成人腹膜后节细胞神经纤维瘤1例暨文献复习

原发性腹膜后节细胞神经纤维瘤在临床上是属于极少见的病例,通常患者就诊时无明显临床症状。本文报道1例43岁成年男性的腹膜后节细胞神经纤维瘤并进行相关文献的复习。已有研究证实影像学检查可对原发性腹膜后肿瘤的良恶性鉴别提供重要依据,节细胞神经纤维瘤的影像学检查亦具有其特征性表现,但最终本病的确诊仍依赖病理及免疫组化技术。影像学检查是确定原发性腹膜后肿瘤的治疗计划的必要措施。目前手术已成为治疗腹膜后节细胞神经纤维瘤的首选治疗。辅以血管外科技术,肿瘤的根治性切除已在临床上广泛开展。通过手术治疗后尽管有二次复发可能,但腹膜后节细胞神经瘤预后仍较好。     

原发性腹膜后副神经节瘤临床诊疗分析

目的:探讨原发性腹膜后副神经节瘤的手术治疗效果,复发及死亡的影响因素。方法:回顾性分析1985年1月至2015年1月30年间于我院治疗的91例原发性腹膜后副神经节瘤的手术患者的临床资料,统计学方法分析腹原发性腹膜后副神经节瘤手术的治疗效果,预后及复发和死亡的相关因素。结果:77例原发性腹膜后良性副神经节瘤患者,l、3、5年总生存率均为98.5%,复发率分别为2%,4.5%,16%。手术根治度与复发相关(X~2=10.368,P=0.01),手术未完整切除复发率高。性别、年龄、肿瘤部位、肿瘤直径与复发无关(P0.05)。14例原发性腹膜后恶性副神经节瘤患者,1、3、5年总生存率分别为78.5%、50%,41.5%。14例恶性副神经节瘤复发率分别为71%,85%,100%。肿瘤级别与腹膜后恶性副神经节瘤预后(X~2=5.536,P=0.019)和复发相关(X~2=5.734,P=0.017),肿瘤级别低,死亡率高,复发率高;远处转移与复发相关(X~2=4.067,P=0.044),远处转移患者复发率高;性别、年龄、肿瘤部位、肿瘤直径、手术根治度与预后及复发无关(P0.05)。结论:良性腹膜后副神经节瘤患者预后较好,手术根治度与复发相关,根治性手术是其主要治疗方式;恶性副神经节瘤预后差,肿瘤分级与其复发及死亡相关,肿瘤分级低复发及死亡率高,远处转移与复发相关,远处转移患者复发率高。     

原发性腹膜后肿瘤46例诊疗分析

目的：总结并探讨原发性腹膜后肿瘤（PRPT）的诊断及治疗方法。方法：回顾并分析2004年1月至2009年12月收治的46例腹膜后肿瘤患者的临床资料及随访结果。结果：良性肿瘤17例,完整切除15例,部分切除2例,复发4例,均再次手术;恶性肿瘤29例,完整切除10例,部分切除15例,活检2例,复发6例,再次手术4例。随访时间0.5至5年,良性肿瘤死于其他疾病3例,死于原发性腹膜后肿瘤1例;恶性肿瘤死亡22例,其中1年内死亡8例,3年内死亡12例,5年内死亡2例。结论：对原发性腹膜后肿瘤,B超、CT及MRI检查是目前诊断PRPT方便、有效的诊断手段,手术治疗是治疗PRPT的首选治疗方式,完整切除肿瘤是影响PRPT治疗效果及其预后的重要因素。对于复发病例应选择再次手术治疗。     

原发性腹膜后肿瘤46例诊疗分析

目的:总结并探讨原发性腹膜后肿瘤(PRPT)的诊断及治疗方法。方法:回顾并分析2004年1月至2009年12月收治的46例腹膜后肿瘤患者的临床资料及随访结果。结果:良性肿瘤17例,完整切除15例,部分切除2例,复发4例,均再次手术;恶性肿瘤29例,完整切除10例,部分切除15例,活检2例,复发6例,再次手术4例。随访时间0.5至5年,良性肿瘤死于其他疾病3例,死于原发性腹膜后肿瘤1例;恶性肿瘤死亡22例,其中1年内死亡8例,3年内死亡12例,5年内死亡2例。结论:对原发性腹膜后肿瘤,B超、CT及MRI检查是目前诊断PRPT方便、有效的诊断手段,手术治疗是治疗PRPT的首选治疗方式,完整切除肿瘤是影响PRPT治疗效果及其预后的重要因素。对于复发病例应选择再次手术治疗。     

