肾上腺血管周上皮样细胞瘤1例报告并文献复习

目的:探讨原发性肾上腺血管周上皮样细胞瘤(PEComa)的临床病理特点及诊断治疗方法。方法:报告2014年11月中国人民解放军总医院泌尿外科诊治原发性肾上腺PEComa 1例患者的临床特征、影像学特点、病理学结果、治疗及随访情况,并结合文献进行分析。结果:文献报道的肾上腺PEComa非常罕见,该病术前多无特异临床症状,容易通过影像学手段发现,但难以确诊,最终诊断依靠病理学表现和免疫组化结果,治疗首选手术切除,术后须长期随访。结论:原发性肾上腺PEComa是一种非常罕见的间叶组织肿瘤,依靠病理学方法诊断。其生物学行为不明确,建议术后长期随访。     

腹膜后和盆腔恶性血管周上皮样细胞肿瘤2例临床病理特征分析并文献复习

目的 探讨恶性血管周上皮样细胞肿瘤(PEComa)的临床病理特点及诊治要点以增加对该疾病的认识。方法 回顾性分析广东医科大学附属医院泌尿外科收治的2例恶性PEComa患者的临床病理资料并复习相关文献。结果 2例恶性PEComa患者均为男性,年龄分别为53、16岁,发生部位分别位于腹膜后、盆腔,均行手术切除肿瘤,经术后病理检查明确诊断。患者1肿瘤组织主要由平滑肌样细胞构成,患者2肿瘤组织主要由上皮样细胞构成,2例患者的肿瘤细胞均可见病理性核分裂象,HMB45、Melan-A、SMA、CD34均表达阳性,随访未见肿瘤复发或转移。分析文献报道的15例腹膜后或盆腔PEComa患者的资料,男性3例、女性12例,其中9例为恶性肿瘤,主要表现为腹痛、腹胀或腰痛,部分患者为体检时发现。结论 恶性PEComa术前诊断较困难,易误诊,最终确诊需要依靠术后病理结果。目前治疗以手术完整切除肿瘤为主,长期随访是关键。     

肝脏血管周上皮样细胞肿瘤临床分析

目的 提高对罕见的肝脏原发性肿瘤——肝脏血管周上皮样细胞肿瘤(PEComa)的诊疗水平.方法 采用文献检索收集1989 ～ 2013年间报道的包括北京大学人民医院1例在内资料齐全的原发性肝脏PEComa患者41例,总结其病例资料,对原发性肝脏PEComa的临床特点、影像学资料、病理特点、治疗及预后进行回顾性分析.结果 肝脏PEComa无特异性临床表现,影像学特征不易识别,主要依靠术后病理确诊.手术是目前公认的主要治疗方法.41例患者中34例为良性,7例为恶性,恶性者预后较差.结论 原发性肝脏PEComa是一种肝脏罕见肿瘤,应提高对其认识和诊疗水平,术后应长期密切随访.     

膀胱血管周上皮样细胞肿瘤临床病理分析

目的 探讨膀胱血管周上皮样细胞肿瘤(PEComa)的临床病理特征,提高对该罕见肿瘤的认识。方法 对5例膀胱PEComa进行组织形态观察、免疫组织化学染色和分子遗传学检测,并结合文献报道的病例总结分析其临床表现与病理学特点、诊断、治疗及预后。结果 本组新报道5例膀胱PEComa,结合检索文献获得的36例进行总结分析显示:41例患者年龄16～78岁,平均(40±14)岁;男性15例,女性26例,男女比例为1∶1.7;肿瘤最大径0.6～14cm,平均(4.2±2.3)cm。部分患者的临床症状类似于膀胱癌,可表现为血尿或腹部不适、腹胀、腹痛等。尽管膀胱PEComa具有特征性的组织形态和免疫表型,但由于罕见,对其认识不足,术前膀胱镜活检诊断困难。目前该肿瘤的治疗方案多样,包括部分膀胱及肿瘤切除术(19/38例)、经尿道膀胱肿瘤电切术(10/38例)、经尿道膀胱肿瘤电切术明确诊断后再行部分膀胱切除术(4/38例)以及膀胱全切除术(3/38例)等。31例患者具有随访结果,随访时间3～266个月,复发率、转移率、死亡率分别为9.7%、9.7%、6.5%。结论 膀胱PEComa非常罕见,其临床表现与膀胱癌相似,术前诊断困难;加强对该肿瘤的认识有助于避免漏诊或误诊。膀胱PEComa的生物学行为尚有不确定性,对局限性病变建议行部分膀胱及肿瘤切除术,术后需长期随访。     

