DEN 24pediatric Lymphomas

Pediatric Lymphomas
PREPARED BY
KELVIN CHACHA
Cervical
Adenopathy
Introduction
• Lymphoma is an uncontrolled malignant proliferation of
Lymphoid cells
• Lymphoid cells are found in primary lymph organs and
secondary lymph organs
• Primary lymph organs – Bone marrow (B cells) and
Thymus (T cells)
• Secobdary lymph organs – Lymph nodes and spleen
Concerns in enlarged LN
• Size >2.5 cm • Fevers >38.5 for 2-4
• Increasing size weeks
over 2-4 weeks • Constitutional
• Matted or fixed symptoms
• Supraclavicular LN • HepatoSplenoMegal
y
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
• Laboratory • Radiology
-CBC with smear -CXR (PA & Lat)
-Chem profile -CT scans neck, chest,
LDH, uric acid abdomen
-PET scan
• Disease specific
-ESR, IL2R for HD
-LP if head/neck NHL
-BMA/Bx
TYPES OF LYMPHOMA
• There 2 types of Lymphoma based on how they appear
microscopically
• Hodgkin's lymphoma
• Non Hodgkin's lymphoma
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
• Rapid CTX response; high risk of tumor lysis and
hyperuricemia
Incidence/Etiology - NHL
• 6% childhood cancer
60% of childhood lymphomas
• Peak age of 5-18; 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
Non-HL Types Commonly Found In
Children
• Burkitt’s Lymphoma and Burkitt’s Like Lymphoma
• Lymphoblastic Lymphoma (Tx as ALL)
• Diffuse large B cell Lymphoma
• Anaplastic Large Cell Lymphoma.
Burkitt’s Lymphoma
• Highly aggressive B-cell non-HL.
• It’s a common childhood malignant in
Africa.
• There are three distinct clinical forms
namely;
• Endemic(African)
• Sporadic and
• Immune deficiency
associated BL.
Clinical Presentations
• Common sites
• Jaw and Facial 50 to 60%:
lymphadenopathy, jaw swelling, single
enlarged tonsil, nasal obstruction,
rhinorrhea, cranial nerve palsies
• Abdomen 35%: pain, distention, jaundice, GI
problems, mass
• Mediastinum (26%): SVC syndrome
+Fever, malaise, night sweats, wt. loss,

Endemic type of BL
• Endemic Burkitt's
lymphoma is seen among
the young children of
equatorial Africa.
• It frequently affects the
jaws of children with
developing molar teeth;
• experts suggest that
growth factors may be
responsible for the site-
specific nature of this
tumor.
Sporadic BL
• Sporadic Burkitt's lymphoma
often involves the abdomen,
and it spreads to the bone
marrow in about 20% of
patients
• When Burkitt's lymphoma

involves the abdomen, it can
cause symptoms such as
abdominal pain, swelling,
nausea, vomiting, and
changes in bowel habits.
• It is a highly malignant
lymphoma, but it is
potentially curable with very
aggressive therapy.
Immunodefecient BL
• Immunodeficient Burkitt's lymphoma affects patients
with abnormal or suppressed immune systems (e.g.,
patients with HIV).
Pathogenesis
• All forms of Burkitt’s lymphoma are associated with a
translocation of the c-myc gene on chromosome 8
Pathogenesis
• EBV is found in all African Burkitt’s Lymphomas and in
25% of the HIV associated Burkitt’s lymphomas but is
rare in Non-endemic or sporadic type of Burkitt’s
• EBV in it self how ever does not appear to be the only

factor in causing the development of Burkitt’s
lymphoma thus not every one who gets infected with
EBV develops Burkitt’s lymphoma
Pathogenesis
Other associated causes of BL
• Chronic malaria infection has been identified as one of
the core factor’s for the development of Burkitt’s
lymphoma since it favors sustained proliferation of B
cells immortalized by EBV
• A weakened immune system fails to control EBV

infection and thus favors proliferation of the infected
cells and thus the high incidence of EBV associated
Burkitt’s lymphomas in immunocompromised patients
Common sites involved
• Bones of the jaw and other facial bones,
• Kidneys,
• Gastrointestinal tract, The abdomen, especially the
ileocecal area, is the most common site of involvement;
• Ovaries,
• Breast,
• Other extranodal sites
Staging
• Group A – Low Risk:
A single facial mass (excluding retro-orbital masses)
less than 10cm
• Group B – High Risk:
Any disease more than Group A but without CNS
disease
• Group C – CNS disease present
• Group D – Bone Marrow Involvement
Investigations
• Tissue biopsy • CT scan/MRI
• FNA (flow • FBP, RFT.LFT
cytometry)
• Serum
• B, T or natural
killer cell electrolytes and
derivation chemistry
• Detection of • HIV serology
markers
(antigens) • BMA
• CSF analysis • Abdominal USS
and CXR
Burkitt lymphoma “starry-sky” pattern
Management
• Chemotherapy
• Radiation
• Surgery
Chemotherapy
• chemotherapy is the treatment of choice
• Alternative name is cytotoxic drugs
• Chemotherapy uses anticancer drugs that are given IV,
IT or orally
• These drugs enter the bloodstream and reach all areas
of the child's body, making this treatment useful for
cancer that has spread widely
Chemotherapy
Cyclophosphamide
1-1.2gm/m² or 40mg/kg/dose
given iv in 100mls of 5%glucose
vincristine
1.5-2mg/m² or 0.05-0.07mg/kg iv by slow push
Methotrexate
20-60mg/m or 0.7-2mg/kg IT (can also be given
oraly,IV)
Chemotherapy
• First-line Chemotherapy: Cyclophosphamide,
Vincristine, and Methotrexate (COM)
• Second-line Chemotherapy: Etoposide, Mesna,

