A 45 year-old woman with 6 months history of low back pain, weight loss, night sweat, fever and galactorrhea was referred to our center. Physical examination revealed multiple and palpable masses in both breasts and galactorrhea without peripheral lymphadenopathy. The rest of physical examination was unremarkable. Ultrasonographic evaluation revealed lobular-shaped, partly ill-defined hypoechoic masses with 2-7 cm diameters and a multi-septated nodular (mottled) appearance without acoustic changes in both breasts. Mammography showed an extremely dense pattern with bilateral ill-defined, hypodense mass-like lesions. Computer tomographic (CT) scans of the chest and abdomen were normal. CBC and LDH were completely normal. The patient had no contributory past or family history.
Breast lumpectomy was performed in another center and the primary result exhibited malignant non-Hodgkin lymphoma; Malt type and immunohistochemistry (IHC) suggested Maltoma (Figure 1).
During the primary work up, her back pain increased and paraplegia occurred. Magnetic resonance imaging (MRI) demonstrated disc protrusion of T8-T9 and spinal surgery was performed immediately. Surgical gross result was extradural soft tissue tumor at T8-T9 with invasion to the bone. Pathologic report revealed malignant high-grade lymphoblastic lymphoma.
She was referred to our department (Medical Oncology and Hematology department of the National Institute of Tuberculosis and Lung disease, NRITLD). First, we suggested reviewing of previous pathology result and surprisingly it was different suggesting dense lymphoid infiltration with diffuse growing pattern and malignant lymphoblastic lymphoma, B-cell type (CD20, CD3, CD5, CD10, BCL2 were positive in IHC staining) (Figure 2).
In the setting of lymphoma staging, bone marrow aspiration and biopsy were performed and bone marrow involvement was highly suspected. Central nervous system involvement was ruled out by lumbar puncture (LP).
The patient was staged IVBE according to the Ann Arbor staging system and International Prognostic Index (IPI) was low-intermediate. We started chemotherapy with hyper CVAD regimen (Course 1: [cyclophosphamide: 300mg/m2; BID days 1-3; vincristine: 2mg/m2 day 1; doxorubicin: 50mg/m2 day 4; dexamethasone 40mg/m2 days 1-4] course 2: [methotrexate: 1000mg/m2 day 1; cytarabine: 3000mg/m2 BID days 2-3], with G-CSF support per 2-3 weeks). CNS prophylaxis was done by methotrexate: 12mg day 2 and cytarabine: 100mg day 7. After 4 courses, the patient did not have any complaint and response evaluation with mammography and bone marrow biopsy was completely normal. She was a candidate for bone marrow transplantation (BMT) due to her stage and IPI but owing to the large number of patients on the waiting list, this process took about 3 months. She developed back pain again and L4/L5 central disc protrusion was seen in MRI. She was admitted to our department. Marked painful swelling of both breasts was detected on physical examination and multiple masses in mammography examination were seen. Chest CT showed minimal pleural effusion in the left side. We treated her by ICE regimen (Ifosfamide 5gr/m2 for 3 days, Etoposide 100mg/m2 for 3 days and Carboplatin AUC 5 day 1) for 8 cycles. Unfortunately, she was expired after 4 months.