04 - Radiol Clin N Am 2007 - Hodgkin's and Non-Hodgkin's Lymphomas.pdf - książki - Bio Med 21 - bibiariel

RADIOLOGIC

CLINICS

OF NORTH AMERICA

Radiol Clin N Am 45 (2007) 69–83

Hodgkin’s and Non-Hodgkin’s

Lymphomas

J¨ rgen Rademaker, MD

- Clinical and radiologic features

Hodgkin’s disease

Non-Hodgkin’s lymphoma

Central nervous system lymphoma

Childhood lymphoma

- Staging

- Assessment of treatment response

- Summary

- References

Lymphomas represent about 4% of the new cases

of cancer diagnosed in the United States every year,

making them the ﬁfth most common type of cancer

and the ﬁfth leading cause of cancer death.

Lymphoma can affect any region of the body.

Most patients present with lymphadenopathy, focal

or diffuse organ involvement, or generalized multi-

organ involvement.

During the past 20 years, advances in radiation

and chemotherapy increased the cure rate, and

now more than 80% of all patients younger than

60 years with newly diagnosed HD are likely to be

cured [5] . Selection of the appropriate therapy is

based on accurate staging. Patients with early stage

disease are treated with abbreviated courses of

chemotherapy followed by involved ﬁeld radiation

therapy, whereas those with advanced-stage disease

receive a longer course of chemotherapy without

radiation therapy. Stage B symptoms and bulky

disease (>10 cm) remain the most important prog-

nostic factors in HD.

More than 80% of patients who have HD present

with lymphadenopathy above the diaphragm that

commonly involves anterior and middle mediasti-

nal nodes with or without disease of the hila

( Figs. 1 and 2 ) [6] . Large mediastinal masses may

invade the chest wall and pericardium directly.

Hilar adenopathy is uncommon without detectable

mediastinal disease. Pleural effusions are often

caused by lymphatic or venous obstruction. Sec-

ondary involvement of the thyroid or parotid gland

is not uncommon, whereas primary involvement is

rather rare ( Fig. 3 ).

Pulmonary involvement is found more often in

HD than in non-Hodgkin’s lymphoma (NHL).

Clinical and radiologic features

Hodgkin’s disease

The incidence of Hodgkin’s disease (HD) is about 4

per 100,000 persons per year, and accounts for less

than 1% of all cancers worldwide. New cases of HD

in the United States were estimated at 7880 and

accounted for 14% of lymphomas [1–3] . HD has

a bimodal incidence curve and occurs more

frequently in two separate age groups, the ﬁrst being

young adulthood, the second being in those over

50 years old. About one third of people with HD

have systemic symptoms, such as low-grade fever,

night sweats, weight loss, pruritus, or fatigue [4] .

People with a history of infectious mononucleosis

have a threefold increased likelihood of developing

HD.

Department of Radiology, Memorial Sloan-Kettering Cancer Center, Cornell University, Weill Medical

College, 1275 York Avenue, New York, NY 10021, USA

E-mail address: rademakj@mskcc.org

doi:10.1016/j.rcl.2006.10.006

radiologic.theclinics.com

Rademaker

Fig. 1. HD. Imaging ﬁndings included axillary (arrow)

and mediastinal lymphadenopathy (arrowhead).

Nodules (with or without cavitation), reticular nod-

ular inﬁltrate, ground glass opacities, or irregular

consolidations are possible appearances [7,8] .

There might be atelectasis secondary to endobron-

chial or nodal obstruction. Many cases present

with hilar lymphadenopathy or mediastinal masses

extending into the adjacent lung. Pulmonary in-

volvement at presentation without associated

mediastinal or hilar lymphadenopathy is unlikely

[9,10] ; however, recurrences in the lung may be

seen without associated lymphadenopathy.

HD also may affect extranodal tissue by direct

invasion or by hematogenous dissemination. The

most commonly involved extranodal sides are

spleen, lung, liver, and bone marrow. Enlargement

of the spleen is not a reliable predictor of disease,

although marked enlargement in the setting of

other sites of involvement makes splenic lym-

phoma involvement likely. One third of patients

with splenic involvement have normal size spleen,

and in one third of those with splenomegaly, the

spleen is not involved by tumor. HD with involve-

ment of the kidneys or the gastrointestinal (GI)

tract is uncommon. Primary bone lesions are rare,

Fig. 3. HD with involvement of the parotid gland. The

T2 weighted axial MR imaging scan demonstrates a

2 cm mass (arrow) in the superﬁcial aspect of the

left parotid gland. The mass enhances after adminis-

tration of gadolinium (not pictured).

but marrow involvement in cases with advanced

disease is not uncommon. Lymph nodes or masses

may reveal calciﬁcations after treatment. Calciﬁca-

tions before treatment suggest aggressive disease,

such as the nodular sclerosing subtype of HD [11] .

Non-Hodgkin’s lymphoma

NHL is a heterogenous group of diseases of either B-

cell or T-cell origin. NHL represents the second fast-

est growing cancer in the United States, and the

most commonly occurring hematologic cancer.

