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A 34-year-old female with extranodal marginal zone lymphoma
A 34-year-old female with no medical history originally presented with hoarseness and a choking sensation in November 2007.
She noted an episode of epistaxis from her left nostril in January 2008. On physical examination, she demonstrated a unilateral swelling of her left neck.
CT of her neck showed extensive soft tissue swelling around the right orbit surrounding the optic nerve and near the vocal cords. Cervical and supraclavicular lymph nodes also were noted.
She underwent left supraclavicular lymph node fine-needle aspiration, which showed a B-cell lymphoma that could have represented a nodal marginal zone B-cell lymphoma with secondary extension into the orbit, although the reverse extension to lymph nodes of the primary extranodal marginal zone B-cell lymphoma of the orbit could not be ruled out.
Histopathology revealed a population of monotonous small lymphocytes with mildly irregular nuclei contour and clumped chromatin, positive for CD20 and bcl-2, and negative for CD23, CD10, CD43, CD5, cyclin D1 and bcl-6. Monoclonal B-cell population was CD19+, CD10–, CD23–, CD5–, Kappa+ and Lambda–. Flow cytometry of the lymph node showed CD19+, CD20+, CD22–, CD10–, CD23–, CD5–, Kappa+ and Lambda–. Staging CT scans showed left supraclavicular lymph nodes, enlarged right internal mammary lymph nodes, splenomegaly, breast nodules and subcutaneous soft tissue nodules.
Figure 1. (CT orbits from Feb. 15, 2008). Axial and coronal reformats from the initial orbital CT show crescentic focus of soft tissue density in the superolateral right orbit, largely inseparable from the lateral rectus muscle (arrow). Multiple other right retrobulbar abnormalities were seen, consistent with clinical suspicion of lymphoma.
Images: M. Ghesani, MD, reprinted with permission.
Bone marrow biopsy was negative. A left laryngeal mass biopsy on Jan. 28, 2008, showed minute fragmented respiratory mucosa with atypical lymphoid infiltrate suspicious for lymphoma. A left false vocal cord biopsy showed squamous and respiratory mucosa with atypical lymphoid infiltrate suspicious for lymphoma, and a biopsy of the left lingual tonsil showed fragments of mucosa involved by large B-cell lymphoma.
Immunohistochemically showed atypical large cell infiltrate, strongly and diffusely positive for B-cell marker CD20 and bcl-2; CD10– and cyclin D1–. Lab work revealed lactate dehydrogenase 199 IU/L and uric acid 5.6 mg/dL. Liver function tests were normal; C-reactive protein 5.23 mg/L, beta-2–microglobulin 3.2 g/mL, serum protein electrophoresis and immunofixation — no monoclonal protein.
PET/CT on Jan. 29, 2008, showed supraclavicular lymphadenopathy and a hypermetabolic nodule in the right breast, mediastinal and groin lymph nodes, cervical lymph nodes and subcutaneous soft tissue nodules. She was started on prednisone 60 mg daily.
After detailed discussion, she started R-CVP. She received a total of six cycles. Post-treatment PET/CT on Aug. 5, 2008, showed no evidence of disease. She then was treated with four cycles of maintenance rituximab (Rituxan, Genentech/Idec).
Interim PET scans did not show any signs of active lymphoma. However, follow-up PET/CT on March 14, 2011, showed a focal mass posterior left of the midline lower back measuring 1.3 cm × 0.8 cm with a standardized uptake value (SUV) of 1.6 at the level of the L5 vertebra.
Figure 2. Staging PET/CT from March 27, 2008 (Figure 2a), shows enhancing right optic nerve thickening and abnormal corresponding hypermetabolic activity (arrow), highly suspicious for lymphomatous involvement. The same scan also demonstrated hypermetabolic right nasopharyngeal soft tissues and hypermetabolic right breast nodules, as well as hypermetabolic lymph nodes in the neck, chest, abdomen and pelvis, all suspicious for lymphomatous involvement. A follow-up scan on May 29, 2008 (Figure 2b), after chemotherapy, showed resolution of right optic nerve thickening with no residual hyperbolic activity, consistent with excellent response to treatment. Previous findings in the neck, chest, abdomen and pelvis showed similar response.