Ifosfamide, Cytarabine (EMIC)
National TX Protocol
Group Number and name of Number of cycles of
intravenous (IV) intrathecal (IT)
cycles of chemotherapy
chemotherapy
A: Low Risk 3 COM 3
B: High Risk 6 COM 3
C: CNS positive 6 COM 6
D: BM Positive 6 alternating COM/ EMIC 6

Sugery
• Surgery should not be done simply to remove a tumor,
as normal organs might be damaged in the process.
• Other reasons for surgery includes:

To obtain tissue for diagnostic tests when other
procedures could not obtain enough tissue
On an emergence basis, to relieve a blockage

(obstruction) in the intestine caused by the tumor mass
Radiation
• Was once the main treatment for children with NHL,
however it is no longer used in the initial Rx of NHL,
except perhaps in an emergency to treat pressure on
the trachea or SC.
• For palliation during late presentation

Prognosis
• International Prognostic Index
The International Prognostic Index (IPI) was designed to
further clarify lymphoma staging.
• The IPI predicts the risk of disease recurrence and
overall survival by taking into account factors such as
• age,
• stage of disease,
• general health (also known as performance status),
• number of extranodal (other than the lymph nodes)
• sites,
• presence or absence of an elevated serum enzyme
named lactate dehydrogenase (LDH).
Differential diagnosis of Abdominal
Masses
• Wilm’s tumour- Nephroblastoma
• Embryonal tumours
• Arising from the kidneys
• They do not cross the midline
• Good prognosis
• Neuroblastoma’s
• Tumour arising from the neuro crest
• Located/ arising from the adrenal glands
• Crosses the midline
• Poor prognosis
• Elevated Urine VMA
• Hepatoblastoma
• Arising from the liver
• Embryonal tumours
• Elevated alpha pheto-protein
DDX
• Splenomegaly
• Sequestration crisis in SCD
• Severe/complicated Malaria
• Leukemia’s
NOTE:
Cautious on your palpation.
Lymphoblastic lymphoma
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
• Tx as ALL
Other Types
Diffuse Large cell Lymphoma
Tx – CHOP (Cyclophosphamide, Adriamycin, Vincristine
and Prednisolone)
Anaplastic Large Cell Large Cell Lymphoma (ALCL)
• Peripheral T-cell presents as painless Lymphadenopathy +/-
skin or subcutaneous tissue involvement
Tx - CHOP
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
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
Hodgkin’s disease with Reed Sternberg cell
often CD20+
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
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
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, chills and night sweating
Staging
A – asymptomatic B – unexplained weight loss >10% in 6mnths +/- fever +/- night sweats
Extralymphatic – thymus, spleen, appendix and Peyer’s patches
Prognosis
FAVORABLE:
<10Cm, Female, favorable subtypes (LP and NS) and
Stage I non-bulky disease
UNFAVORABLE:
Persistently elevated ESR;
LD histopathology;
bulky disease--largest dimension >10cm;
B symptoms;
Treatment For HL
• 1st line
• Adriamycin, Bleomycin, Vinblastine, Dacarbazine
(ABVD).
• If good response →ABVD +/- radiotherapy if localized
disease
• 2nd line
• If poor response→EPIC(Etoposide, ifosfamide,
prednisone, Cisplatin) +/- radiotherapy if a localized
disease
Approach
• 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
Continue
• 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
• and know that such patients should be evaluated
thoroughly if fever develops
Side effects
• Nausea, vomiting, diarrhea, loss of appetite
• Alopecia
• Tumor Lysis syndrome-after a rapid destruction of the
tumor » Hyperuricemia
with renal failure (Prevented by allopurinol and
plenty of fluids). Breakdown products may affect
kidneys, heart, CNS.
Side effects
• Damage to other normal cells that are dividing rapidly-
bone marow,linings of mouth (mouth sores),
GIT,reproductive cells
• Hemorrhagic cystitis
• Cardiotoxic
• Vincristine is neurotoxic
SIDE EFFECT OF EACH
CHEMO THERAPY

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Pediatric Lymphomas

+Fever, malaise, night sweats, wt. loss,

• When Burkitt's lymphoma

• EBV in it self how ever does not appear to be the only

• A weakened immune system fails to control EBV

• Second-line Chemotherapy: Etoposide, Mesna,

B: High Risk 6 COM 3

C: CNS positive 6 COM 6

D: BM Positive 6 alternating COM/ EMIC 6

• Other reasons for surgery includes:

On an emergence basis, to relieve a blockage

• For palliation during late presentation

**More favorable: Stage I or II, head/neck, peripheral

• Recognize the need for evaluation of supraclavicular

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