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Uncommon Hematologic Malignancies

CASE 2. CALCIFICATION IN UNTREATED PRIMARY MEDIASTINAL LARGE B-CELL LYMPHOMA WITH SCLEROSIS

Caritas St. Elizabeth’s Medical Center of Boston, Tufts University School of Medicine, Boston, MA

A 23-year-old white male was admitted to our hospital with a diagnosis of superior vena cava syndrome. He presented with a 1-week history of swelling of his face and neck, discomfort on swallowing, headaches, and pressure in the head. On the day of admission, he noticed bluish discoloration of the skin over his chest. He denied fevers, weight loss, chills, night sweats, dyspnea, or wheezing. His past medical history was remarkable for cleft lip repair, exercise-induced ventricular tachycardia, and right inguinal herniorrhaphy. Physical examination revealed suffused conjunctivae, swollen face, neck and upper extremities, and dilated anterior chest wall veins. No enlarged lymph nodes or hepatosplenomegaly were present. The blood counts and chemistry profile were normal except for serum lactate dehydrogenase of 1,359 U/L (range, 313 to 618 U/L). The erythrocyte sedimentation rate was 33 mm/first hour. A chest radiograph showed an anterior mediastinal mass further detailed on a computed tomography (CT) scan of the chest, which showed a 9 x 8 x 10 cm mediastinal mass with areas of cystic change and calcification (Fig 1), compressing the trachea and superior vena cava and displacing the aorta. Subsequent serum ß-human chorionic gonadotrophin and -fetoprotein were normal. A CT-guided needle biopsy revealed large-cell lymphoma with fibrosis and scattered areas of calcification (Fig 2A and B). Immunoperoxidase stains were strongly positive for CD20 (Fig 2C) and leukocyte common antigen but negative for CD3, CD43 and CD45R0 (UCHL-1). Flow cytometry revealed B lymphocytes with monoclonal kappa immunoglobulin light chain restriction. Subsequent CT of his abdomen and pelvis were unrevealing. ⁶⁷gallium scan showed a large area of increased activity in the mediastinum. The bone marrow biopsy ruled out lymphoma involvement. A diagnosis of primary mediastinal large B-cell lymphoma with sclerosis was established and he was treated with three-weekly chemotherapy with cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab. Within a few days, his symptoms improved with the resolution of the swelling over his face and neck and skin discoloration. The ⁶⁷gallium scan became negative after two cycles of chemotherapy. The restaging CT scans after six cycles of chemotherapy revealed a residual mass (5 x 2 x 7 cm). He was started on involved field radiotherapy to the mediastinum.

View larger version (99K): [in this window] [in a new window]   Fig 1.

View larger version (91K): [in this window] [in a new window]   Fig 2.

AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST

The authors indicated no potential conflicts of interest.

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