Lymphoma

Dr. Aynalem Yemane

 Lymphoma
• The third most common cancer among children
• It is the most common cancer in adolescents
– >25% of newly diagnosed cancers in persons 15-19 yr
old.
• 2 category
– Hodgkin lymphoma (HL)
– Non-Hodgkin lymphoma (NHL),
Hodgkin Lymphoma
 EPIDEMIOLOGY
• Bimodal age distribution
– 1ST peak: 15-35 yr of age
– 2nd peak: > 50 yr.
• A male predominance is found among young children, but
lessens with age.
 Etiology
• Infectious agents
– Human herpesvirus 6
– Cytomegalovirus
– Epstein-Barr virus (EBV): 4-fold higher risk
• Gene mutation
 CLINICAL MANIFESTATIONS

• Lymphadenopathy
– Painless, non tender, firm, rubbery
– Common site: cervical or supraclavicular
• Usually some degree of mediastinal involvement
• Symptoms and signs of airway obstruction
• HSM
– Rarely encountered
• Involvement below the diaphragm is rare and occurs in approximately 3% of
all cases.
• Metastatic effect
– Pleural or pericardial effusion
– BM infiltration
– Hepatocellular dysfunction
• Systemic symptoms (B symptoms)
– Important in staging
– Unexplained fever >38°C (100.4°F),
– Weight loss >10% total body weight over 6 mo
– Drenching night Sweats.
 Lab
• Complete blood cell count
• Erythrocyte sedimentation rate
• Measurement of serum ferritin
• Excisional biopsy: diagnostic
• Reed-Sternberg cells are pathognomonic
• Bone marrow aspiration
Histologic classification
Imaging

• CXR: mediastinal
mass
• CT scans of the
neck, chest,
abdomen, and pelvis
Staging
 TREATMENT

• Determined by
– Disease stage
– Presence or absence of B symptoms
– The presence of bulky nodal disease
Radiation therapy
• Prolonged remission and cure
rates
• Used in low-stage HL.
• Causes significant long-term
morbidity
• including growth retardation,
thyroid dysfunction, and cardiac
and pulmonary toxicity.
Combination chemotherapy
– Complete response rate of 70-
80%
– Cure rate of 40-50% in
patients with advanced-stage
disease.
• The combination chemotherapy regimens includes
– COPP: Cyclophosphamide, vincristine [Oncovin],
procarbazine, and prednisone or
– ABVD: doxorubicin [Adriamycin], bleomycin, vinblastine,
and dacarbazine
– ABVE-PC
– BAVD : Brentuximab , doxorubicin [Adriamycin],
vincristine, dacarbazine
• A complete response is defined as
– Complete resolution of disease on clinical examination and
imaging studies or
– At least 70-80% reduction of disease and a change from
initial positivity to negativity on PET scanning.
 RELAPSE and Prognosis
• Most occur within the 1st 3 yr after diagnosis
• Can occur late as 10 yr.
• Poor prognostic factors
– Tumor bulk
– Stage at diagnosis
– Extralymphatic disease
– Presence of B symp
• Early stage disease
– Even free survival (EFS) of 85-90%
– Overall survival (OS) at 5 yr of >95%.
• Advanced stage disease
– EFS :80-85%
– OS : 90%)
NHL
• Results from malignant proliferation of cells of lymphocytic
Lineage.
• Generally restricted to lymphoid tissue such as lymph nodes,
Peyer’s patches and spleen
• Bone marrow involvement in children.
• Bone and primary CNS lymphomas are rare presentations.
 EPIDEMIOLOGY
• 8–10% of all malignancies in children between 5–19 years of
age.
• 60% of all lymphomas in children and adolescents.
• Male : Female : 2.5:1 overall and over 3:1 in ages 5–14.
• Similar across all ages, but peaks at ages 15–19 years,
 Risk factors
• Genetic:
– Immunological defects: Agammaglobulinemia, SCID,
ataxia-telangiectasia, Wiskott–Aldrich syndrome
• Post-transplant immunosuppression
• Drugs
• Radiation
• Viral: Epstein–Barr virus , human immune deficiency virus
 PATHOLOGIC CLASSIFICATION
• Classification is based on :
– Histologic
– Immunophenotypic
– Genetic features
– Clinical presentation and course
 Cont…
Cell origin Common site of Incidence Genome alteration
involvement

