肿瘤的CNR,对各序列图像质量进行评分.最后,将各序列MRI的诊断和评价结果与手术病理相对照.结果胰腺-肿瘤的CNR:多时相动态增强3-D FLASH T1WI中,以胰腺实质期的胰腺-肿瘤CNR最高(9.7),其同期所获得的图像质量也最好(3.54±0.64),均优于平扫各序列(P＜0.05).对胰腺肿瘤的评价:动态增强3-D FLASH T1WI在检出胰腺癌及评价癌肿胰周血管受累、邻近器官受侵及转移灶方面均为最优(P＜0.05);MRCP对胰腺癌导致的胰胆管受侵最敏感,其次是True-FISP T2WI.综合各扫描序列的敏感性和准确性均优于任何单一的平扫、水成像或动态增强扫描序列.结论胰腺肿瘤的MR检查,多种扫描序列各有优缺点,联合应用能发挥最大潜力.理想的检查组合应包括:平扫屏气FS-FLASH T1WI、TSE T2WI、高分辨力的厚层及薄层MRCP、屏气的三维梯度回波序列多时相动态增强扫描.     

Pancreatic perivascular epithelioid cell tumor: A case report with clinicopathological features and a literature review

Perivascular epithelioid cell tumor(PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. This paper described a case of pancreatic PEComa, which was initially suspected as neuroendocrine carcinoma by biopsy, and therefore surgical treatment was recommended due to undetermined diagnosis. Examination of the surgical specimen under a microscope showed that the tumor cell's morphology was epithelioid or spindle-shaped, and ranged in a nested pattern. Additionally, these cells had a large extent of acidophilic cytoplasm, no mitotic figures, and expressed HMB-45, melan-p, and smooth muscle actin immunohistochemically. Pathological examination indicated that PEComa originated from the pancreas, but symptoms related to tuberous sclerosis were absent. Since PEComa is extremely rare in the pancreas, it is likely to be ignored in differential diagnosis. In conclusion, our article highlighted the clinicopathological features of PEComa, and we conducted a literature review focusing on PEComa so as to deepen the understanding of this tumor type.     

First pancreatic perivascular epithelioid cell tumor (PEComa) treated by mTOR inhibitor

BackgroundPerivascular epithelioid cell tumor, an extremely rare mesenchymal tumor, could be ubiquitous but rarely arises from pancreas. Surgery is considered the most appropriate treatment. Nevertheless, activation of mTOR pathway seems to be a common pathogenic event in PEComas paving the way to chemotherapy by mTOR inhibitor.MethodA 17 year-old man presented a hypervascular tumor of 55 mm, located in the head of pancreas without bile duct or pancreatic duct compression.ResultsHistopathology showed epithelioid cells with clear or focally granular eosinophilic cytoplasm with melanocytic (HMB-45, Melan-A) and myoid markers which confirmed diagnosis of PEComa. Given the absence of worrisome feature, we ruled out surgery and decided to initiate treatment with Sirolimus, an mTOR inhibitor. After 3.5 years, we showed a significant reduction in size of the tumor.ConclusionThis first case of pancreatic PEComa treated by mTOR inhibitor without surgery suggests a good efficiency of this therapy.     

Primary pancreatic perivascular epithelioid cell tumor (PEComa): A surgical enigma. A systematic review of the literature

Background

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor, with distinctive morphology and expression of myo-melanocytic markers.Current scientific literature reported just 24 cases of pancreatic PEComas. With our systematic review, we aimed at improving our understanding of the disease, focusing on the knowledge gained on epidemiology, etiology, clinical presentation, diagnosis, treatment and prognosis.

Improving recognition of hepatic perivascular epithelioid cell tumor:Case report and literature review

A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial resection of segment 3 of the liver.The lesion pathologically showed eosinophilic proliferation,in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A,thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor(PEComa).A PEComa arising from the liver is relatively rare.Moreover,the name ‘PEComa' has not yet been widely recognized,and the same disease entity has been called epithelioid angiomyolipoma(EAML),further diminishing the recognition of PEComa.In addition,PEComa imaging findings mimic those of malignant liver tumors,and clinically,this tumor tends to enlarge.Therefore,a PEComa is difficult to diagnose.We conducted a systematic review of PEComa and EAML cases and discuss the results,including findings useful for differentiating perivascular epithelioid cell tumors from malignant liver tumors.     