The incidence of NHL in the United States has in-

creased by 50% over the past 15 years, and the cause

for this increase is not known. In 2004, new cases of

NHL in the United States were estimated at 54,370,

resulting in 4% of all cancer deaths.

Overall, NHL has a worse prognosis than HD.

Unlike HD, which commonly spreads through con-

tiguous groups of lymph nodes, NHL is infre-

quently localized at the time of diagnosis and

frequently involves extranodal sites of disease.

For decades, NHL was classiﬁed by morphology

and histology. In 1994, the Revised European-Amer-

ican Lymphoma (REAL) classiﬁcation introduced

additional immunophenotypic, genetic, and clini-

cal characteristics. This system was modiﬁed further

for the currently accepted classiﬁcation introduced

by the World Health Organization (WHO) [12] .

The WHO classiﬁcation differentiates between B

lymphoid neoplasms (including follicular lym-

phoma and diffuse large B-cell lymphoma) and

T- and NK lymphoid neoplasms (including anaplas-

tic large-cell lymphoma) [13] . NHL is actually a com-

plex group of almost 40 distinct entities, and their

classiﬁcation in the WHO classiﬁcation scheme is

Fig. 2. Large anterior mediastinal mass (M) in HD. The

scan demonstrates the growth pattern of the tumor

with extension into the right anterior pleural space

(arrows). The pericardial effusion is probably from

lymphomatous extension into the pericardium

(arrowhead). Pleural effusions often result from venous

or lymphatic obstruction by enlarged lymph nodes.

Lymphomas 71

summarized in Box 1 . The box is included in this ra-

diologic review to illustrate the spectrum of diseases

summarized under the name lymphoma. The classi-

ﬁcation incorporates histologic ﬁndings, immuno-

phenotype, cytogenetic, and molecular data. Many

diseases are deﬁned better, ultimately helping clini-

cians better understand and treat disease. Most

NHLs develop from B-cells.

Substantial progress has been made in under-

standing the molecular pathogenesis of several

forms of NHL [ 11,14 ]. Several forms of NHL are as-

sociated closely with an infectious agent, which is

not surprising, because most NHLs arise from B-

cells. Burkitt’s lymphoma is associated with Ep-

stein-Barr virus, MALT lymphoma with Helicobacter

pylori [15,16] , and primary effusion lymphoma

with human herpes virus 8, the etiologic agent of

Kaposi’s sarcoma. Molecular translocations also

have been identiﬁed for many forms of NHL and

not only reﬂect pathogenesis, but also serve as

markers for molecular diagnosis and monitoring.

Molecular translocations include translocation t

(11;14) for mantle cell lymphoma and transloca-

tion t (14;18) for follicular lymphoma [1] .

Box 1: World Health Organization classiﬁcation scheme for lymphoma

B-cell lymphoma/leukemias

Chronic lymphocytic leukemia/small lymphocytic lymphoma

B-cell prolymphocytic leukemia

Lymphoplasmacytic lymphoma/Waldenstr ¨ m’s macroglobulinemia

Splenic marginal zone B-cell lymphoma

Hairy cell leukemia

Plasma cell myeloma/plasmocytoma

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT)

Nodal marginal zone B-cell lymphoma

Follicular lymphoma

Mantle cell lymphoma

Diffuse large B-cell lymphoma

Mediastinal (thymic) large B-cell lymphoma

Primary effusion lymphoma

Burkitt’s lymphoma/leukemia

Pre-B-cell lymphoblastic leukemia/lymphoma

T-cell and NK-cell lymphomas/leukemias

T-cell prolymphocytic leukemia

T-cell large granular lymphocytic leukemia

Aggressive NK-cell leukemia

Adult T-cell leukemia/lymphoma (human T lymphotropic virus type 1-positive)

Extranodal NK-cell/T-cell lymphoma, nasal type

Enteropathy type T-cell lymphoma

Hepatosplenic T-cell lymphoma

Subcutaneous panniculitis-like T-cell lymphoma

Blastic NK-cell lymphoma

Mycosis fungoides/S´ zary syndrome

Primary cutaneous CD30-positive T-cell lymphoproliferative disorders

Primary cutaneous anaplastic large cell lymphoma

Lymphomatoid papulosis

Angioimmunoblastic T-cell lymphoma

Peripheral T-cell lymphoma, unspeciﬁed

Anaplastic large cell lymphoma

Pre-T-cell lymphoblastic leukemia/lymphoma

Hodgkin’s lymphoma

Nodular lymphocyte-predominant

Classic Hodgkin’s lymphoma

Nodular sclerosis

Mixed cellularity

Lymphocyte depleted

Lymphocyte rich

Adapted from Harris NL, Jaffe ES, Diebold J, et al. The World Health Organization classiﬁcation of neoplastic diseases

of hematopoietic and lymphoid tissues. Report of the Clinical Advisory Committee meeting, Arlie House, Virginia,

November 1997. Ann Oncol 1999;10:1419–32.

