原发性肝脏平滑肌肉瘤诊断和治疗体会

目的探讨原发性肝脏平滑肌肉瘤（PHLMS）的诊断和鉴别诊断。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的肝脏病理切片。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检查。     

巨噬细胞与血管内皮生长因子在消化道平滑肌肿瘤中的表达及意义

目的 探讨消化道平滑肌肿瘤组织中巨噬细胞(CD68)和血管内皮生长因子(VEGF)的表达和相互关系。方法 应用SP免疫组织化学方法对74例消化道平滑肌肿瘤及22例正常组织进行CD68及VEGF的检测和分析。结果 CD68在平滑肌瘤、平滑肌肉瘤及正常平滑肌组织中的阳性率分别为82.22％、37.93％、22.73％,平滑肌瘤中的阳性表达率显著高于平滑肌肉瘤和正常组织(P<0.05);VEGF在平滑肌瘤和平滑肌肉瘤中的阳性表达率为15.56％、82.76％,平滑肌肉瘤中的阳性率显著高于平滑肌瘤(P<0.05);在平滑肌肉瘤和平滑肌瘤中VEGF和CD68的表达呈显著负相关(r s分别为-0.7292和-0.6998,P<0.05)。结论 消化道平滑肌瘤中有CD68的高表达,肉瘤中则有VEGF的高表达,与肿瘤的生物学特性有关,CD68和VEGF的表达在消化道平滑肌肿瘤中有等级相关性。     

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     

低度恶性子宫内膜间质肉瘤14例病理特点分析

采用HE及免疫组织化学染色法分析14例低度恶性子宫内膜间质肉瘤(LGESS)的临床病理特点,结果HE染色示肿瘤组织成巢团样浸润,肿瘤细胞呈圆形、卵圆形或梭形,肿瘤内有大量小血管;免疫组织化学染色(8例)示CD10均阳性,角蛋白均阴性;4例波形蛋白阳性,3例ki-67抗体阳性指数<40%;2例伴性索样分化者ER、PR阳性,3例伴平滑肌分化者中2例SMA阳性,1例结蛋白阳性.提示LGESS确诊主要依靠组织病理学和免疫组织化学检查;CD10是一种比较特异的标记.     

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和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:一、临床资料:     

肝上皮样血管平滑肌脂肪瘤2例

目的:探讨肝上皮样血管平滑肌脂肪瘤的病理诊断及鉴别诊断.方法:收集武钢总医院病理科2007-2009年手术切除肝上皮样血管平滑肌脂肪瘤标本2例,对2例肝上皮样血管平滑肌脂肪瘤相关临床资料、病理学特征及免疫组织化学进行观察分析并复习相关文献.结果:2病例均为女性,巨检境界较清楚,镜下见肿瘤主要由弥漫的上皮样细胞构成,排列...     

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.     

老年人腹主动脉瘤血管平滑肌细胞相关蛋白表达及巨噬细胞浸润的病理学特点

目的 观察血管平滑肌细胞(VSMC)表达变化及巨噬细胞浸润在老年人腹主动脉瘤中的病理学特点. 方法 对15例老年人腹主动脉瘤与6例正常腹主动脉组织行HE染色、VanGieson法染色和免疫组织化学染色.用免疫组织化学染色检测α-平滑肌肌动蛋白(α-SMA)、组织蛋白酶B及CD68蛋白表达. 结果 老年人腹主动脉瘤病变处胶原容积百分比(9.3±1.9)%,较正常主动脉的(5.3±1.8)%增高(P＜0.05).老年人腹主动脉瘤中组织蛋白酶B和CD68的表达增强分别为0.38+0.07和0.51±0.12,α-SMA表达减弱为0.23±0.05,与正常腹主动脉(分别为0.13±0.06和0.01±0.01,0.33±0.05)比较.差异有统计学意义(P＜0.05). 结论 VSMC相关蛋白表达水平改变及巨噬细胞浸润可能参与了老年人腹主动脉瘤血管壁的破坏.     

