原发性肝脏平滑肌肉瘤的病理及免疫组织化学观察

目的探讨原发性肝脏平滑肌肉瘤(primary hepatic leiomyosarcoma,PHLMS)的病理学及免疫组织化学表现。方法应用组织病理学、免疫组织化学方法观察3例原发性肝脏平滑肌肉瘤。结果组织学观察瘤细胞呈梭形,可见核分裂;免疫组织化学结果Smooth Muscle Actin( )、CD117(-)、S-100(-)、Vimentin(-)。结论原发性肝脏平滑肌肉瘤是一种罕见的恶性间叶肿瘤,其诊断主要依靠免疫组织化学方法。     

β-catenin和WT1在子宫间叶性肿瘤中的表达和临床意义

目的探讨β-联蛋白（β-catenin）和WT1在子宫间叶性肿瘤中的表达及其临床意义。方法采用免疫组织化学EnVision染色法对25例子宫内膜间质肉瘤、9例平滑肌肉瘤、16例平滑肌瘤（包括7例高度富于细胞平滑肌瘤）、8例正常子宫肌壁和6例正常子宫内膜中的β-catenin和WT1的蛋白表达水平进行检测。结果β-cate—nin在子宫内膜间质肉瘤细胞核表达的阳性率高于平滑肌肉瘤（P=0．004）和平滑肌瘤（P〈0．001）。正常子宫肌壁平滑肌细胞细胞核中无β-catenin表达。WT1阳性表达见于正常子宫内膜的间质细胞、子宫肌壁的平滑肌细胞和平滑肌瘤细胞。可旧1在平滑肌肉瘤和子宫内膜间质肉瘤的肿瘤细胞中的表达强度较平滑肌瘤细胞减弱（P分别为0．007和0．002）。结论β-catenln和WTI表达可能与子宫恶性间叶性肿瘤,尤其是子宫内膜间质肉瘤的发生和发展有关。细胞核的β-catenin蛋白表达有助于子宫内膜间质肉瘤与平滑肌肿瘤的鉴别诊断。     

重型肝炎

慢性重型乙型肝炎的病理形态学表现及诊断——张立洁等（北京首都医科大学附属北京佑安医院病理科100069）；《中华肝脏病杂志》2007，15（5）：323-325[目的：观察并总结慢性重型乙型肝炎（CSHB）的病理形态学特点。方法：用常规HE染色、组织化学Masson三色、Sweet网织、D-PAS染色及免疫组织化学CK7、CD68、     

成人肝脏未分化胚胎性肉瘤1例

来源于间叶组织的肿瘤称为肉瘤,原发性肝肉瘤极为罕见,约占原发性肝内恶性肿瘤的1.3%,包括淋巴肉瘤、血管肉瘤、平滑肌肉瘤等多种类型,肝脏未分化肉瘤又称胚胎肉瘤（undifferentiated embryonal sarcoma of liver,USL）,好发于儿童及青少年,成年病例极为罕见,我科于2006年曾收治1例USL患者,现报告如下。     

原发性肝脏平滑肌肉瘤1例报告

<正>原发性肝脏平滑肌肉瘤在所有肝脏恶性肿瘤中极为罕见,其血供丰富,进展快,无特异性临床及影像学表现,诊断依赖免疫组织化学。结合我院收治的1例该病例,复习国内外相关文献,总结其影像学特点、病理学特征、诊断思路及治疗方法。1病例资料患者,女,22岁,因"乏力、腹胀、肝功能异常10 d,肝占位5d"入院。查体:消瘦、贫血貌,眼睑苍白,上腹部膨隆,肝肋下可触及约3 cm,质硬,移动性浊音阴性。强化CT示肝右叶见截面     

