O'Suoji Chibuzo, Welch Jennifer J G, Perkins Sherrie L, Smith Lynette M, Weitzman Sheila, Simko Stephen J, Galardy Paul J, Bollard Catherine M, Gross Thomas G, Termuhlen Amanda M
Division of Pediatric Hematology/Oncology, West Virginia University, Charleston, West Virginia.
Division of Pediatric Hematology/Oncology, Hasbro Children's Hospital, Alpert Medical School Brown University, Providence, Rhode Island.
Pediatr Blood Cancer. 2016 May;63(5):794-800. doi: 10.1002/pbc.25881. Epub 2016 Jan 5.
Non-Hodgkin lymphoma (NHL) is a relatively common malignancy in pediatric patients; however, a small subgroup have unusual lymphoma subtypes for the pediatric population.
The Children's Oncology Group Rare and Cutaneous NHL registry's (protocol ANHL 04B1) main objectives were to determine the pathologic, biologic, and clinical features of rare and cutaneous pediatric NHL and establish a bank of centrally reviewed tissue specimens. We report the clinical data, treatment data, and outcome for rare pediatric NHL.
In 101 lymphomas, there is a 97.8% concordance between the reviewing study pathologists and an 87.6% concordance between the central and institutional pathology review. Samples in the specimen bank include primary tumor tissue that is snap frozen, in paraffin blocks, or H&E-stained and unstained paraffin slides as well as blood, serum, and bone marrow. This descriptive analysis shows that children with pediatric follicular lymphoma, mucosa-associated lymphoid tissue, nodal marginal zone lymphoma, primary cutaneous, primary central nervous system lymphoma, and subcutaneous panniculitis-like T-cell lymphomas have 100% survival at a median of 2 years from enrollment. There are early deaths, mostly from progressive disease, in subjects with peripheral T-cell (not otherwise specified), NKT, and hepatosplenic T-cell lymphomas.
This registry provides high-quality biologic specimens with clinical data to investigators working on the biology of these unusual pediatric diseases.
非霍奇金淋巴瘤（NHL）在儿科患者中是一种相对常见的恶性肿瘤；然而，一小部分患者具有在儿科人群中不常见的淋巴瘤亚型。
儿童肿瘤学组罕见和皮肤NHL登记处（方案ANHL 04B1）的主要目标是确定罕见和皮肤儿科NHL的病理、生物学和临床特征，并建立一批经过集中审查的组织标本库。我们报告了罕见儿科NHL的临床数据、治疗数据和结果。
在101例淋巴瘤中，审查研究病理学家之间的一致性为97.8%，中央和机构病理审查之间的一致性为87.6%。标本库中的样本包括速冻的原发性肿瘤组织、石蜡块、苏木精和伊红染色及未染色的石蜡切片，以及血液、血清和骨髓。这项描述性分析表明，患有儿科滤泡性淋巴瘤、黏膜相关淋巴组织、结边缘区淋巴瘤、原发性皮肤、原发性中枢神经系统淋巴瘤和皮下脂膜炎样T细胞淋巴瘤的儿童在入组后2年的中位生存期内生存率为100%。在外周T细胞（未另行指定）、自然杀伤T细胞和肝脾T细胞淋巴瘤患者中存在早期死亡，主要死于疾病进展。
该登记处为研究这些不常见儿科疾病生物学的研究人员提供了高质量的生物学标本和临床数据。