原发性肾上腺非霍奇金淋巴瘤（附9 例报告）

目的:探讨原发性肾上腺淋巴瘤(Primary Adrenal Lymphoma,PAL)的临床特点、提高对PAL的认识。方法:回顾分析解放军总医院1995年12月至2007年6月收治的9例PAL的临床表现、实验室检查、影像学特点、组织病理类型以及治疗方法等临床资料,并结合国内外文献进行分析。结果:9例患者中,1例因常规体检发现,8例因腹痛、腹胀或腰痛就诊发现;其中单侧3例,双侧6例,实验室检查无明显异常,影像学检查仅发现肾脏肿瘤,但术后病理组织学诊断为非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL),其中8例弥漫大B细胞淋巴瘤,1例T细胞淋巴瘤;7例患者术后均接受了CHOP或RCHOP方案化疗为主的综合治疗,2例常规治疗;随访至2010年2月,1例弥漫性大B细胞淋巴瘤患者存活4年,1例在术后3年2个月死亡,余7均在2年内死亡。结论:PAL是一种罕见的、恶性程度较高的肿瘤,临床表现和影像学检查缺乏特异性,组织病理学及免疫组织化学是明确诊断的好方法。术前确诊肾上腺原发性非霍奇金淋巴瘤可避免手术,联合化疗应为治疗首选。     

应用基因芯片技术研究原发性腹膜后脂肪肉瘤组织特异性候选基因

目的:原发性腹膜后脂肪肉瘤是一种少见的恶性肿瘤,发病率大约占全部恶性肿瘤的1％,但其却是腹膜后最常见的软组织肿瘤.通过研究原发性腹膜后脂肪肉瘤和正常脂肪组织间的基因表达差异,可以寻找到与原发性腹膜后脂肪肉瘤发生、发展相关的候选基因并为其防治研究提供线索.方法:应用基因芯片技术检测2例原发性腹膜后脂肪肉瘤和相应的正常脂肪组织全基因组序列,分析其基因表达差异.结果:两组间的差异表达基因3828个,其中表达上调有1837个基因,表达下调有1991个基因.其中涉及细胞增殖、黏附、凋亡以及信号通路等多种生物学过程.结论:通过基因表达谱芯片筛选出与原发性腹膜后脂肪肉瘤发生和进展密切相关的基因为其基础研究及临床早期诊断、预防和治疗提供潜在的分子标记和靶基因.     

颅内原发性黑色素瘤的影像学诊断

目的：探讨颅内黑色素瘤的影像学特点。方法：回顾性分析5例经手术病理及临床证实的颅内原发性黑色素瘤的CT和MR影像学表现。结果：CT表现为类圆形高密度影4例,低密度影1例。增强扫描肿瘤出现不均匀强化。MR检查,4例T1WI肿瘤表现为高信号,1例T1wI肿瘤表现为不均匀低信号;5例增强扫描肿瘤明显增强。结论：MRI有助于颅内黑色素瘤的诊断,在一定程度上可以区分原发性和转移性黑色素瘤。     

副神经节瘤伴腰椎转移一例报道并文献复习

目的:探讨腹膜后非嗜铬副神经节瘤(副节瘤)的诊断与治疗方法.方法:诊断为肾上腺癌并腰椎转移的患者,女,38岁;手术采用经胸腹联合切口,切除第9肋,打开膈肌,由于肾脏和肿瘤粘连紧密,行腹膜后肿瘤及左肾联合切除术.结果:肿瘤完整切除,肿瘤10cm× 9cm× 7cm.病理诊断为倾向来源于肾上腺的副神经节瘤.腰椎手术后,病检证实是副神经节瘤转移.结论:恶性副神经节瘤对化疗和放疗不敏感,手术是唯一有效的治疗方法.     

