Pediatric lymphoma

  • 文章类型: Journal Article
    小儿型滤泡性淋巴瘤是年轻人群中常见的一种罕见且新近被认识的淋巴肿瘤。尽管其临床过程缓慢且局部淋巴结受累,它的特点是其高级组织病理学特征。这种疾病和几个实体之间的重叠特征使得接近这个独特的实体具有极大的挑战性,所有这些特征都反映在WHO2016年淋巴恶性肿瘤分类所强调的严格诊断标准中.尽管它具有很高的组织学特征,它的治愈率仍然很高,复发和转化很少发生。有趣的是,结节性疾病切除术后,有几例病例已经缓解,可能消除对化疗和放疗的需要,并防止疾病幸存者后期治疗的长期发病率。
    Pediatric-type follicular lymphoma is an uncommon and newly recognized entity of lymphoid neoplasm commonly encountered in the young population. Despite its indolent clinical course and localized nodal involvement, it has been characterized by its high-grade histopathological features. The overlapping features between this disease and several entities have made approaching this unique entity significantly challenging, with all such features being reflected in the strict diagnostic criteria highlighted by the WHO 2016 lymphoid malignancy classification. Despite its characteristic high-grade histology, its cure rates have remained high, with relapse and transformation rarely occurring. Interestingly, several cases have achieved remission following nodal disease resection, possibly eliminating the need for chemotherapy and radiation and preventing long-term morbidities from later approaches in disease survivors.
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  • 文章类型: Case Reports
    UNASSIGNED:伯基特淋巴瘤(BL)是儿童时期常见的肿瘤,通常以散发形式出现在腹部或骨盆中。在少数情况下,BL可以表现为中枢神经系统受累,通常作为次要网站。很少,BL主要出现在硬膜外腔,并伴有背痛,或者不太常见,急性脊髓病。此演示文稿是外科紧急情况,需要警惕的管理。
    UNASSIGNED:我们描述了一例小儿BL主要出现在硬膜外腔内,并表现为3岁男孩的进行性行走困难。进展到完全无法行走,下肢深肌腱反射缺失,新的尿失禁促使脊柱MRI检查,显示病变从T5延伸到T10,并包裹在脊柱的前部和后部,有脊髓压迫的证据。患者从T5到T10接受了减压椎板切除术,并部分切除了肿瘤的后部。显微镜检查显示突出的“星空”图案,具有丰富的有丝分裂图。免疫组织化学证实了BL的诊断。患者术后10个月,继续接受化疗,部分神经系统得到改善。术后10个月无复发。
    未经证实:这似乎是最年轻的原发性椎旁BL急性脊髓病患者。治疗应包括脊髓的手术减压,然后是各种所述的化疗方案之一。术前分期和神经功能与预后相关。
    UNASSIGNED: Burkitt lymphoma (BL) is a common tumor of childhood that usually arises in the abdomen or pelvis in its sporadic form. In a minority of cases, BL can present with CNS involvement, usually as a secondary site. Rarely, BL can arise primarily in the epidural space and present with back pain, or less commonly, acute myelopathy. This presentation is a surgical emergency and requires vigilant management.
    UNASSIGNED: We describe a case of pediatric BL arising primarily within the epidural space and presenting with progressive difficulty walking in a 3-year-old boy. Progression to complete inability to walk, absent lower extremity deep tendon reflexes, and new urinary incontinence prompted MRI of the spine, which showed a lesion extending from T5 to T10 and wrapping around the anterior and posterior portions of the spine with evidence of spinal cord compression. The patient underwent decompressive laminectomies from T5 to T10 and partial debulking of the posterior portions of the tumor. Microscopic examination showed a prominent \"starry sky\" pattern with abundant mitotic figures. Immunohistochemistry confirmed the diagnosis of BL. The patient is 10 months post-op and continues to undergo chemotherapy with partial neurologic improvement. He was free of recurrence 10 months post-operative.
    UNASSIGNED: This appears to be the youngest described patient presenting with acute myelopathy in primary paraspinal BL. Management should include surgical decompression of the spinal cord followed by one of the various described chemotherapeutic regimens. Preoperative staging and neurologic function correlate with prognosis.
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  • 文章类型: Journal Article
    The aim of this study was to integrate the available data regarding pediatric Burkitt\'s lymphoma (BL) of the oral cavity. A systematic review was conducted following PRISMA guidelines, through a specific search strategy. Twenty-nine publications were included in this study, resulting in a total of 144 cases. Oral BL was predominantly found in males (75.7%). The mandible was the most involved site (37.5%), and all cases clinically exhibited a swelling. Presence of EBV was observed in 33.3% of the cases, and 4 cases reported HIV-positive patients (33.3%). Chemotherapy was the leading treatment choice for oral BL (94.9%), and the overall 5-year survival was 54.3%. Regarding the quality assessment of the studies, most (19 studies; 65.5%) were classified as an overall moderate risk of bias. In conclusion, the clinicopathological characteristics of oral BL in the pediatric population comprise the sporadic and intermediate subtypes. Despite its aggressiveness, this malignancy presents a moderate overall survival.
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