LBL Immature Mediastinum 19% t(5;14) translocation

B(10%) and T (20%)
cell(90%)
BL Mature B cell Abdomen , Head 21% t(8;14) (90%)
and Neck t(8;22) translocation
(10%).
DLBCL Mature B cell Abdomen 37% t(8;14) translocation
(30%) often have a
complex (80%)
karyotype
Atypical large B Mature T(70%) 17% t(2;5) translocation
cell lymphoma Mature B (90%)
cell( 10%)
 CLINICAL FEATURES

• The clinical manifestations of childhood NHL depend primarily on

– Pathological subtype
– Sites of involvement.
• Tumors which grow rapidly can cause symptoms based on size and
location.
• Approximately 70% of children present with advanced-stage disease
including involvement of GI, bone marrow and central CNS.
Abdomen
• BL and DLBCL commonly have primary tumors in the abdomen.
• Is the primary site in 35% of childhood NHL.
• Common site
– The ileocecal region
– Appendix
– Ascending colon
– Some combination of these sites.
Abdomen
• Present with:
– Abdominal pain and distention
– Nausea, vomiting and bowel changes
– Palpable masses
– Intussusception
• Lymphoma is the most frequent cause of intussusception in children over 6years of age
– HSM, obstructive jaundice, ascites, or peritonitis.
– Bleeding and perforation of the intestine, although rare, may occur.
Head and Neck (13%)
• Present with:
– Enlargement of the cervical node(s) and parotid gland,
– jaw swelling
– Unilateral tonsillar hypertrophy.
• The endemic form of BL has a much higher incidence of facial
involvement, with around 50–60% at presentation
Mediastinum(26%)
• Patients with large mediastinal masses may have superior
mediastinal syndrome
• SMS present with
– Distended neck veins
– Edema of the neck and face
– Marked dyspnea, orthopnea
– Dizziness, headache, dysphagia, epistaxis, altered mental
status and syncope
• Mediastinal tumors may lead to pleural effusion through
– Direct pleural involvement or
– Compression of lymphatics by the mediastinal mass.
• The presence of significant pericardial effusion may cause
cardiac tamponade.
• Lymphoblastic lymphoma (LL) commonly presents with an
intrathoracic or mediastinal mass along with spread to other sites,
e.g., bone marrow, meninges and gonads.
• BL and anaplastic large cell lymphoma (ALCL) involve the
mediastinum less commonly.
Bone Marrow
• BL and LL have a predilection for spreading to the bone marrow.
• Rarely, ALCL and DLBCL will present with bone marrow
involvement.
• Bone marrow involvement is consistent with acute leukemia in
25%
Central Nervous System
• More common with BL and LL
• Worse overall survival
Other Primary Sites(11%)
• Skin and subcutaneous tissue
• Orbit
• Thyroid
• Bone (with or without hypercalcemia)
• Kidney
• Epidural space, breast and gonads.
Constitutional Symptoms
• Fever and weight loss are relatively uncommon except in ALCL.
• Weight loss may also occur secondary to mechanical bowel
obstruction.
DIAGNOSIS
 MANAGEMENT
• Emergency Treatment of onchologic emergencies
– Superior vena cava (SVC) syndrome
– Respiratory airway compression
– Tumor lysis syndrome (TLS) secondary to……….
• Chemotherapy
• Radiation Therapy
– only indicated for acute life-threatening complications refractory to
initial chemotherapy
• Surgical Therapy
– The role in NHL treatment is limited.
– It should be performed on patients that total resection can be achieved
 PROGNOSIS
• Excellent for most forms
• Patients with localized disease have a 90-100% chance of survival
• With advanced disease have a 70-95% chance of survival.
THANK YOU