肝脏肉瘤6例报道

肝脏恶性肿瘤包括起源于上皮细胞的原发性肝癌利起源于间叶组织的肉瘤，肝脏肉瘤临床上极为少见，容易被误诊。我院1990年1月至1992年12月收治肝脏肉瘤6例，其中男性5例，女性1例。年龄36-60岁。原发于肝脏者4例，转移者2例。平滑肌肉瘤3例，血管内皮细胞肉瘤1例，恶性淋巴瘤1例，粘液样肉瘤1例。现将病例报告如下：     

原发性肌源性肺肉瘤

目的 :报告我院 1981～ 1992年 ,经手术切除 ,根据光镜、电镜及免疫组化观察确诊的原发性肌源性肺肉瘤 6例。方法 :年龄 3～ 6 5岁 ,男性 4例 ,女性 2例 ,其所中包括横纹肌肉瘤 4例 ,肺平滑肌肉瘤和肺血管平滑肌肉瘤各 1例。结果 :6例肌源性肺肉瘤在光镜下各有其形态特点 ,免疫组化观察结果为 :结蛋白 (Desmin)及肌动蛋白 (Actin)染色瘤细胞全部呈阳性表达 ,肌红蛋白 (mb)染色肺横纹肌肉瘤瘤细胞呈阳性表达。肺平滑肌肉瘤及肺血管平滑肌肉瘤为阴性 ;S 10 0蛋白和细胞角蛋白 (Cytoker atin)全部为阴性。 3例横纹肌肉瘤电镜观察 :胞质内可见肌原纤维。结论 :原发性肺肉瘤的组织发生及其发病率 ,以及免疫组化染色在肺肉瘤诊断中的作用。     

原发性小肠肿瘤44例临床分析

原发性小肠肿瘤非常少见，临床早期诊断困难。目的：探讨原发性小肠肿瘤的临床常见表现、诊断和治疗方法。方法：回顾性分析1990年1月-2007年1月北京友谊医院确诊的44例原发性小肠肿瘤患者的临床资料。结果：原发性小肠肿瘤患者男女比例为3：1。主要临床表现为柏油样便、腹部包块、腹痛和肠梗阻症状。原发性小肠肿瘤以空肠最为常见，良恶性比为1：3．4。44例原发性小肠肿瘤患者中，29例（65．9％）为间质瘤，其中良性6例，低度恶性7例，高度恶性16例；5例（11．4％）为非霍奇金淋巴瘤；4例（9．1％）为腺癌；3例（6．8％）为平滑肌瘤；1例（2_3％）为平滑肌肉瘤；1例（2．3％）为类癌；1例（2．3％）为绒毛管状腺瘤。所有患者均行手术治疗。结论：原发性小肠肿瘤临床表现无特异性，病理类型多样，结合常见临床表现和有效检查方法有助于尽早诊断该病。     

原发性肺平滑肌肉瘤8例诊治分析并文献复习

目的探讨原发性肺平滑肌肉瘤的临床、病理特点,诊断和治疗,提高其诊治效果。方法总结分析我院收治的8例原发性肺平滑肌肉瘤患者的诊治及预后情况。结果无手术死亡,8例患者均恢复出院,术后随访死亡2例,其余仍健在。结论原发性肺平滑肌肉瘤是一种少见的肺部恶性肿瘤,早期无特殊症状,极易误诊。手术切除是治疗原发性肺平滑肌肉瘤的有效方法,肿瘤的大小、局部受侵程度、癌细胞分化程度与预后明显相关。     

原发性肺大B细胞淋巴瘤的临床病理研究

目的探讨原发性肺弥漫性大B细胞淋巴瘤的组织学特点和免疫表形特征。方法运用组织形态学和免疫组织化学方法研究我院1例肺弥漫性大B细胞淋巴瘤患者的临床表现、病理特点和免疫组化标记特点。结果肉眼观察肿瘤为灰白色不规则分叶状团块,切面灰白色,鱼肉样,有大量坏死灶;显微镜观察被覆呼吸上皮下中心母细胞样细胞弥漫增生,浸润肺组织,瘤细胞坏死明显。免疫组化：CD20（＋）,CD79α（＋）,CD3（-）,CD45RO（-）,PCK（-）。结论弥漫性大B细胞淋巴瘤是一种少见的恶性肿瘤,而原发于肺的DLBCL更极为罕见。该病缺乏典型的临床表现,极易误诊。肺弥漫性大B细胞淋巴瘤的诊断和鉴别诊断,要以组织病理学形态为基础并与免疫组织化学相结合。     

