Primary Burkitt Lymphoma of the Chest Wall

IntroductionBurkitt lymphoma (BL) originating in the skin and soft tissue is very rare. To our knowledge, this case of primary sporadic BL presenting as an isolated chest wall mass arising from the soft tissue in an adult may be the first report.Case ReportA previously healthy 33-year-old Caucasian man presented with a 1-month history of a painful lump over the left breast that he initially noticed as a small “pimple-like” lesion in the area. After a week, the skin lesion became larger, erythematous, and painful. At a local hospital, he underwent an incision and drainage procedure for a presumed chest wall abscess. Several days after debridement, a similar lump recurred around the incised area, which rapidly grew in size. He also started experiencing fever and chills for which he was readmitted with a diagnosis of necrotizing chest wall infection. A second debridement with excisional biopsy of the chest wall revealed atypical lymphoid cells, prompting transfer to our institution. Upon transfer, a large, gaping, erythematous and indurated wound with indistinct, thickened borders and extensive edema and necrosis of subcutaneous tissue and musculature of almost the entire left chest wall was noted. No palpable peripheral lymphadenopathy or organomegaly were observed. He underwent minimal debridement and partial excision. The histopathology revealed atypical lymphocytes with prominent nucleoli, deeply basophilic cytoplasm, and abundant lipid vacuoles in a “starry-sky” pattern. The lymphoid population was CD-20 and CD-10 positive, negative for CD-5 and BCL-2, nearly 100% Ki-67 positive, and indeterminate for light chain restriction. Molecular cytogenetic analysis revealed fusion signals with IgH/Myc t(8;14) dual fusion probe, supporting the diagnosis of BL. Staging positron emission tomography (PET)/computed tomography (CT) scan showed a large subcutaneous defect of the left hemithorax involving the dermis, subcutaneous tissue, and musculature, measuring 19.3 × 13.9 × 31.0 cm, with maximal SUV of 9.8 and an average of 6.2. No additional involved sites were seen. The bone marrow biopsy showed minimal involvement by BL and abnormal hybridization pattern for IgH/Myc t(8;14) and Epstein-Barr virus, while the peripheral blood and cerebrospinal fluid showed no involvement. HIV and hepatitis serologies were negative. Three days after surgery, chemotherapy with granulocyte colony-stimulating factor (G-CSF) support was initiated for high-risk disease. He received CODOX-M (intravenous cyclophosphamide, doxorubicin, vincristine, methotrexate; intrathecal cytarabine, methotrexate) as cycle 1 followed by IVAC (intravenous ifosfamide, etoposide, cytarabine; intrathecal methotrexate) with rituximab as cycle 2. He developed tumor lysis without end-organ damage. However, a few days after completion of cycle 2, he developed neutropenic fever and pneumonia, and died in septic shock.DiscussionPrimary chest wall tumors are uncommon. Approximately 50% are malignant, and chest wall lymphoma accounts for < 2%, with extranodal diffuse large B-cell lymphoma being the most common. Primary skin and soft tissue involvement of the chest wall in the absence of detectable lymphadenopathy and visceral disease in an adult by BL has not yet been reported. While there are isolated reports of skin and soft tissue involvement, they were in the setting of immunodeficiency state and were felt to be the result of either iatrogenic tumor seeding after nodal biopsies or local tumor invasion as a manifestation of recurrent disease. This patient's clinical presentation began with the development of an isolated rapidly enlarging chest wall mass that progressed despite surgical debridements.ConclusionThis case illustrates a primary sporadic BL originating in skin and soft tissue in an adult. Whether this case represents a BL that began in the skin and soft tissue and spread to the bone marrow, or began in the bone marrow and spread to the chest wall cannot be determined. The role of tumor debulking procedure is uncertain, although aggressive chemoimmunotherapy with central nervous system (CNS) prophylaxis is warranted as with other BL presentations.