Clinical Manifestations and Treatment of Marginal Zone Lymphomas (Extranodal/Malt, Splenic, And Nodal)

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Chapter 33 Clinical Manifestations and Treatment of Marginal Zone Lymphomas (Extranodal/Malt, Splenic, And Nodal)

Carlos A. Ramos

Table 33-1 Staging Systems for Gastrointestinal Lymphomas

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* In case of more than one visible lesion synchronously originating in the gastrointestinal tract, select the characteristics of the more advanced lesion.

† Anatomic designation of lymph nodes as regional according to site: (a) stomach: perigastric nodes and those located along the ramifications of the celiac artery (i.e., left gastric artery, common hepatic artery, splenic artery); (b) duodenum: pancreaticoduodenal, pyloric, hepatic, and superior mesenteric nodes; (c) jejunum/ileum: mesenteric nodes and, for the terminal ileum only, the ileocolic as well as the posterior cecal nodes; (d) colorectum: pericolic and perirectal nodes and those located along the ileocolic, right, middle, and left colic, inferior mesenteric, superior rectal, and internal iliac arteries.

Table 33-2 ISCL/EORTC Staging System for Cutaneous Lymphomas Other Than Mycosis Fungoides and Sézary Syndrome4

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* The ISCL/EORTC proposes to defer any stage groupings of the TNM classification until further information is available to validate specific stage grouping strategies.

† Definition of body regions: Head and neck: inferior border—superior border of clavicles, T1 spinous process. Chest: superior border—superior border of clavicles; inferior border—inferior margin of rib cage; lateral borders—midaxillary lines, glenohumeral joints (inclusive of axillae). Abdomen/genital: superior border—inferior margin of rib cage; inferior border—inguinal folds, anterior perineum; lateral borders—midaxillary lines. Upper back: superior border—T1 spinous process; inferior border—inferior margin of rib cage; lateral borders—midaxillary lines. Lower back/buttocks: superior border—inferior margin of rib cage; inferior border—inferior gluteal fold, anterior perineum (inclusive of perineum); lateral borders—midaxillary lines. Each upper arm: superior borders—glenohumeral joints (exclusive of axillae); inferior borders—ulnar/radial-humeral (elbow) joint. Each lower arm/hand: superior borders—ulnar/radial-humeral (elbow) joint. Each upper leg (thigh): superior borders—inguinal folds, inferior gluteal folds; inferior borders—mid-patellae, midpopliteal fossae. Each lower leg/foot: superior borders—mid-patellae, midpopliteal fossae.

‡ Definition of lymph node regions: Peripheral sites: antecubital, cervical, supraclavicular, axillary, inguinal-femoral, and popliteal. Central sites: mediastinal, pulmonary hilar, paraortic, iliac.

Table 33-3 Chronic Antigenic Stimulation and Extranodal Marginal Zone Lymphoma

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* Estimated by assuming cases of thyroid histiocytic lymphoma were ENMZL because these were published before the REAL classification.

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Figure 33-1 EXTRANODAL MARGINAL ZONE LYMPHOMAS OF MUCOSA-ASSOCIATED LYMPHOID TISSUE (MALT LYMPHOMAS).

A to C, An example of a MALT lymphoma in the parotid gland is illustrated with various stains. The glandular tissue is overrun by lymphoid cells, which disrupt and destroy the gland. The resulting structure is referred to as a lymphoepithelial lesion (A). These can be more clearly identified with a keratin stain (B) and with a B-cell stain such as CD20 (C), which illustrate the glandular remnant and the B-cell proliferation. D, MALT lymphoma of the stomach is commonly associated with H. pylori infection, which can be identified by special stains (top).

Suggested Treatment Approach to Extranodal Marginal Zone Lymphoma

Stage I/II

Gastric H. pylori–positive

• Antibiotic therapy

• Repeat endoscopy in 3-6 months

• If still H. Pylori–positive and no progression, alternative antibiotic therapy

• Repeat endoscopy in 3 months

Nongastric, gastric H. pylori–negative or not responding to antibiotic therapy

• Antibiotic trial for stage I gastric, ocular, or cutaneous?

• Involved field radiation therapy (30 Gy)

Stage III/IV or Relapsed after Antibiotic and Radiation Therapy

• Expectant observation until indication to treat

• Rituximab ± single-agent alkylator/purine analogue or combination chemotherapy (CVP/FND)

Suggested Treatment Approach to Splenic Marginal Zone Lymphoma

HCV-Positive

• Interferon-α and ribavirin (or other anti-HCV therapy)

HCV-Negative (or Not Responding to Anti-HCV Therapy) or Relapsed

• Expectant observation until indication to treat

• Splenectomy

• Rituximab ± single-agent alkylator/purine analogue or combination chemotherapy (CVP/FND)

• If contraindication to splenectomy and systemic therapy, low-dose splenic irradiation

References

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