Limited stage I disease is not necessarily indicative of an excellent prognosis in childhood anaplastic large cell lymphoma

Attarbaschi, Andishe; Mann, Georg; Rosolen, Angelo; Williams, Denise; Uyttebroeck, Anne; Márky, Ildikó; Lamant, Laurence; Horibe, Keizo; Wróbel, Grażyna; Beishuizen, Auke; Wößmann, Wilhelm; Reiter, Alfred; Mauguen, Audrey; Deley, Marie-Cécile Le; Brugières, Laurence

doi:10.1182/blood-2010-12-324012

Cited by 24 publications

(20 citation statements)

References 19 publications

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“…2 Children with PTNFL have an excellent response to surgical excision alone without subsequent progression or recurrence of disease. 3 However, as seen in our PTNFL cohort, adults with PTNFL continue to be treated with chemoimmunotherapy and/or radiotherapy, because of its morphological overlap with high-grade FL, and the lack of definite evidence that these cases are biologically equivalent to PTNFL in children. In the current study, we provide strong evidence that PTNFL is frequently driven by aberrant activation of the MAPK pathway, rather than by genetic alteration of epigenetic modifiers as seen in typical FL.…”

Section: Discussionmentioning

confidence: 86%

“…MAP2K1 mutations were present at a median VAF of 0.12 by WES (range, 5% to 24%). All MAP2K1 mutations were known activating mutations in the negative regulatory (F53Y, Q56P [3], K57E [3], K57R) region and the catalytic core (C121S) domain, encoded by exons 2 and 3, respectively (supplemental Figure 2). PTNFLs (10%) had known activating mutations in MAPK1, which encodes extracellular signal-regulated kinase 2 (ERK2) and is immediately downstream of MEK1 in the MEK/ERK pathway, and 1 PTNFL had a known activating mutation in RRAS.…”

Section: Org Frommentioning

confidence: 99%

“…Patients with PTNFL consistently have indefinite remissions if treated with surgical excision alone. 3 However, most adults with PTNFL continue to be treated with standard chemoimmunotherapy and/or radiation therapy, irrespective of BCL2 translocation status, because of its histological mimicry of high-grade typical FL. Therefore, objective biological markers are needed to distinguish PTNFL from typical FL in both children and adults in order to avoid potentially unnecessary treatment.…”

Section: Introductionmentioning

confidence: 99%

“…In contrast to the well-characterized landscape of typical FL, [3][4][5][6][7][8][9][10][11][12][13][14][15][16] the molecular genetic features of PTNFL are essentially undefined. Therefore, we performed comprehensive mutation analysis and copy number variant analysis on PTNFLs from children and adults to address this problem.…”

Section: Introductionmentioning

confidence: 99%

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Pediatric-type nodal follicular lymphoma: a biologically distinct lymphoma with frequent MAPK pathway mutations

Louissaint

Schafernak

Geyer

et al. 2016

Blood

132

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Section: Discussionmentioning

confidence: 86%

Section: Org Frommentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Pediatric-type nodal follicular lymphoma: a biologically distinct lymphoma with frequent MAPK pathway mutations

Louissaint

Schafernak

Geyer

et al. 2016

Blood

132

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“…7 However, recent studies recommend a "watch-and-wait" strategy for cases in which a complete lymph node (LN) resection is achieved in stage I patients. 8 The genetic alterations involved in the pathogenesis of nodal PTFL are currently largely unknown. Due to the indolent disease course, it has been questioned whether nodal PTFL represents a true lymphoma or rather an atypical "monoclonal" lymphoid hyperplasia.…”

Section: Introductionmentioning

confidence: 99%

Genome-wide analysis of pediatric-type follicular lymphoma reveals low genetic complexity and recurrent alterations of TNFRSF14 gene

Schmidt

Gong

Marafioti

et al. 2016

Blood

123

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Key Points• PTFL is a monoclonal B-cell neoplasia with low genomic complexity and recurrent TNFRSF14 mutations/ deletions.• The genetic profiles of conventional t(14;18) 2 and t(14;18) 1 FL are similar but distinct from PTFL.Pediatric-type follicular lymphoma (PTFL) is a variant of follicular lymphoma (FL) with distinctive clinicopathological features. Patients are predominantly young males presenting with localized lymphadenopathy; the tumor shows high-grade cytology and lacks both BCL2 expression and t(14;18) translocation. The genetic alterations involved in the pathogenesis of PTFL are unknown. Therefore, 42 PTFL (40 males and 2 females; mean age, 16 years; range, 5-31) were genetically characterized. For comparison, 11 cases of conventional t(14:18) 2 FL in adults were investigated. Morphologically, PTFL cases had follicular growth pattern without diffuse areas and characteristic immunophenotype. All cases showed monoclonal immunoglobulin (IG) rearrangement. PTFL displays low genomic complexity when compared with t(14;18) 2 FL (mean, 0.77 vs 9 copy number alterations per case; P < .001). Both groups presented 1p36 alterations including TNFRSF14, but copy-number neutral loss of heterozygosity (CNN-LOH) of this locus was more frequently observed in PTFL (40% vs 9%; P 5 .075). TNFRSF14 was the most frequently affected gene in PTFL (21 mutations and 2 deletions), identified in 54% of cases, followed by KMT2D mutations in 16%. Other histone-modifying genes were rarely affected. In contrast, t(14;18) 2 FL displayed a mutational profile similar to t(14;18) 1 FL.In 8 PTFL cases (19%), no genetic alterations were identified beyond IG monoclonal rearrangement. The genetic landscape of PTFL suggests that TNFRSF14 mutations accompanied by CNN-LOH of the 1p36 locus in over 70% of mutated cases, as additional selection mechanism, might play a key role in the pathogenesis of this disease. The genetic profiles of PTFL and t(14;18) 2 FL in adults indicate that these are two different disorders. (Blood. 2016;128(8):1101-1111

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Observation of Alectinib‐ and Crizotinib‐ included chemotherapy in children with ALK‐positive anaplastic large cell lymphoma: A single institutional experience

Pei

Zhang

et al. 2022

Cancer Medicine

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Approximately one‐third children with anaplastic large cell lymphoma (ALCL) relapse after completion of chemotherapy, particularly for those high‐risk patients. The introduction of novel therapeutic modalities is much needed for these sub‐group patients. Two groups ( n = 3, n = 4) of ALCL patients were treated with crizotinib‐ and alectinib‐included ALCL‐99 therapy, respectively, achieving complete remission rates of 66.7% and 100%. Two patients of crizotinib group relapsed, while none relapsed among the alectinib‐treated patients. Adding alectinib instead of crizotinib sufficiently suppressed and maintained the deep NPM ‐ ALK molecular response. ALK inhibitors were well tolerated with only grade 1 adverse events in both groups. Though a relatively small case number, this study raised the possibility that alectinib‐included therapeutic regimens may benefit the early response, in‐depth molecular remission, and persistent remission to some extent. Further studies are warranted to validate our preliminary findings.

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Limited stage I disease is not necessarily indicative of an excellent prognosis in childhood anaplastic large cell lymphoma

Cited by 24 publications

References 19 publications

Pediatric-type nodal follicular lymphoma: a biologically distinct lymphoma with frequent MAPK pathway mutations

Pediatric-type nodal follicular lymphoma: a biologically distinct lymphoma with frequent MAPK pathway mutations

Genome-wide analysis of pediatric-type follicular lymphoma reveals low genetic complexity and recurrent alterations of TNFRSF14 gene

Observation of Alectinib‐ and Crizotinib‐ included chemotherapy in children with ALK‐positive anaplastic large cell lymphoma: A single institutional experience

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