肺血管肉瘤的临床病理观察

目的 探讨肺原发性血管肉瘤的临床病理特点.方法 对我院收治的1例,发生于肺的血管肉瘤的临床表现、组织形态学及免疫组化进行分析,并复习相关文献.结果 患者男性,67岁,临床表现主要为咳嗽和痰中带血.光镜下上皮样或梭形肿瘤细胞形成不规则且相互吻合的血管腔隙,伴大片的出血坏死.免疫组织化学染色显示肿瘤细胞CD34、CD31和vim阳性;CK( AE1/AE3)、TTF-1和S-100阴性.结论 原发性肺血管肉瘤具有和其他部位血管肉瘤相似的组织形态学特点,免疫组织化学是其必要的辅诊手段.其临床表现无明显特异性,早期诊断困难.     

Giant hepatic metastasis from gastrointestinal stromal tumor of the rectum 12 years after surgery

Gastrointestinal stromal tumors are non-epithelial neoplasms that arise from the gastrointestinal tract. Their variable cytologic atypia makes it difficult to predict their prognosis. We report a case of right hepatectomy for a giant metastasis detected 12 years after the surgical treatment of a rectal neoplasm, histologically demonstrated as a low-grade leiomyosarcoma initially, having morphological and immunohistochemical features of low malignancy. Histological examination of the hepatic metastases demonstrated that the tumors were composed of spindle cells similar to those in the rectal neoplasm. Immunohistochemical staining of the hepatic metastases with Ki-67 revealed stronger than the primary tumor. In conclusion, although histological and immunohistochemical analyses provide useful prognostic information, the prognosis of gastrointestinal stromal tumors is difficult to predict. Therefore, a patient with gastrointestinal stromal tumor diagnosed as low-grade malignancy requires carefully long-term follow-up.     

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 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 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.     

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.     

Primary hepatic leiomyosarcoma with liver metastasis of rectal cancer

Primary hepatic leiomyosarcoma is a particularly rare tumor with a poor prognosis. Curative resection is currently the only effective treatment, and the efficacy of chemotherapy is unclear. This represents the first case report of a patient with primary hepatic leiomyosarcoma co-existing with metastatic liver carcinoma. We present a 59-year-old man who was diagnosed preoperatively with rectal cancer with multiple liver metastases. He underwent a curative hepatectomy after a series of chemotherapy regimens with modified FOLFOX6 consisting of 5-fluorouracil, leucovorin and oxaliplatin plus bevacizumab, FOLFIRI consisting of 5-fluorouracil, leucovorin and irinotecan plus bevacizumab, and irinotecan plus cetuximab. One of the liver tumors showed a different response to chemotherapy and was diagnosed as a leiomyosarcoma following histopathological examination. This case suggests that irinotecan has the potential to inhibit the growth of hepatic leiomyosarcomas. The possibility of comorbid different histological types of tumors should be suspected when considering the treatment of multiple liver tumors.     

The First Case of Metastatic Pancreatic Leiomyosarcoma Derived from the Urinary Bladder Diagnosed Using an Endoscopic Ultrasound-guided Fine-needle Biopsy

We herein report the first case of metastatic pancreatic leiomyosarcoma derived from the urinary bladder diagnosed by an endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB) in a 65-year-old woman. The patient had undergone total cystectomy for bladder leiomyosarcoma. Four years thereafter, a nodule was observed in her left lung on chest computed tomography. Suspecting primary lung cancer, pulmonologists at our hospital recommended a thoracoscopic lung biopsy, which the patient refused. Five years post-cystectomy, fluorodeoxyglucose positron emission tomography revealed enlargement of the left lung nodule and a new mass in the pancreatic head. She was referred to our department for the pathological diagnosis of a pancreatic head mass by an EUS-FNB. The EUS-FNB yielded adequate pancreatic tissue for an immunohistochemical analysis. A diagnosis of metastatic pancreatic lesion originating from the urinary bladder was made. In atypical pancreatic tumors, the utilization of an EUS-FNB and immunohistochemical analysis can help establish an accurate diagnosis.