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A rare occurrence of primary T-cell lymphoma of the breast in pregnancy

Primary T-cell lymphoma of the breast is a very rare clinical entity. We report herein an extremely rare case of primary T-cell lymphoma of the breast during pregnancy. A 24-year-old woman in the 7th month of her fi rst pregnancy was admitted to our hospital with a three month history of progressive enlargement of her left breast and left axillary lymph nodes. She denied fever, night sweats or weight loss. Her past medical history was signifi cant for Evans syndrome diagnosed fi ve years earlier at our hospital. Although her hemolytic anemia responded well to steroid, thrombocytopenia was refractory to both steroid and intravenous immunoglobulin treatment. Eventually a complete response was achieved after subsequent splenectomy. The hematologic values remained normal during her regular follow-up. Her physical examination revealed a 6  8 cm, hard irregular mass in the upper outer quadrant of the left breast and ipsilateral enlarged axillary lymph nodes. No other enlarged peripheral lymph nodes were found. The hematologic and biochemical parameters were within normal limits. The peripheral blood smear examination was normal. An obstetrical ultrasonography showed a single viable intrauterine pregnancy of 27 weeks gestational age. Abdominal ultrasonograhy was normal. A core needle biopsy of the breast mass revealed diffuse infi ltration of atypical lymphoid cells with hyperchromatic nuclei and scant cytoplasm. Immunohistochemically, the tumor cells were positive for vimentin, CD3 and leukocyte common antigen, and negative for CD4, CD8, CD10, CD20, CD30, S-100, terminal deoxynucleotidyl transferase, desmin and cytokeratin, which suggested T-cell lymphoma. A bone marrow biopsy showed normal hematopoietic cells. The patient started chemotherapy with standard-dose CHOP-21 regimen (cyclophosphamide, doxorubicin, vincristine, and prednisolone). We observed a dramatic tumor regression after the fi rst cycle of the treatment, and she achieved a complete clinical response after the second cycle. Eight weeks after the initiation of chemotherapy, a cesarean section was performed, which yielded an apparently healthy male infant weighing 2450 g. After delivery, computed tomography (CT)-scans showed no evidence of disease spread. Four additional courses of chemotherapy were administered, after which the patient was reevaluated. Her physical examination and CT-scans and magnetic resonance imaging (MRI) of the brain were normal. Bilateral mammography and ultrasound showed benign fi ndings. Seven months after the completion of chemotherapy she was hospitalized again because of fever, productive cough, and shortness of breath. On physical examination she was in moderate respiratory distress and chest radiography and CTscan showed infi ltrates in the right lower and right middle lobes. In a matter of hours, the patient developed hypoxemic respiratory failure that required mechanic ventilation. She was transferred to the intensive care unit and empirically treated with broad spectrum antibiotics and anti-fungal agents. She developed multiple organ failure and died 15 days after her admission. Before the Acta Oncologica, 2011; 50: 1262–1263