lunes, 26 de agosto de 2019

Childhood Non-Hodgkin Lymphoma Treatment (PDQ®) 8/8 –Health Professional Version - National Cancer Institute

Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version - National Cancer Institute

National Cancer Institute



Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version



Rare NHL Occurring in Children




Low-grade or intermediate-grade mature B-cell lymphomas—such as small lymphocytic lymphoma, mucosa-associated lymphoid tissue (MALT) lymphoma, mantle cell lymphoma, myeloma, or follicular cell lymphoma—are rarely seen in children. The most recent World Health Organization (WHO) classification has identified pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma as entities separate from their adult counterparts.[1]
In an attempt to learn more about the clinical and pathologic features of these rare types of pediatric non-Hodgkin lymphoma (NHL), the Children's Oncology Group (COG) opened a registry study (COG-ANHL04B1). This study banks tissue for pathobiology studies and collects limited data on clinical presentation and outcome of therapy.[2]


Pediatric-type Follicular Lymphoma

Pediatric-type follicular lymphoma is a disease that genetically and clinically differs from its adult counterpart and is recognized by the WHO classification as a separate entity from follicular lymphoma observed commonly in adults.[1] The genetic hallmark of follicular lymphoma is t(14;18)(q32;q21) involving BCL2; however, this translocation must be excluded to make the diagnosis of pediatric-type follicular lymphoma.[1,3-5] Pediatric-type follicular lymphoma predominantly occurs in males, is associated with a high proliferation rate, and is more likely to be localized disease.[3,6,7] In pediatric-type follicular lymphoma, a high-grade component (i.e., grade 3 with high proliferative index such as Ki-67 expression of >30%) resembling diffuse large B-cell lymphoma can frequently be detected at initial diagnosis but does not indicate a more aggressive clinical course in children. Unlike follicular lymphoma in adults, pediatric-type follicular lymphoma does not transform to diffuse large B-cell lymphoma.[1,3,5,7,8] Limited-stage disease is observed with pediatric-type follicular lymphoma, with cervical lymph nodes and tonsils as common sites, but disease has also occurred in extranodal sites such as the testis, kidney, gastrointestinal tract, and parotid gland.[3-5,8-10]

Tumor biology

Pediatric-type follicular lymphoma appears to be molecularly distinct from follicular lymphoma that is more commonly observed in adults. The pediatric type lacks BCL2rearrangements; BCL6 and MYC rearrangements are also not present. The TNFSFR14mutations are common in pediatric-type follicular lymphoma, and they appear to occur with similar frequency in adult follicular lymphoma.[7,11] However, MAP2K1 mutations, which are uncommon in adults, are observed in as many as 43% of pediatric-type follicular lymphomas. Other genes (e.g., MAPK1 and RRAS) have been found to be mutated in cases without MAP2K1 mutations, suggesting that the MAP kinase pathway is important in the pathogenesis of pediatric-type follicular lymphoma.[12,13] Translocations of the immunoglobulin locus and IRF4, mutations in IRF8, and abnormalities in chromosome 1p have also been observed in pediatric-type follicular lymphoma.[11,14,15]

Treatment options for pediatric-type follicular lymphoma

Pediatric-type follicular lymphoma is rare in children, with only case reports and small case series to guide therapy. The outcome of pediatric-type follicular lymphoma is excellent, with an event-free survival (EFS) of about 95%.[3,5-8,10] Unlike in adult follicular lymphoma, the clinical course is not dominated by relapses.[3,5,8,9]
Treatment options for pediatric-type follicular lymphoma include the following:
  1. Surgery only.
  2. Multiagent chemotherapy with or without rituximab.
Studies suggest that for children with stage I disease who had a complete resection, a watch-and-wait approach without chemotherapy may be indicated. Patients with higher-stage disease also have a favorable outcome with low-intensity and intermediate-intensity chemotherapy, with 94% EFS and 100% overall survival (OS) rates with a 2-year median follow-up.[2,3,6,7] Although the number of pediatric patients with pediatric follicular-type lymphoma is too small to perform meaningful clinical trials, studies of adult patients with follicular lymphoma support the addition of rituximab to standard regimens (refer to the Follicular Lymphoma section in the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information).
For patients with BCL2-rearranged tumors, treatment similar to that of adult patients with follicular lymphoma is administered (refer to the Follicular Lymphoma section in the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information).

