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。     

肝脏未分化胚胎性肉瘤4例

目的:探讨肝脏未分化胚胎性肉瘤(undifferen-tiated embryonal sarcoma of the liver,UESL)的诊断与治疗.方法:回顾性总结分析本院2005-2011年住院手术治疗并经术后病理组织学检查确认的4例UESL患者的临床资料.结果:男1例,女3例,年龄分别为6岁、14岁、14岁和46岁.既往无肝病背景,其临床表现主要为腹部包块、腹痛和发热等.术前常规化验检查显示肝功能基本正常,肝炎病毒标志物和甲胎蛋白等肿瘤标志物均为阴性.术前影像学检查确诊较困难,术后病理组织学检查均确诊为UESL.所有患者均行根治性手术切除,其中2例因出现发热和腹膜炎体征而疑有肿瘤破裂出血,转行急诊手术治疗,并于术后联合行化学药物辅助治疗.结论:UESL是一种少见的高度恶性肿瘤,无特异性临床表现,术前难以确诊;治疗上仍以手术完整切除肿瘤为首选,联合化疗等综合辅助治疗可进一步提高疗效.     

肝脏未分化胚胎性肉瘤的临床病理学研究进展

肝脏未分化胚胎性肉瘤(UES)是发生于肝脏的一种独特的高度恶性的间叶源性肿瘤,常累及儿童及青少年.UES具有发病隐匿、术前确诊率低、术后易复发、预后差等特点,缺乏特异性诊断标志物,分子生物学特征尚待研究,主要依靠病理检查明确诊断.对于不能手术切除,或切除后复发的UES患者,肝移植治疗已经有长期生存的病例报道.本文拟从U...     

肝未分化胚胎性肉瘤1例

一、病例资料患者男性,16岁,上腹部不适伴纳差2个月.2个月前在无明显诱因情况下出现上腹部不适,食欲减退,自觉肝区发硬,无明显肿胀、疼痛,无明显寒战、发热,无恶心、呕吐,无腹痛、腹泻.人院查体:神志清楚,发育正常,营养良好;全身皮肤黏膜无黄染及出血点,浅表淋巴结未扪及,肝区哮隆、饱满,有压痛和叩击痛,腹软,移动性浊音阴性.实验室检查:血常规:白细胞计数11.6× I09/L,单核细胞计数1.19×109/L,略升高;血红蛋白87 g/L,稍低.     

肝右叶未分化胚胎性肉瘤1例

患者,男,58岁,因\"全身乏力1月余\"于2011年6月在当地医院MRI检查,提示肝脏Ⅷ段占位,考虑恶性可能.7月4日PET-CT提示:全脏器反位,肝脏Ⅷ段略低密度影,大小约5 cm×11 cm×8 cm,考虑恶性,腹膜后多发淋巴结转移,胆囊及左肾结石,双肾囊肿.     

原发性肝未分化肉瘤的病理特征(附2例报告)

目的探讨原发性肝未分化肉瘤的病理特征。方法对2例原发性肝未分化肉瘤患者的的病理检查资料作分析。结果肿瘤呈胚胎性间叶性分化,异型性明显并可出现多核瘤巨细胞,核分裂多见,间质为丰富的黏液样基质;肿瘤边缘可见包绕的肝细胞和扩张的胆管,在瘤细胞质内或细胞外基质中可见PAS阳性的嗜酸性球形小体;免疫组化检查瘤细胞Vim、desmin、SMA均可阳性。结论原发性肝未分化肉瘤可能来自原始多潜能干细胞,其组织学呈现为肿瘤形态多样性和分化方向不确定性。     

未分化结缔组织病致继发性膜性肾病1例

未分化结缔组织病(UCTD)患者中有20%～40%发展成一种确定的结缔组织病(CTD);50%～60%的患者仍然维持在UCTD状态;10%～20%的患者症状逐渐减轻,但始终未发展成一种肯定的CTD。目前报道的CTD累及肾脏时通常临床表现轻微,多为镜下血尿或少量蛋白尿,且对肾脏受累病理类型报道较少。本文报道1例确诊UCTD合并继发性膜性肾病(MN)     