Rademaker

Although there are many forms of NHL, the 13

most common types account for 88% of the cases

in the United States. The most common forms of

NHL in adults in the western world are [4] :

Diffuse large B-cell lymphoma 31%

Follicular lymphoma 22%

Small lymphocytic lymphoma 6%

Mantle cell lymphoma 6%

Peripheral T cell lymphoma 6%

Extranodal marginal zone B-cell lymphoma

of MALT type 5%

Others (including composite lymphomas)

24%

Note that composite lymphoma is deﬁned as si-

multaneous occurrence of two histologically differ-

ent types of lymphoma situated in one location.

NHL can be divided into two groups: indolent

lymphomas, which grow more slowly and have

fewer symptoms (eg, follicular lymphoma, MALT),

and aggressive lymphomas, which grow more

quickly (eg, diffuse large cell lymphoma, Burkitt’s

lymphoma, Mantle cell lymphoma). The follicular

lymphomas are the most common subtype of indo-

lent NHL. Most of these patients present with ad-

vanced disease (stage 3 or 4) and reveal an

indolent clinical course with a median survival of

6 to 10 years. Only 10% to 15% of patients have lim-

ited disease. It remains controversial whether pa-

tients with limited disease can be cured, because

relapses occur even after 10 to 20 years. Diffuse large

B-cell NHL represents an aggressive type, and at least

40% of patients were cured with standard regimens

of chemotherapy and prolonged follow-up [13] .

There is a higher incidence of extranodal disease

and marrow involvement in patients who have

NHL than in patients who have HD. Extranodal

lymphatic tissue like Waldeyer’s ring or spleen

and nonlymphatic organs like liver, GI tract [17–

19] , lung, bone, and CNS often are involved with

NHL. Parotid and thyroid gland ( Fig. 4 ), breast,

bones, testes, or leptomeninges ( Fig. 5 ) may be in-

volved [20–26] . Certain forms of lymphoma like

primary effusion lymphoma/body cavity lym-

phoma, enteropathy-type T-cell lymphoma, or

MALT lymphoma might present with localized

and rather typical imaging ﬁndings [17–19,27] .

Pulmonary involvement and pleural disease may

be seen without mediastinal involvement. Solid

pleural masses are infrequent but may be encoun-

tered with NHL. Isolated chest wall soft tissue

masses are not common and are usually a manifes-

tation of NHL.

Involvement of liver ( Fig. 6 ) or spleen [28]

( Fig. 7 A) may present with focal lesions, and the

imaging features are similar to metastatic disease.

Diffuse involvement of liver and spleen is more

Fig. 4. Diffuse large cell lymphoma with involvement

of the thyroid and spleen (not pictured). Small

amount of regular thyroid tissue (arrowhead) is

depicted within the large neck mass (arrow).

difﬁcult to detect despite advances in imaging

[29] . Enlargement of the liver is suggestive of inﬁl-

tration. Enlargement of the spleen is not a reliable

predictor of disease, although marked enlargement

in the setting of other sites of involvement makes

splenic lymphoma involvement likely.

Small bowel, stomach, or colon often are

involved with NHL [30] , and disease may be

multicentric. The stomach is the most commonly

involved organ in primary and secondary

lymphoma ( Fig. 8 ). Secondary involvement of

the pancreas also is seen often in patients who

have NHL [31] ( Fig. 9 ). Renal involvement with

Fig. 5. Leptomeningeal lymphoma. Sagittal T1

weighted MR scan after administration of gadoli-

nium reveals plaque-like and conﬂuent leptomenin-

geal disease (arrow) along the lower thoracic spinal

cord and conus medullaris.

Lymphomas 73

Fig. 6. Residual mass in large cell lymphoma. The

initial scan from 2003 (A) revealed a large heteroge-

nous low-attenuation mass involving the caudate

lobe extending to the conﬂuence of the hepatic veins

(arrow). Three years after treatment, there is a much

smaller partially calciﬁed residual mass in the apex of

the liver (B, arrow).

Fig. 7. Diffuse large-cell lymphoma with splenic in-

volvement (A, arrow), paragastric lymphadenopathy

(A, arrowhead), retroperitoneal lymphadenopathy

(not pictured), and enhancing bilateral renal masses

(B, small arrow).

NHL may occur as solid masses (see Fig. 7 B),

diffuse inﬁltrations with nephromegaly, or by

invasion of retroperitoneal disease [32] . The

genitourinary tract is very rarely involved at

presentation. Retroperitoneal and mesenteric

disease [33] may present with subtle ﬁndings

mimicking peritoneal carcinomatosis, with

enlarged lymph nodes, or with conglomerate

masses ( Figs. 10–12 ).

There is an increased incidence of NHL in immu-

nocompromised patients, such as patients with

AIDS or patients receiving immunosuppressive

medications for an organ transplant [34–36] . Extra-

nodal sites (GI tract, central nervous system [CNS],

Fig. 8. Gastric NHL (MALT lym-

phoma). (A) Stomach with

a large lesser curvature mass (ar-

rows) and circumferential thick-

ening of the gastric wall

(arrowheads). (B) Positron emis-

sion tomography (PET) with F18-

FDG usually does not visualize

extranodal B-cell lymphoma

of the mucosa-associated

lymphoid tissue (MALT)-type.

This is a rare case of a

FDG-avid MALT lymphoma

(SUV 17.5) (different patient

than patient in A).

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