Marginal Zone Lymphoma (Including MALT Lymphoma)

Marginal zone lymphoma is a type of indolent lymphoma that is rare in pediatric patients. Marginal zone lymphoma can present as nodal or extranodal disease and almost always as low-stage (stage I or stage II) disease. It is unclear whether the marginal zone lymphoma that is observed in pediatric patients is clinicopathologically different from the disease that is observed in adults. Most extranodal marginal zone lymphoma in pediatrics presents as MALT lymphoma and may be associated with Helicobacter pylori (gastrointestinal) or Chlamydophila psittaci (conjunctival), previously called Chlamydia psittaci.[16,17]

Treatment options for marginal zone lymphoma (including MALT lymphoma)

Treatment options for marginal zone lymphoma (including MALT lymphoma) include the following:
  1. Surgery only.
  2. Radiation therapy.
  3. Rituximab with or without chemotherapy.
  4. Antibiotic therapy, for MALT lymphoma.[17,18]
Most pediatric marginal zone lymphomas require no more than local therapy involving curative surgery and/or radiation therapy.[16,19] Treatment of MALT lymphoma of the gastric mucosa may also include antibiotic therapy, which is considered standard treatment in adults. However, the use of antibiotic therapy in children has not been well studied because there are so few cases.
Evidence (treatment of marginal zone lymphoma):
  1. In the largest retrospective study of pediatric patients (aged 18 years or younger) with marginal zone lymphoma (N = 66), the overall 5-year EFS was 70%, and OS was 98%. Patients primarily fell into the following two WHO-defined groups:[20][Level of evidence: 3iiiA]
    • Nodal (32%): Nearly all patients were male, with localized primary tumors in the head and neck. The treatment for all patients was resection (complete or incomplete) followed by observation. The EFS was 94%, and the OS was 100%.
    • Extranodal (67%): 57% of patients were male, and 27% of patients had a preexisting condition, which was immune compromising in most patients. The treatment options included chemotherapy, radiation, rituximab, resection, and observation. The EFS was 64%, and the OS was 97%. The only two deaths resulted from treatment-related complications of stem cell transplantation; both patients had an underlying immunodeficiency. Of note, 9 of 12 patients with extranodal marginal zone lymphoma who were managed with resection only remained in a first continuous complete remission with no further therapy; the other 3 patients who relapsed had their disease successfully salvaged.
Although the number of pediatric patients with MALT lymphoma is too small to perform meaningful clinical trials, studies of adult patients support the use of rituximab with or without chemotherapy (refer to the Marginal Zone Lymphoma section in the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information).
Intralesional interferon-alpha for conjunctival MALT lymphoma has been described.[21]

Primary Central Nervous System (CNS) Lymphoma

Other types of NHL that may be rare in adults and are exceedingly rare in pediatric patients include primary CNS lymphoma. Because of the small numbers of patients, it is difficult to ascertain whether the disease observed in children is the same as the disease observed in adults.
Reports suggest that the outcome of pediatric patients with primary CNS lymphoma (OS, 70%–80%) may be superior to that of adults with primary CNS lymphoma.[22-25]
Most children have diffuse large B-cell lymphoma, although other histologies can be observed.

Treatment options for primary CNS lymphoma

Treatment options for primary CNS lymphoma include the following:
  1. Chemotherapy.
Therapy with high-dose intravenous methotrexate and cytosine arabinoside is the most successful, and intrathecal chemotherapy may be needed only when malignant cells are present in the cerebrospinal fluid.[26]
There is a case report of repeated doses of rituximab, both intravenous and intraventricular, being administered to a 14-year-old boy with refractory primary CNS lymphoma, with an excellent result.[27] This apparently good outcome needs to be confirmed, and similar results have not been observed in adults. It is generally believed that rituximab does not cross the blood-brain barrier.
(Refer to the PDQ summary on Primary CNS Lymphoma Treatment for more information about treatment options for non–AIDS-related primary CNS lymphoma.)