儿童肺原发未分化高级别多形性肉瘤1例并文献复习

目的总结儿童肺原发未分化高级别多形性肉瘤(UHGPS)的临床特点。方法报告1例小儿肺原发UHGPS,并对相关文献进行回顾分析。结果患儿女,5岁1个月,临床表现为腹痛、发热伴阵发性咳嗽;胸部CT示右肺中叶肿块,行肺叶切除术;肿瘤位于右肺中叶,包膜不明显;镜下主要由密集的梭形细胞呈束状、车辐状排列,多形区见多少不等的组织细胞样、奇异形、多核巨细胞,具有单个或多个深染的不规则核,核仁明显;瘤细胞Vimentin、CD68、α1-ACT、Lysozyme局灶阳性,CKpan、SMA、Desmin、CD34、CD117、Myo D1、Myogenin、HMB45、S-100、CD45、CD99及ALK均阴性,Ki67约80%阳性;术后9 d出院,密切随访4个月无肿瘤复发转移。结论儿童肺原发UHGPS临床及辅助检查无特异性,病理结合免疫组合有助于诊断,治疗主要是外科手术,预后差。     

未分化肺癌1例误诊分析

患者,女,40岁,反复四肢关节酸痛4年,曾多次查血红细胞沉降率明显高于正常,抗“O”正常,ENA多肽抗体均阴性,四肢关节X片无异常,被诊断为类风湿关节炎。本次因体检发现右上肺阴影而入院手术治疗。入院前曾用雷公藤多甙及激素、云克等治疗,疗效均欠佳,未曾完全缓解过。2002年7月体检发现右上肺阴影直径约3cm左右,对照患者1998年所摄胸片,发现同一部位当时即有阴影直径约1.5cm左右,4年后的胸片显示该阴影在增大,2002年7月给于手术治疗,术后病理证实是肺未分化癌,术后3月胸片及肺CT未发现肺内及肺外转移灶,四肢关节酸痛不治而愈。讨论近年来肺…     

Undifferentiated （embryonal） sarcoma of liver in adult： A case report

We report a case of undifferentiated (embryonal) sarcoma of the liver (UESL), which showed cystic formation in a 20-year-old man with no prior history of any hepatitis or liver cirrhosis. He was admitted with abdominal pain and a palpable epigastric mass. The physical examination findings were unremarkable except for a tenderness mass and the results of routine laboratory studies were all within normal limits. Abdominal ultrasound and computed tomography (CT) both showed a cystic mass in the left hepatic lobe. Subsequently, the patient underwent a tumor excision and another two times of hepatectomy because of tumor recurrence. Immunohistochemical study results showed that the tumor cells were positive for vimentin, alpha-1-antichymotrypsin (AACT) and desmin staining, and negative for alphafetoprotein (AFP), and eosinophilic hyaline globules in the cytoplasm of some giant cells were strongly positive for periodic acid-Schiff (PAS) staining. The pathological diagnosis was UESL. The patient is still alive with no tumor recurrence for four months.     

Undifferentiated liver embryonal sarcoma in adults: A report of four cases and literature review

AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. RESULTS: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laborator...     

Undifferentiated embryonal sarcoma of liver in an old female：Case report and review of the literature

The clinical characteristics of undifferentiated（embryonal） sarcoma of the liver（UESL） were investigated and the best treatment modalities were recommended.Both histology and immuno-histochemistry demonstrated the cellular features of this peculiar tumor.The tumor size was 12 cm × 9 cm × 8 cm in the right liver lobe.The patient underwent surgical resection of the tumor.The postoperative recovery was uneventful and she died eight months after diagnosis.The tumor showed mixed spindle and polygonal cells within the myxoid matrix.Some tumor cells contained eosinophilic hyaline globules that were positive for resistant diastase.Immunohistochemistry showed positive vimentin.Stellate and spindle cells were positively stained with alpha-1-antichymotrypsin（AACT） and CD68.This case indicates that UESL is not obviously differentiated in old-aged adults.     