肝脏血管周上皮样细胞瘤误诊1例报告并文献复习

目的介绍1例肝脏血管周上皮样细胞瘤(perivascular epithelioid cell tumor,PEComa)的诊疗过程,并进行文献复习。方法患者男性,26岁,因反复右上腹部疼痛不适3月,发现肝占位2周入院,术前诊断肝脏占位性病变,怀疑肝癌。遂于全麻下行腹腔镜下右肝后叶切除术,术后病理诊断为PEComa。结果患者痊愈出院,术后随访1月余无明显不适,无复发。结论PEComa是起源于间叶组织的一种少见肿瘤,由特征性的血管周上皮样细胞(perivascular epithelioid cell,PEC)组成。肝脏PEComa罕见,仅有文献个案报道。肝PEComa缺乏特征性的临床特点和体征,CT及MRI表现不典型,临床较易误诊为原发性肝细胞癌(hepatocellular carcinoma,HCC)。积极手术治疗,能取得较好疗效。     

原发性甲状腺淋巴瘤的诊治进展

目的探讨原发性甲状腺淋巴瘤的诊断与治疗进展。方法通过阅读近几年国内外的原发性甲状腺淋巴瘤相关文献,对其诊断与治疗进展进行归纳总结。结果原发性甲状腺淋巴瘤的发病与桥本甲状腺炎等慢性炎症的刺激相关,术前诊断主要依靠超声引导下的穿刺活检,治疗方式主要取决于其病理学类型及肿瘤分期。手术切除主要用于病理活检及解除压迫症状,放疗及化疗是其主要的治疗方式。结论原发性甲状腺淋巴瘤是一种比较罕见的甲状腺恶性肿瘤,熟悉并了解其临床病理学特征,对于术前诊断、明确其病理学类型和分期,以及选择合理的治疗措施,均具有重要的指导意义。     

肝脏血管周上皮样细胞瘤临床诊治分析

目的探讨肝脏血管周上皮样细胞瘤(PEComa)的临床特点、诊治原则和预后。方法回顾性分析2013年1月至2019年7月于中国医科大学附属盛京医院手术治疗且术后病理证实为肝脏PEComa的23例患者临床资料,结合文献讨论肝脏PEComa的临床特点、诊断情况、治疗及预后。结果23例患者中女16例,男7例,中位年龄为50岁;肿瘤直径4.6(1.0~22.5)cm,以单发为主(87.0%),8例位于左半肝,11例位于右半肝,1例包含左、右半肝,1例位于尾状叶,1例肝、肾多发,1例肝、腹膜后多发,术前仅4例诊断为PEComa。Melan-A、HMB-45和SMA阳性率分别为92.9%(13/14)、85.7%(12/14)和76.9%(10/13)。所有患者术后均恢复良好后出院,随访期内未见复发。结论肝脏PEComa是一种相对罕见的肝脏肿瘤,多为良性,影像学表现有一定特征性,但依赖于术后病理学和免疫组织化学检查确诊。术前能够确诊的良性PEComa可定期复查或保守治疗,手术切除预后较好。     

巨大原发性肝血管肉瘤1例并文献复习

目的：探讨原发性肝血管肉瘤的临床病理特点、诊断及治疗方法。
方法：报告1例原发性肝血管肉瘤,并结合文献复习。
结果：患者临床症状及辅助检查无特异性,术前未能明确诊断,行手术切除治疗,病理检查示肿瘤主要由梭形细胞被覆的肿瘤血管样腔隙构成,特征为形成相吻合的血管通路;免疫组化表达血管内皮细胞标记,诊断为原发性肝血管肉瘤。术后化疗3次,随访7个月死于全身衰竭。
结论：肝血管肉瘤是一种罕见的高度恶性肿瘤,预后差,术前诊断非常困难,典型CT征象有助于本病的诊断,确诊依赖病理学检查和免疫组化;手术联合化疗有助于延长生存期。

     

1例原发于肝脏的血管周上皮样细胞肿瘤的诊治分析附文献复习

目的结合近期收治的1例肝脏血管周上皮样细胞肿瘤（perivascular epitheliod cell tumor,PEComa）的病例特点,总结该疾病的诊治体会。方法回顾分析2013年9月笔者所在医院成功收治的1例肝脏原发PEComa的诊治过程,并结合既往文献报道,总结和复习该疾病的临床特点以及处理方法。结果肝脏PEComa较为罕见,起病隐匿,症状缺乏特异性,临床诊断困难,多依赖病理学检查证实,手术切除是有效的治疗手段。结论提高本病影像学特征的认识有助于术前肿瘤性质的判断,本病预后较好,但术后仍需要长期密切观察随访。     