原发性肠系膜肿瘤的诊断与治疗

目的:探讨原发性肠系膜肿瘤的诊断及治疗方法。方法:对各种类型的原发性肠系膜肿瘤的临床表现、术前检查及诊断、治疗等进行对比分析。结果:原发性肠系膜肿瘤临床表现多样、复杂,缺乏特异性诊断依据,但在B超引导下细针穿刺等有助于其诊断。实性肿瘤多为恶性,手术切除率低。囊性肿瘤多为良性,手术切除率高。结论:手术是治疗本病的唯一有效手段。     

γδT细胞在肿瘤免疫治疗中的研究进展

癌症是威胁人类健康最主要的疾病之一,目前临床上主要采用的手术/放化疗联合治疗的疗效有限,因此如何进一步提高肿瘤临床疗效仍是巨大的挑战。免疫细胞过继是肿瘤治疗技术中迅速发展的一种生物治疗技术,通过输入自身或同种"抗肿瘤免疫效应细胞"达到直接杀伤肿瘤或增强机体自身细胞免疫功能的作用。由于γδT细胞对肿瘤细胞具有强有力的直接细胞毒性,并可与其他免疫细胞协同作用发挥其抗肿瘤活性,因而成为了细胞免疫治疗中新的研究热点。本文主要叙述了γδT细胞与肿瘤相关的基础研究及临床试验的最新进展。     

Association of Pheochromocytoma and Ganglioneuroma: Unusual Finding in Neurofibromatosis Type 1

ObjectiveTo report a rare case of association of pheochromocytoma and ganglioneuroma in an asymptomatic patient with neurofibromatosis type 1 (NF1) and to discuss the importance of annual biochemical and imaging studies.MethodsWe present the clinical, laboratory, and pathology findings in a 41-year-old woman with NF1 and review the pertinent literature.ResultsA 41-year-old woman with NF1 presented for a routine gynecologic examination, at which time a right adrenal mass (4 by 3 cm) was discovered by abdominal ultrasonography and confirmed by abdominal computed tomographic scans and magnetic resonance imaging. The patient was normotensive and complained only of discrete essential tremors. Biochemical studies showed a serum epinephrine level of 195 pg/mL (normal,<100) and a 24-hour urine epinephrine excretion of 55 μg (normal,<20), findings consistent with pheochromocytoma. Metaiodobenzylguanidine scintigraphy revealed uptake in the right adrenal gland, with no evidence of metastatic lesions. Before surgical treatment, the patient received an α-adrenergic antagonist for 30 days. Laparoscopic excision of the right adrenal gland yielded excellent postoperative results. Surgical pathology revealed a multinodular mass composed of pheochromocytoma and ganglioneuroma. In patients with NF1 (von Recklinghausen’s disease), a tumor consisting of pheochromocytoma and ganglioneuroma is rare and may be more aggressive than pheochromocytoma alone. An asymptomatic catecholamine-producing tumor may cause substantial morbidity and mortality, especially in patients who are undergoing surgical intervention or are under other stressors.ConclusionThe current guidelines for managing patients with NF1 are an annual history and physical examination. Because of the increased prevalence of pheochromocytoma and ganglioneuroma in patients with NF1, and the potential associated adverse effects, we emphasize the importance of periodic clinical evaluation with biochemical testing and imaging studies. (Endocr Pract. 2007;13:647-651)     

Comment je raisonne devant une masse rétropéritonéale

The retroperitoneal masses are various and heterogeneous and include tumors from different organs (kidneys), the primary retroperitoneal tumors and metastasis. The primary retroperitoneal tumors are rare, frequently malignant with a delayed diagnosis. More than half of them are sarcoma. Rarity and heterogeneity of retroperitoneal tumors explain the difficulties of diagnostic. The goal of this article is to review the imaging characteristics of CT scan and RMN of retroperitoneal mass and to clarify the diagnosis. The management of sarcoma requires an expert multidisciplinary team (pathologist, radiologist, surgeon, oncologist) and pathology analysis by retroperitoneal transcutaneous biopsy.     

