肝原发性平滑肌肉瘤（附三例报告及免疫组化分析）

本文报告了３例肝原发性平滑肌肉瘤，男性１例，女性２例，平均年龄３８．７岁，占同期肝恶性肿瘤检出率的０．６８％，占同期肝癌检出率的２５％，临床表现为上腹痛，右上腹包块，肝大。本组病例还进行了Ｍａｓｓｏｎ三色染色及免疫组化染色。本文结合文献对肝原发性平滑肌肉瘤的组织发生，临床病理特点及参数，鉴别诊断进行了讨论。     

肝原发性血管肉瘤（附三例报告）

本文报告了３例肝原发性血管瘤，男性２例，女性１例，平均年龄３２．７岁，占同期肝脏恶性肿瘤检出率的０．６８％（３／４３６），临床表现为上腹痛及包块，血清ＨＢｓＡｇ及ＡＦＰ阴性，本组资料还进行了网状纤维染色及免疫组织化学标记，本文讨论肝血管肉瘤的临床病理特点，诊断及鉴别诊断。     

肝原发性血管肉瘤（附三例报告）

本文报告了３例肝原发性血管瘤，男性２例，女性１例，平均年龄３２．７岁，占同期肝脏恶性肿瘤检出率的０．６８％（３／４３６），临床表现为上腹痛及包块，血清ＨＢｓＡｇ及ＡＦＰ阴性，本组资料还进行了网状纤维染色及免疫组织化学标记，本文讨论肝血管肉瘤的临床病理特点，诊断及鉴别诊断。     

原发性肝血管肉瘤1例报告并文献复习

原发性肝血管肉瘤（primary heparic angiosarcoma, PHA）是一种罕见的恶性间叶组织肿瘤，发生率为1.4-2.5/10^7，约占原发性肝肿瘤的0.4%，但却是肝肉瘤中最多见的，约占36%。我院2003年诊断1例，现结合文献，就其临床表现、病理特点、诊断及鉴别诊断报告如下。     

p57及nm23在肢体平滑肌肉瘤组织中的表达及临床意义

目的：检测肢体平滑肌肉瘤组织中p57及nm23的表达，分析p57及nm23对平滑肌肉瘤的诊断、治疗及预后的意义。方法：应用免疫组化方法检测63例平滑肌肉瘤组织中p57及nm23的表达，并对所有病例的5年存活情况进行随访。结果：63例平滑肌肉瘤患者中有23例（36．51％）p57表达阳性，31例（49．21％）nm23表达阳性，p57及nm23的表达与平滑肌肉瘤的发生及患者的预后相关（P〈0．01）；nm23还与平滑肌肉瘤的转移具有相关性（P〈0．01）。结论：p57及nm23可作为两个新的指标，用于平滑肌肉瘤的诊断、治疗及预后判断。     

小肠原发性恶性肿瘤54例临床分析

（目的）总结小肠原发性恶性肿瘤的诊断及治疗。（方法）回顾分析54例经手术病理证实原发性小肠恶性肿瘤的临床资料。（结果）平均发病年龄41．3岁，30岁～60岁占70．4％。腺癌中位发病年龄49岁，十二指肠为其好发部位，占52．2％（12／23）。平滑肌肉瘤为51岁，空肠占54．5％（6／11）。淋巴肉瘤40岁，回肠占73．7％（14／19）。常见症状为腹痛51．9％（28／54）和腹块46．2％（25／54）。术前确诊率13．0％。术后5年生存率25．3％。（结论）小肠原发性恶性肿瘤以腹痛腹块为主要表现。全小肠X线钡餐检查、胃镜、ERCP等检查诊断意义较大。治疗以手术为首选。     

口腔粘膜原发性恶性黑色素瘤的临床病理分析

对16例口腔粘膜原发性恶性黑色素瘤进行了临床病理及免疫组织化学分析。结果表现，本组病例占同期全身恶性黑色素瘤的10．2%（16/156）发病年龄30～66岁，平均46．3岁，男女之比为1．29：1。9例（56．3％）于发病前有口腔粘膜黑斑病史。本组发生于牙龈、牙槽粘膜9例（56．3％），上腭部6例（37．5％）。除HE染色外，还进行了S-100蛋白及HMB-45的免疫组织化学染色，阳性率分别为100％和87．5％。应用Giemsa液复染，代替常现的苏木素液复染，使免疫组化染色结果更易判断。本文结合国内外文献对本病的临床病理特点及癌前病变、早期诊断进行了讨论。     

原发性肝平滑肌肉瘤一例

本文报告1例原发性肝平滑肌肉瘤,并复习有关文献,对临床、病理特征作了简单介绍。肝脏原发性恶性肿瘤中癌瘤多见。肉瘤较少,仅占肝恶性肿瘤的1—2%。其中平滑肌肉瘤更为罕见,据目前国际文献仅有10余例个案报告。未见国内报告。我院遇见1例,报告如下。     

