Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Cutaneous diffuse Large B-cell lymphoma
- Radiologic Findings
- Chest CT images show multifocal subcutaneous soft tissue lesions with reticular margins and enhancement in left upper back. Enlarged right supraclavicular lymph nodes are also noted. These lesions and LNs show increased FDG uptake.
- Brief Review
- Primary cutaneous B-cell lymphomas (PCBCLs) were defined as B-cell lymphomas manifesting on the skin without evidence of extracutaneous disease. PCBCLs are much less common than primary cutaneous T-cell lymphomas (PCTCLs) and represent approximately 20% to 25% of all primary cutaneous lymphomas. For many years, there has been confusion regarding the terminology and classification of PCBCL, which may have come to an end with the publication of the new World Health Organization-European Organization for Research and Treatment of Cancer(WHO-EORTC) classification for PCBCL. This classification distinguishes 3 main types of PCBCL: primary cutaneous marginal zone B-cell lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL, LT).
PCMZL is presented as solitary or multiple papules, plaques, or nodules preferentially localized on the extremities, and sometimes associated with Borrelia burgdorferi infection. It shows frequent cutaneous relapses and rare extracutaneous dissemination. Histopathology reveals patchy or diffuse infiltrates composed of small B cells, including marginal zone (centrocyte-like) cells, lymphoplasmacytoid cells, and plasma cells. Immunophenotype incudes monotypic cIg, CD79a+, Bcl-2+, CD5-, cyclin D1-, Bcl-6-, CD10-, MUM-1+ (on plasma cells). Five-year survival is more than 95%.
PCFCL shows solitary or grouped tumors presenting on the head or on the trunk, and cutaneous relapses occur in 20%. Extracutaneous dissemination is found in 5% to 10%. Histopathology of PCFCL reveals follicular, follicular and diffuse, or diffuse infiltrates composed of neoplastic follicle center cells, usually a mixture of centrocytes and variable numbers of centroblasts. Monotypic sIg or absence of sIg, CD20+, CD79a+, Bcl-6+, Bcl-2-, MUM-1-, CD10짹, FOXP1- (짹) are possible as immunophenotype. Five-year survival is 95%.
PCLBCL is presented as solitary or multiple tumors presenting mainly on the leg(s) and rarely at other sites. It shows frequent relapses and extracutaneous dissemination. Diffuse infiltrates with a predominance or confluent sheets of of centroblasts and immunoblasts Immunophenotype are seen on histopathologic examination, and monotypic sIg and/or cIg, CD20+, CD79a+, Bcl-6+(-), CD10-, Bcl-2+, MUM-1+, FOXP1+ are detected as immunophenotypes.
This case is primary cutaneous diffuse large B-cell lymphoma, involving mainly trunk and right arm. No involvement of legs was detected, and the patient was considered as cutaneous diffuse large B-cell lymphoma, not otherwise specified. He is going on the chemotherapy with regimen of R-CHOP.
- References
- Nancy J. Senff et al, European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood, 1 Sep 2008, Vol 112, Number 5, 1600-1609.
- Keywords
- chest wall, lymphoproliferative disease,