原发性肝脾T细胞淋巴瘤三例报告并文献复习

 目的 探讨原发性肝脾T细胞淋巴瘤的临床表现、病理学特征和免疫表型特点。方法 对3例患者的临床资料进行回顾性分析并对相关文献进行复习。结果 患者肝脾明显肿大,伴有发热、全血细胞减少及肝功受损;病理示瘤细胞弥漫浸润肝/脾脏红髓血窦;免疫表型：瘤细胞表达CD+2、CD+3、CD-4、CD-8,TCR-γ／δ（+）。结论 肝脾T细胞淋巴瘤是较为罕见的外周T细胞淋巴瘤,预后较差,应注意与恶性组织细胞病鉴别。     

肝脏原发性淋巴瘤

原发性肝脏淋巴瘤临床上较为罕见,迄今为止,文献共报道29例。作者等报告经病理证实的肝脏原发性淋巴瘤9例,均为男性,平均年龄55岁,并对本病的临床表现、实验室检查、病理和诊疗等略作讨论,结果如下。9例原发性肝脏淋巴瘤患者中8例 AFP正常,7例 CEA 正常,7例碱性磷酸酶升高。8例 LDH 升高,5例 SGOT 升高,4例血清白蛋白稍偏低;7例患者作腹部 CT,均呈孤立性肿块,其中6例在肝右叶,1例在肝左叶。     

原发性肝癌21例误诊分析

本文分析误诊的21例原发性肝癌。误诊为肝脓疡、肝炎或肝硬化、肝细胞增生、血管瘤和胰腺癌。误诊原因是1,未严格按照肝细胞癌诊断标准诊断。2,未作AFP与SGPT动态观察。3,过于相信个别的辅助检查结果。分析结果提示:如果HBsAg阳性的肝病患者AFP升高,必须密切随访。参照显像检查的结果必须结合临床表现。如果肝脓疡疗效不佳应作剖腹探查,以免误诊。     

经皮超声引导下腹腔肿块穿刺活检确诊小肠淋巴瘤 1 例并文献复习

目的：探讨小肠原发性恶性淋巴瘤（primary malignant lymphoma of small intestine）的临床特点和诊治方法.方法：回顾性分析我院收治的1例小肠原发性恶性淋巴瘤的临床资料,分析其临床、病理及治疗的特点.结果：不明原因腹腔肿块,在超声引导下腹腔穿刺,取病理行免疫组化,获得组织学证据.结论：该病起病隐匿,临床表现缺乏特异性,容易漏诊或误诊,缺乏有用的确诊手段,超声诊断对提高确诊率很有帮助.     

肝、胆、胰、脾

经多种途径射频消融术治疗肝脏肿瘤，MDR1特异性核酶逆转肝癌多药耐药的实验研究,经肝门血管解剖和靶肝段染色引导下肝段切除术加术中灌注化疗治疗原发性肝癌,肝切除术治疗原发性肝癌破裂出血26例分析,肝外伤的诊治（附70例报告）     

肝外病灶为首发现表现的原发性肝癌（附4例临床分析）

本报告4例肝原发病灶症状尚不明显而转移病灶的症状为其首发临床表现的原发性肝癌。4例病例的共同特点是就诊时仅发现有肝外转移病灶，而肝内未能发现占位性病变，AFP均＞400ng/ml。初诊均误诊为良性肿瘤。确诊依据为病理检查，病人虽属晚期，因其肝内病灶出现较晚，生存期相对较长。     

肝细胞腺瘤（附9例临床病理特征分析）

目的 研究分析肝细胞腺瘤临床病理学特征有助于提高临床诊治水平。方法 复阅所有病例临床病理资料,AFP、PCNA、P53蛋白检测采用免疫组化ABC法。结果 本组患者男性4例,女性5例,年龄20—58岁(平均34.5岁),肿瘤直径3.2—16.5cm(平均11.4cm),MRI提示肝脏有等高信号占位性病变,组织病理形态学呈良性改变,AFP、PCNA、P53蛋白染色均为阴性。结论 肝细胞腺瘤术前易误诊为原发性肝癌或海绵状血管瘤,如就诊患者为年轻女性,有或无腹痛,实验室检查指标正常,影像学提示肝脏占位性病变,临床应考虑肝细胞腺瘤的诊断,必要时行肝穿刺活检。     

肝动脉栓塞加部分脾栓塞治疗原发性肝癌伴脾亢的临床研究

对原发性肝癌伴肝硬化脾亢患者行肝动脉化疗栓塞加部分脾栓塞与单纯肝动脉化疗栓塞对照研究。结果显示，肝动脉化疗栓塞加部分脾栓塞疗效满意。动态观察血常规、肝功能及各项免疫指标，发现该组血细胞下降较单纯肝动脉化疗栓塞组明显减少，两组比较有显著差异（Ｐ＜０．０１），再次治疗的间隔时间缩短。长期观察发现，肝动脉化疗栓塞加部分脾栓塞组治疗前后肝功改善也较单纯肝动脉化疗栓塞组明显。提示肝动脉化疗栓塞加部分脾栓塞是治疗原发性肝癌伴肝硬化脾亢患者的有效方法之一     