Peripheral T-cell Lymphoma

Peripheral T-cell lymphoma, excluding anaplastic large cell lymphoma, is rare in children.
Mature T-cell/natural killer (NK)–cell lymphoma or peripheral T-cell lymphoma has a postthymic phenotype (e.g., terminal deoxynucleotidyl transferase negative), usually expresses CD4 or CD8, and has rearrangement of T-cell receptor genes, either alpha-beta and/or gamma-delta chains. The most common phenotype observed in children is peripheral T-cell lymphoma–not otherwise specified, although angioimmunoblastic lymphoma, enteropathy-associated lymphoma (associated with celiac disease), subcutaneous panniculitis-like lymphoma, angiocentric lymphoma, and extranodal NK/T-cell peripheral T-cell lymphoma have been reported.[28-32]
A Japanese study described extranodal NK/T-cell lymphoma–nasal type as the most common peripheral T-cell lymphoma subtype among Japanese children (10 of 21 peripheral T-cell lymphoma cases). In adults, extranodal NK/T-cell lymphoma–nasal type is generally Epstein-Barr virus (EBV) positive, and 60% of the cases observed in Japanese children were EBV positive.[33]
Although very rare, gamma-delta hepatosplenic T-cell lymphoma may be seen in children.[31] This tumor has also been associated with children and adolescents who have Crohn disease and have been treated with immunosuppressive therapy; this lymphoma has been fatal in all cases.[34]

Treatment options for peripheral T-cell lymphoma

Optimal therapy for peripheral T-cell lymphoma is unclear for both pediatric and adult patients.
Treatment options for peripheral T-cell lymphoma include the following:
  1. Chemotherapy.
  2. Radiation therapy.
  3. Allogeneic or autologous stem cell transplantation (SCT).
There have been four retrospective analyses of treatment and outcome for pediatric patients with peripheral T-cell lymphoma. The studies have reported the following:
  • The United Kingdom Children's Cancer Study Group (UKCCSG) reported on 25 children diagnosed over a 20-year period with peripheral T-cell lymphoma, with an approximate 50% 5-year survival rate.[28] The UKCCSG also observed that the use of acute lymphoblastic leukemia–like therapy, instead of NHL therapy, produced a superior outcome.
  • The COG reported on 20 patients older than 8 years who were treated on Pediatric Oncology Group NHL trials.[29] Eight of ten patients with low-stage disease achieved long-term disease-free survival compared with only four of ten patients with high-stage disease.
  • A study of Japanese children with peripheral T-cell lymphoma (N = 21) reported a 5-year OS rate of 85.2%. Treatment for peripheral T-cell lymphoma included chemotherapy (n = 18), radiation therapy (n = 2), and autologous (n = 2) and allogeneic (n = 9) SCT.[33]
  • The Berlin-Frankfurt-Münster study group reported 38 cases of peripheral T-cell lymphoma acquired over a 26-year period.[31][Level of evidence: 3iiiDiii] Patients with peripheral T-cell lymphoma–not otherwise specified (n = 18), most with advanced disease (stage III [n = 10] and stage IV [n = 5]), were usually treated with anaplastic large cell lymphoma protocols and had a 10-year EFS rate of 61%. Patients with NK/T-cell lymphoma (n = 9) fared poorly, with a 10-year EFS rate of 17%. This series also included five patients with hepatosplenic T-cell lymphoma and five patients with subcutaneous panniculitis-like T-cell lymphoma.

