Devita, Hellman, and Rosenberg's Cancer

LWBK1006-30 LWW-Govindan-Review December 12, 2011 19:35

Chapter 30•Lymphomas 419

Question 30.27. Primary mediastinal (thymic) large B-cell lymphomas (PMBCLs) are char-

acterized by all of the following, EXCEPT:

A. Involve the thymus. Tumor cells correspond to B cells in the thymic

medulla and are Ig negative but express B-cell–associated antigens

CD19, CD20, CD22, CD79a, and CD45. Most cases are BCL6 posi-

tive and Ig heavy, and light-chain genes are rearranged, whereas BCL2

and BCL6 are germ line.

B. Express FIG1 and amplified REL oncogene on chromosome 9p and

overexpress the MAL gene at chromosome 2p13-15.

C. GEP of PMBCL is similar to DLCBL with overexpression of the MAL

and FIG1 genes.

D. OS at 5 years in patients aged less than 65 years treated with MACOP-

B/VACOP-B, R-CHOP, and CHOP is 87%, 81%, and 71%, respec-

tively (Vancouver series). Addition of RT did not improve survival in

this series.

Question 30.28. Which statement is FALSE about natural killer (NK)/T-cell neoplasms?

A. Extranodal NK/T-cell lymphoma is EBV+, is common in Asia and

Peru, and can affect children and adults. Hemophagocytic syndrome

may occur.

B. Extranodal sites involve the nose, palate, upper airway, gastrointesti-

nal tract, and skin.

C. Atypical cells are CD2+, CD56+, surface CD3–, cytoplasmic CD3+,

and many are CD4– CD8– and express cytoplasmic granule proteins

(granzyme B and TIA-1).

D. Relapse after RT alone is rare. Patients with disseminated disease are

cured by CHOP therapy.

Question 30.29. Which statement is FALSE about T-cell neoplasms?

A. Enteropathy-type T-cell lymphoma occurs in adults with gluten-

sensitive enteropathy (antigliadin antibodies). Outcomes in patients

with advanced celiac disease are very poor.

B. Anaplastic large T/null cell lymphoma expresses ALK-1, CD30, and

usually CD25; therapy with DLCBL active regimen without Rituxan

is used; the outcome of ALK– patients is worse (31% to 37%) than

that of ALK+patients (71% to 95%). Responses have been seen with

CD30-directed monoclonal antibodies.

C. Subcutaneous panniculitis T-cell lymphoma is a cytotoxic T-cell neo-

plasm that infiltrates subcutaneous tissues. Hemophagocytic syn-

drome is common with fever and serositis, myalgias, and joint pains.

Disease prognosis is very good with chemotherapy.

D. Hepatosplenic T-cell lymphoma is rare, and diagnosis is often dif-

ficult. Patients present with organomegaly and sinusoidal infiltrates

in the liver, spleen, or marrow. It is CD2+CD3+CD5–, CD4–CD8–

CD16+CD56+/–. TCRg and TCRd genes are rearranged; isochro-

mosome 7 is common and trisomy 8 may be seen.