High-grade B-cell Lymphoma with <i>MYC</i> and <i>BCL2</i> Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass
Follicular lymphoma, the second most common non-Hodgkin lymphoma (NHL), primarily affects adults and shows an indolent clinical course. Rare cases of follicular lymphoma transform to a high-grade B-cell lymphoma with <i>MYC</i> and <i>BCL2</i> rearrangements or “double-hit ly...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2020-03-01
|
| Series: | Diagnostics |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-4418/10/3/157 |
| _version_ | 1817996357985632256 |
|---|---|
| author | Anna Shestakova Sherif Rezk Dara Ghasemizadeh Ali Nael Xiaohui Zhao |
| author_facet | Anna Shestakova Sherif Rezk Dara Ghasemizadeh Ali Nael Xiaohui Zhao |
| author_sort | Anna Shestakova |
| collection | DOAJ |
| description | Follicular lymphoma, the second most common non-Hodgkin lymphoma (NHL), primarily affects adults and shows an indolent clinical course. Rare cases of follicular lymphoma transform to a high-grade B-cell lymphoma with <i>MYC</i> and <i>BCL2</i> rearrangements or “double-hit lymphoma”. Transformation to a “double-hit lymphoma” portends a worse prognosis and requires aggressive treatment. We report a comprehensive clinical, pathologic and radiographic review of a patient with previously undiagnosed low-grade follicular lymphoma that transformed into a “double-hit lymphoma”. The patient presented with a large heterogeneous mass 16 x 19 cm involving pancreatic head and neck and a mildly enlarged inguinal lymph node. Positron emission tomography (PET) study demonstrated Fluorodeoxyglucose (<sup>18</sup>F) (FDG)-avid peripancreatic mass. Tissue biopsy demonstrated a high-grade B-cell lymphoma with rearrangements t(14;18) and <i>MYC</i>, leading to the diagnosis of high-grade B-cell lymphoma with <i>MYC</i> and <i>BCL2</i> rearrangements. Excisional biopsy of an inguinal lymph node demonstrated low-grade follicular lymphoma. Clonality studies demonstrated the same immunoglobulin clone V7-4 in inguinal lymph node and peripancreatic mass. Therefore, diagnosis of a high-grade B-cell lymphoma with <i>MYC</i> and <i>BCL2</i> rearrangements that transformed from a low-grade follicular lymphoma was rendered. It is ultimately important to establish a tissue-based diagnosis at the different sites that are involved with lymphoma. Patient proceeded with the aggressive treatment with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab (EPOCH-R) treatment. |
| first_indexed | 2024-04-14T02:21:11Z |
| format | Article |
| id | doaj.art-916f598448c149f59aee79499aa7da98 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-04-14T02:21:11Z |
| publishDate | 2020-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-916f598448c149f59aee79499aa7da982022-12-22T02:18:02ZengMDPI AGDiagnostics2075-44182020-03-0110315710.3390/diagnostics10030157diagnostics10030157High-grade B-cell Lymphoma with <i>MYC</i> and <i>BCL2</i> Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic MassAnna Shestakova0Sherif Rezk1Dara Ghasemizadeh2Ali Nael3Xiaohui Zhao4Department of Pathology, University of California, Irvine, CA 92868, USADepartment of Pathology, University of California, Irvine, CA 92868, USADepartment of Radiological Sciences, University of California, Irvine, CA 92868, USADepartment of Pathology, University of California, Irvine, CA 92868, USADepartment of Pathology, University of California, Irvine, CA 92868, USAFollicular lymphoma, the second most common non-Hodgkin lymphoma (NHL), primarily affects adults and shows an indolent clinical course. Rare cases of follicular lymphoma transform to a high-grade B-cell lymphoma with <i>MYC</i> and <i>BCL2</i> rearrangements or “double-hit lymphoma”. Transformation to a “double-hit lymphoma” portends a worse prognosis and requires aggressive treatment. We report a comprehensive clinical, pathologic and radiographic review of a patient with previously undiagnosed low-grade follicular lymphoma that transformed into a “double-hit lymphoma”. The patient presented with a large heterogeneous mass 16 x 19 cm involving pancreatic head and neck and a mildly enlarged inguinal lymph node. Positron emission tomography (PET) study demonstrated Fluorodeoxyglucose (<sup>18</sup>F) (FDG)-avid peripancreatic mass. Tissue biopsy demonstrated a high-grade B-cell lymphoma with rearrangements t(14;18) and <i>MYC</i>, leading to the diagnosis of high-grade B-cell lymphoma with <i>MYC</i> and <i>BCL2</i> rearrangements. Excisional biopsy of an inguinal lymph node demonstrated low-grade follicular lymphoma. Clonality studies demonstrated the same immunoglobulin clone V7-4 in inguinal lymph node and peripancreatic mass. Therefore, diagnosis of a high-grade B-cell lymphoma with <i>MYC</i> and <i>BCL2</i> rearrangements that transformed from a low-grade follicular lymphoma was rendered. It is ultimately important to establish a tissue-based diagnosis at the different sites that are involved with lymphoma. Patient proceeded with the aggressive treatment with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab (EPOCH-R) treatment.https://www.mdpi.com/2075-4418/10/3/157double-hit lymphoma 1follicular lymphoma 2ct scan 3 |
| spellingShingle | Anna Shestakova Sherif Rezk Dara Ghasemizadeh Ali Nael Xiaohui Zhao High-grade B-cell Lymphoma with <i>MYC</i> and <i>BCL2</i> Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass Diagnostics double-hit lymphoma 1 follicular lymphoma 2 ct scan 3 |
| title | High-grade B-cell Lymphoma with <i>MYC</i> and <i>BCL2</i> Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass |
| title_full | High-grade B-cell Lymphoma with <i>MYC</i> and <i>BCL2</i> Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass |
| title_fullStr | High-grade B-cell Lymphoma with <i>MYC</i> and <i>BCL2</i> Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass |
| title_full_unstemmed | High-grade B-cell Lymphoma with <i>MYC</i> and <i>BCL2</i> Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass |
| title_short | High-grade B-cell Lymphoma with <i>MYC</i> and <i>BCL2</i> Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass |
| title_sort | high grade b cell lymphoma with i myc i and i bcl2 i rearrangement arising from follicular lymphoma presentation as a large peripancreatic mass |
| topic | double-hit lymphoma 1 follicular lymphoma 2 ct scan 3 |
| url | https://www.mdpi.com/2075-4418/10/3/157 |
| work_keys_str_mv | AT annashestakova highgradebcelllymphomawithimyciandibcl2irearrangementarisingfromfollicularlymphomapresentationasalargeperipancreaticmass AT sherifrezk highgradebcelllymphomawithimyciandibcl2irearrangementarisingfromfollicularlymphomapresentationasalargeperipancreaticmass AT daraghasemizadeh highgradebcelllymphomawithimyciandibcl2irearrangementarisingfromfollicularlymphomapresentationasalargeperipancreaticmass AT alinael highgradebcelllymphomawithimyciandibcl2irearrangementarisingfromfollicularlymphomapresentationasalargeperipancreaticmass AT xiaohuizhao highgradebcelllymphomawithimyciandibcl2irearrangementarisingfromfollicularlymphomapresentationasalargeperipancreaticmass |