原发性气管和支气管淀粉样变1例

患者女,32岁.间断性咳嗽伴胸部憋闷1年余,加重1个月.查体:T 36.5℃,P 82次/min,R 22次/min,ESR20mm/h.无紫绀,气管左偏,心浊音界左移,左肺呼吸音极低.心、肝、脾、肾及皮肤均无异常发现.胸部X线及CT示左肺不张,纵隔显著左移(图1).纤维支气管镜检查:气管左侧壁延至隆突,左主支气管开口处有淡黄色隆起物,呈细小颗粒状,左主支气管管口堵塞.取送病理为淀粉样变.予以强地松60mg,每日1次,治疗2个月,症状明显减轻,X线胸片示左肺膨张良好,纵隔位置正常(图2).复查纤维支气管镜:气管、隆突和左主支气管开口处仍可见散在颗粒状隆起物,但较前稀疏,左主气管口通畅且呈不规则状.     

胰腺肝样腺癌1例报告并文献复习

目的 报道1例胰腺肝样腺癌，探讨血清甲胎蛋白AFP阳性的胰腺肝样腺癌的组织学表现、临床特点及其与预后的关系。方法 对1例女性病人的临床资料复查及病理切片行免疫组织化学标记，选用的一抗有AFP、α1-AAT、α1-ACT、CEA、EMA、AE1／AE3、SYN、CgA、NSE、S．100、CD34、PCNA、C-erbB-2，并复习相关文献。结果 肿瘤具有2种结构：①具有与肝细胞癌相似的组织学表现，实体状，小梁状，大梁状，血管血窦丰富，免疫组织化学染色AFP弥漫性强阳性，而CEA、EMA灶性阳性，癌细胞为CD34阳性的血管分割成小梁状。②腺癌组织学表现为实体巢状或弥散分布，以及肿瘤性的乳头状上皮、筛状结构，免疫组织化学染色CEA、EMA阳性，而AFP灶性阳性。结论 胰腺肝样腺癌具有独特的病理形态和临床特点，是原发性胰腺癌少见的特殊亚型，具有易于复发或转移、预后差的临床特点。     

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。     

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。     

双胞胎肝母细胞瘤3例报告并文献复习

  目的  分析肝母细胞瘤的家族遗传倾向, 为临床遗传咨询及早诊断、早治疗提供依据。  方法  收集中国医科大学附属盛京医院2005年6月至2011年11月间收治的3例双胞胎肝母细胞瘤患儿的资料, 结合患儿生化、影像、治疗方案、生存期、家族史及相关文献报道分析遗传相关因素。  结果  本文中3例双胞胎患儿均为单发, 无家族史, 其中1例为早产儿。3例患儿均采用手术治疗, 经病理证实为肝母细胞瘤, 术后1例患儿因失血性休克死亡, 余2例采用不同的化疗方案, 均获得完全缓解。自1969年至今, 已有8例关于兄弟姐妹同患此病的文献报道。8组文献中5组患儿家族中有肿瘤(多数为结肠癌)及家族性结肠息肉病史, 其中3组还有基因异常改变。  结论  肝母细胞瘤是一种罕见的小儿肝脏恶性肿瘤, 具有家族遗传倾向, 与结肠癌及家族性结肠息肉综合征密不可分, 在诊疗过程中, 有必要加强家属的认知, 做好预防保健, 争取早发现早诊断早治疗。      

原发性肝鳞状细胞癌1例报告

患者,男性,52岁。2004年6月因间歇性高热、全身乏力、上腹饱胀1月余,B超发现肝左叶占位病变3天入院。发热时体温达40-42℃,体温可自行下降。发热至人院时体重下降10kg。既往患肝内、外胆管结石症5年,2002年9月行胆囊摘除术,曾于2003年3月行经十二指肠胆总管取石术。     

肝肉瘤样癌1例并文献复习

目的：探讨肝肉瘤样癌患者的临床及病理学特征、诊断、鉴别诊断、治疗和预后。方法：结合我院收治的肝肉瘤样癌1例临床资料,分析国内外文献报道的该病临床特点、影像学表现、病理学特点,治疗和预后。结果：大部分该病患者均有腹痛和发热的临床表现,病理学上肿瘤都包含明确的恶性上皮性成分和梭形细胞肉瘤样成分且上皮和间叶性两种标记物表达阳性,愈后差。结论：肝肉瘤样癌是一种罕见的恶性肿瘤,恶性程度高,浸润性强,病因不明,目前无有效治疗,预后很差。     

An unusual case of aggressive systemic mastocytosis mimicking hepatic cirrhosis

Hepatic involvement in aggressive systemic mastocytosis（ASM） is relatively common, and the main clinical features of this disease include hepatomegaly, portal hypertension, ascites, and fibrosis. Cirrhosis is a rare ASM symptom. We report an ASM case that initially mimicked cirrhosis based on clinical and radiographic analyses. The portal tract was expanded by mononuclear inflammatory cells, and an increase in collagen amount was observed in routine histological sections of the biopsied liver. A diagnosis of systemic mastocytosis（SM） was made after ancillary tests for mast cells using bone marrow aspirates. Extensive involvement of the liver and gastrointestinal tract was observed. Clinicians and pathologists need to consider ASM as a diagnosis or differential diagnosis in a clinical case of cirrhosis with unknown etiology. The diagnosis can be confirmed or disregarded by immunohistochemical staining and molecular analysis.     

