Pediatric Non-Hodgkin Lymphoma Differential Diagnoses

  • Author: J Martin Johnston, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Apr 8, 2011
 
 

Differential Diagnoses

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Contributor Information and Disclosures
Author

J Martin Johnston, MD  Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Pediatric Hematology/Oncology, Backus Children's Hospital; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, California Nanosystems Institute and Molecular Biology Institute, University of California, Los Angeles, David Geffen School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

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Incidence of lymphoma as a function of age per 100,000 population. Data are from the Surveillance, Epidemiology, and End Results (SEER) for 1990-1994.
Massive mediastinal T-lymphoblastic lymphoma. Note compression of the left mainstem bronchus and the pulmonary atelectasis.
Non-Hodgkin lymphoma of the terminal ileum. Note the doughnut sign, ie, intraluminal contrast material surrounded by a grossly thickened bowel wall. This appearance is highly suggestive of small noncleaved cell lymphoma (Burkitt type).
Malignant pleural effusion. Non-Hodgkin lymphoma of the terminal ileum was diagnosed; the doughnut sign (ie, intraluminal contrast material surrounded by a grossly thickened bowel wall) was present. A diagnosis of stage 3 Burkitt lymphoma was established by means of pleurocentesis. (The bone marrow was normal.) The patient was treated successfully and never required an abdominal procedure.
Massive left pleural effusion as a complication of an upper anterior mediastinal T-lymphoblastic lymphoma. Note the atelectatic left lung. The diagnosis was established by means of thoracentesis. This patient had presented with bilateral parotid gland enlargement.
Table 1. Modified LSA2 L2 Therapy in Children's Cancer Group Protocol 552
PhaseDrugRoute
InductionCyclophosphamide, vincristine, daunorubicinIV
Ara-C, methotrexateIT
PrednisonePO
ConsolidationAra-CIV or SC
6-thioguaninePO
MethotrexateIT
L-asparaginaseIM
BCNUIV
PhaseCycleDrugRoute
Maintenance*16-thioguaninePO
CyclophosphamideIV
2HydroxyureaPO
DaunorubicinIV
3MethotrexatePO
BCNUIV
4Ara-CIV or SC
VincristineIV
Source.—Children's Cancer Group.



Ara-C = cytarabine; BCNU = 1,3-bis(2-chloroethyl)-1-nitrosourea, or carmustine; IM = intramuscular; IT = intrathecal; IV = intravenous; PO = oral; SC = subcutaneous.



* A minimum of 5 repeated courses (total duration of therapy >18 mo) are noted. Each course of intrathecal methotrexate (day 0 of each course) consists of 4 cycles of rotating drug pairs that are administered every 2 weeks after blood counts have recovered.



Table 2. Therapy for Stage III and IV non–B-Cell Disease* According to BFM Protocol 86
PhasesDrugRoute
InductionPrednisone, 6-mercaptopurinePO
Vincristine, daunorubicin, cyclophosphamide, Ara-CIV
L-asparaginaseIM
MethotrexateIT
Consolidation6-mercaptopurinePO
Methotrexate with leucovorin rescueIV
MethotrexateIT
Re-inductionDexamethasone, 6-thioguaninePO
Vincristine, doxorubicin, cyclophosphamide, Ara-CIV
L-asparaginaseIM
MethotrexateIT
Maintenance†6-mercaptopurine, methotrexatePO
Source.—Berlin-Frankfurt-Munster Group.



Ara-C = cytarabine; IT = intrathecal; IV = intravenous; PO = oral; SC = subcutaneous.



* Diagnoses included lymphoblastic lymphoma of the T-cell or precursor B-cell type, immunoblastic T-cell lymphoma, and other peripheral T-cell lymphomas. Of note, patients with Ki-1+ anaplastic LCLs were not included.



† Continued until 24 months after diagnosis.