原发性腹膜后巨大淋巴管瘤1例报告并文献复习

目的：提高原发性腹膜后淋巴管瘤的诊治水平。方法：报道1例原发性腹膜后淋巴管瘤患者的诊治过程,术前诊断腹膜后囊肿,行单孔腹腔镜双侧腹膜后囊肿切除术。结果：术后病理（腹膜后囊肿）检材由扩张的淋巴管组成,囊壁由内皮细胞、平滑肌及纤维组织组成,符合腹膜后淋巴管瘤。术后随访6个月未见复发。结论：原发性腹膜后淋巴瘤是一种良性病变,临床罕见,确诊需结合术后病理和影像学表现,手术切除是最佳治疗方式。     

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??Primary retroperitoneal perivascular epithelioid cell tumor coexisting with renal angiomyolipoma and tuberous sclerosis: a case report and literature review WU Wen-ming, QIU Hui-zhong, WU Bin, et al.Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China Corresponding author: QIU Hui-zhong, E-mail: qiuhzpumc@yahoo.com.cn Abstract??Objective??The first domestic report of primary retroperitoneal perivascular epithelioid cell tumor ( PEComa). and discuss the clinical pathologic features of the disease. Methods A 29 year-old female patient was admitted in April 2009 in the general surgery department of Peking Union Medical College Hospital and was diagnosed as primary retroperitoneal perivascular epithelioid cell tumor located in the pelvic cavity coexisting with renal angiomyolipoma and tuberous sclerosis. The clinical, imaging, pathology features and treatment of the patient was analyzed retrospectively and related literatures were reviewed. Results The patient had not special symptoms and signs. The imaging methods can easily find the tumor but hard to differentiate it . The final diagnosis depends on the pathologic features and immunohistochemistry techniques. Among present literatures, scarce cases of retroperitoneal PEComa were reported. Conclusions Primary retroperitoneal PEComa is a very rare tumor that arises from mesenchymal tissues. The diagnosis of PEComa mainly depends on the pathological features. Because of the uncertain biologic behavior retroperitoneal PEComa should be followed up for a long term.     

Synchronous transperitoneal laparoscopic resection of right retroperitoneal schwannoma and left kidney monotypic PEComa in the presence of a duplicated inferior vena cava (IVC)

A 21-year-old female presented with a retroperitoneal mass in the right side and a centrally located renal mass in the left kidney with an aberrant vascular anatomy of a double inferior vena cava. The 2 tumors were radically excised by a transperitoneal laparoscopic approach in one session. Histopathologic examination revealed the right retroperitoneal lesion to be a schwannoma and the renal mass to be a cluster of monotypic neoplasms with perivascular epithelioid cell differentiation (PEComas). The latter is a rare tumor.     

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目的 分析肝脏血管周上皮样细胞瘤的临床特点.方法 2007年10月首都医科大学附属北京友谊医院普外科诊治1例肝脏血管周上皮样细胞瘤,对其临床、影像、病理学特点进行分析及相关文献复习.结果 肝脏血管周上皮样细胞瘤无临床症状,无特征性影像学表现,术前难于确诊.组织学上由单一上皮样细胞构成,不合血管及脂肪成分,免疫组化呈特征性黑色素生成(HMB-45)阳性.目前文献报道肝脏血管周上皮样细胞瘤发生在肝脏者尚不足10例.结论 肝脏血管周上皮样细胞瘤是一种非常罕见的间叶组织来源肿瘤.主要依靠病理进行诊断.肝脏血管周上皮样细胞瘤生物学行为不确定,建议术后长期随访.     

Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case

Perivascular epithelioid cell neoplasms, also known as “PEComas”, are unusual mesenchymal tumors, exhibiting perivascular epithelioid cell differentiation and characterized by a mixed myogenic and melanocytic phenotype. “PEComas not otherwise specified” (PEComas-NOS) are especially rare; consequently, there are no published large series, but only case reports. These tumors are rarely located retroperitoneally, with only about 15 such cases reported. We report a case of pulmonary diffuse lymphangioleiomyomatosis with large retroperitoneal PEComa-NOS in a 66-year-old woman. Treatment consisted only of tumor resection, without additional adjuvant therapy. We emphasize the importance of correct immunohistochemistry diagnosis, initiation of recommended treatment, and surveillance of this unique family of tumors.     