Diagnostic yield of percutaneous image-guided tissue biopsy of focal hepatic lesions in cancer patients: ten percent are not metastases from the primary malignancy

BACKGROUND:

The diagnostic yield was evaluated of percutaneous image‐guided tissue biopsy of hepatic lesions identified on computed tomography performed for staging of a primary malignancy, and it was determined how often the biopsy result was unexpectedly negative, benign, or secondary to a second unknown malignancy.

METHODS:

In a retrospective investigation from 1998 through 2008, 580 patients with primary malignancies had indeterminate focal hepatic lesions and underwent percutaneous image‐guided biopsy; 369 patients had lesions in their liver at first cross‐sectional imaging, performed for staging; 211 patients had a negative liver imaging study, followed by the subsequent appearance of at least 1 indeterminate suspicious lesion. The results of percutaneous image‐guided tissue biopsies were compared with the histology of the primary malignancy.

RESULTS:

Liver biopsies were performed in 580 patients (288 men and 292 women; age, 25‐92 years; mean age, 61 years). The most common primary malignancies were pancreatic (n = 96), breast (n = 85), melanoma (n = 57), esophageal (n = 51), lung (n = 47), colorectal (n = 37), and urothelial tumors (n = 26). Biopsy results were positive for malignancy in 528 (91%) cases. Among the positive biopsies, 29 (5%) cases had pathology results different from the primary tumor. Of the 52 biopsies negative for malignancy, 20 yielded a specific benign diagnosis, and 32 were nondiagnostic.

CONCLUSIONS:

If all liver lesions had been assumed to be metastases, as expected secondary to the known primary tumor, then the true or presumed alternate diagnosis would have been missed in 60 (10.3%) of the 580 cases. The authors did not attempt to determine whether actual clinical management changed based on these 60 liver biopsy results, so this number is an upper bound on management change. On the basis of these results, and given the minimal complication rate of liver biopsy, the authors suggest that liver biopsy should still be performed in the types of cases studied here, despite the finding that the vast majority of biopsies produced the expected result and presumably did not change patient management. Cancer 2011;. © 2011 American Cancer Society.     

精氨酸酶-1在原发性肝癌及肝转移癌鉴别中的作用

目的： 探讨精氨酸酶-1(Arg-1)在原发性肝癌(PHC)和肝转移癌(HM)鉴别中的意义。方法：123例肝癌患者行超声引导下细针穿刺活检,标本制作成细胞块进行免疫组化染色,观察Arg-1表达情况。所有患者行手术切除后进行组织病理学诊断,结合细胞学、血清肿瘤标记物、临床及影像学随访结果进行最终判定。计算采用Arg-1鉴别PHC和HM的敏感度和特异性。结果：123例患者中PHC 78例,HM 45例。在PHC患者中,Arg-1呈高表达(83.3%)且以强染色为主(60.3%),高、中度分化肝细胞癌患者中阳性表达率(92.9%)高于低分化患者(76.9%),在胆管细胞型肝癌患者中不表达,在混合型肝癌患者中阳性表达率为75%;在HM患者中,Arg-1阳性表达率较低(8.9%),存在于结直肠癌、胰腺癌、乳腺癌肝转移患者中且均为弱染色,其他原发部位肝转移患者未见Arg-1阳性表达。Arg-1用于PHC和HM鉴别的敏感度为83.3%,特异性为91.1%。结论：细针穿刺活检标本行Arg-1免疫组化染色用于PHC和HM鉴别的敏感度和特异性均较高,值得进一步推广。     

Hepatic uptake of a bone scan agent (99mTc-MDP) in a patient with amyloidosis associated with plasma cell dyscrasia

This case report describes diffuse hepatic uptake of a diphosphonate bone scan agent (Technetium-99m-MDP) in a patient with amyloidosis associated with a plasma cell dyscrasia. Amyloid infiltration is one of the more rare, but well recognized, causes of hepatic uptake of bone scan tracer. Other causes are also discussed in this case report.     

The accuracy of liver scanning in the detection of metastatic disease.

Isotopic scanning of the liver to detect hepatic metastases is a commonly used clinical tool in assessing the extent of malignant disease and the proper mode of therapy. Opinions vary widely as to the efficacy of this test. To determine the accuracy of our scanning technique the clinical and laboratory data on 429 patients who had hepatic scans (technetium sulfur colloid) between February 1971 and May 1973 was reviewed. Of these patients, 125 had a confirmatory procedure (needle biopsy - 15, abdominal exploration - 69, autopsy - 41) done within 6 weeks of their scan. Nineteen patients who had negative scans were proven to have hepatic metastases. Fourteen patients had positive scans which could not be substantiated by one or more of the above confirming examinations. Thus the frequency of error was 25%. Regarding other tests - alkaline phosphatase and bilirubin - no better correlation could be made with respect to the presence or absence of metastases. From the data available on this small group of patients the liver scan is sufficiently inaccurate to preclude relying upon it for the selection of therapy in cancer patients. When the presence of hepatic metastases changes the type of therapy a confirmatory laparotomy or biopsy should be considered.     