阴道平滑肌肉瘤三例

阴道肉瘤很少见,仅占阴道恶性肿瘤的２％以下［１］,包括平滑肌肉瘤、纤维肉瘤和葡萄状肉瘤。我院从１９８８年至１９９８年的１０年间,仅收治３例阴道平滑肌肉瘤患者,占同期阴道恶性肿瘤的０．７８％,现对其临床特点及治疗方法进行探讨。临床资料例１　患者５４岁,以接触性出血１年余,阴道口脱出肿物１０天就诊。妇查：阴道后壁上段可见一５．５ｃｍ×４ｃｍ的肿物,触血（＋＋）,宫颈、宫体未见异常,左侧阴道旁组织增厚达中线。行肿物活检,病理报告为平滑肌肉瘤。临床诊断：阴道平滑肌肉瘤Ⅱ期。于１９９１年４月５日入院,入院…     

肛管直肠少见恶性肿瘤60例分析

1986～1995年，收治直肠平滑肌肉瘤、直肠恶性淋巴瘤、直肠类癌、肛管直肠恶性黑色素瘤、一穴肛源癌、基底细胞癌、肛管鳞癌、原发性肛周粘液腺癌等8种少见的肛管直肠恶性肿瘤共60例。占同期肛管直肠恶性肿瘤的4．4％（60／1359）。并就这些肿瘤的诊断与治疗进行了讨论。发现这些肿瘤均位于距肛缘8cm以下范围内，故强调肛指及活检的重要性。     

CDK-inhibitors-associated kinase activity: a possible determinant of malignant potential in smooth muscle tumors of the external soft tissue.

There has been accumulating histological observation of leiomyoma and leiomyosarcoma of the external soft tissue regarding their differential diagnosis. The definitive diagnostic tools have not been established, however, nor have the pathological mechanisms of cell proliferation in these tumors been clarified. Herein, expression of the cyclin-dependent kinase inhibitors (CKIs), p21, p27 and p57 and their associated kinase activities were examined in 61 cases of soft tissue smooth muscle tumors. Immunohistochemical staining showed that all 3 inhibitor proteins were expressed in all cases of leiomyoma and leiomyosarcoma, but that the mean values of their labeling indices (LIs) were higher in the cases of leiomyosarcoma. In addition, the LIs of p21 and p27 were inversely correlated in total cases. Immunoblotting revealed that these proteins are expressed at higher levels in tumors, in particular, in leiomyosarcoma. When CKIs were immunoprecipitated from tissue extracts, cyclin/cdk protein complexes associated with, at least, 1 CKI were detectable only in tumor tissues. Furthermore, cdk2 or cdk4 kinase activity manifested by these cyclin/cdk/CKI complexes (CKI-associated kinase activity) was detectable exclusively from leiomyosarcoma, but not from leiomyoma. Among the cases of leiomyosarcoma, cdk2 activity was generally found associated either with p21 or p27, but not both. Statistical analysis indicated that p21- and p27 LIs are predictive of positive or negative clinical outcome, respectively. In conclusion, the participation of CKIs in active cyclin/cdk complexes in a reciprocal and redundant manner and subsequent CKI- associated kinase activity are the characteristic profiles of malignant phenotype in these tumors. Moreover, immunohistochemical detection of CKIs may provide a useful tool for evaluating patients' prognosis.     

消化道肿瘤肝脏转移的临床病理学分析(附55例报告)

作者对55例消化道肿瘤肝脏转移的病理学特征进行了分析。其结果表明:(1)55例消化道肿瘤转移肝脏的有38例(69.1％)来自结肠,证明结肠肿瘤大部分是由门静脉血流转移至肝脏;(2)55例病人血清学检查中CEA升高的有35例(63.5％),提示:CEA是辅助诊断消化道肿瘤转移的有用指标;(3)B超、CT和MRI是探测消化道肿瘤转移肝脏的非常有益的手段,(4)55例病人血清中AFP检测均为阴性,而术中病人肝脏均无肝硬变改变;这表明与原发性肝癌不同;(5)外科手术治疗仍然是消化道肿瘤肝脏转移的最佳方法,本组病人中有48例患者进行再手术切除,术后患者最长的已生存9年。     

Pancreatic and gastric metastases of leiomyosarcoma arising in the left leg

Pancreatic or gastric metastases from other primary malignancies are rare, especially from leiomyosarcoma. We report a case of leiomyosarcoma in the left lower leg with metastases to the pancreas and stomach. A 61-year-old man had liver cirrhosis caused by hepatitis C virus infection and was followed up by his primary physician. Two years before presentation at our hospital, he had undergone surgical resection of leiomyosarcoma in the left lower leg and systemic chemotherapy for multiple metastatic tumors in the lung. On admission, endoscopic examination and computed tomography were performed for a routine checkup to exclude esophageal varices and liver tumor. Although the patient had no specific symptoms, multiple gastric and pancreatic metastases were identified by endoscopy and computed tomography, respectively. In general, metastases to the pancreas and stomach are rare. We discuss the clinical and diagnostic findings of pancreatic and gastric metastases by reviewing previously reported cases.     