Case report of primary retroperitoneal hydatid cyst

Hydatid cyst develops in retroperitoneal space without accompanied lesion in other organs is defined as primary retroperitoneal hydatid cyst. It is extremely rare though hydatid cyst may affect every organ in human body. A 15 years old boy presented with a giant retroperitoneal cyst and hydatid cyst was suspected. Thorough examination did not revealed lesions in liver, lung, kidney or other organs. The diagnosis was confirmed through laparotomy. The diagnosis is difficult, but the possibility should be considered before operation to prevent spillage or fatal anaphylactic shock. Living in an endemic area, ultrasonography, CT or serology test is helpful in the diagnosis of this disease. Total cystectomy is the ideal surgery, if not possible or at high risk, partial pericystectomy is acceptable.     

Structure-guided engineering of human thymidine kinase 2 as a positron emission tomography reporter gene for enhanced phosphorylation of non-natural thymidine analog reporter probe

Positron emission tomography (PET) reporter gene imaging can be used to non-invasively monitor cell-based therapies. Therapeutic cells engineered to express a PET reporter gene (PRG) specifically accumulate a PET reporter probe (PRP) and can be detected by PET imaging. Expanding the utility of this technology requires the development of new non-immunogenic PRGs. Here we describe a new PRG-PRP system that employs, as the PRG, a mutated form of human thymidine kinase 2 (TK2) and 2'-deoxy-2'-18F-5-methyl-1-β-L-arabinofuranosyluracil (L-18F-FMAU) as the PRP. We identified L-18F-FMAU as a candidate PRP and determined its biodistribution in mice and humans. Using structure-guided enzyme engineering, we generated a TK2 double mutant (TK2-N93D/L109F) that efficiently phosphorylates L-18F-FMAU. The N93D/L109F TK2 mutant has lower activity for the endogenous nucleosides thymidine and deoxycytidine than wild type TK2, and its ectopic expression in therapeutic cells is not expected to alter nucleotide metabolism. Imaging studies in mice indicate that the sensitivity of the new human TK2-N93D/L109F PRG is comparable with that of a widely used PRG based on the herpes simplex virus 1 thymidine kinase. These findings suggest that the TK2-N93D/L109F/L-18F-FMAU PRG-PRP system warrants further evaluation in preclinical and clinical applications of cell-based therapies.     

Circular dichroism and fluorescence spectroscopic analyses of a proline-rich glycoprotein from human parotid saliva

A proline-rich glycoprotein (PRG) was isolated from human parotid saliva and examined by circular dichroism and fluorescence spectroscopy. Addition of guanidine hydrochloride to PRG labeled with an extrinsic dansyl probe had no effect on the fluorescence spectra's 511 nm lambda-max location. Thermodynamic calculations supported the contention that PRG has no significant tertiary structure. Circular dichroism results for PRG were simulated by computer and a secondary structure composed of 70% random coil and 30% beta-form conformation was predicted. Circular dichroism of PRG failed to detect either poly-L-proline type I or II structures. Deglycosylation of PRG had no measurable effect on the circular dichroism spectrum, indicating that the carbohydrate side chains had little influence on PRG secondary structure. Based upon mathematical calculations, beta-turns were predicted around three glycosylated Asn residues of PRG. These collective data suggest that PRG is composed of a disordered polypeptide chain with at least three of the N-linked Asn residues participating in some type of beta-turn.     

A novel p53-inducible apoptogenic gene,PRG3, encodes a homologue of the apoptosis-inducing factor (AIF)

The p53 tumor suppressor protein induces cell cycle arrest or apoptosis in response to cellular stresses. We have identified PRG3 (p53-responsive gene 3), which is induced specifically under p53-dependent apoptotic conditions in human colon cancer cells, and encodes a novel polypeptide of 373 amino acids with a predicted molecular mass of 40.5 kDa. PRG3 has significant homology to bacterial oxidoreductases and the apoptosis-inducing factor, AIF, and the gene was assigned to chromosome 10q21.3-q22.1. Expression of PRG3 was induced by the activation of endogenous p53 and it contains a p53-responsive element. Unlike AIF, PRG3 localizes in the cytoplasm and its ectopic expression induces apoptosis. An amino-terminal deletion mutant of PRG3 that lacks a putative oxidoreductase activity retains its apoptotic activity, suggesting that the oxidoreductase activity is dispensable for the apoptotic function of PRG3. The PRG3 gene is thus a novel p53 target gene in a p53-dependent apoptosis pathway.     