A case of primary hepatic leiomyosarcoma presenting with multiple subcutaneous scalp mass]

Leiomyosarcoma is an uncommon tumor which arises from various sites including uterus, stomach, retroperitoneum, superficial soft tissues, bladder, kidney, and lung. Primary hepatic leiomyosarcoma is a very rare tumor and fewer than 70 cases of primary hepatic leiomyosarcoma have been reported since the first publication in Japan. And there was only one case report of cutaneous metastasis from hepatic leiomyosarcoma. We recently experienced a case of primary hepatic leiomyosarcoma presenting as subcutaneous palpable mass. Herein we report this case with a review of literatures.     

Review of the literature laparoscopic surgery for metastatic hepatic leiomyosarcoma associated with smooth muscle tumor of uncertain malignant potential: Case report

Metastatic hepatic leiomyosarcoma is a rare malignant smooth muscle tumor.We report a case of metastatic hepatic leiomyosarcoma associated with smooth muscle tumor of uncertain malignant potential(STUMP).A 68-year-old female presented with a liver mass(60 mm × 40 mm, Segment 4).She underwent left salpingooophorectomy for an ovary tumor with STUMP in a broad ligament 6 years ago.Though FDG-PET showed obvious metabolically active foci, abnormal metabolically active foci other than the lesion were not detected.A malignant liver tumor was strongly suspected and laparoscopic partial liver resection was performed with vessel-sealing devices using the crush clamping method and Pringle maneuver.Immunohistochemical findings revealed metastatic liver leiomyosarcoma associated with STUMP in a broad ligament.This case is an extremely rare case of malignant transformation from primary STUMP to metastatic hepatic leiomyosarcoma.It provides important evidence regarding the treatment for metastatic hepatic leiomyosarcoma associated with STUMP.     

A patient with von Recklinghausen's disease associated with polymyositis, asymptomatic pheochromocytoma, and primary hepatic leiomyosarcoma

A 72-year-old woman with von Recklinghausen's disease was referred to our hospital because of pain and muscle weakness in her thighs. She had elevated serum values of creatine kinase, aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, and aldolase. Based on these results, a diagnosis of polymyositis was made. Treatment with prednisolone improved muscle strength, and laboratory values returned to normal. Computed tomography, magnetic resonance imaging of the abdomen, and 131I-metaiodobenzyl guanidine MIBG scintigraphy demonstrated a tumor 3 cm in diameter in the region of the left adrenal gland. Endocrinologic investigation disclosed elevation of serum and urine catecholamines. Since the blood pressure was normal, nonfunctioning pheochromocytoma was diagnosed clinically. The nonhypertensive course was attributed to reduced vascular response to noradrenaline. Serum lactate dehydrogenase. alkaline phosphatase. and asparate aminotransferase became elevated, and abdominal computed tomography showed a well-defined mass measuring 13 x 12 x 10 cm in the right lobe of the liver. The patient underwent right trisegmentectomy and left adrenalectomy. Histologically the adrenal tumor was a typical pheochromocytoma. The hepatic tumor was a leiomyosarcoma consisting of elongated spindle-shaped atypical cells arranged in intersecting bundles. Immunohistochemically, the cells of this tumor were reactive for alpha-smooth muscle actin and vimentin. The leiomyosarcoma recurred and metastasized to the liver. Eight months after onset of symptom, the patient developed hepatic coma and died. The mean age at presentation with pheochromocytoma in von Recklinghausen's disease patients age is 42 years. Our patient was considerably older. To the best of our knowledge this is the first report of a patient with von Recklinghausen's disease developing polymyositis. asymptomatic pheochromocytoma, and primary hepatic leiomyosarcoma and illustrates the need to remain aware of the possibility of cancer in von Recklinghausen's disease.     

