Abstract
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 MYC and BCL2 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 (18F) (FDG)-avid peripancreatic mass. Tissue biopsy demonstrated a high-grade B-cell lymphoma with rearrangements t(14;18) and MYC, leading to the diagnosis of high-grade B-cell lymphoma with MYC and BCL2 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 MYC and BCL2 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.
Highlights
Follicular lymphoma is the second most common non-Hodgkin lymphoma and it accounts for approximately 20% of all non-Hodgkin lymphoma [1]
We report a comprehensive clinical, pathologic including molecular clonality studies and a radiologic review of a case of low-grade follicular lymphoma that progressed to a so-called “double-hit lymphoma” with BCL2/IGH and MYC rearrangements
The patient proceeded with the recommended dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab (DA-EPOCH-R) treatment
Summary
Follicular lymphoma is the second most common non-Hodgkin lymphoma and it accounts for approximately 20% of all non-Hodgkin lymphoma [1]. The gold standard to diagnose transformation requires a biopsy from the involved lymph nodes or extranodal tissue that demonstrates sheets of large tumor cells and loss of the follicular architecture These tumors resemble diffuse large B-cell lymphoma (DLBCL) or other aggressive lymphomas, and have high proliferative index, as indicated by Ki-67 immunostaining. We report a comprehensive clinical, pathologic including molecular clonality studies and a radiologic review of a case of low-grade follicular lymphoma that progressed to a so-called “double-hit lymphoma” with BCL2/IGH and MYC rearrangements. The next-generation sequencing of the immunoglobulin variable region determined the same clone, V7-4, in low-grade follicular lymphoma involving the inguinal lymph node and high-grade lymphoma with BCL2/IGH and MYC rearrangements involving the peripancreatic region. In order to establish accurate diagnosis, it is important to biopsy different sites that are involved with the lymphoma
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