Non‐anaplasticperipheral T‐cell lymphomas (PTCL) are rare tumors in children, adolescents, and young adults (CAYA) with poor prognosis and scarce genetic data. We analyzed lymphoma tissue from 36 patients up to 18 years old with PTCL, not otherwise specified (PTCL‐NOS), hepatosplenic T‐cell lymphoma, Epstein–Barr virus (EBV)‐positive T‐lymphoproliferative diseases, subcutaneous panniculitis‐like T‐cell lymphoma, and other PTCL types. Twenty‐three patients (64%) had at least one genetic variant detectable, including TET2, KMT2C, PIK3D, and DMNT3A. TP53 and RHOA variants, commonly found in adults, were not identified. Eight of 20 (40%) CAYA PTCL‐NOS had no detectable mutations. The genetic findings suggest that CAYA PTCL differ from adult cases.
/24 ч в целях уменьшения токсичности. Впервые в лечении детей и подрост-ков со зрелоклеточными В-НХЛ использовано противоопухолевое действие ритуксимаба (Мабтера в дозе 375 мг/м 2 внутривенно). Из 83 больных, включенных в анализ с 2004 по 2009 г., клинико-гематологическая ремиссия достигнута у 77 (92,8 %); при меди-ане наблюдения 51,6 мес ремиссия продолжалась у 23 (85,2 %) больных В-ОЛ, у 32 (88,9 %) (Мабтера)
Thymic carcinoma (TC) belongs to a group of rare thymic epithelial tumours (TETs) arising from the anterior mediastinum. One of the most relevant established prognostic factors is complete surgical resection. Given the high invasive potential of TC a lot of patents at the diagnosis present with a locally advanced or metastatic disease, therefore complete resection could not be achieved, and a role of systemic therapy is increasing. Due to the rarity of this tumor type in children and adolescents, treatment approaches based on the guidelines for management of TETs in adults. In this article we report a case of TC in a 2-year-old boy. The literature review describes the current histological classification of the TET, approaches for systemic chemotherapy and local control in patients with TC.
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