原发性肝脏弥漫性大B细胞淋巴瘤的特点分析--附4例报告及文献复习

目的报道4例原发性肝脏非霍奇金淋巴瘤，分析其病理组织形态学特点及临床表现。方法组织形态学结合免疫组织化学研究，对4例原发性肝脏非霍奇金淋巴瘤的临床表现、病理形态学特点进行探讨。结果本组原发性肝脏非霍奇金淋巴瘤均为弥漫性大B细胞淋巴瘤，患者为中老年人；组织学检查，病变具有身体其他部位弥漫性大B细胞淋巴瘤的特点；免疫组织化学染色显示，瘤细胞HCVAb、CD20、CD79a和CD30阳性表达，HBVAb、CD3和CD45RO阴性表达。结论原发性肝脏淋巴瘤发病率低，临床表现类似于肝脏炎症性疾病表现，并常被误诊为急性肝炎等疾病，导致临床处理不当，延误治疗。在结合临床检查的基础上，通过组织形态学和免疫组化检查可以作出正确的诊断。     

肝内胆管癌23例临床特点和诊治分析

[目的]探讨肝内胆管癌的临床特点、诊治和预后,以进一步提高其诊治水平。[方法]回顾性分析1993年12月至2009年8月经病理证实的肝内胆管癌23例患者临床资料,其中19例经开腹手术,包括单纯肝脏活检术7例,姑息性手术9例,根治性切除术3例,另4例经影像学定位下经皮肝脏穿刺活检。[结果]全组临床表现无特异性,合并肝内胆管结石或有肝内胆管结石手术史者47.9%（11/23）,伴肝硬化者8.7%（2/23）,血清CA19-9明显升高者52.2%（12/23）,B超、CT诊断符合率分别为39.1%（9/23）、81%（17/21）。保守或姑息手术治疗者1年生存率15%（3/20）,3例行根治性手术者1年生存率100%（3/3）。[结论]肝内胆管癌无特异性临床表现,根治性手术可明显延长其生存期。     

Veno-occlusive disease of the liver in children following chemotherapy for acute myelocytic leukemia

Three children developed acute veno-occlusive disease of the liver following combination chemotherapy for acute myelocytic leukemia. The clinical presentation was similar in all three, with acute onset of hepatomegaly and thrombocytopenia in the absence of significant transaminasemia or icterus. In all three patients, radionuclide imaging with technetium-99m sulfur colloid showed hepatosplenomegaly, decreased liver uptake, and increased splenic activity. The results of liver biopsy established the diagnosis, revealing marked centrilobular congestion with hemorrhage into the spaces of Disse, atrophy of central hepatic cords, and edema of the walls of the central and sublobular veins. Each patient showed marked improvement following temporary cessation of chemotherapy. The diagnosis of veno-occlusive disease is suggested by the triad of: (1) clinical signs and symptoms; (2) scintigraphic findings; and (3) temporal relationship to chemotherapy.     

Primary systemic amyloidosis

Opinion statement Patients with unexplained heart failure, hepatomegaly, nephrotic syndrome, or peripheral neuropathy should be evaluated for primary systemic (amyloid lightchain, or AL) amyloidosis by first seeking evidence of a clonal plasma cell disorder with serum and urine immunofixation studies, as well as a bone marrow biopsy. Immunostaining of the marrow biopsy for lambda and kappa isotypes will usually demonstrate a dominant clonal population of plasma cells if immunofixation studies are negative (less than 10% of cases). Tissue diagnosis of amyloidosis should be sought by biopsy of the abdominal fat or an involved organ. In addition, patients with stable myeloma or monoclonal gammopathy of undetermined significance who develop such conditions or become progressively ill should be evaluated for amyloidosis. We recommend that newly diagnosed patients with AL amyloidosis, who meet criteria for autologous hematopoietic cell transplantation, be considered for highdose melphalan with stem cell support. Criteria usually include adequate cardiac, pulmonary, and hepatic function. AL amyloidosis patients treated with autologous transplantation frequently achieve durable complete remissions of the plasma cell disease and marked improvement in amyloid-related organ dysfunction. AL amyloidosis patients with dominant cardiac amyloid, who are without symptomatic pleural effusions and have no history of cardiac syncope or symptomatic arrhythmias, may be considered for autologous transplantation but are at increased risk of peritransplant mortality. Autologous transplantation should not routinely be offered to patients with dominant cardiac amyloid with recurrent effusions or histories of syncope or arrhythmias or to patients older than 50 years of age with more than two major organ systems involved (eg, heart, kidneys, liver, and peripheral nerves). We recommend that AL patients with isolated advanced cardiac or hepatic amyloidosis be considered for solid organ replacement followed by autologous transplantation. Otherwise, AL patients who are elderly or ineligible for autologous transplantation may be treated with oral melphalan (Alkeran, GlaxoWellcome, Middlesex, UK) and prednisone; however, because the response rate is only about 25% and the prognosis poor, such patients might also be enrolled on clinical trials of emerging therapies.     

