Pediatric Lymphomas

Posted by e-Medical PPT Thursday, February 9, 2012
Concerns in enlarged LN: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

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

Staging Evaluation:
Staging Evaluation 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

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 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 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 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 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, Staging of NHL:I Single tumor /node NOT in mediastinum or abdomen II 1-2 nodes same side of diaphragm or resectable GI primary III 2+ nodes both sides of diaphragm; intrathoracic or extensive intra-abd IV Any of above with CNS and/or BM 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

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

NHL chemotherapy overview:Low-stage NHL’s are treated with CHOP (+/- rituximab – anti-CD20) Higher-stage lymphoblastic lymphomas are treated on leukemia protocols Higher-stage non-lymphoblastic NHLs require extremely aggressive chemotherapy with significant infectious risks, but still have generally good remission rates High-dose chemotherapy with stem cell rescue is considered an option for relapse, though without the success rates of HD; T cell disease probably requires an allogeneic response...

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