A case of primary leiomyosarcoma of the liver presenting with acute bleeding]

Leiomyosarcoma is a rare tumor of the liver. It usually arises from many other organs including uterus, gastrointestinal tract, retroperitoneum, and soft tissues. Primary hepatic leiomyosarcoma progresses very slowly and is not associated with chronic liver disease. When the tumor is detected early enough to be treated by operation, the prognosis is favorable. While several cases of primary hepatic leiomyosarcoma have been reported in Korea, there was no case associated with acute bleeding. We report a 80-year old male patient with huge primary hepatic leiomyosarcoma, who presented with acute bleeding and IVC obstruction. The patient was treated by embolization and IVC stenting.     

Primary Leiomyosarcoma of the Liver: Diagnostic Efficacy of Endoscopic Retrograde Cholangiopancreatography

Abstract: A small primary leiomyosarcoma of the liver detected in a 58-year-old man is presented in this paper. In addition to the findings obtained by gray scale ultrasonogruphy and computed tomography, the possible diagnosis of this very rare tumor was suggested by endoscopic retrograde cholangiopancreatography (ERCP). The characteristic findings of leiomyosarcoma of the liver obtained by ERCP were skipping strictures at the intrahepatic bile ducts. The ERCP findings and diagnosis were verified bv pathological studies of the resected material. This appears to be the first report discussing the usefulness of ERCP in vnaking a diagnosis of primary leiomyosarcoma of the liver.     

CASE REPORT: A long-term survivor of jejunal leiomyosarcoma with liver metastasis: Effective transcathetel arterial embolization for hepatic metastatic foci

We report a case of jejunal leiomyosarcoma with liver metastases in a 52-year-old Japanese male. An echogram demonstrated multiple cystic liver masses in April 1991. The diagnosis of metastatic leiomyosarcoma was made on the basis of characteristic hepatic angiography images and liver biopsy findings. The jejunal leiomyosarcoma was resected and unresectable liver metastatic foci were treated repeatedly with transcathetel arterial embolization. Transcathetel arterial embolization was considered to be effective in this case as the patient survived 4 years and 9 months after the first treatment.     

Primary hepatic leiomyosarcoma: A report of two cases.

Two cases of primary hepatic leiomyosarcoma are reported. Review of the literature reveals only seven previous reports. Cases presented predominantly with either hepatomegaly or ascites. Neither history nor physical and laboratory examination distinguished these patients with primary leiomyosarcomas from those with other hepatic tumors. Analysis of the cases reviewed suggests that aggressive hepatic surgery may improve prognosis.     

Metastasis of uterine leiomyosarcoma to the pancreas--usefulness and limitations of EUS-FNA

A woman in her 60's presented with a tumor of the pancreatic body. Pan-hysterectomy had been performed under a diagnosis of uterine leiomyoma 11 years previously. A sample obtained by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) revealed the histopathological proliferation of spindle-shaped bundles of atypical cells, and immunohistochemical staining demonstrated that these cells were positive for KIT. Therefore, distal pancreatectomy was performed under a diagnosis of pancreatic gastrointestinal stromal tumor (GIST). Immunohistochemical staining of surgical specimens demonstrated that the tumor cells were positive for desmin and negative for KIT and CD34. The low-grade leiomyosarcoma in pathological specimens of the uterine myoma obtained 11 years previously histologically resembled the pathological findings of the pancreatic specimens except for atypical nuclei and mitotic cells. Therefore, the final diagnosis was extremely rare metastatic leiomyosarcoma of the pancreas. Herein, we report metastasis of uterine leiomyosarcoma to the pancreas and discuss the usefulness and limitations of EUS-FNA.