A bulky single mass of the liver that revealed a primary hepatic leiomyosarcoma

Primary hepatic leiomyosarcoma is a very rare disease, with only few cases reported in the literature. Here in, we present a case of primary hepatic leiomyosarcoma in woman age 56, revealed by abdominal pain and hepatomegaly. Computed tomography and magnetic resonance imaging (MRI) demonstrated a large mass in the right hepatic lobe associated with lung metastasis. Fine needle hepatic biopsy showed a leiomyosarcoma. Right hepatectomy was performed followed by six cycles of systemic chemotherapy (doxorubicin + ifosfamide). This combined treatment may be the optimum management of this disease. This patient experienced eighteen months of survival despite a very bulky tumor.     

Primary Non-Hodgkin's Lymphoma of the Liver: Case Report

Abstract

This paper describes a rare occurrence of primary lymphoma of the liver in a young female and demonstrates the possibility of making the correct diagnosis by ultrasonically guided fine needle aspiration biopsy. A 32-year old female suffering from upper abdominal pain, hepatomegaly, nausea, anorexia and weight loss for almost 2 months was admitted to our Department. After a clinical and instrumental (lab exams, ultrasonography, computed tomography) evaluation, we reached the correct diagnosis of hepatic primary non-Hodgkin's lymphoma by means of ultrasonically guided fine needle aspiration biopsy. Two weeks after hospitalization the patient was treated with 8 cycles of CHOP chemotherapy and then with α-2b interferon immunotherapy. The hepatic ultrasonography and CT abdominal scan showed the complete absence of the lym-phomatous lesions 36 months later. Up to February 1998, the patient was well and led a normal life. We conclude that the CHOP chemotherapy plus interferon immunotherapy were effective and well tolerated with a complete response 38 months following diagnosis.     

B lymphocytic lymphoma (large cell) of possible splenic marginal zone origin presenting with prominent splenomegaly and unusual cordal red pulp distribution

Two cases of large cell lymphoma, B-cell type, primarily involving the red pulp of the spleen rather than the white pulp are described. A number of unusual features suggest that this may be a lymphoma originating from a distinct splenic B-cell lymphocyte whose origin may be the marginal zone of the spleen or the splenic cords. The patients presented with splenomegaly, cytopenias, and no peripheral lymphadenopathy. The gross appearance of the spleens was beefy red without tumor nodules. The tumor cells were primarily in the splenic cords and surrounding residual normal white pulp. There was a minimal hemic phase. The tumor cells had abundant cytoplasm, surface IgM, IgD, kappa, and FC receptors, tartrate-resistant acid phosphatase, but no alkaline phosphatase or interleukin-2 receptors. They had a similar DNA aneuploidy. The most unusual feature was that tumor cells in both cases had phagocytic properties. These lymphomas may be clinically more indolent than their follicular center counterparts.     

Primary lymphoma of the liver. Ten cases and a review of the literature

Ten adult white patients (21-75 years old; six women, four men) presented with large cell lymphoma of the liver. Each complained of abdominal pain and/or an abdominal mass, and five had B-symptomatology of weight loss, fever (one patient), and night sweats (three patients). At laparotomy (eight patients) or by computerized tomography, liver-spleen scan and lymphangiogram (two patients with percutaneous liver biopsy only), the liver was the sole site of involvement, although subsequent staging procedures revealed bone marrow involvement in three patients. Initial biopsy interpretation was incorrect in four cases which were diagnosed as poorly differentiated carcinoma. Although uncommon, the differential diagnosis of primary liver lymphoma should be entertained when patients present with solitary (three cases) or multiple (six cases) liver defects, particularly when alpha-fetoprotein and carcinoembryonic antigen levels are normal. One patient had diffuse hepatomegaly. Treatment included biopsy (eight patients) or resection (two patients) followed by combination chemotherapy. All patients are alive from 0 to over 10 years (mean, 2.4; median, 1.8 years): six in complete remission, two with less than 6-months follow-up, and two with recurrent lymphoma. Examination of this group of patients along with 19 cases identified in the literature suggests that this is a more treatable disease than primary liver carcinoma.     