Table 3. Clinical Risk Groups in the International Trial for Patients With SNCCL (Children's Cancer Group study 5961)
Clinical GroupSubjects,



Estimated %



Definition
A10All resected stage I or abdominal stage II tumors
B65Unresected stage I or II tumor, stage III, tumor, or stage IV with no CNS involvement and < 25% marrow blasts
C25CNS involvement or >25% marrow blasts
Table 4. Standard Therapy for Subjects in the International Trial for Patients With SNCCL, Group A*
DrugRoute
PrednisonePO
Vincristine, cyclophosphamide, doxorubicinIV
Filgrastim (G-CSF), to enhance neutrophil recoverySC or IV
G-CSF = granulocyte colony-stimulating factor; IV = intravenous; PO = oral; SC = subcutaneous.



* See Table 3 for the definition of group A. All subjects received 2 cycles.



Table 5. Standard Therapy for Subjects in International Trial for Patients With SNCCL, Group B*
PhaseDrugRoute
ReductionPrednisonePO
Vincristine, cyclophosphamideIV
Methotrexate/hydrocortisoneIT
PhaseCyclesDrugRoute
Induction2, starting 7 d after reductionPrednisonePO
Vincristine, methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/hydrocortisoneIT
Filgrastim (G-CSF)SC or IV
Consolidation2Methotrexate with leucovorin rescue, Ara-C
Methotrexate/hydrocortisone, Ara-C/hydrocortisone
Filgrastim (G-CSF)
Maintenance1PrednisonePO
Vincristine, methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/hydrocortisoneIT
Ara-C = cytarabine; G-CSF = granulocyte colony-stimulating factor; IT = intrathecal; IV = intravenous; PO = oral, SC = subcutaneous.



* See Table 3 for the definition of group B.



Table 6. Standard Therapy for Subjects in International Trial for Patients With SNCCL, Group C*
PhaseDrugRoute
ReductionPrednisonePO
Vincristine, cyclophosphamideIV
Methotrexate/Ara-C/hydrocortisoneIT
Induction, cycle 1 starting 7 d after reductionPrednisonePO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/Ara-C/hydrocortisoneIT
Filgrastim (G-CSF)SC or IV
Induction, cycle 2PrednisonePO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/Ara-C/hydrocortisoneIT
Filgrastim (G-CSF)SC or IV
Consolidation, 2 cycles†High-dose Ara-C, etoposide (VP-16)IV
Filgrastim (G-CSF), days 7-21SC or IV
High-dose methotrexate with leucovorin rescueIV
Methotrexate/Ara-C/hydrocortisoneIT
Maintenance 1PrednisonePO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/Ara-C/hydrocortisoneIT
Maintenance 2Ara-C, etoposide (VP-16)IT
Maintenance 3PrednisonePO
Vincristine, cyclophosphamide, doxorubicinIV
Maintenance 4Ara-C, etoposide (VP-16)IV
Ara-C = cytarabine; G-CSF = granulocyte colony-stimulating factor; IT = intrathecal; IV = intravenous; PO = oral, SC = subcutaneous.



* See Table 3 for the definition of group C.



† For patients with CNS involvement, during consolidation cycle 1 only.



Table 7. Prephase Therapy for Ki-1+ Anaplastic LCLs in All Patients According to the BFM-90 Protocol
DrugRoute
PrednisonePO
CyclophosphamideIV
Methotrexate/Ara-C/prednisoloneIT
Ara-C = cytarabine; IT = intrathecal; IV = intravenous; PO = oral.
Table 8. Subsequent Therapy for Ki-1+ Anaplastic LCLs According to the BFM-90 Protocol
CycleDrugRoute
AMethotrexate with leucovorin rescue, ifosfamide, etoposide (VP-16), Ara-CIV
Methotrexate/Ara-C/prednisoloneIT
BDexamethasonePO
Methotrexate with leucovorin rescue, Ara-C, doxorubicinIV
Methotrexate/Ara-C/prednisoloneIT
AADexamethasonePO
Vincristine, high-dose methotrexate with leucovorin rescue, ifosfamide, Ara-C, etoposide (VP-16)IV
Methotrexate/Ara-C/prednisoloneIT
BBDexamethasonePO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/Ara-C/prednisoloneIT
CCDexamethasonePO
Vindesine, high-dose Ara-C, etoposide (VP-16)IV
Methotrexate/Ara-C/prednisoloneIT
Ara-C = cytarabine; IT = intrathecal; IV = intravenous; PO = oral.
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