A new, second nodular focus measuring 7 mm × 4 mm was seen at the level of the left gluteal muscle.
Because of suspicion for disease recurrence, the patient underwent restaging PET/CT on June 2, 2011. The previously seen subcutaneous soft tissue nodule in the left lower back/gluteal area had increased in size to 1.3 cm × 1.3 cm, with SUV of 3.2.
There were new adjacent subcutaneous soft tissue nodules overlying the gluteus muscle measuring 1.8 cm × 1.5 cm and 0.9 cm × 1 cm, with SUV of 2.9. A left gluteal nodule had increased to 1 cm × 0.7 cm, with SUV of 3. There was no radiographically significant or hypermetabolic lymphadenopathy. There was no evidence to suspect recurrence of right optic nerve lymphoma.
She underwent a biopsy of this soft tissue nodule, which showed extranodal marginal zone lymphoma.
Discussion
Extranodal marginal zone lymphoma, also called low grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), is an extranodal lymphoma that arises in a number of epithelial tissues, including the stomach, salivary gland, lung and small bowel.
For treatment purposes, extranodal MALT is separated into those developing in the stomach (gastric MALT) and those developing in nongastric extranodal locations (nongastric MALT). This distinction is primarily based upon the role of Helicobacter pylori in the development of gastric MALT and the efficacy of H. pylori eradication in the treatment of gastric MALT.
Figure 3a. Follow-up PET/CT from March 14, 2011, shows a new hypermetabolic subcutaneous nodule (arrow) in the left lower back/gluteal soft tissues. The patient had no history of subcutaneous injections in that area, and the possibility of lymphomatous involvement could not be excluded. There was no evidence of recurrence in the right orbit or anywhere else in the body.
Figure 3b. A repeat scan on June 22, 2011), showed an increase in size, number and hypermetabolism of subcutaneous left lower back/gluteal soft tissue nodules. Subsequent percutaneous biopsy revealed B-cell lymphoma.
Pretreatment evaluation should include a thorough physical exam, determination of the patient’s performance status by the ECOG or Karnofsky performance scales, lab testing — including a complete blood count with differential and platelet count — chemistries with liver and renal function and electrolytes, and lactate dehydrogenase level. Patients also should be tested for hepatitis B before the use of rituximab therapy. Imaging should include a contrast-enhanced CT scan of the chest, abdomen and pelvis. Patients with multifocal disease should have a bone marrow aspiration and biopsy.
Locoregional radiation therapy is the primary treatment option for limited-stage disease. Surgery typically is used for diagnostic purposes only, but may play a role in the treatment of tumors in areas not conducive to radiation therapy (eg, lungs). The addition of adjuvant chemotherapy or antibiotic therapy has not demonstrated improved DFS or OS. There are occasional patients with stage I disease who are not ideal candidates for radiation therapy and may be initially observed closely.
The treatment of patients with advanced-stage nongastric extranodal marginal zone lymphoma is not clearly defined, and most data in this population come from retrospective series or extrapolation of data from other indolent non-Hodgkin’s lymphoma.
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For more information:
- Munir Ghesani, MD, is an attending radiologist at St. Luke’s-Roosevelt Hospital Center and Beth Israel Medical Center, an associate clinical professor of radiology at Columbia University College of Physicians and Surgeons, and a HemOnc Today section editor. Amit Patel, MD, is a fellow in hematology/oncology at St. Luke’s-Roosevelt Hospital. Sam Altman, MD, is a resident in radiology at St. Luke’s-Roosevelt Hospital Center. Drs. Ghesani, Patel and Altman report no relevant financial disclosures.