肝脏淋巴瘤CT诊断及临床分析

目的 肝脏淋巴瘤临床罕见,既往容易误诊误治.本研究通过分析5例肝脏淋巴瘤的CT表现及临床特点,以期提高对其诊治水平.方法 回顾性分析2009-02-11-2016-04-30经3家医院(淄博市第一医院1例,上海市金山区中西医结合医院1例,复旦大学附属中山医院3例)诊治的5例病理及临床治疗证实的肝脏淋巴瘤患者的CT及临床资料.结果 5例中原发2例,继发3例.3例表现为肝内边界较清楚的结节和(或)肿块,最大直径34～107 mm,1例多发,2例单发;另外2例呈肝内边界模糊的多发斑片状“地图样”浸润.CT平扫病灶呈较均匀低或稍低密度,CT值为19.8～43.4 Hu.增强5例肿瘤动脉期均轻度强化,门脉期呈轻到中度持续强化,但弱于正常肝组织,平均CT值45.6～63.9 Hu,延迟期强化均减弱呈较低密度.4例肿瘤中见“血管漂浮征”;3例见腹腔、腹膜后肿大淋巴结,较均匀中度强化;临床3例出现低热及浅表淋巴结肿大;2例出现腹痛、腹胀及消瘦;1例扪及腹部包块;1例无特殊症状.5例均无明显肝硬化,4例出现肝功能异常,2例合并乙型肝炎.2例原发性者经外科手术治疗及内科治疗,3例继发性者经中西医结合内科综合治疗.4例经随访分别生存16～39个月,平均生存21个月,1例术后随访至7个月,未见复发转移.结论 肝脏淋巴瘤罕见而预后不佳,临床以低热、全身消耗症状、肝脏肿块及腹痛为主,CT可以显示病灶的一些病理学变化,对其诊断具有较大价值.     

A case of hepatocellular carcinoma with extrahepatic portal vein obstruction due to tumor invasion, responsive to oral administration of UFT

A 38-year-old male was admitted to Ikeda Municipal Hospital because of thrombocytopenic purpura and abnormal liver function. Computed tomography and ultrasonography of the liver showed a 5 by 5 cm mass in the right lobe of the liver. Hepatic angiography revealed a hypervascular tumor in the right lobe of the liver and extrahepatic portal venous obstruction. A diagnosis of hepatocellular carcinoma with extrahepatic portal venous obstruction due to tumor invasion was made. Endoscopic examination showed marked esophageal varices with red color sign. Oral administration of UFT at a daily dose of 400 mg was started. Though ascites and frequent hemorrhage from esophageal varices were observed, these symptoms disappeared completely after 8 months administration of UFT. Endoscopic examination revealed disappearance of the esophageal varices, and recanalization of the extrahepatic portal vein was confirmed by the second hepatic angiography. Computed tomography showed no growth of the liver tumor. These data suggest that UFT was effective in this case.     

Cystadenoma of the liver treated by hepatic resection

The aim of this study is to report a case of cystadenoma in the liver and to discuss the difficulty of differential diagnosis from malignant tumor and the importance of complete resection of the lesion. The patient had epigastric pain and abdominal distension. Hepatic ultrasonography, abdominal CT, magnetic resonance imaging, and angiography showed the presence of a large cystic tumor occupying the right hepatic lobe indicating malignant cystadenocarcinoma. Needle biopsy of the cyst wall with sampling of the cyst fluid was performed, which revealed no malignant cells. However, CEA and CA19-9 were markedly elevated in the cyst fluid. A right trisegmentectomy via laparotomy was performed successfully. The resected tumor was 11.5x9x9 cm in size and weighed 1,240 g. The cut surface revealed the tumor with multiple cysts, in some parts of which large papillary projections into lumina were seen. Though the gross features were similar to those of cystadenocarcinoma, the final histological diagnosis was benign cystadenoma of the liver. The postoperative course was uneventful, and the patient is in good condition six years postoperatively without evidence of recurrence of the disease.     

Role of intra-arterial hepatic chemotherapy in the treatment of colorectal cancer metastases

Hepatic metastases are common with colorectal cancer. The primary blood supply to hepatic metastases is the hepatic artery. Regional chemotherapy utilizing the hepatic artery is one treatment option for liver metastases. The advantage of hepatic arterial chemotherapy is that high concentrations of the therapeutic drug are obtained in the liver with minimal systemic toxicity. Recently, systemic chemotherapy regimens have been added to hepatic arterial infusional chemotherapy to treat hepatic metastases. Due to the high response rates in the liver, resection rates are increasing in patients originally thought to have unresectable liver disease. Hepatic arterial chemotherapy has also been used in the adjuvant setting after resection of all liver metastases in order to minimize hepatic recurrences. The role of hepatic arterial infusional therapy in treating hepatic colorectal metastases includes treating patients with both resectable and unresectable metastases in the adjuvant, neoadjuvant, or palliative settings.