小肠原发性肿瘤33例临床分析

目的 :探讨小肠肿瘤临床特点和早期诊断手段。方法 :回顾性分析 33例原发性小肠肿瘤患者的临床资料。结果 :小肠良性肿瘤 5例均为平滑肌瘤 ;恶性肿瘤 2 8例 ,其中平滑肌肉瘤 13例 (4 6 4 % ) ,淋巴肉瘤 11例 (39 2 % ) ,腺癌 3例 (10 7% ) ,类癌 1例 (3 5 % )。临床上无特异性症状及可靠的诊断方法 ,内窥镜和全消化道钡餐造影是主要诊断手段。 5 3 6 %的恶性病例在手术时已有远处转移 ,仅 13例(4 6 4 % )行根治性切除。根治切除术后 5年生存率为 4 6 1%。结论 :早期诊断、早期治疗是提高小肠恶性肿瘤患者生存率的关键 ,对无远处转移者应争取行根治术。     

11例原发性肾肉瘤临床治疗分析

目的:探讨成人原发性肾肉瘤的临床表现、病理特点和预后影响因素.方法:收集1970年1月～2000年12月收治的成人肾脏肿瘤752例,对其中11例原发性肾肉瘤进行回顾性分析.结果:11例中8例以肿块、消瘦为首发症状:11例术后病理确诊为:恶性纤维组织细胞瘤5例,平滑肌肉瘤3例,脂肪肉瘤2例,低分化肉瘤1例.术后无残余瘤组平均生存期为5.8年,残余瘤组术后平均生存10.6个月;恶性纤维组织细胞瘤平均生存期为6个月、平滑肌肉瘤和脂肪肉瘤分别为3.5和10.2年.结论:肿块、消瘦是肾肉瘤的最常见临床症状,病理类型及术后有无残余瘤是决定预后的重要因素.     

Clinicopathologic Patterns of Adult Renal Tumors in Pakistan

Background: Renal cancer is a serious public health problem which may be under reported and registeredin our setup, since the Karachi cancer registry documented only 43 cases out of 4,268 incident cancer cases over3 year duration. Therefore we aimed to determine the clinicopathologic characteristics of adult renal tumors inour setup. Materials and Methods: The study was conducted in histopathology department, Liaquat NationalHospital and included total of 68 cases of adult renal tumors over 4 years. Detailed histopathologic characteristicsof tumors were analyzed. Results: Mean age of patients was 56.4 (18-84) years. Renal cell carcinoma (RCC) wasthe most common cell type (78%) cases; followed by transitional/urothelial carcinoma (12.5%), leiomyosarcoma(4.7%), oncocytoma (1.6%), squamous cell carcinoma (1.6%) and high grade pleomorphic undifferentiatedsarcoma (1.6%). Among 50 RCC cases; 62% were conventional/clear cell RCC (CCRCC) type followed bypapillary RCC(PRCC), 24%; chromophobe RCC(CRCC), 6% and sarcomatoid RCC(SRCC), 8%. Mean tumorsize for RCC was 7.2 cm. Most RCCs were intermediate to high grade (60% and 40% respectively). Capsularinvasion, renal sinus invasion, adrenal gland involvement and renal vein invasion was seen in 40%, 18%, 2% and10% of cases respectively. Conclusions: We found that RCC presents at an earlier age in our setup compared toWestern populations. Tumor size was significantly larger and most of the tumors were of intermediate to highgrade. This reflects late presentation of patients after disease progression which necessitates effective measuresto be taken in primary care setup to diagnose this disease at an early stage.     