Abdominopelvic perivascular epithelioid cell tumor with overt malignancy: a case report

Perivascular epithelioid cell tumor (PEComa) is a group of rare tumors composed of epithelioid cells with characteristic perivascular distribution and co-expression of the melanogenic marker HMB-45 and muscular markers. There are no documented parameters referring to the biologic behavior of PEComa. We report an abdominopelvic PEComa with overt malignancy in a 16-year-old girl. Histologically, the tumor showed the typical morphophenotypic characteristics of PEComa. Though the cytologic appearance of the tumor cells was relatively bland, the extensive necrosis, presence of lymph node metastases, and surrounding tissue invasion were all indicative of malignancy. Relapse of the tumor with multiple lymphadenopathy shortly after debulking surgery for the primary lesion, and postoperative adjuvant chemotherapy, further denoted its aggressive behavior.     

Perivascular epithelioid cell tumor of the liver： A report of two cases and review of the literature

Perivascular epithelioid cell tumor （PEComa） is a rare tumor which arises from mesenchymal tissues. It is predominant in the uterus, but very rare in the liver. To the best of our knowledge, less than 5 cases of PEComa of the liver have been reported. Herein we present two pathologically proven cases of PEComa of the liver, retrospectively analyze their clinical and imaging features, and review the literature.     

A perivascular epithelioid cell tumor of the stomach:An unsuspected diagnosis

Perivascular epithelioid cell tumor(PEComa) is a rare mesenchymal neoplasia and currently well recognized as a distinct entity with characteristic morphological,immunohistochemical and molecular findings.We report a case of PEComa arising in the antrum of a 71-year-old female with melena.The tumor,located predominantly in the submucosa as a well delimited nodule,measured 3.0 cm in diameter and was completely resected,with no evidence of the disease elsewhere.Histologically,it was composed predominantly of e...     

Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor

Approximately 10% of patients with gastrointestinal stromal tumors （GIST）develop other neoplasms, either synchronously or metachronously. In this report we describe coexistence of a gastrointestinal stromal tumor and a hepatic perivascular epithelioid cell tumor （PEComa） in a 51-year-old woman with no evidence of tuberous sclerosis. A subcapsular hepatic nodule （0.8 cm in diameter） was found during surgery for symptomatic gastric neoplasm （15 cm in diameter） arising from the lesser curvature. Both tumors revealed histomorphological and immunohistochemical features confirming a diagnosis of a small incidental hepatic PEComa and a high risky extramural gastric GIST, respectively. The patient remained disease-free 25 mo after surgery with no evidence of tumor recurrence or new neoplasms. To our knowledge, this is the first report of PEComa in a patient with GIST. Hepatic lesions detected synchronously or metachronously in patients with GISTs may represent histogenetically distinct lesions and should be sampled to confirm or exclude metastatic GISTs.     

First Description of a PEComa (Perivascular Epithelioid Cell Tumor) of the Colon: Report of a Case and Review of the Literature

We describe a young female patient suffering from a PEComa (perivascular epithelioid cell tumor) of the cecum, incidentally found at an examination made by her family physician. The perivascular epithelioid cell tumor is a very rare tumor, until today reported in a few cases in falciform ligament, uterus, jejunum, terminal ileum, rectum, liver, kidney, lung, pancreas, prostate, and soft tissue of the thigh. This tumor is part of a new group of tumors, comprised of angiomyolipoma, lymphangiomyolipoma, and clear-cell myomelanocytic "sugar" tumor. Defined by coexpression of melanocytic (HMB-45) and muscle markers (smooth muscle actin and desmin) the perivascular epithelioid cell tumor does not have predictable histopathologic behavior. Some cases of metastasis are described, comorbidities such as tuberous sclerosis of the brain "Bourneville" and lymphangioleiomyomatosis have to be excluded. The therapy consists of the radical resection. An adjuvant therapy is not known. Recommended is a close and long-term follow-up clinically and by CT scan.     

胰腺双部位实性假乳头肿瘤1例

目的:总结1例胰腺双部位实性假乳头肿瘤(SPT)的临床病理特点,探讨其可能起源.方法:手术行标准胰十二指肠切除术和胰体尾及脾切除术,分析该病例SPT的临床病理特点,并行多个抗体的免疫组织化学检查.结果:患者术后血糖5.5-8.9 mmol/L,第5天开始恢复饮食.未发生胰漏、胆漏及腹腔感染等并发症.大体形态的囊实性比例不尽相同,但镜下肿瘤细胞形态学一致,均确诊为SPT,对各个免疫表型的表达具有异质性,其中VIM、S100、AAT、CyclinD1、PR及Nestin 均呈阳性.结论:SPT可能起源于胰腺干细胞及与其发育密切相关的胚胎神经嵴的神经前体细胞,由干细胞发育过程中分化不成熟所致.