Abdomen

The primary site in 35% of cases is abdominal—the ileocecal region, appendix, ascending colon, or some combination of these sites. These patients usually present with:

• Abdominal pain

• Nausea and vomiting

• Constipation or diarrhea

• Abdominal distention

• Palpable mass

• Intussusception

• Peritonitis

• Acute gastrointestinal bleeding

• Obstructive jaundice

• Hepatosplenomegaly

• Right iliac fossa mass.

Table 16-2. Histologic Classification, Relative Frequency of Histologic Type, Correlation with Immunophenotype, Common Sites of Involvement and Genetic Features in Childhood Non-Hodgkin Lymphoma

Histology

Immunophenotype

Common Sites of Involvement

Frequency

Genetic Features

Lymphoblastic lymphoma (LBL): 4 Convoluted 4 Non-convoluted

Precursor T (90% of LBL)

Precursor-B (10% of LBL)

Small non-cleaved cell lymphomas (diffuse undifferentiated):

Burkitt lymphoma (BL) Burkitt-like (BLL)

HLA-DR ,TdT positive CD 10, 19, 20, 22, HLA-DR, TdT positive

B-cell SIgM, (SIgG, SIgA occasionally)

77, 79a TdT negative

Large cell Lymphomas Subtypes

Diffuse large cell: B; T

Anterior mediastinum (50-70%), 30-35%

pleural effusion, sometimes cervical, axillary, inguinal lymphadenopathy. Occasional abdominal involvement.

Abdomen with or without ascites 40-50%

(90%). Most frequent pathology causing intussusception in children >6 years of age.

Endemic BL: jaw

Usually abdomen but may present 15-20%

with unusual sites of involvement e.g., mediastinum.

t(10;14), t(11;14), t(1;14), TCR/TALI, t(1;19), t(8;14), (q24,q11), c-myc /TCRa TCR/RHOMB2, TCR/LCK

t(8;14), c-myc/ IgH t(8;22), c-myc/ Ig Lambda t(2;8), Ig Kappa/c-myc P53 mutation BLL:

bcl-2 rearrangement 30%, bcl-6 rearrangement

Anaplastic large cell: usually T, CD 30 (Ki-1), 25, 71 Ia

NPM/ALK sometimes

B-lineage.

Often skin, CNS, lymph nodes, lung, testes, muscle, GI tract, bone t(2;5) (p23;q35), t(1;5)

Synonyms in various classifications:

1. ML lymphoblastic synonyms: WHO/REAL: precursor T-cell neoplasm: precursor T-lymphoblastic lymphoma/leukemia (T-LBL); Rappaport: poorly differentiated lymphocytic; Kiel: T-lymphoblastic; Lukes-Collins: convoluted T-lymphocytic; working formulation: lymphoblastic, convoluted, or nonconvoluted.

2. ML small noncleaved Burkitt synonyms: WHO: Burkitt lymphoma/Burkitt cell leukemia, typical Burkitt; subtype of mature B-cell neoplasms; REAL: Burkitt lymphoma subtype of peripheral B-cell lymphoma; Rappaport: undifferentiated lymphoma, Burkitt type; Kiel: Burkitt lymphoma; Lukes-Collins: small noncleaved follicular center cell; working formulation: small noncleaved cell, Burkitt type.

3. ML small noncleaved Burkitt-like synonyms: WHO: Burkitt lymphoma/Burkitt cell leukemia, Burkitt-like variant; REAL: a subtype of peripheral B-cell neoplasms assigned as a provisional entity of high-grade B-cell lymphoma Burkitt-type: Rappaport: undifferentiated non-Burkitt; Kiel: not listed; Lukes-Collins: small non-cleaved follicular center cell; working formulation: small noncleaved cell, non-Burkitt.

4. ML large-cell synonyms: WHO/REAL: diffuse large-B-cell lymphoma, a subtype of peripheral B-cell neoplasm (multiple variants); Rappaport: diffuse histiocytic; Kiel: centroblastic, B-immunoblastic, large-cell anaplastic B-cell; Lukes-Collins: large-cell cleaved, noncleaved, or immunoblastic; working formulation: large-cell lymphoma.

5. Anaplastic large-cell lymphoma: WHO: anaplastic large-cell lymphoma (T/null), a subtype of mature T-cell neoplasms, predominantly nodal and predominantly extranodal; REAL: anaplastic large-cell (CD30+) lymphoma (T/null), a subtype of peripheral T-cell and NK cell neoplasm; Rappaport: not listed; Lukes-Collins: T-immunoblastic sarcoma; working formulation; not listed.

Abbreviation: ML, malignant lymphoma.

Table 16-3. Epidemiologic, Immunologic, and Molecular Features of Endemic and Sporadic Burkitt Lymphoma

Feature Endemic Sporadic

Epidemiologic

Population affected Age affected Organ involvement

Epstein-Barr virus CNS involvement

Immunologic IgM secretion Fc and C3 CALLA

IgH gene rearrangement Molecular

Breakpoints on chromosome 8 Site of cell origin

African

Children (peak age 7 years) Jaw, orbit, paraspinal, abdomen, ovary Present in >97% of cases More frequent than bone marrow involvement

Little or none

DH or JH

Upstream of c-myc

Germinal center of lymph node

Worldwide

Young adults (peak age: 11 years) Abdomen, marrow, nasopharynx, ovary Present in 15-20% of cases Less frequent than bone marrow involvement

Prominent

IgH switch region Within c-myc Bone marrow

Bleeding and perforation of the intestine occur infrequently in patients with Burkitt lymphoma.

Lymphoma is the most frequent anatomic lesion causing intussusception in children over 6 years of age. When this disease presents insidiously, it may clinically and radiologically resemble Crohn disease.

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