Lymphomas in Children

Lymphomas
• Case history
• Epidemiology
• NHL
• HL
 Case history
• 3 yr old male child presented with neck swelling for
3 months.
• No other complaints.
• No history of TB exposure.
• With RX also,swelling not reducing in size.
O/E:
• Cervical lymphadenopathy bilaterally
• Bilateral axillary lymphnodes.
• No other physical findings.
 Case history
On investigations:
• Hb- 8gm%, WBC 10000/cumm, platelets 100000/cumm
• ESR –25
• CXR: normal
• Mantoux test –ve
• Uric acid + LDH- Normal
• USG abdomen: normal
• Lymph node biopsy showed Reed Sternberg cells with
lymphocyte predominance.
• Bone marrow aspn + biopsy: normal
Reed-Sternberg
cell

DX: Hodgkin’s lymphoma

(Lymphocyte predominance)
 Lymphoma
• Lymphomas results from malignant proliferation
of cells of lymphocytic or histiocytic lineage.
• 3rd most common group of cancers.
• 10-15% of newly diagnosed cancers in childrens
and adolescents comprise of Lymphoma.
• 60% of these are NHL and 40% Hodgkin’s
lymphomas.
Cancer in adult and children, UK, 1986-1995
 Non-Hodgkin’s Lymphoma:
epidemiology
• Lymphoma = 10-15% of all pediatric cancers
• NHL occurs in 60% of lymphoma cases
• M:F = 3:1
• Peak incidence between 7-11 years of age
• Striking geographical differences in incidences
rates and distribution of subtypes of NHL eg.
Burkitt’s.
 Non-Hodgkin’s Lymphoma:
epidemiology
• EBV is almost invariably associated with
Burkitt’s.
• Increased risk with T-cell deficiency
– Congenital immunodeficiency syndromes like Ataxia
Telengiectasia, Wiskott-aldrich syndrome.
– Acquired immunodeficiency syndrome eg: AIDS, Post
transplant
– Immunosuppressive drug therapy
 Non-Hodgkin’s Lymphoma:
classification
• Low-, intermediate-, and high-grade lesions
• 90% of children with NHL have high-grade
disease at presentation
• High-grade
– Large cell lymphoma
– Lymphoblastic lymphoma (usually T celled)
Burkitt’s
– Small cell noncleaved lymphoma
(Usually B cell derived) Non-Burkitt’s
 Non-Hodgkin’s Lymphoma
• Large cell lymphoma
– 27% pediatric cases
– t(2;5) anomaly
– Rare presentation in
H&N
(Involves skin commonly)

From, Diagnostic Surgical Pathology of the Head and Neck,

W.B.Saunders, p 762.
 Non-Hodgkin’s Lymphoma
• Lymphoblastic lymphoma
– 29% pediatric
cases
– t(7 or 14)
– Mediastinal mass or
cervical or generalized
lymphadenopathy.

From, Diagnostic Surgical Pathology of the

Head and Neck, W.B.Saunders, p 759.
 Non-Hodgkin’s Lymphoma

• Small cell noncleaved

– 34% pediatric cases
– Burkitt’s lymphoma
• Epstein-Barr virus
• t(8;2,14,22)
• Mandible
• Head and Neck
• Abdomen

From, Surgical Pathology of the Head and Neck,

Lippincott Williams & Wilkins, p 161.
 Non-Hodgkin’s Lymphoma: C/F
• Presenting symptoms are varied and depend upon the
location of tumor, extent of disease, and histologic subtype.
• Children usually present with extranodal diseases involving
the mediastinum, abdomen, or head and neck region.
• Presentation in the H&N in 29% of cases
– Cervical lymphadenopathy
– Salivary gland, larynx, sinuses, orbit, scalp
– Endemic Burkitt’s as jaw mass in 50% cases.
– Associated symptoms
• Fever, night sweats, weight loss
Non-Hodgkin’s Lymphoma: C/F
• Mediastinum is involved in about 25%
cases.
• Large anterior mediastinal mass can cause:
1. superior-venacaval syndrome
• Can involve Bone marrow and develop
meningeal and gonadal involvement.
• Pleural and pericardial effusion.
Non-Hodgkin’s Lymphoma: C/F
• Abdomen: primary site in 35% of cases.
• Ileocecal region, appendix, colon and
retroperitoneal region.
• Usually present with abdominal pain, vomiting,
abdominal distension, palpable mass,
intussusception, ascites, obstructive jaundice or
hepatosplenomegaly.
• Can cause spinal cord compression with epidural
mass.- surgical emergency.
 Non-Hodgkin’s Lymphoma: Dx
• Evaluation
– H&P
– Biopsy
• Tonsillectomy
• Lymph node
– Staging w/u
• Blood studies- CBC
• Biochemistry RFT (BUN, creatinine and uric acid), LFT, serum
eletrolytes, serum LDH.
• Lumbar puncture
• Bone marrow biopsy
• CXR, CT chest
• USG abdomen , CT abdomen/pelvis
• Bone scan
 Non-Hodgkin’s Lymphoma:
Management
Principles of therapy:
• Multi-agent chemotherapy directed to the stage and
histologic subtype-cornerstone of Rx.
• Surgery has very limited role except for Dx.
• Irradiation is of the primary site is usually restricted
to emergency situation like cord compression, cranial
nerve palsy.
• Drug selection and duration is based on the stage and
histologic subtypes.
• Usually sensitive to chemotherapy.
 Non-Hodgkin’s Lymphoma:
management
• Multiagent Chemotherapy
– Cyclophosphamide
– Doxorubicin
– Vincristine 6 cycles of COMP or
– Prednisone
3 cycles of COMP + 6
– +/- Methotrexate months of 6-MP and
Methotrexate
 Non-Hodgkin’s Lymphoma: Rx
• Survival at 5 years
– Overall Stage I and II NHL = 85-95%
– Overall Stage III and IV NHL = 65-75%
– Stage III and IV BL = 75-85%
 Non-Hodgkin’s Lymphoma:
complications
• Complication arising from space occupying
nature of the tumor.
1. Superior venacava syndrome
2. Pericardial effusion
3. Small bowel obstruction, gastric bleeding or
perforation.
• Metabolic complication eg. Tumor lysis
syndrome.
 Hodgkin’s Disease
• Less common than NHL
• More frequently in 15-20 y/o population
• 4% under 10 years
• M:F = 3:1

