Pedi Lymphomas Flashcards
What is the most common lymphoproliferative
malignancy of the head and neck in pediatric
patients?
Lymphoma
What are common risk factors for lymphoma?
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)
What cell types give rise to non-Hodgkin
lymphoma?
B cells: 85%
T cells: 15%
What is the typical age distribution of
non-Hodgkin lymphoma?
Incidence increases with age.
According the National Cancer Institute, what are
the most common types of non-Hodgkin
lymphoma of childhood?
Burkitt [t(8:14)] and Burkitt-like lymphomas
Lymphoblastic lymphoma
Diffuse large B-cell lymphoma
Other (anaplastic large cell lymphoma)
How do pediatric lymphomas most commonly
manifest?
● 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)
What are the two symptom classes used for
staging lymphomas?
Class A: Asymptomatic (better prognosis)
Class B: Weight loss, fever, night sweats (poorer prognosis)
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?
Salivary glands, larynx, Waldeyer ring, paranasal sinuses,
orbit, and scalp
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?
Biopsy or tissue specimen (excisional biopsy) for histology,
cytology, immunohistochemistry and genotyping
What infectious agent has been shown to be
associated with both endemic (85%) and sporadic
(15%) cases of Burkitt lymphoma?
Epstein-Barr virus
What chromosomal translocation is commonly
noted in Burkitt lymphoma?
t(8;14)(q24;q32)
The 5-year survival rate in pediatric cases of non-
Hodgkin lymphoma is 80%; this rate varies
depending on what important prognostic factor(s)?
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
How is pediatric non-Hodgkin lymphoma clinically
staged?
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%)
What is the mainstay of management for non-
Hodgkin lymphoma?
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.
What age group(s) is(are) most affected by
Hodgkin lymphoma?
Bimodal distribution: Teenage adolescents and middle-age
adults
Which sex confers a higher risk for Hodgkin
lymphoma?
Male, 2:1
What causative agent is commonly associated
with Hodgkin lymphoma?
Epstein-Barr virus
What tumors are most commonly associated with
Epstein-Barr virus infection?
Burkitt lymphoma (non-Hodgkin lymphoma)
Hodgkin lymphoma
Nasopharyngeal carcinoma
How does Hodgkin lymphoma typically manifest?
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.
What is the pathognomonic histopathology
found in Hodgkin lymphoma?
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.
What is the histologic classification used for
Hodgkin lymphoma?
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
What is the clinical classification system used to
stage Hodgkin lymphoma?
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
In addition to a thorough physical examination,
what radiographic studies are important for the
workup of a patient with suspected Hodgkin
lymphoma?
Chest X-ray
Chest and abdominal CT scan
PET/CT
What is the overall 5-year survival rate for patients
with Hodgkin lymphoma?
~ 80%
What prognostic factors are important in Hodgkin
lymphoma?
Better: Lymphocytic predominance types Worse: The presence of Reed-Sternberg cells, higher Ann Arbor stage, presence of class B symptoms
What is the management strategy for primary
treatment of Hodgkin lymphoma?
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.
