Pedi Lymphomas

Question 1

What is the most common lymphoproliferative
malignancy of the head and neck in pediatric
patients?

Answer 1

Lymphoma

Question 2

What are common risk factors for lymphoma?

Answer 2

Radiation, Epstein-Barr virus, human immunodeficiency
virus (HIV) or other immunosuppressive disorders, organ
transplantation, common immunodeficiency syndromes
(e.g., Wisckott-Aldrich syndrome, ataxia-telangiectasia,
X-linked lymphoproliferative disease), organic toxins (e.g.,
phenols, benzene), autoimmune disorders (e.g., celiac
disease, rheumatoid arthritis)

Question 3

What cell types give rise to non-Hodgkin

lymphoma?

Answer 3

B cells: 85%

T cells: 15%

Question 4

What is the typical age distribution of

non-Hodgkin lymphoma?

Answer 4

Incidence increases with age.

Question 5

According the National Cancer Institute, what are
the most common types of non-Hodgkin
lymphoma of childhood?

Answer 5

Burkitt [t(8:14)] and Burkitt-like lymphomas
Lymphoblastic lymphoma
Diffuse large B-cell lymphoma
Other (anaplastic large cell lymphoma)

Question 6

How do pediatric lymphomas most commonly

manifest?

Answer 6

● Rapid enlargement of extranodal tissue (e.g., Waldeyer
ring) or nodal enlargement (persisting > 4 to 6 weeks,
generally > 2 cm in diameter; nodes > 1 cm should be
considered suspicious) that may or may not be painful
● Mass effect is dependent on location (e.g., nasal
obstruction, dysphagia, airway compromise, superior
vena cava syndrome).
● Hepatosplenomegaly
● Central nervous system (CNS) involvement (cranial nerve
palsies, mental status changes, rarely with seizures)

Question 7

What are the two symptom classes used for

staging lymphomas?

Answer 7

Class A: Asymptomatic (better prognosis)

Class B: Weight loss, fever, night sweats (poorer prognosis)

Question 8

Most non-Hodgkin lymphomas in the pediatric
population arise in the abdomen; only 5 to 10%
are located in the head and neck. What are the
most common subsites in the head and neck?

Answer 8

Salivary glands, larynx, Waldeyer ring, paranasal sinuses,

orbit, and scalp

Question 9

Non-Hodgkin lymphoma workup includes a careful
history and physical examination; imaging such as
CT and gallium-67 scanning; and laboratory workup
including cerebrospinal fluid analysis, urinalysis, CBC,
and serum chemistries. However, definitive diagnosis
rests on what key step?

Answer 9

Biopsy or tissue specimen (excisional biopsy) for histology,

cytology, immunohistochemistry and genotyping

Question 10

What infectious agent has been shown to be
associated with both endemic (85%) and sporadic
(15%) cases of Burkitt lymphoma?

Answer 10

Epstein-Barr virus

Question 11

What chromosomal translocation is commonly

noted in Burkitt lymphoma?

Answer 11

t(8;14)(q24;q32)

Question 12

The 5-year survival rate in pediatric cases of non-
Hodgkin lymphoma is 80%; this rate varies

depending on what important prognostic factor(s)?

Answer 12

Age: Worse outcome if patient is < 12 months or > 15 years
of age

Site of disease: Worse in mediastinum, with CNS involve-
ment

Tumor burden or lactate dehydrogenase (LDH) levels
Unusual chromosomal abnormalities

Question 13

How is pediatric non-Hodgkin lymphoma clinically

staged?

Answer 13

I Single extranodal tumor or single anatomic area (nodal) excluding the mediastinum or abdomen

II Single extranodal site with regional lymph node involvement; two or more nodal areas on the same side of
the diaphragm; two single extranodal tumors + /- regional node disease on the same side of the diaphragm;
primary GI tract tumor (usually ileocecal + /- involvement of the associated mesenteric nodes)

III Two single extranodal tumors + /- regional node disease on opposite sides of the diaphragm; two or more
nodal areas on opposite sides of the diaphragm; all extensive primary intra-abdominal disease; unresectable;
all primary intrathoracic tumors (mediastinal, pleural, thymic); all primary paraspinal or epidural tumors
regardless of other sites

IV Any initial CNS or bone marrow involvement (< 25%)

Question 14

What is the mainstay of management for non-

Hodgkin lymphoma?

