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Pediatric Lymphomas

Pediatric Lymphomas. Resident Education Lecture Series. Cervical adenopathy. Size >1-2 cm Increasing size over 2-4 weeks Matted or fixed Supraclavicular LN. Fevers >38.5 for 2-4 weeks Constitutional symptoms HSM. Concerns in enlarged LN. When to biopsy. Supraclavicular node

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Pediatric Lymphomas

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  1. Pediatric Lymphomas Resident Education Lecture Series

  2. Cervical adenopathy

  3. Size >1-2 cm Increasing size over 2-4 weeks Matted or fixed Supraclavicular LN Fevers >38.5 for 2-4 weeks Constitutional symptoms HSM Concerns in enlarged LN

  4. When to biopsy • Supraclavicular node • Increasing size over 2-4 weeks • Constitutional symptoms • Asymptomatic enlarged node-not decreasing in size over 6 weeks or not normal after 8-12 weeks

  5. Laboratory -CBC with smear -Chem profile LHD, uric acid Disease specific -ESR, IL2R for HD -LP if head/neck NHL -BMA/Bx for all NHL, only IIB or higher HD Radiology -CXR (PA & Lat) -CT scans neck, chest, abdomen -Gallium, bone scan -PET scan Staging Evaluation

  6. Lymphoma Staging • Murphy  Ann Arbor • I: tumor at one site (nodal or extranodal -- “E”) • II: two or more sites; same side of body (or resectable GI primary) • III: both sides of body but not IV (& unresec. GI & mediastinal for NHL) • IV: CNS or marrow involvement (Murphy); lung, liver, marrow, or bone for Ann Arbor (< 25% marrow) • “B” sxs are defined for HD, as is “bulky disease” • Head and neck (possibility of CNS involvement) is a further consideration for NHL • PET or gallium

  7. LYMPHOMA HODGKINS NON-HODGKINS (40%) (60%) BURKITT’S LYMPHOMA LARGE CELL LYMPHOMA LYMPHOBLASTIC LYMPHOMA (<15%) (30-40%) (40-50%) IMMUNOBLASTIC ANAPLASTIC (50%) (50%)

  8. Non-Hodgkin’s Lymphoma • Malignant solid tumor of immune system • Undifferentiated lymphoid cells • Spread: aggressive, diffuse, unpredictable • Lymphoid tissue; BM and CNS infiltration • High growth fraction and doubling time • Dx and Rx ASAP • Rapid CTX response; tumor lysis concern

  9. Incidence/Etiology - NHL • 6% childhood cancer 60% of childhood lymphomas • Peak age of 5-15; M:F ratio of 2.5:1 • Increased with • SCIDS, HIV, EBV • post t-cell depleted BMT • post solid organ transplant • Geographic, viral, genetic & immunologic factors

  10. Types of NHL Lymphoblastic (30-35%) • 90 % immature T cells (very similar to T-ALL) • remainder pre-B phenotype (as in ALL) • 50-70% anterior mediastinum • neck, supraclavicular, axillary adenopathy • Classic: older child with intussusception

  11. Small non-cleaved cell (40-50%) --Mature B-cell phenotype --Burkitt's and non-Burkitt's --90% abdomen --Ascites and intusussception --Endemic in Africa (Burkitt's), with EBV 97%

  12. Burkitt Facts • 100 new cases/year in US, 2-3:1 male:female; mean age 11 years (in non-endemic form) • small, noncleaved cell; mature B phenotype; intraabdominal (sporadic) or jaw (endemic) most common primary site • 90% have t(8;14) • (8 ~ c-myc; 14 ~ heavy chains) • others are 8;2 or 8;22 • (2, 22 ~ light chains) • Extremely rapidly-growing; tumor lysis issues

  13. Burkitt Prognosis Adult Data: Stage: EFS OS I-II 91% 78% IV 25% 25% but in patients < 40 yo 70% 60% Pediatric Data: Localized > 90% Disseminated (but not B- ALL) 80-90% on newer protocols

  14. Large-cell lymphoma (15-20%) • Anaplastic (Ki-1) lymphoma – ALK fusion protein • Diffuse Large B-cell lymphoma (DLBCL) • frequent Mediastinal involvement • More like Hodgkin lymphoma than other NHLs • “Peripheral T-cell” lymphoma • Often involves skin, CNS, lymph nodes, lung, testes, muscles, and GI tract