Ganglioneuroma Manifesting as an Incidental Adrenal Mass in an Adult With Turner’s Syndrome

ObjectiveTo report a case of ganglioneuroma masquerading as an incidental adrenal mass in an adult patient with Turner’s syndrome.MethodsWe present the clinical, laboratory, radiologic, and pathologic findings in this patient.ResultsA 31-year-old woman with Turner’s syndrome who had previously been treated with growth hormone replacement had an incidentally discovered mass, apparently arising from the left adrenal gland. The mass was “silent” clinically and biochemically, but imaging characteristics were not reassuring for a benign cortical adenoma. Because of uncertainty regarding the nature of the mass, it was removed laparoscopically; during this procedure, it was noted to be intimately associated with, but anatomically distinct from, the left adrenal gland. The pathology report confirmed the presence of a benign ganglioneuroma.Conclusion: Although ganglioneuroma has previously been noted to be associated with Turner’s syndrome (especially in pediatric patients), to the best of our knowledge this is the first report of a ganglioneuroma manifesting as an incidental adrenal mass in an adult patient with Turner’s syndrome. (Endocr Pract. 2005;11:382-384)     

PET imaging of herpes simplex virus type 1 thymidine kinase (HSV1-tk) or mutant HSV1-sr39tk reporter gene expression in mice and humans using [18F]FHBG

The herpes simplex virus type 1 thymidine kinase (HSV1-tk) positron emission tomography (PET) reporter gene (PRG) or its mutant HSV1-sr39tk are used to investigate intracellular molecular events in cultured cells and to image intracellular molecular events and cell trafficking in living subjects. The expression of these PRGs can be imaged using 18F- or 124I-radiolabeled acycloguanosine or pyrimidine analog PET reporter probes (PRPs). This protocol describes the procedures for imaging HSV1-tk or HSV1-sr39tk PRG expression in living subjects with the acycloguanosine analog 9-4-[18F]fluoro-3-(hydroxymethyl)butyl]guanine ([18F]FHBG). [18F]FHBG is a high-affinity substrate for the HSV1-sr39TK enzyme with relatively low affinity for mammalian TK enzymes, resulting in improved detection sensitivity. Furthermore, [18F]FHBG is approved by the US Food and Drug Administration as an investigational new imaging agent and has been shown to detect HSV1-tk transgene expression in the liver tumors of patients. MicroPET imaging of each small animal can be completed in approximately 1.5 h, and each patient imaging session takes approximately 3 h.     

Investigation of cis/trans proline isomerism in a multiply occurring peptide fragment from human salivary proline-rich glycoprotein.

The solution-state conformations of eight proline-containing peptide fragments found in human salivary proline-rich glycoprotein (PRG) were investigated in 2 x distilled water (treated with metal ion chelating resin) using 13C-nuclear magnetic resonance (NMR) and circular dichroism (CD) spectroscopy. The peptide sequences and acronyms were as follows: PRG9-2 = NH2-G(1)-P(2)-CONH2, PRG9-3 = NH2-G(1)P(2)-P(3)-CONH2, PRG9-4 = NH2-G(1)-P(2)-P(3)-P(4)-CONH2, PRG9-5 = NH2-G(1)-P(2)-P(3)-P(4)-H(5)-CONH2, PRG9-6 = NH2-G(1)-P(2)-P(3)-P(4)-H(5)-P(6)-CONH2, PRG9-7 = NH2-G(1)-P(2)-P(3)-P(4)-H(5)-P(6)-G(7)-CONH2, PRG9-8 = NH2-G(1)-P(2)-P(3)-P(4)-H(5)-P(6)-G(7)-K(8)-CONH2 and PRG9-9 = NH2-G(1)-P(2)-P(3)-P(4)-H(5)-P(6)-G(7)-K(8)-P(9)-CONH2. Sequence-specific resonance assignments from the 13C-NMR spectra indicated that the trans proline isomer dominated the conformations of the peptides. CD results clearly showed the presence of the poly-L-proline II helix as the major conformation in PRG9-3----PRG9-5, supplemented by beta- and/or gamma-turns in PRG9-6----PRG9-9. These data suggest that in "metal free" water, native PRG could contain several small poly-L-proline II helices along with beta- and/or gamma-turns. Since proline is the major amino acid present in native PRG, these localized conformations may contribute to PRG's global conformation and act as a primary force in determining its biological activities.