Discordant lymphoma consisting of mediastinal large B-cell lymphoma and nodular sclerosis Hodgkin lymphoma in the right supraclavicular lymph nodes: a case report

Abstract

BackgroundDiscordant lymphoma is defined by the simultaneous presence of two or more distinct types of lymphomas at different anatomic sites. With fewer than 20 studies reporting cases of discordant lymphoma to date, the incidence of this condition is believed to be very low.Case PresentationHere, we report a case of discordant lymphoma in a 34-year-old female patient that involved mediastinal large B-cell lymphoma and nodular sclerosis Hodgkin lymphoma in the right supraclavicular lymph nodes. The patient presented with a mass in the mediastinum and enlargement of the right supraclavicular lymph nodes, but no obvious signs of lymphoma. Histological examination revealed that the encapsulated mediastinal mass contained medium- or large-size tumor cells with lightly stained cytoplasm and round vesicular nuclei as well as a high percentage of mitotic cells; strongly positive immunohistochemical staining for PAX5, CD20, and CD79a also was observed. Examination of biopsied right supraclavicular lymph node tissues revealed separation by collagen fibers, extensive inflammatory cell infiltration, and large-size tumor cells, such as Reed–Sternberg cells. These tissues stained strongly positive for PAX5 and CD30, weakly positive for CD15, and negative for Epstein-Barr viral RNA. We also found monoclonal gene rearrangement in the immunoglobulin heavy chain gene in the mediastinal large B-cell lymphoma, but no monoclonal gene rearrangement in the nodular sclerosis Hodgkin lymphoma. These findings suggested that these two lymphomas were not of a common clonal origin. The patient was treated by surgical excision of the mediastinal mass followed by radio-chemotherapy, and no metastasis or recurrence occurred during a follow-up period of 32 months.ConclusionA review of previously reported cases indicated that the clinical manifestations and pathological features of discordant lymphoma are diverse due to variation in the types of lymphomas involved. Physicians must have an awareness of discordant lymphoma to avoid incorrect and missed diagnoses, especially considering that the true incidence may not be as low as previously believed.Electronic supplementary materialThe online version of this article (doi:10.1186/s13000-015-0450-6) contains supplementary material, which is available to authorized users.

Synchronous discordant Epstein-Barr virus (EBV)–positive nodal T/NK-cell lymphoma and EBV-positive diffuse large B cell lymphoma in a patient with a history of EBV-positive Burkitt lymphoma

“…B and T cell discordant lymphomas are described only rarely in the existing literature. Our patient's combination of EBV-positive primary nodal T/ NK-cell lymphoma and EBV-positive diffuse large B cell lymphoma (both existing within lymph nodes) appears to be unique among published reports of discordant lymphoma, but other cases have described peripheral T cell lymphomas found in combination with Hodgkin lymphoma [2]. In at least one of these cases, the discordant lymphoma displayed strong EBV positivity [13].…”

“…The diagnosis of discordant lymphoma requires evidence of at least two unique lymphomas in at least two anatomically distinct sites. These sites may be two different lymph nodes, as in our case and others, or may include non-lymphoid tissues such as the nasopharynx, skin, salivary glands, stomach, abdominal cavity, mediastinum, and bone marrow [2,[12][13][14][15][16][17][18]. Because not all lymphoma patients will undergo biopsies at multiple anatomic locations, there is significant potential for under-diagnosis, especially in patients who are asymptomatic or without diffuse lymphadenopathy at presentation.…”

“…The pathogenesis of discordant lymphoma is poorly understood, in part because of its rarity. Zhang et al estimated that fewer than 20 reports of discordant lymphoma have been published since the phenomenon was first classified in 1974, though they suggested that it is likely under-diagnosed [2,11]. The diagnosis of discordant lymphoma requires evidence of at least two unique lymphomas in at least two anatomically distinct sites.…”

“…The development of multiple distinct lymphomas within a single patient is an uncommon phenomenon with significant variability in both the patient presentation and in the diagnostic features of the lymphomas themselves. These cases are classified according to temporality (two lymphomas occurring simultaneously are considered synchronous, while those occurring sequentially are metachronous), anatomic location (two lymphomas existing at a single anatomic site comprise a composite lymphoma, while those existing at different anatomic sites comprise a discordant lymphoma), and the identifying features of the component lymphomas (including morphology, immunohistochemical staining pattern, and molecular features) [1,2].…”

Promising Response to Lenalidomide-Combination Therapy in a Discordant Lymphoma Consisting of EBV-Positive Diffuse Large B-Cell Lymphoma and Angioimmunoblastic T-Cell Lymphoma: A Case Report

“…Discordant lymphomas, which are defined as two distinct types of lymphomas coexisting at different anatomic sites, 9 are relatively infrequent, particularly those containing T cell and B cell lymphomas. Here, we report a rare case of synchronous discordant lymphoma in a 77-year-old man that involved AITL and EBV-positive DLBCL that was successfully treated with a lenalidomide-combination regimen.…”