Undifferentiated embryonal sarcoma of the liver: case report and literature survey

Undifferentiated embryonal sarcoma (UES) of the liver was first identified as an independent clinicopathologic type of sarcoma in 1978. It is an uncommon hepatic tumor, of mesenchymal origin, usually observed in children, and cases in adults are rare: to our best knowledge, reports of only 51 cases have been published in the past 50 years. We present a case of UES of the liver in a previously healthy 22 year-old woman, admitted to our hospital due to a palpable mass in the right upper abdomen. On admission, laboratory studies showed mildly elevated aspartate aminotransferase, alkaline phosphatase, and γ-GPT. Hepatitis and tumor markers were negative. Ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) showed a large mass involving the right lobe and the medial segment of the liver, with compression of the bile duct. Right trisectionectomy with bile duct resection and reconstruction was performed. Microscopically, the tumor was composed of pleomorphic spindle cells in a myxoid stroma with focal staining of S-100 by immunohistochemistry. The histologic diagnosis was UES. Adjuvant therapy with vincristine, actinomycin-D, and cyclophosphamide was performed, and at 14 months of follow-up, the patient is alive without any evidence of recurrence. The clinical and histopathological features, as well as the therapeutic choices for adult UES, are described for this patient and in the literature of the past 50 years.     

Undifferentiated embryonal sarcoma of the liver presenting as a hemorrhagic cystic tumor in an adult

BACKGROUND:Undifferentiated embryonal sarcoma (UES) of the liver is a rare,highly malignant neoplasm with a poor prognosis occurring almost exclusively in late childhood (6-10 years of age).Only a few cases have been reported in adults accounting for less than 1% of all primary liver neoplasms.METHODS:A 47-year-old woman presented with a palpable mass in the left upper abdomen.Magnetic resonance imaging revealed a 12×10 cm cystic mass with hemorrhage in the left lateral segment of the liver.The initial impr...     

Non-random p53 mutations in pediatric undifferentiated (embryonal) sarcoma of the liver.

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare hepatic tumor in children. Its pathogenesis is largely unknown, but lines of evidence suggest common links to that of mesenchymal hamartoma of the liver (MH). Previously, we found a p53 mutation in a case of pediatric USL. Recently, there was another published report, demonstrating further evidence of p53 alterations in the adult cases. In this study, we analyzed in three cases of pediatric USL and two cases of MH by using PCR-SSCP and direct sequencing technique. The study identified missense mutations in all three cases of USL, but none of MH. The mutations were found specifically in tumor tissue and not detected in the surrounding normal hepatic tissue. Mutation points were localized in exon 7 (Gly245Ser), exon 6 (Arg196Pro), and exon 8 (Arg273Pro), respectively. Immunohistochemical study of p53 protein expression revealed strong immunoreactivity in cases of USL and negative staining in MH. In summary, this study provided a novel data suggesting that mutations of p53 in pediatric USL are not random genetic events and highly possible to be involved in its tumorigenesis.     

Soft tissue sarcoma with metastasis to the stomach: a case report

Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common site of metastasis is the lung, with other sites being bone, the brain, and the liver. Metastasis to the gastrointestinal tract has rarely been documented. We present an unusual case of high-grade pleomorphic sarcoma with metastasis to the stomach, complicated by upper gastrointestinal bleedin...     

Undifferentiated embryonal sarcoma of the liver. Case report of an adult woman

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare hepatic tumor and is generally considered an aggressive neoplasm with unfavorable prognosis. USL has a predilection for children and young adults in the first two decades of life, although a few cases have been reported in adults. The present report describes occurrence of such tumor in a 24-year-old woman who came for treatment in our hospital. She underwent exploratory laparotomy, and tumorectomy was performed. Histological examination resulted in diagnosis of USL. The patient died one month after her operation. Clinical and histopathologic features of this tumor are discussed.     

Nonleukemic myeloid sarcoma of the liver: a case report and review of literature

Myeloid sarcoma (MS) is a neoplasm of immature granulocytes, monocytes, or both involving extramedullary sites. MS with no evidence of leukemia (nonleukemic MS) is very rare and the initial diagnosis can be difficult. This report describes an unusual case of nonleukemic MS of the liver in a 16-year-old patient presenting as debilitating hepatomegaly. A liver biopsy revealed diffuse infiltration by neoplastic cells of myeloid lineage (CD68, myeloperoxidase). A bone marrow biopsy showed no evidence of medullary involvement. The patient subsequently developed heart failure. Autopsy revealed infiltration of most organs by neoplastic cells but failed to identify abnormal myeloid cells in bone marrow.