Cutaneous T-cell Lymphoma

Primary cutaneous lymphomas are very rare in pediatric patients (1 case per 1 million person-years), but the incidence increases in adolescents and young adults. All histologies of NHL have been observed to involve the skin. More than 80% of cutaneous lymphomas are T-cell or NK-cell phenotype.[35]
Subcutaneous panniculitic T-cell lymphomas are very rare lymphomas with panniculitis-like infiltration of subcutaneous tissue by cytotoxic T-cells.[36-38] Subcutaneous panniculitic T-cell lymphoma can be observed with malignant T cells, expressing alpha-beta chain T-cell receptor or gamma-delta T-cell receptor rearrangements.
In adults, the gamma-delta subtype of subcutaneous panniculitic T-cell lymphoma is associated with a more aggressive course and carries a worse prognosis than does the alpha-beta subtype of subcutaneous panniculitic T-cell lymphoma.[39] Morbidity and mortality are frequently related to the development of hemophagocytic syndrome, which was reported in one series in adults to occur in 17% of patients with alpha-beta subcutaneous panniculitic T-cell lymphoma and in 45% of patients with gamma-delta subcutaneous panniculitic T-cell lymphoma. The 5-year OS rate is 82% for alpha-beta subcutaneous panniculitic T-cell lymphoma and 11% for gamma-delta subcutaneous panniculitic T-cell lymphoma.[39] Subcutaneous panniculitic T-cell lymphoma is heterogeneous in the pediatric age group and does not necessarily follow the course observed in adults. In a series of 11 pediatric patients with subcutaneous panniculitis-like T-cell lymphoma, most presented with multifocal disease (often on the trunk) and systemic symptoms (fever), and there was a frequent association with hemophagocytic syndrome.[40]
The diagnosis of primary cutaneous anaplastic large cell lymphoma can be difficult to distinguish pathologically from more benign diseases such as lymphomatoid papulosis.[41] Primary cutaneous lymphomas are now thought to represent a spectrum of disorders, distinguished by clinical presentation.
Mycosis fungoides is rarely reported in children and adolescents,[42-44] accounting for about 2% of all cases. Patients present with low-stage disease, and it appears that the hypopigmented, CD8-positive variant of mycosis fungoides is more common in children than in adults.[45]; [46][Level of evidence: 3iiiDii]

Treatment options for cutaneous T-cell lymphoma

Because of the rarity of cutaneous T-cell lymphoma, no standard treatments have been established. Management and treatment of cutaneous T-cell lymphoma should be individualized and, in some cases, watchful waiting may be appropriate. Treatment may only be necessary if hemophagocytic syndrome develops.[47]
The best treatment for T-cell lymphomas with primarily pannicular involvement is not known. Treatment options include high-dose steroids, bexarotene, denileukin diftitox, multiagent chemotherapy, and hematopoietic SCT.[38,47-52]
An oral retinoid (bexarotene) has been reported to be active against subcutaneous panniculitis-like T-cell lymphomas in a series of 15 patients from three institutions.[49] In a series of 11 pediatric patients, aggressive polychemotherapy was used in all patients. Nine of 11 patients sustained clinical remission, with a median follow-up of 3.5 years.[40] In general, however, the optimal therapy for non–anaplastic large cell lymphoma cutaneous T-cell lymphoma in childhood is unclear.
Primary cutaneous anaplastic large cell lymphoma usually does not express ALK and may be treated successfully with surgical resection and/or local radiation therapy without systemic chemotherapy.[53] There are reports of surgery alone also being curative for ALK-positive cutaneous anaplastic large cell lymphoma, but extensive staging and vigilant follow-up is required.[54,55]
Mycosis fungoides occurring in pediatric patients may respond to various therapies, including topical steroids, retinoids, radiation therapy, or phototherapy (e.g., narrow-band ultraviolet B treatment), but remission may not be durable.[45,56-58]

