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1578 Part XI: Malignant Lymphoid Diseases Chapter 95: General Considerations for Lymphomas 1579

TABLE 95–5. The Deauville 5-Point Scale for Assessment a solitary mass lesion anywhere. The histopathology of primary extra-
nodal lymphoma is usually either marginal zone lymphoma of MALT
of Positron Emission Tomography/Computed Tomography or DLBCL. Follicular lymphoma and several other histologic subtypes
Imaging in Lymphoma Patients 16 of lymphoma may also occur. Therapy usually involves a combination
Deauville FDG Uptake* of multidrug chemotherapy and a lymphocyte-directed monoclonal
Score antibody, such as rituximab-cyclophosphamide, hydroxydoxorubicin,
1 No significant FDG uptake in tumor site(s) above vincristine (Oncovin), and prednisone (R-CHOP). Selection of the best
background. regimen depends on the histopathologic subtype of lymphoma and
the location of the disease. Radiotherapy is used less commonly in the
2 FDG uptake in tumor site(s) less than that in the medi-
astinal blood pool management of lymphoma than in the past because of concerns about
induction of secondary malignancies and delayed cardiopulmonary
3 FDG uptake in tumor site(s) greater than the mediasti- toxicities, although it still has a role in treatment of localized, stages I
num but less than the liver
to II lymphomas, and for consolidation of bulky adenopathy (>10 cm)
4 FDG uptake in tumor site(s) moderately higher than in selected settings.
†
in the liver An unanswered pathogenetic question concerning primary extra-
5 FDG uptake in tumor site(s) markedly higher than nodal lymphoma is the propensity for both sites of paired organs (e.g.,
†
that in the liver and/or new FDG-avid lesions likely to ovaries, testicles, breasts, ocular adnexa, adrenal glands, kidneys, and
be lymphoma ureters) to be affected simultaneously. It is also curious that several
X New areas of uptake unlikely to be related to lymphoma of these sites (e.g., kidney) are normally devoid of significant accu-
mulations of lymphatic tissue. If the transformed lymphocyte arises
FDG, 2-fluorodeoxyglucose. outside these tissues, it must have a tropism for both paired organs,
*The Deauville 5-point scale scores the most intense uptake in a site perhaps because of expression of site-specific adhesion molecules or
of initial disease. addressins. 147
† It has been recommended that the Deauville score of 4 be applied to
uptake in tumor site(s) that is less than twice as high as the maximum CENTRAL NERVOUS SYSTEM
standard uptake value (SUV) in a large region of normal liver whereas Primary lymphomas originating in and confined to the leptomenin-
the score of 5 be used if the tumor uptake is more than twice the 148 149–151 152
maximum SUV in the liver. ges, brain, or spinal cord are uncommon. They almost always
are of an aggressive histologic subtype, usually DLBCL. 151,153 Spinal cord
compression typically presents with back pain, followed by extremity
weakness, paresis, and paralysis. Leptomeningeal spread may present
is on a clinical trial or not, and by the clinical setting (e.g., initial vs.
relapsed/refractory disease; complete response or not). For curable his- with cranial nerve palsies and signs of meningeal irritation, for example,
tologies such as HL and DLBCL, the likelihood of relapse decreases over headache and stiff neck. Intracerebral mass lesions may present with
time and visits are reduced from every 3 months during the first 2 years, headaches, lethargy, papilledema, focal neurologic signs, or seizures.
to every 6 months for the next 3 years, and then annually thereafter. Intracerebral lymphoma increased dramatically after the onset of the
Incurable histologies are observed every 3 to 6 months, determined by human immunodeficiency virus epidemic as a result of the association
pretreatment risk factors, whether the patient is being managed con- with AIDS-related aggressive lymphomas (Chap. 81). The incidence of
servatively, and whether treatment has achieved a complete remission intracerebral lymphoma has slowed in AIDS patients because of more
or not. At each visit, a history, physical examination, CBC, metabolic successful antiviral therapy. Primary pituitary (or hypothalamic) extra-
panel, and serum lactate dehydrogenase are performed. The role, if any, nodal lymphoma may result in hypopituitarism. Diabetes insipidus or
of surveillance radiographic imaging for patients in remission is con- anterior pituitary failure may occur. The lesion may invade the sella tur-
154–156
troversial. All groups strongly discourage surveillance monitoring with cica or other neighboring bone and nervous tissue.
PET/CT imaging for patients in remission because of the high rate of
false-positive findings in this setting, which lead to unnecessary anxiety, EYE
expense, and biopsy procedures. 15–17,33 The Lugano IWG guidelines also Ophthalmic lymphoma, the most common orbital malignancy, includes
discourage surveillance CT imaging for patients with HL and DLBCL in lymphoma localized to the eyelid, conjunctiva, lacrimal sac, lacri-
complete remission at the end of therapy. In contrast, NCCN guidelines mal gland, orbit, or intraocular space. 157–159 This location accounts for
recommend that contrast-enhanced CT imaging be performed no more approximately 7 percent of all extranodal lymphomas. The most fre-
160
frequently than every 6 months for 2 years after the end of therapy for quent subtype is extranodal marginal zone lymphoma of MALT. Bilat-
DLBCL and HL, and then be discontinued. eral involvement occurs in 10 percent of cases. The most common site of
ocular lesions is the periorbital soft tissues, particularly the conjunctival
PRIMARY EXTRANODAL LYMPHOMA mucosal surfaces and the area surrounding the lacrimal gland. These
lesions typically have a low-risk of progression and commonly have the
Lymphomas involving extranodal sites most commonly occur simulta- histology of a marginal zone lymphoma of MALT or follicular center
neously with nodal involvement, either at the time of diagnosis or dur- cell lymphoma and may be associated with C. psittaci (see “Infectious
ing the course of the disease. Extranodal involvement that occurs as the Agents” above). In a Danish study, approximately 50 percent of orbital
only initial evidence of lymphoma after staging procedures is referred and ocular adnexal lymphomas were of the marginal zone lymphoma of
to as primary extranodal lymphoma. The presence of a tumor or mass MALT subtype; DLBCL was the most common intraocularly. 91,160 Lym-
outside of the lymph nodes is usually not considered lymphoma until a phoma arising in the lacrimal sac was usually DLBCL. There has been
biopsy is done and the histopathology establishes the diagnosis. On the a striking increase in incidence rates for lymphoma of the eye over the
other hand, solitary extranodal lymphomas can occur in virtually any past 30 years. 24,157–159,161 Patients with marginal zone lymphoma of MALT
organ or tissue and should be considered in the differential diagnosis of of the ocular orbit may relapse or have progression of disease after

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