Answer 14

Chemotherapy: CHOP (cyclophosphamide, doxorubicin
[hydroxydaunorubicin, Adriamycin], vincristine (Oncovin),
prednisone (or prednisolone)
Radiation can be used for localized disease or emergencies

involving respiratory, nervous system, or vascular compro-
mise.

Question 15

What age group(s) is(are) most affected by

Hodgkin lymphoma?

Answer 15

Bimodal distribution: Teenage adolescents and middle-age

adults

Question 16

Which sex confers a higher risk for Hodgkin

lymphoma?

Answer 16

Male, 2:1

Question 17

What causative agent is commonly associated

with Hodgkin lymphoma?

Answer 17

Epstein-Barr virus

Question 18

What tumors are most commonly associated with

Epstein-Barr virus infection?

Answer 18

Burkitt lymphoma (non-Hodgkin lymphoma)
Hodgkin lymphoma
Nasopharyngeal carcinoma

Question 19

How does Hodgkin lymphoma typically manifest?

Answer 19

About 80% of patients have cervical lymphadenopathy and ~
40% with class B symptoms (night sweats, fever, weight loss).
It can present with respiratory distress or superior vena cava
syndrome resulting from mediastinal involvement.

Question 20

What is the pathognomonic histopathology

found in Hodgkin lymphoma?

Answer 20

Reed-Sternberg cells (“owl-eyes” = two or more nuclei with
two or more large nucleoli)
Eosinophilic inclusions
Large, clonal, multinucleated cells
B cell in origin, derived from germinal centers.

Question 21

What is the histologic classification used for

Hodgkin lymphoma?

Answer 21

The World Health Organization (WHO) 2008 Classification:
1. Nodular lymphocytic predominant
2. “Classic” Hodgkin lymphoma: Rye modification of the
Lukes-Butler classification:
● Lymphocytic predominance (< 10%): Typically manifests
with localized disease; high concentration of normal-
appearing lymphocytes, uncommon Reed-Sternberg cells, and no fibrosis; better prognosis
● Mixed cellularity (20 to 40%): Typically manifests with extranodal involvement; mixed infiltrate; Reed-Stern-
berg cells more common

● Lymphocytic depletion (< 15%): Common in HIV pa-
tients; diffuse fibrosis, few lymphocytes, bizarre Reed-
Sternberg cells; poor prognosis

● Nodular sclerosis (40 to 80%): Most common; lymphoid
nodules separated by bands of collagen containing the
Reed-Sternberg cells (lacunar cell variant); more
commonly affects females than males

Question 22

What is the clinical classification system used to

stage Hodgkin lymphoma?

Answer 22

Ann Arbor Staging:
● Stage I: Single lymph node region or single extralymphatic
organ or site (IE)
● Stage II: Two or more lymph node regions on the same
side of the diaphragm or localized involvement of an
extralymphatic organ or site (IIE) in addition to one or
more lymph node region on the same side of the
diaphragm
● Stage III: Involvement of lymph node regions on both
sides of the diaphragm, with or without involvement of
an extralymphatic organ or site (IIIE), involvement of the
spleen (IIIS), or both
● Stage IV: Diffuse or disseminated involvement of one or
more extralymphatic organs or sites, with or without
associated lymph node involvement

Question 23

In addition to a thorough physical examination,
what radiographic studies are important for the
workup of a patient with suspected Hodgkin
lymphoma?

Answer 23

Chest X-ray
Chest and abdominal CT scan
PET/CT

Question 24

What is the overall 5-year survival rate for patients

with Hodgkin lymphoma?

Answer 24

~ 80%

Question 25

What prognostic factors are important in Hodgkin

lymphoma?

Answer 25

Better: Lymphocytic predominance types
Worse: The presence of Reed-Sternberg cells, higher Ann
Arbor stage, presence of class B symptoms

Question 26

What is the management strategy for primary

treatment of Hodgkin lymphoma?

Answer 26

Chemotherapy: MOPP (mechlorethamine, Oncovin [vin-
cristine], prednisone, procarbazine); ABVD (Adriamycin

[doxorubicin], bleomycin, vinblastine, dacarbazine), fol-
lowed by targeted radiation therapy.

Some authorities suggest single-modality radiation therapy
for early disease.