References
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  25. Thorer H, Zimmermann M, Makarova O, et al.: Primary central nervous system lymphoma in children and adolescents: low relapse rate after treatment according to Non-Hodgkin-Lymphoma Berlin-Frankfurt-Münster protocols for systemic lymphoma. Haematologica 99 (11): e238-41, 2014. [PUBMED Abstract]
  26. Abla O, Weitzman S, Blay JY, et al.: Primary CNS lymphoma in children and adolescents: a descriptive analysis from the International Primary CNS Lymphoma Collaborative Group (IPCG). Clin Cancer Res 17 (2): 346-52, 2011. [PUBMED Abstract]
  27. Akyuz C, Aydin GB, Cila A, et al.: Successful use of intraventricular and intravenous rituximab therapy for refractory primary CNS lymphoma in a child. Leuk Lymphoma 48 (6): 1253-5, 2007. [PUBMED Abstract]
  28. Windsor R, Stiller C, Webb D: Peripheral T-cell lymphoma in childhood: population-based experience in the United Kingdom over 20 years. Pediatr Blood Cancer 50 (4): 784-7, 2008. [PUBMED Abstract]
  29. Hutchison RE, Laver JH, Chang M, et al.: Non-anaplastic peripheral t-cell lymphoma in childhood and adolescence: a Children's Oncology Group study. Pediatr Blood Cancer 51 (1): 29-33, 2008. [PUBMED Abstract]
  30. Wang ZY, Li YX, Wang WH, et al.: Primary radiotherapy showed favorable outcome in treating extranodal nasal-type NK/T-cell lymphoma in children and adolescents. Blood 114 (23): 4771-6, 2009. [PUBMED Abstract]
  31. Kontny U, Oschlies I, Woessmann W, et al.: Non-anaplastic peripheral T-cell lymphoma in children and adolescents--a retrospective analysis of the NHL-BFM study group. Br J Haematol 168 (6): 835-44, 2015. [PUBMED Abstract]
  32. Maciejka-Kemblowska L, Chaber R, Wrobel G, et al.: Clinical features and treatment outcomes of peripheral T-cell lymphoma in children. A current data report from Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG). Adv Med Sci 61 (2): 311-316, 2016. [PUBMED Abstract]
  33. Kobayashi R, Yamato K, Tanaka F, et al.: Retrospective analysis of non-anaplastic peripheral T-cell lymphoma in pediatric patients in Japan. Pediatr Blood Cancer 54 (2): 212-5, 2010. [PUBMED Abstract]
  34. Rosh JR, Gross T, Mamula P, et al.: Hepatosplenic T-cell lymphoma in adolescents and young adults with Crohn's disease: a cautionary tale? Inflamm Bowel Dis 13 (8): 1024-30, 2007. [PUBMED Abstract]
  35. Senerchia AA, Ribeiro KB, Rodriguez-Galindo C: Trends in incidence of primary cutaneous malignancies in children, adolescents, and young adults: a population-based study. Pediatr Blood Cancer 61 (2): 211-6, 2014. [PUBMED Abstract]
  36. Weisenburger DD, Savage KJ, Harris NL, et al.: Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood 117 (12): 3402-8, 2011. [PUBMED Abstract]
  37. Gallardo F, Pujol RM: Subcutaneous panniculitic-like T-cell lymphoma and other primary cutaneous lymphomas with prominent subcutaneous tissue involvement. Dermatol Clin 26 (4): 529-40, viii, 2008. [PUBMED Abstract]
  38. Mellgren K, Attarbaschi A, Abla O, et al.: Non-anaplastic peripheral T cell lymphoma in children and adolescents-an international review of 143 cases. Ann Hematol 95 (8): 1295-305, 2016. [PUBMED Abstract]
  39. Willemze R, Jansen PM, Cerroni L, et al.: Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood 111 (2): 838-45, 2008. [PUBMED Abstract]
  40. Oschlies I, Simonitsch-Klupp I, Maldyk J, et al.: Subcutaneous panniculitis-like T-cell lymphoma in children: a detailed clinicopathological description of 11 multifocal cases with a high frequency of haemophagocytic syndrome. Br J Dermatol 172 (3): 793-7, 2015. [PUBMED Abstract]
  41. Kumar S, Pittaluga S, Raffeld M, et al.: Primary cutaneous CD30-positive anaplastic large cell lymphoma in childhood: report of 4 cases and review of the literature. Pediatr Dev Pathol 8 (1): 52-60, 2005 Jan-Feb. [PUBMED Abstract]
  42. Kim ST, Sim HJ, Jeon YS, et al.: Clinicopathological features and T-cell receptor gene rearrangement findings of mycosis fungoides in patients younger than age 20 years. J Dermatol 36 (7): 392-402, 2009. [PUBMED Abstract]
  43. Hodak E, Amitay-Laish I, Feinmesser M, et al.: Juvenile mycosis fungoides: cutaneous T-cell lymphoma with frequent follicular involvement. J Am Acad Dermatol 70 (6): 993-1001, 2014. [PUBMED Abstract]
  44. Castano E, Glick S, Wolgast L, et al.: Hypopigmented mycosis fungoides in childhood and adolescence: a long-term retrospective study. J Cutan Pathol 40 (11): 924-34, 2013. [PUBMED Abstract]
