Neoplastic diseases of the head & neck in children

Pediatric neoplasm is next only to trauma as the most common cause of death. The cervicofacial malignancies presents a unique challenge owing to potential adverse effects of both the disease process and the treatment employed on critical developing head and neck structures. This study comprised of 106 children below 12 years age group conducted during 1999–2003 at JNMC, Aligarh. Of 106 cases 70 were benign neoplasms and 36 malignant neoplasms. Nasopharynx was the commonest site of involvement. Nasopharyngeal angiofibroma was the commonest benign tumour and lymphoma was the commonest malignant neoplasm. Team approach is required for mitigation of the disease process.     

鼻腔NK/T细胞淋巴瘤T-bet基因扩增及特异表达的研究

目的 探索鼻腔NK／T细胞淋巴瘤（N-NK／T—L）特异基因改变及其表达变化。方法 对同一病例的N-NK／T-L肿瘤细胞和外周血白细胞进行限制性酶切路标基因组扫描（RIGs），并结合相应虚拟基因组扫描（VGS）生物信息分析，及基因库检索确定异常基因信号；通过对上述结果发现的异常基因进行克隆和探针制备，对3例新鲜N—NK／T-L肿瘤组织和1例外周血标本进行了Southern和斑点印迹杂交分析，并对20例石蜡组织N-NK／T-L以及对照组包括17例同部位B细胞淋巴瘤、3例正常脾组织和5例慢性炎性鼻黏膜组织进行了原位杂交检测。结果 RLGS和VGS法显示N-NK／T-L肿瘤细胞中存在T-bet基因异常；经Southem和斑点印迹杂交检测证明在3例新鲜N-NK／T-L肿瘤细胞中有T-bet基因扩增；原位杂交显示T-bet基因在20例N-NK／T-L中阳性表达者为18例（90．0％），而B细胞淋巴瘤中仅为2例（11．8％），两者比较差异有统计学意义（P〈0．01）。在3例脾组织和5例慢性炎性鼻黏膜组织中均未有T-bet表达。结论T．bet基因在N-NK／T-L肿瘤细胞中存在基因扩增和过表达，T-bet基因的异常表达可能在N-NK／T—L的发生中起一定作用，并有必要对其是否可作为分类依据和辅助诊断的指标做进一步研究。     

网膜非霍奇金淋巴瘤合并肾功能衰竭一例

患者男，52岁。因腹胀、乏力、纳差、下腹部持续性隐痛月余，渐加重，于2004年1月30日就诊于当地医院。彩超示中等量腹水；CT见腹膜弥漫性不规则增厚，大量腹水，脾稍大，胆胰未见异常；结肠镜检查未见异常；多次腹水细胞学检查见大量淋巴细胞、红细胞，少量间皮细胞，未见癌细胞；血、腹水结核酶标抗体及肿瘤标记物在正常范围；肝肾功能正常。给予多种抗生素及抗结核药物治疗无效，且尿量进行性减少。再次腹穿，腹水细胞涂片送我院会诊，见大量淋巴及淋巴样细胞，考虑为恶性肿瘤，遂转入我院。     

原发性骨淋巴瘤诊疗现状及进展

原发性骨淋巴瘤是一种罕见的结外淋巴瘤，缺少特征性的临床症状和影像学表现。化疗、放疗或联合治疗是原发性骨淋巴瘤患者常用的治疗方法。原发性骨淋巴瘤预后优于其他结外淋巴瘤。文章就原发性骨淋巴瘤的临床表现、病理诊断、治疗方法进行综述，以加深对原发性骨淋巴瘤的理解。     

Peripheral T-cell lymphoma – are we making progress?

Peripheral T cell lymphoma (PTCL) is a rare subtype of non-Hodgkin lymphoma. PTCLs are heterogeneous in terms of biology, but generally have more aggressive features and poorer outcomes than aggressive B-cell lymphomas when treated with combination chemotherapy. While the best long-term results are still seen with intensive chemotherapeutic approaches, significant progress has been made with molecular profiling identifying genetic drivers of PTCL that could serve as therapeutic targets. Tailoring therapy to different subtypes of PTCL may lead to more individualized approaches with the hope of improved outcomes. In this paper, we review current therapies for treatment of PTCL, newly identified molecular markers, and the role of emerging therapy and novel combinations of existing agents.     

Breast cancers with round lumps: Correlations between imaging and anatomopathology

A round lump with a well-defined outline is, in most cases, benign. However, in 10 to 20% of all cases, a round and well-defined lump may correspond to a cancer. Most often, it consists of grade III infiltrating ductal carcinoma (IDC). Other histological sub-types may provide round masses with smooth contours: colloid carcinoma (still called mucinous carcinoma), medullary carcinoma, intramammary metastases, intra-cystic papillary carcinoma, lymphoma and high-grade phyllode tumours.     

Immunoglobulin G4–Related Lymphadenopathy

Immunoglobulin G4–related lymphadenopathy (IgG4-RLAD) occurs in the setting of extranodal IgG4-related disease (IgG4-RD), an immune-mediated process described in many organ systems characterized by lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and fibrosis. Although the morphologic features in the lymph node sometimes resemble those seen at the extranodal sites, 5 microscopic patterns have been described, most of which resemble reactive lymphoid hyperplasia. This morphologic variability leads to unique diagnostic challenges and a broad differential diagnosis. As IgG4-RD may be exquisitely responsive to steroids or other immunotherapy, histologic recognition and inclusion of IgG4-RLAD in the differential diagnosis is vital.     

Integrating novel systemic therapies for the treatment of mycosis fungoides and Sézary syndrome

Novel systemic therapies are generally prescribed to patients with advanced-stage disease or those with early-stage disease refractory to skin-directed therapies. In general, systemic chemotherapy should be reserved for patients who fail to respond to biological agents. Such biological agents include interferon alfa, bexarotene, histone deacetylase inhibitors (vorinostat, romidepsin), brentuximab vedotin and mogamulizumab. Extracorporeal photopheresis is particularly effective for patients with Sézary Syndrome. Allogeneic transplantation is becoming increasing used for younger patients. Novel agents in advanced development include the monoclonal antibody IPH4102,duvelisib,and the new modified formulation of denileukin diftitox. The choice of agents for patients is typically a balance of patient factors (age, co-morbidities, geographic location), relative efficacy and toxicity.     

MicroRNAs in hematological malignancies

MicroRNAs (miRNAs) have become one of the hottest topics in biology over recent years, but remarkably have only been formally recognized for just over 10 years. These endogenously produced short (19–24 nt) non-coding RNAs have introduced an entirely new paradigm in our understanding of gene control and it is now evident that miRNAs play a crucial regulatory role in many, if not all, physiological and pathological processes. In this review we provide an overview of the role and potential clinical utility for miRNAs in hematological malignancies and their function in normal hematopoiesis. Although still in its infancy, the miRNA field has already added much to our understanding of hematological processes, and provides us with novel tools as both biomarkers and therapeutic agents for hematological malignancies.