A prospective trial of short‐fractionation radiotherapy for the palliation of liver metastases

The purpose of this study was to prospectively examine the effectiveness and tolerability of a simple radiotherapy technique for the palliation of symptomatic liver metastases. Twenty‐eight patients with symptomatic liver metastases were enrolled from seven centres, and received targeted (partial or whole) liver irradiation consisting of 10 Gy in two fractions over 2 days. Symptoms at baseline were hepatic pain (27 patients), abdominal distension (19), night sweats (12), nausea (18) and vomiting (eight). Twenty‐two patients (76%) had failed previous treatment with chemotherapy, hormonal therapy and/or high‐dose steroids. Symptoms and potential toxicities were prospectively assessed at the time of treatment, then 2, 6 and 10 weeks later. Individual symptom response rates were 53?66% at 2 weeks. Partial or complete global symptomatic responses were noted in 15 patients (54%) overall. The treatment was well tolerated with two patients (7%) experiencing grade 3 toxicity (one vomiting and one diarrhoea); however, four patients reported temporary worsening of pain shortly after treatment. This simple and well‐tolerated treatment achieves useful palliation.     

Management of apparent single foci of epithelial ovarian cancer

Four patients with proven ovarian cancer were found to have single lesions in the liver parenchyma after completing chemotherapy. They otherwise appeared to be in complete clinical remission. Percutaneous needle biopsy was sometimes misleading and dangerous. Laparoscopy and laparotomy were required for optimum safe diagnosis, staging, and treatment. Definitive resection appeared to be beneficial but usually only as a palliative form of debulking. Subsequent adjuvant combination chemotherapy may sometimes improve the duration of useful remissions. Unusual sites of metastatic ovarian cancer appear to be increasing in frequency. Successful management of late recurrences in the liver is possible, provided the diagnosis is immediately suspected and one utilizes both definitive surgery and chemotherapy.     

The distribution of acidic coomassie blue-stained proteins from uninvolved human liver, hepatoma, normal colon, primary colon cancer, and colon metastases to the liver, determined by two-dimensional protein electrophoresis

The distribution of Coomassie blue-stained proteins from uninvolved regions of 4 human livers, from 1 hepatocellular carcinoma, and from 4 samples each of uninvolved colon, primary adenocarcinoma of the colon, and colon cancer metastatic to the liver was analyzed by two-dimensional protein electrophoresis. From a comparison of acidic proteins between pI 3.5 and 6.5, we conclude (1) that the majority (66 of 82) of denoted acidic proteins from 4 normal liver samples were represented in the hepatocellular carcinoma. Fifty-one of 58 proteins denoted in the 4 colon samples were detected in each of the 4 primary colon cancers; (2) that the "normograms" of proteins from normal colon and normal liver differed in many details, and their dissimilar patterns identified the source of the sample; (3) that hepatoma and primary adenocarcinoma of the colon were easily distinguished by their distribution of proteins; (4) that colon cancer metastatic to the liver contained a majority (50/58) of acidic proteins enumerated in primary colon cancer. These results indicate that uninvolved liver and colon and their primary or secondary cancers can be identified by their distribution of electrophoresed acidic proteins.     

Hepatic metastasectomy in children

BACKGROUND: There are little data regarding the safety and efficacy of hepatic metastasectomy for solid tumors in childhood. We reviewed our institutional experience to assess operative mortality and morbidity, technique of resection, local control, and survival in pediatric patients undergoing liver resection for metastases. METHODS: All pediatric patients who underwent hepatic resection for metastatic disease from August 1988 to July 2005 were retrospectively identified and clinical data were collected. RESULTS: Fifteen patients were identified during this period and primary malignancies included neuroblastoma (7), Wilms tumor (3), osteogenic sarcoma (2), malignant gastric epithelial tumor (1), and desmoplastic small round cell tumor (2). Twelve patients underwent anatomical hepatic resections and 3 had wedge resections. There were no intraoperative or postoperative deaths. The 2 postoperative complications included 1 wound infection and 1 bile collection. The median follow-up after hepatic resection was 1.6 years (0.2-7 years). Three patients remain alive. Eleven patients died of progressive disease; 4 patients suffered local recurrence. One patient died from enterocolitis and sepsis and was without evidence of malignancy at the time of death. CONCLUSIONS: Hepatic metastasectomy in children is feasible and is associated with a low operative mortality and morbidity. In this small group of patients anatomic hepatectomy was associated with better local control compared with wedge resection. Overall prognosis in these patients remains poor and the decision to perform hepatic metastasectomy should be highly selective.