Pediatric lymphoma

  • 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
    Attempts to establish a concise classification of lymphoblastic lymphomas (LBLs) have gained momentum in recent years, mainly due to the expanding possibilities of immunohistochemical and genetic characterization of different disease entities. Thus, cases of immature lymphoid malignancies with unusual immunopathological features have been reported during the last years, suggesting the need for new LBL classification concepts. To further characterize and demonstrate the extended spectrum of LBL, we present an unusual pediatric case of LBL that could not be categorized into one of the subgroups and exhibited a benign course after surgical treatment and subsequent chemotherapy. A mediastinal tumor of a 6-year-old Caucasian boy was examined by clinical staging, light microscopy, immunohistochemistry and PCR assays. The tumor cells reacted with TdT and had a positive cytoplasmic immunoreactivity for CD3. Further T- aand NK-cell markers CD1a, CD4, CD8, CD10, and CD56 reacted positively, but CD57, CD16 and CD 30 (Ber H2) were all negative. CD34 as a marker for bipotential B/T-precursors was also positive. B cell markers (CD20, CD22, Cd79a and IgM) were all negative. No clonal B cell Ig or T cell gamma chain rearrangements were detectable. Epstein Barr virus and other Herpes Virus DNA were not detected using a sensitive PCR assay. The applied chemotherapy was tolerated well and a complete remission of the tumor was achieved (observation period three years after the initial diagnosis). Localization, morphology, and the expressions markers made the tumor a typical member of the LBL group. However, our case represents a rare pediatric lymphoma derived from a thymic precursor committed to T/NK-cell differentiation and a favourable outcome after chemotherapy.
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