• Association with EBV

• In developing countries, reported to have occurred
in young children frequently.
 Hodgkin’s Disease: types
• Nodular Sclerosing • Lymphocyte Predominant

From, Diagnostic Surgical Pathology of the Head and Neck,

W.B.Saunders, p 750 & 764.
 Hodgkin’s Disease: types
• Mixed Cellularity • Lymphocyte Depleted

From, Diagnostic Surgical Pathology of the Head and Neck,

W.B.Saunders, p 750.
 Hodgkin’s Disease: types
• Reed-Sternberg cells are not pathognomonic of
Hodgkin’s lymphoma .Can be found in reactive
lymphoid hyperplasias, NHL, non-lymphoid
malignancies. Sometimes in EBV infection.
 Hodgkin’s Disease: C/F
• Presentation
– Asymmetric lymphadenopathy—90%
• Firm, rubbery
• Supraclavicular fossa
• Progressive, painless enlargement.
– 60% have mediastinal disease at diagnosis.
– Spleen, liver
– Constitutional symptoms—1/3 of cases
• Fever > 38 degree C, night sweats, anorexia, weakness, weight
loss of > 10% of body wt.
 Hodgkin’s Disease: C/F
• Any peripheral lymph node 1 cm. In size or more if doesn’t show
signs of regression after 6 weeks of observation should be
biopsied.
• Biopsy should be taken from “central” node as possible within a
group of enlarged nodes as edge of Hodgkin’s lymph node often
appear “reactive”.
Atypical presentations:
• Nephrotic syndrome
• Autoimmune hemolytic anemia
• Dermatomyositis
• Acute dysautonomia
• Central pontine myelinolysis
 Hodgkin’s Disease: Dx
• Evaluation
– H&P
– Chest xray to exclude mediatinal
adenopathy./ or CT chest
– CBC, ESR, serum copper, ALP
assay, TSH and T4
– USG or CT scan of Abdomen
– Spinal fluid, peritoneal ,pericardial
or pleural fluid exam.: cytochemical,
immunologic, cytogenetic, and
molecular
– LDH, serum uric acid
– Biochemistry: LFT, RFT
-- Biopsy = Reed-Sternberg cells
– Bone marrow biopsy
Hodgkin’s Disease: staging
Hodgkin’s Disease: Management
• Localized disease ( stages I, II and IIIA)
– Extended field XRT

• Disseminated disease ( stages IIIB and IV)

– MOPP = nitrogen mustard, vinblastine,
procarbazine, prednisone
– ABVD = adriamycin, bleomycin, vincristine,
dacarbazine
Hodgkin’s Disease: Management
• Radiation therapy can cause growth retardation,
thyroid failure, cardiac and pulmonary
dysfunction, and an increased risk of breast
cancer. So, chemotherapy is preferred by any
centers.
• Chemotherapy: minimum of six cycles were
given. Significant cumulative toxicity can occur,
including sterility, second malignancies and
cardiac and pulmonary dysfunction.
 Hodgkin’s Disease: prognosis
• Survival
– Stages I, II, and III = 90%
– Stage IV = 75-80%
 Comparison between HL and
NHL
Hodgkin’s disease NHL
• Gradual progression • Rapid progression
• Contiguous spread • Can spread anyway
• Histologic subtype not as • Crucial
imp as in NHL
• BM biosy only in advanced • BM biopsy is standard
disease. workup.
• Osseous involvement is rare. • 5-15% of initial presentation.
• Liver involvement is rare • 40-60% in small lymphocytic
(5%) and small cleaved cell
lymphomas.
• CNS disease is rare.
• 25% of pts with BM disease.
Thank You!!