Perivascular Epithelioid Cell Tumor of the Retroperitoneum in a Young Woman Resulting in an Abdominal Chyloma

Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm which appears to have predominancy for young, frequently Asian, women. The neoplasm is composed chiefly of HMB-45-positive epithelioid cells with clear to granular cytoplasm and usually showing a perivascular distribution. These tumors have been reported in various organs under a variety of designations. Malignant PEComas exist but are very rare. The difficulty in determining optimal therapy, owing to the sparse literature available, led us to present this case. We report a retroperitoneal PEComa discovered during emergency surgery for abdominal pain in a 28-year-old Asian woman. The postoperative period was complicated by chylous ascites that was initially controlled by a wait-and-see policy with total parenteral nutrition. However, the chyle production gradually increased to more than 4 l per day. The development of a bacterial peritonitis resulted in cessation of production of abdominal fluid permitting normal nutrition without chylous leakage. Effective treatment for this rare complication of PEComa is not yet known; therefore, we have chosen to engage in long-term clinical follow-up.     

Clear cell myomelanocytic tumor of the urinary bladder

Clear cell myomelanocytic tumors are a recently described neoplastic growth considered to be a member of the family of perivascular epithelioid cell tumor. These tumors have a predilection for falciform ligament/ligamentum teres. We report an additional case arising from the muscularis propria of the urinary bladder in a 33-year-old woman. The tumor consisted of clear to eosinophilic, epithelioid, and spindled cells arranged in fascicles or packets. A delicate vascular stroma was found among the nests. Immunohistochemically, the tumor cells were typically positive for HMB-45 and smooth muscle actin but negative for S-100 protein, Melan-A, desmin, and pan-cytokeratin. The patient has been free of the disease since the excision of the tumor 6 years ago. This case, in association with the expanding list of perivascular epithelioid cell tumor reported in different sites, suggests that this type of neoplasm may be ubiquitous.     

Diagnostically Challenging Epithelioid Soft Tissue Tumors

In this article, we focus on the histologic features, differential diagnosis, and potential pitfalls in the diagnosis of epithelioid sarcoma, alveolar soft part sarcoma, clear-cell sarcoma, ossifying fibromyxoid tumor, and malignant extrarenal rhabdoid tumor. Numerous other soft tissue tumors also may have epithelioid variants or epithelioid features. Examples include epithelioid angiosarcoma, epithelioid malignant peripheral nerve sheath tumor, epithelioid gastrointestinal stromal tumor, and perivascular epithelioid cell tumor, among others.     

Paracolic recurrence: the importance of wide excision of the spermatic cord at retroperitoneal lymph node dissection.

PURPOSE: Four patients who underwent retroperitoneal lymph node dissection elsewhere and subsequently had radiographic evidence of expanding ipsilateral paracolic recurrence were referred to our institution for treatment. We evaluated ipsilateral spermatic cord metastatic involvement at retroperitoneal lymph node dissection and identified the possible etiology of these unusual recurrences. MATERIALS AND METHODS: Between January 1988 and February 1998, 34 of 685 patients who underwent a total of 702 retroperitoneal lymph node dissections had metastatic disease in the spermatic cord specimen. Variables examined in this group of patients included other disease sites, lymphovascular invasion in the primary tumor, histopathological findings of the primary tumor and retroperitoneal disease, clinical and pathological stage, disease side and the specific site of anatomical involvement of metastatic disease within the spermatic cord specimen, that is spermatic vessels and/or surrounding lymphatic tissue. RESULTS: Of these 34 positive spermatic cord specimens 18 were in primary retroperitoneal lymph node dissections and 16 were in post-chemotherapy specimens. Histopathological evaluation in 25 (74%) and 9 (26%) of the 34 primary tumors showed a mixed germ cell pattern and pure embryonal carcinoma, respectively. Similarly 9 of the 34 retroperitoneal lymph node specimens (26%) showed pure embryonal cell carcinoma and the remainder showed mixed histopathological findings. Disease was clinical stage I in 13 cases (38%) and lymphovascular invasion was absent in the primary tumor in 11 (32%). Despite disease in the spermatic cord specimen there was none at the primary landing zone in 2 patients (6%), including 1 in whom the spermatic cord was the only disease site. In 12 positive spermatic cord specimens (35%) disease was identified in the surrounding perivascular and lymphatic tissue without gonadal vessel involvement. CONCLUSIONS: Metastatic disease in the spermatic vessels and/or surrounding lymphatic tissue represents a possible site of recurrence when incompletely excised. The lack of lymphovascular invasion in the primary tumor does not preclude metastatic disease in the spermatic cord specimen. Complete and wide excision of the spermatic cord and surrounding lymphatic tissues at retroperitoneal lymph node dissection is necessary and may prevent paracolic recurrence.