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1580 Part XI: Malignant Lymphoid Diseases Chapter 95: General Considerations for Lymphomas 1581

(e.g., R-CHOP) typically do not penetrate the eye or brain, rendering require excisional or incisional biopsy. They may be associated with
these regimens ineffective. In recent years, many neuro-oncologists have pleural effusions and involvement of neighboring ribs. 176
advised treating intraocular lymphomas similarly to primary DLBCL Primary endobronchial lymphoma is a rare occurrence and may
of the brain with high-dose methotrexate based-chemotherapy with or follow lung transplantation. It can lead to airway obstruction as an early
without intrathecal treatment and/or whole-brain and eye radiother- sign.
apy. A large study conducted at 17 European centers has not confirmed
the expected efficacy of this aggressive approach for vitreoretinal
lymphoma, however. 158 HEART
Primary cardiac lymphoma may involve the heart or pericardium or
PARANASAL SINUSES both. Patients may present with dyspnea, edema, arrhythmia, or peri-
Localized NHL involving the nasal cavity and/or paranasal sinuses cardial effusion. The effusion may result in cardiac tamponade. Lym-
phomatous masses may be found in the right atrium (most common),
may be DLBCL, T-cell lymphoma, or NK/T-cell lymphoma. 74,162–168 pericardium, right ventricle, left atrium, or left ventricle. Most cases are
The nasal cavity is the predominant site of involvement in T-cell and a B-cell lymphoma; less than 5 percent are of T-cell lineage. 177–179
NK/T-cell lymphoma, whereas sinus involvement without nasal dis-
ease is common in B-cell lymphoma. Systemic B symptoms are more
frequently observed in NK/T-cell lymphoma. Based on in situ hybrid- GASTROINTESTINAL TRACT
ization studies, there is a strong association of EBV with NK/T-cell Gastrointestinal lymphoma is the most common form of extranodal
lymphoma. These lymphomas may involve the frontal, maxillary, eth- lymphoma, accounting for one-third of cases. The most commonly
180
moid, and sphenoid sinuses and typically involve bone. They present involved site is the stomach, followed by the small bowel, ileum, cecum,
with local pain, upper airway obstruction, rhinorrhea, facial swelling, colon, and rectum. Lymphoma of the stomach typically causes dyspep-
or epistaxis. They may extend into the periorbital area causing propto- tic symptoms and sometimes anorexia or early satiety. Hemorrhage
sis, visual loss, or diplopia. These lymphomas typically are DLBCL in is unusual but if present suggests a high-grade lymphoma. Diagnosis
the United States and Western Europe and more often T- and NK-cell typically is made by endoscopic biopsy. 180,181 H. pylori infection has
lymphomas in Asia. Nasal NK/T lymphomas are typically treated with been implicated in the pathogenesis of MALT gastric lymphoma. At
88
combination chemotherapy including l-asparaginase plus radiother- endoscopy, mild to severe gastritis is common. Multiple biopsies are
apy. 162,169 In contrast, patients with primary sinus lymphoma usually important to obtaining adequate material to determine the presence of
have DLBCL. A series of 80 patients with primary sinonasal DLBCL H. pylori. MALT lymphoma is common, but DLBCL also may arise
treated with R-CHOP chemotherapy demonstrated a long-term pro- de novo or may be found in the background of a MALT lymphoma.
181
gression-free survival rate of 50 to 60 percent, with only a single patient If both subtypes of lymphoma are present, the treatment should be
experiencing CNS relapse. 170
directed at the large B-cell lymphoma.
In the bowel, the small intestine, rectum, and colon may be
SKIN involved, in that order of frequency. 88,182 The intestinal location most
The three main types of cutaneous B-cell lymphomas are primary cuta- often involved is the ileocecal region followed by small bowel, large
182
neous marginal zone B-cell lymphoma, primary cutaneous follicular bowel, and multiple intestinal sites. Primary esophageal lymphoma
183
center lymphoma, and primary cutaneous large B-cell lymphoma (leg is rare. Primary colonic lymphoma is associated with symptoms of
type) as defined by the WHO–European Organization for Research and diarrhea, lower gastrointestinal bleeding, and nausea and vomiting sec-
Treatment of Cancer. Primary cutaneous marginal zone B-cell and ondary to low-grade obstruction. The most common disease location is
171
primary cutaneous follicle center lymphoma are indolent types with an the cecum, followed by the right colon, and the sigmoid colon. 184
excellent prognosis that should be treated primarily with nonaggressive Rare cases of lymphoma may be confined to the liver. Right
therapies, including simple excision, glucocorticoid injections or local upper quadrant pain is the most common symptom. In about half of
radiotherapy. Primary cutaneous large B-cell lymphoma (leg type) is a cases there is a history of previous inflammatory liver disease, such as
diffuse dermal infiltrate of neoplastic B cells with extension to both the hepatitis C. 185–188
papillary dermis and the subcutaneous fat. 172,173 It typically presents as a Primary extranodal lymphoma of the pancreas may present with
solitary soft-tissue mass and mimics a soft-tissue sarcoma until biopsy abdominal pain, nausea, vomiting, obstructive jaundice, and weight
clarifies the diagnosis. It is an aggressive lymphoma that should be loss, and very rarely signs of pancreatitis. 189–191
treated primarily with aggressive chemotherapy (Chap. 98). Chap. 103 The gallbladder may be the site of primary extranodal lymphoma
discusses classical T-cell cutaneous lymphomas, particularly mycosis and may present with right upper quadrant pain or other symptoms and
fungoides. signs consistent with cholecystitis. 192,193 It may extend into the bile ducts
with jaundice and other signs of bile duct obstruction. 194
CHEST AND LUNG
Primary pulmonary lymphoma may present as a pulmonary nodule GENITOURINARY
or mass and may be associated with hilar lymph node enlargement. Testicular
The histopathology is usually marginal zone B-cell lymphoma of the Primary lymphoma of the testes typically presents as a painless enlarge-
mucosa-associated lymphoid tissue or DLBCL, though lymphomatoid ment of the testis in an older man (Fig. 95–5). A hydrocele may also
granulomatosis may also present in this fashion. 174,175 Lung biopsy is be present. 195–198 The histologic type usually is a DLBCL. At presenta-
usually required to make a definitive diagnosis. Pleural effusions may tion, two-thirds of cases are localized to the testicle or to the testicle and
occur as a result of either central lymphatic obstruction or pleural pelvic or abdominal lymph nodes. After orchiectomy has established
seeding. the diagnosis, patients are staged with a special focus on the remain-
Primary chest wall lymphoma may present as local pain or may ing testicle. If sonography of the remaining testicle demonstrates a solid
be accompanied by fever, sweating, and dyspnea. These masses usually mass, it should be assumed to be lymphoma. Patients presenting with

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