  15. “low grade” lymphomas – rare in children • Follicular • marginal zone/MALT • primary CNS (often seen with HIV infection) • peripheral cutaneous (mycosis fungoides)

  16. Clinical Presentations • Abdomen: (35%): pain, distention, jaundice, GI problems, mass • Head/neck (13%): lymphadenopathy, jaw swelling, single enlarged tonsil, nasal obstruction, rhinorrhea, cranial nerve palsies • Mediastinum (26%): SVC syndrome • CNS (rare): HA, V, irritability, papilledema +Fever, malaise, night sweats, wt. loss,

  17. Prognosis affected by… • Incomplete remission in first 2 mos. Rx • Large tumor burden (LDH >1000) • Stages III and IV: CNS or BM involvement • Delay in treatment • Relapse **More favorable: Stage I or II, head/neck, peripheral nodes, GI tract

  18. NHL Treatment • Surgery for diagnostic bx or second look • Radiation Therapy: emergency airway obstruction or CNS complication – may be used for local control of residual mass • Chemotherapy: Combination chemo is usual, with overall cure rates 60-80+%; high risk of tumor lysis and hyperuricemia • Relapse: Re-induction, followed by BMT

  19. Hodgkin’s Disease • Immune system malignancy, involving B or T lymphocytes • Reed-Sternberg cells • Spread: slow, predictable, with extension to contiguous lymph nodes • Infiltration to non-lymphoid organs is rare

  20. Hodgkin’s disease with Reed Sternberg cell often CD20+

  21. Incidence and Etiology • Hodgkin’s 5% of childhood cancers • Bimodal peaks, at 15-35 and >50; rare < 5 • M:F ratio of 3:1; variation r/t geography and SES, and type • Increased in immunologic disorders, HIV, EBV

  22. Types of Hodgkin’s Lymphoma • Nodular sclerosing (NS), 40-60%, lower cervical, supraclavicular, mediastinal nodes • Mixed cellularity (MC), 15-30%; advanced disease with extranodal involvement • Lymphocyte predominance (LP), 5-15%, presents as localized disease • Lymphocyte depletion (LD) (<5%); widespread disease

  23. Clinical Presentation • Painless lymph node swelling (90%) that persists despite antibiotic therapy • Palpable non-tender, firm, mobile, rubbery nodes; Mediastinal adenopathy (60%); SVC • Bulky: when mass is > 1/3 thorax diameter • B symptoms: Fever of >38C for 3 days, drenching night sweats, 10% weight loss

  24. Mediastinal masses • Risk for anesthesia (esp. if tracheal compression > 50% by CT) • Least invasive diagnostic procedure therefore indicated (incl. thoracentesis) • Emergent steroids or RT generally acceptable prior to biopsy • HD and DLBCL tend to have areas of necrosis and therefore look more “bumpy” than T-ALL

  25. Prognosis FAVORABLE: <10, F, favorable subtypes (LP and NS) and Stage I non-bulky disease UNFAVORABLE: Persistently elevated ESR; LD histopathology; bulky disease--largest dimension >10cm; B symptoms;

  26. Treatment and Prognosis • Dependent on age, stage, and tumor burden • RT alone, CTX alone • RT: varies from involved field for localized disease to extended field to total nodal irradiation, inverted Y plus mantle • most often multimodal therapy, with low-dose involved field RT and multi-agent CTX • Combined modality 70-90% LT cure

  27. Hodgkin Px and Rx • Splenectomy generally no longer used • Exact type and ratio of combined modality therapy changes… due to differences in success rates for salvage therapy and concerns for late effects of therapy • Second malignancy risks • Sterility risks

  28. From ABP Certifying Exam Content Outline • Know how to evaluate a child with an acute cervical lymphadenopathy • Know the differential diagnosis of neck masses: • lymphoma, • cystic hygroma, • thyroglossal duct cyst • branchial cleft abnormalities

  29. From ABP Certifying Exam Content Outline, continued • Recognize the need for evaluation of supraclavicular lymph node enlargement • Identify the chest x-ray as an important part of the initial evaluation of the patient with an unexplained lymphadenopathy • Know that overwhelming sepsis is a serious complication in patients with Hodgkin disease who have undergone splenectomy, • and know that such patients should be evaluated thoroughly if fever develops

  30. Credits • Meghen Browning MDAnne Warwick MD MPH

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