Childhood and adolescent non-Hodgkin lymphoma: New insights in biology and critical challenges for the future

Abstract

Pediatric non-Hodgkin lymphoma (NHL) is a common and fascinating group of diseases with distinctive underlying genetic events that characterize the major histologic subtypes: diffuse large B-cell lymphoma, Burkitt lymphoma, anaplastic large cell lymphoma and lymphoblastic lymphoma. With systematic improvements in therapy over recent decades, the vast majority of children with NHL of all subtypes are now cured. The similarities and differences between adult and childhood presentations of disease, and whether or not some subtypes of NHL and leukemia are the same or different disease entities, are interesting questions that will be addressed with advances in our understanding of the molecular and genetic bases of these diseases. As is the case with other pediatric malignancies, growing emphasis is now being placed on the development of less toxic, targeted therapeutic approaches, and this review highlights some of the biological discoveries that will potentially open these avenues.

Diffuse large B‐cell lymphoma in the young in Japan: A study by the Osaka Lymphoma Study Group

“…Malignant lymphomas are the third most common malignancies in childhood, and non-Hodgkin's lymphoma (NHL) represents *6-10% of pediatric malignancies [1,2]. Pediatric NHLs comprise four major subtypes; Burkitt lymphoma, lymphoblastic lymphoma, diffuse large B-cell lymphoma (DLBCL), and anaplastic large cell lymphoma [1,2].…”

“…Pediatric NHLs comprise four major subtypes; Burkitt lymphoma, lymphoblastic lymphoma, diffuse large B-cell lymphoma (DLBCL), and anaplastic large cell lymphoma [1,2]. DLBCL is the commonest lymphoma among adults, but ranks third among pediatric NHLs.…”

Outcome of children and adolescents with lymphoblastic lymphoma

“…29 Over 80% of patients with T-LBL do not have marrow involvement at diagnosis by morphologic examination of bilateral marrow aspirates and biopsies. 2 However, in a series of 99 children with T-LBL, more than two thirds of them had neoplastic lymphoblasts detected by flow cytometry. This finding implies that a more sensitive method for investigating neoplastic cells in the BM at diagnosis may contribute to a more appropriate risk indeed an extremely poor prognosis.…”

“…T-cell lymphoblastic lymphomas (T-LBL) most commonly involve the anterior mediastinum and supradiaphragmatic lymph nodes. 2,9 Precursor B-lymphoblastic lymphomas (pB-LBL) are usually localized to peripheral lymph nodes and extranodal sites such as skin, soft tissues, and bone, with a predilection for the head and neck regions. 10,11 Whether LBL and ALL in childhood are biologically identical or rather distinct disorders is not entirely clear.…”

“…[1][2][3] The current World Health Organization (WHO) classification assigns tumours of hematopoietic and lymphoid tissues in T lymphoblastic leukemia/lymphoma and B lymphoblastic leukemia/lymphoma. 4,5 Of note, only approximately 10% of LBL express B-cell markers in contrast to approximately 85% of acute lymphoblastic leukemia (ALL).…”