Contributions
Abstract: PB1461
Type: Publication Only
Session title: Aggressive Non-Hodgkin lymphoma - Clinical
Background
Primary lymphoid organs are bone marrow and thymus.The secondary lymphoid organs are spleen,lymphonodes and lymphoid tissue associated to mucous membranes.Cecal appendix,therefore,can be site of NHL such as Burkitt lymphoma.BL is an aggressive NHL characterized by translocation and deregulation of the MYC gene on chromosome 8 and the heavy chain gene on chromosome 14.The t(8;14) is the most common translocation in BL occurring in 70-80% of cases.BL is derived from germinal center B cells and hystologicaly is characterized by a monomorphic population of matur lymphocytes with basophilic cytoplasm and prominent vacuoles.The result is a “starry sky” apparence due to macrophages containing cellular debris.The malignant cells express B-cell markers including CD19-CD20-CD79A-PAX5 and for germinal center markers CD10 and bcl6 but are negative for bcl2.There are only 30 cases of appendiceal Burkitt lymphoma in literature.In this cohort,the patients are predominantly male with an average age of 20 years.The majority of cases clinically presented as classical acute appendicitis,the remainder of cases presented atypical clinic but radiological signs of appendicitis.
Aims
In february 2020,a 44-years old man underwent appendicectomy for the diagnosis of acute appendicitis.Intraoperatively,a significant inflamed,edematous appendix without perforation was identified.Biopsy histology demonstrated marked expansion by lymphoid infiltrate composed by monomorphic intermediate-sized and the characteristic starry sky appaerance.In immunochemistry,the cells are CD20+ CD10+ BCL6+ BCL2- cMyc + with Ki67 proliferative index equal to 90%.The translocation t(8;14) in FISH confirms the diagnosis of Burkitt lymphoma.The patient had no B syntoms and was subjected to bone marrow biopsy without marrow infiltration by Burkitt Lymphoma.Total body CT and whole body PET did not demonstrate evidence of residual disease at any nodal or extranodal side.CSF was negative for neoplastic cells.LDH was normal.The Ann Arbor staging classification corresponde to stage IE, low risk.
Methods
The patient was started on a multiagent steroid and chemotherapy regimen(R-CODOX-M-IVAC)and medicated lumbar punctures.Our therapeutic program is to subject the patient to other cycles of therapy and to peripheral stem cells collection according to NCCN guidelines.
Results
Ours is one of the rare cases of appendiceal BL.This case allows us to underline the importance of hystology after appendicectomy to discover incidental diagnosis of NHL and other pathology with poor prognosis if not treated.
Conclusion
Approximately 1% of appendicetomies have an incidental finding of an appendiceal neoplasm.A primary appendiceal lymphoma is extremely rare. The predominant PAL histological subtype was DLBCL followed by Burkitt lymphoma.Treatment for Burkitt Lymphoma is stratified on patient age and stage.In adult,current recomandations include multiagent regimens with intensive chemotherapy and immunotherapy with anti CD20.Newer anti-CD20,anti-CD19,CD22 monoclonal antibody are under investigation.Follow up is carried out in the basis of the disease responce according to the Lugano criteria.
Keyword(s): Burkitt's lymphoma
Abstract: PB1461
Type: Publication Only
Session title: Aggressive Non-Hodgkin lymphoma - Clinical
Background
Primary lymphoid organs are bone marrow and thymus.The secondary lymphoid organs are spleen,lymphonodes and lymphoid tissue associated to mucous membranes.Cecal appendix,therefore,can be site of NHL such as Burkitt lymphoma.BL is an aggressive NHL characterized by translocation and deregulation of the MYC gene on chromosome 8 and the heavy chain gene on chromosome 14.The t(8;14) is the most common translocation in BL occurring in 70-80% of cases.BL is derived from germinal center B cells and hystologicaly is characterized by a monomorphic population of matur lymphocytes with basophilic cytoplasm and prominent vacuoles.The result is a “starry sky” apparence due to macrophages containing cellular debris.The malignant cells express B-cell markers including CD19-CD20-CD79A-PAX5 and for germinal center markers CD10 and bcl6 but are negative for bcl2.There are only 30 cases of appendiceal Burkitt lymphoma in literature.In this cohort,the patients are predominantly male with an average age of 20 years.The majority of cases clinically presented as classical acute appendicitis,the remainder of cases presented atypical clinic but radiological signs of appendicitis.
Aims
In february 2020,a 44-years old man underwent appendicectomy for the diagnosis of acute appendicitis.Intraoperatively,a significant inflamed,edematous appendix without perforation was identified.Biopsy histology demonstrated marked expansion by lymphoid infiltrate composed by monomorphic intermediate-sized and the characteristic starry sky appaerance.In immunochemistry,the cells are CD20+ CD10+ BCL6+ BCL2- cMyc + with Ki67 proliferative index equal to 90%.The translocation t(8;14) in FISH confirms the diagnosis of Burkitt lymphoma.The patient had no B syntoms and was subjected to bone marrow biopsy without marrow infiltration by Burkitt Lymphoma.Total body CT and whole body PET did not demonstrate evidence of residual disease at any nodal or extranodal side.CSF was negative for neoplastic cells.LDH was normal.The Ann Arbor staging classification corresponde to stage IE, low risk.
Methods
The patient was started on a multiagent steroid and chemotherapy regimen(R-CODOX-M-IVAC)and medicated lumbar punctures.Our therapeutic program is to subject the patient to other cycles of therapy and to peripheral stem cells collection according to NCCN guidelines.
Results
Ours is one of the rare cases of appendiceal BL.This case allows us to underline the importance of hystology after appendicectomy to discover incidental diagnosis of NHL and other pathology with poor prognosis if not treated.
Conclusion
Approximately 1% of appendicetomies have an incidental finding of an appendiceal neoplasm.A primary appendiceal lymphoma is extremely rare. The predominant PAL histological subtype was DLBCL followed by Burkitt lymphoma.Treatment for Burkitt Lymphoma is stratified on patient age and stage.In adult,current recomandations include multiagent regimens with intensive chemotherapy and immunotherapy with anti CD20.Newer anti-CD20,anti-CD19,CD22 monoclonal antibody are under investigation.Follow up is carried out in the basis of the disease responce according to the Lugano criteria.
Keyword(s): Burkitt's lymphoma