Rare non‐Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric‐type follicular lymphoma, marginal zone lymphoma, and nonanaplastic peripheral T‐cell lymphoma

Abstract

Pediatric‐type follicular (PTFL), marginal zone (MZL), and peripheral T‐cell lymphoma (PTCL) account each for <2% of childhood non‐Hodgkin lymphoma. We present clinical and histopathological features of PTFL, MZL, and few subtypes of PTCL and provide treatment recommendations. For localized PTFL and MZL, watchful waiting after complete resection is the therapy of choice. For PTCL, therapy is subtype‐dependent and ranges from a block‐like anaplastic large cell lymphoma (ALCL)‐derived and, alternatively, leukemia‐derived therapy in PTCL not otherwise specified and subcutaneous panniculitis‐like T‐cell lymphoma to a block‐like mature B‐NHL‐derived or, preferentially, ALCL‐derived treatment followed by hematopoietic stem cell transplantation in first remission in hepatosplenic and angioimmunoblastic T‐cell lymphoma.

Importance of clinical correlation in the diagnosis of pediatric B‐cell lymphomas

“…For PTFL, recent data would support surgical excision or simply observation. OS rates for PTFL are close to 100% 22,25 . For LBCL with IRF4 , the 5‐year OS rates are also up to 100% 23,20 …”

Clinical observation of pediatric-type follicular lymphomas in adult: Two case reports

“…The cell-rich tumor area had a single cell composition, it mainly consisted of medium-sized blastoid cells which characterize the irregular nucleus and unclear nucleolus. The mitosis was easy to found, and some cases contain more typical immature centroblastic cells[ 7 ]. In terms of immunophenotype, tumor cells expressed mature B cell markers, namely CD20, PAX5, and CD79a.…”

Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis Resembling Recurrent Follicular Lymphoma: A Case Report

“…In contrast to FL in adulthood, which is usually of low-grade morphology and an incurable disease despite diverse treatment approaches, the prognosis and outcome of pediatric FL seems to be extraordinarily good after limited chemotherapy or complete resection followed by observation only [9]. Children with unresectable FL have an excellent outcome with conventional multidrug chemotherapy, but the regimen is associated with acute and long-term toxicity; therefore, multinational collaborations aiming for treatment reduction have yet to be established [9]. Cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone (CHOP)-like cycles with or without rituximab are effective in cases that are not completely resectable [9].…”

“…While FL accounts for 25% of non-Hodgkin's lymphomas (NHL) cases in adulthood, it rarely occurs in children and adolescents (＜2% of cases) [8]. In pediatric FL, tissues are negative or weakly positive for the BCL-2 protein [9]. Moreover, most adult patients present with disseminated disease at initial diagnosis, but children usually present with localized disease often confined to the peripheral lymph nodes only [8].…”

“…Moreover, most adult patients present with disseminated disease at initial diagnosis, but children usually present with localized disease often confined to the peripheral lymph nodes only [8]. In contrast to FL in adulthood, which is usually of low-grade morphology and an incurable disease despite diverse treatment approaches, the prognosis and outcome of pediatric FL seems to be extraordinarily good after limited chemotherapy or complete resection followed by observation only [9]. Children with unresectable FL have an excellent outcome with conventional multidrug chemotherapy, but the regimen is associated with acute and long-term toxicity; therefore, multinational collaborations aiming for treatment reduction have yet to be established [9].…”