原发性心脏肿瘤的超声心动图诊断价值

目的:探究原发性心脏肿瘤的超声心动图诊断特征及价值。方法:选择2012年4月-2014年6月收治的均经手术病理确诊的155例原发性心脏肿瘤患者,回顾性分析患者的超声心动图、病理学等临床资料,总结其超声心动图特征。结果:155例原发性心脏肿瘤患者中,良性肿瘤患者121例,其中男性48例,女性73例,女性多于男性（P<0.05）。恶性肿瘤患者34例,男性15例,女性19例,女性多于男性,但差异性不显著（P>0.05）。良性肿瘤患者的比例显著高于恶性肿瘤患者,具有明显差异（P<0.05）。良性黏液瘤多发于成人以及老年人,良性非黏液瘤以儿童、成人多见,恶性肿瘤多发于40岁以上成年人;黏液瘤在良性肿瘤中比例为62.8%,比例最大,良性非黏液瘤包括纤维瘤（3.3%）、横纹肌瘤（7.4%）、脂肪瘤（16.5%）、乳头状弹性纤维瘤（8.3%）以及血管瘤（1.7%）,恶性肿瘤包括血管性肉瘤（17.6%）、横纹肌肉瘤（17.6%）、纤维组织细胞瘤（14.7%）、间叶肉瘤（8.8%）、平滑肌肉瘤（17.6%）以及未分类肉瘤（23.5%）。在良性肿瘤患者中,同时累及左心房与左心室的比例为79.3%,同时累及右心房与右心室的比例为11.6%,心包受累比例为5.8%,其他部位3.3%,各个部位受累比例具有明显差异性（P<0.05）。在恶性肿瘤患者中,受累比例最高的是右心房与右心室,其次是左心室与左心房、心包,其他部位为11.8%,各个部位受累比例具有明显差异性（P<0.05）。良性肿瘤与恶性肿瘤患者在各个部位受累的比例差异性明显,具有统计学意义（P<0.05）。结论:利用超声心动图检测原发性心脏肿瘤患者,能够较清晰区分不同病理的肿瘤患者的受累部位,对临床研究具有一定的指导作用。     

Endothelial Cell Proliferation and Vascular Endothelial Growth Factor Expression in Primary Colorectal Cancer and Corresponding Liver Metastases

Background: . Colorectal carcinoma (CRC) is one of the major causes of cancer death worldwide. Data fromthe literature indicate differences between the proliferation rate of endothelial cells relative to the morphologygrowth type, possibly due to origin of specimens (autopsy material, surgery fragments) or quantification methods.Vascular endothelial growth factor (VEGF) is a factor that stimulates the proliferation of endothelial cells. It isexpressed in more than 90% of cases of metastatic CRC. Aim: The aim of this study was to evaluate the endothelialcell proliferation and VEGF expression in primary tumors and corresponding liver metastases. Materials andMethods: Our study included 24 recent biopsies of primary tumors and corresponding liver metastases of CRCcases. CD34/ Ki67 double immunostaining and RNA scope assay for VEGF were performed. Results: In theprimary tumors analysis of VEGFmRNA expression indicated no significant correlation with differentiationgrade, proliferative and non-proliferative vessels in the intratumoral and peritumoral areas. In contrast, in thecorresponding liver metastases, VEGFmRNA expression significantly correlated with the total number of nonproliferativevessels and total number of vessels. CD34/ Ki67 double immunostaining in the cases with poorlydifferentiated carcinoma indicated a high number of proliferating endothelial cells in the peritumoral area anda low number in the intratumoral area for the primary tumor. Moderately differentiated carcinomas of colonshowed no proliferating endothelial cells in the intratumoral area in half of the cases included in the study, forboth, primary tumor and liver metastasis. In well differentiated CRCs, in primary tumors, a high proliferationrate of endothelial cells in the intratumoral area and a lower proliferation rate in the peritumoral area were found.A low value was found in corresponding liver metastasis. Conclusions: The absence of proliferative endothelialcells in half of the cases for the primary tumors and liver metastases in moderately differentiated carcinomasuggest a vascular mimicry phenomenon. The mismatch between the total number of vessels and endothelialproliferation in primary tumors indicate that a functional vascular network is already formed or the existenceof some mechanisms influenced by other angiogenic factors.     

Histopathology peer review of high-grade soft tissue sarcoma: the Scandinavian Sarcoma Group experience

From 1981 to 1986, a total of 240 patients with a primary soft tissue sarcoma with malignancy grade III or IV were entered into an adjuvant chemotherapy multicenter trial conducted by the Scandinavian Sarcoma Group (SSG). Histopathologic peer review of all the specimens was performed by an expert pathology committee. The most common soft tissue sarcoma after review was malignant fibrous histiocytoma (MFH) (40%), followed by synovial sarcoma (15%), leiomyosarcoma (9%), liposarcoma (8%), and malignant Schwannoma (6%). In 25% of the cases the histologic type of sarcoma was reclassified, and in 40% of the cases the malignancy grade was changed. By survival analyses, the reclassification of malignancy grade seemed to be valid. Also, grading highly malignant soft tissue sarcoma in two grades (III and IV) increased the prognostic information. Of 164 tumors from the centers with the most reported cases (five centers with 25 to 51 tumors each), eight tumors were found to be ineligible for the adjuvant study (5%); of 76 tumors from 13 centers with few tumors (one to 16 tumors per center), 12 tumors were ineligible (16%). We conclude that histologic peer review is important in studies of soft tissue sarcoma.