  45. Boulos S, Vaid R, Aladily TN, et al.: Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: a case series of 34 patients. J Am Acad Dermatol 71 (6): 1117-26, 2014. [PUBMED Abstract]
  46. Cervini AB, Torres-Huamani AN, Sanchez-La-Rosa C, et al.: Mycosis Fungoides: Experience in a Pediatric Hospital. Actas Dermosifiliogr 108 (6): 564-570, 2017 Jul - Aug. [PUBMED Abstract]
  47. Johnston EE, LeBlanc RE, Kim J, et al.: Subcutaneous panniculitis-like T-cell lymphoma: Pediatric case series demonstrating heterogeneous presentation and option for watchful waiting. Pediatr Blood Cancer 62 (11): 2025-8, 2015. [PUBMED Abstract]
  48. McGinnis KS, Shapiro M, Junkins-Hopkins JM, et al.: Denileukin diftitox for the treatment of panniculitic lymphoma. Arch Dermatol 138 (6): 740-2, 2002. [PUBMED Abstract]
  49. Mehta N, Wayne AS, Kim YH, et al.: Bexarotene is active against subcutaneous panniculitis-like T-cell lymphoma in adult and pediatric populations. Clin Lymphoma Myeloma Leuk 12 (1): 20-5, 2012. [PUBMED Abstract]
  50. Rojnuckarin P, Nakorn TN, Assanasen T, et al.: Cyclosporin in subcutaneous panniculitis-like T-cell lymphoma. Leuk Lymphoma 48 (3): 560-3, 2007. [PUBMED Abstract]
  51. Gibson JF, Alpdogan O, Subtil A, et al.: Hematopoietic stem cell transplantation for primary cutaneous γδ T-cell lymphoma and refractory subcutaneous panniculitis-like T-cell lymphoma. J Am Acad Dermatol 72 (6): 1010-5.e5, 2015. [PUBMED Abstract]
  52. Chen CC, Teng CL, Yeh SP: Relapsed and refractory subcutaneous panniculitis-like T-cell lymphoma with excellent response to cyclosporine: a case report and literature review. Ann Hematol 95 (5): 837-40, 2016. [PUBMED Abstract]
  53. Kempf W, Pfaltz K, Vermeer MH, et al.: EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood 118 (15): 4024-35, 2011. [PUBMED Abstract]
  54. Hinshaw M, Trowers AB, Kodish E, et al.: Three children with CD30 cutaneous anaplastic large cell lymphomas bearing the t(2;5)(p23;q35) translocation. Pediatr Dermatol 21 (3): 212-7, 2004 May-Jun. [PUBMED Abstract]
  55. Oschlies I, Lisfeld J, Lamant L, et al.: ALK-positive anaplastic large cell lymphoma limited to the skin: clinical, histopathological and molecular analysis of 6 pediatric cases. A report from the ALCL99 study. Haematologica 98 (1): 50-6, 2013. [PUBMED Abstract]
  56. Koh MJ, Chong WS: Narrow-band ultraviolet B phototherapy for mycosis fungoides in children. Clin Exp Dermatol 39 (4): 474-8, 2014. [PUBMED Abstract]
  57. Laws PM, Shear NH, Pope E: Childhood mycosis fungoides: experience of 28 patients and response to phototherapy. Pediatr Dermatol 31 (4): 459-64, 2014 Jul-Aug. [PUBMED Abstract]
  58. Heng YK, Koh MJ, Giam YC, et al.: Pediatric mycosis fungoides in Singapore: a series of 46 children. Pediatr Dermatol 31 (4): 477-82, 2014 Jul-Aug. [PUBMED Abstract]

Changes to This Summary (08/06/2019)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Anaplastic Large Cell Lymphoma
Added text to state that although remissions can be achieved with single-agent therapy, central nervous system (CNS) progressions after therapy have been observed in patients with recurrent anaplastic large cell lymphoma. In one series, four of five patients who developed CNS progressions achieved complete remissions with either radiation therapy or high-dose methotrexate (cited Ruf et al. as reference 35).
Lymphoproliferative Disease Associated With Immunodeficiency in Children
Added Twist et al. as reference 27.
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

About This PDQ Summary



Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood non-Hodgkin lymphoma. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Childhood Non-Hodgkin Lymphoma Treatment are:
  • Louis S. Constine, MD (James P. Wilmot Cancer Center at University of Rochester Medical Center)
  • Alan Scott Gamis, MD, MPH (Children's Mercy Hospital)
  • Thomas G. Gross, MD, PhD (National Cancer Institute)
  • Kenneth L. McClain, MD, PhD (Texas Children's Cancer Center and Hematology Service at Texas Children's Hospital)
  • Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)
  • Nita Louise Seibel, MD (National Cancer Institute)
  • Malcolm A. Smith, MD, PhD (National Cancer Institute)
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”
The preferred citation for this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/hp/child-nhl-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389181]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.


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