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Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 645

The isolated extranodal relapse of the isolated extranodal non- Hodgkin lymphoma: A case report

1 Department of Internal Medicine, Sehitkamil State Hospital, Gaziantep, Turkey
2 Department of Internal Medicine, Dr. Ersin Arslan State Hospital, Gaziantep, Turkey
3 Department of Medical Biology, University of Gaziantep, Gaziantep, Turkey
4 Department of Medical Oncology, University of Gaziantep, Gaziantep, Turkey

Date of Web Publication9-Oct-2015

Correspondence Address:
Gazi Comez
Department of Internal Medicine, Sehitkamil State Hospital Gaziantep, TR-27310
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.147706

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 > Abstract 

Diffuse large lymphomas of B-cell origin (DLBCL) comprise approximately one-third of all non-Hodgkin lymphomas (NHLs) and extranodal involvement is detected in 50% of these cases at initial diagnosis. Primary malignant lymphoma of the adrenal gland is extremely rare. Here we report a 64-year-old male patient with nasopharyngeal lymphoma who had been in remission for 2 years. An adrenal mass was detected on a control abdominal computed tomography (CT) at one of his follow-up visits. The biopsy showed DLBCL. Since the tumor was solitary without any other nodal involvement, a new/de novo primary tumor was considered. Metachronous NHLs develop between 3 months and 15 years after a primary NHLs and VDJ (variable, diversity, joining) rearrangement gene analysis of the tumor tissue is recommended to discriminate recurrence from a metachronous NHLs. VDJ rearrangement gene analysis was consistent with the recurrence of the original neoplasm.

Keywords: Non-Hodgkin lymphoma, relapse, VDJ rearrangement gene analysis

How to cite this article:
Comez G, Goktepe MF, Oztuzcu S, Sevinc A, Camci C. The isolated extranodal relapse of the isolated extranodal non- Hodgkin lymphoma: A case report. J Can Res Ther 2015;11:645

How to cite this URL:
Comez G, Goktepe MF, Oztuzcu S, Sevinc A, Camci C. The isolated extranodal relapse of the isolated extranodal non- Hodgkin lymphoma: A case report. J Can Res Ther [serial online] 2015 [cited 2020 Jul 16];11:645. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/645/147706

 > Introduction Top

Lymphomas are the second most common malignancies of the head and neck. Most of the lymphomas localized in the nasopharyngeal region are non-Hodgkin lymphomas (NHLs). [1] Diffuse large lymphomas of B-cell origin (DLBCL) comprise approximately one-third of all NHL and extranodal involvement is detected in 50% of these cases at the time of diagnosis. Gastrointestinal tract, skin, kidney, soft tissues, orbita, and the adrenal gland are the most common anatomical sites of extranodal involvement. [2],[3] Here we report a case of primary nasopharyngeal NHL who relapsed with solitary adrenal involvement. Our aim was to evaluate the discrimination of recurrence from a metachronous NHLs via VDJ (variable, diversity, joining) rearrangement gene analysis.

 > Case report Top

A 64-year-old male patient presented to the medical oncology outpatient clinic because of nasal pain and difficulty in breathing. Magnetic resonance imaging (MRI) of the nasopharynx revealed a mass lesion of approximately 50 × 40 × 30 mm in size. Biopsy from the mass showed DLBCL with positive CD 20 (+) staining. Imaging studies were otherwise unremarkable. The patient received the CHOP (cyclophosphamide: 750 mg/m 2 , adriamycin: 50 mg/m 2 , vincristine: 1.4 mg/m 2 , and prednisolone: 100 mg) combination chemotherapy regimen. The nasopharyngeal MR and other imaging studies were consistent with remission after chemotherapy. The patient was followed periodically after completion of six cycles of CHOP chemotherapy. The abdominal computed tomography (CT) that was performed in the second year of remissionshowed a 57 × 59 mm mass lesion in the left adrenal gland. Abdominal MRI confirmed the diagnosis. Biopsy from the lesion revealed DLBCL (CD 20 (+)). Other body scans were unremarkable. VDJ rearrangement gene analysis was consistent with recurrence. With these findings the case was considered as relapsed DLBCL and treated with a combination of again CHOP plus rituximab. An abdominal CT scan performed after three courses of chemotherapy demonstrated a decrease in the size of the mass lesion to 30 × 20 × 15 mm in size. The patient received three additional courses to complete the therapy. However, no further significant change in lesion size was detected in the abdominal CT scan. Therefore, the patient received radiotherapy for the treatment of the mass lesion in the left adrenal gland. The patient died due to acute myocardial infarction.

 > Discussion Top

Approximately 10-20% of the primary NHLs are located in the head and neck and among these NHL cases. [4] DLBCLs are the most common primary lymphomas of the adrenal gland. Adrenal gland involvement is detected by various scanning procedures in 4% of NHL cases with diffuse involvement. Postmortem examination revealed adrenal gland involvement in 25% of these patients. [5],[6]

As in our case presented here, it is a real challenge to discriminate between a secondary primary lymphoma and recurrence of the original tumor in patients with remission. Studies show that if the time to relapse is approximately 3 years or shorter and if the histopathological characteristics are similar, the case should be evaluated as a recurrent neoplasm. Similarly, if the time to relapse is 3 years or longer and if the histopathological characteristics are different, it would be a right approach to consider the new tumor as a secondary primary lymphoma. [7],[8] Several reports in the literature highlight the difficulty of discriminating between a second primary lymphoma and recurrent lymphoma. Comparison of histological characteristics alone is insufficient in many cases to distinguish new lymphomas from recurrent lymphomas. Recurrent NHLs may be located at different anatomical sites, but histologically share the same characteristics with the primary tumor. And, the newly formed tumor may share the same histological properties with the primary tumor, but may not always indicate recurrent tumor. [9],[10] It should be noted that histological properties and time to remission do not always help to discriminate between second primary and recurrent lymphomas. VDJ rearrangement gene analysis technique enables a reliable comparison of the clonal origins of B-cell lymphomas through the analysis of the B-cell Ig H genes. Each immunoglobulin molecule consists of two identical heavy chains and two identical light chains. This region represents the germline organization of the heavy chain locus. The locus includes V (variable), D (diversity), J (joining), and C (constant) segments. During B cell development, a recombination event at the deoxyribonucleic acid (DNA) level joins a single D segment with a J segment; the fused D-J exon of this partially rearranged D-J region is then joined to a V segment. Mature B cells in the lymph nodes undergo switch recombination, so that the fused V-D-J gene segment is brought in proximity to one of the IGHG, IGHA, or IGHE gene segments and each cell expresses either the gamma, alpha, or epsilon heavy chain. Potential recombination of many different V segments with several J segments provides a wide range of antigen recognition. Application of VDJ rearrangement analysis in clinical research enables the characterization of the immunophenotype of the metachronous tumor, thus making it possible to discriminate a second primary tumor from a recurrent lymphoma. Furthermore, this technique has the potential to improve treatment of lymphoma patients because management of recurrent lymphomas differs from that of new lymphomas. Also, in humans, VDJ repertoire is almost entirely set by whatever genetic controls determine the selection of one V, D, or J segment over another, with only a small bias of preferential pairing of certain Ds with certain Js. However, VDJ rearrangement analysis present some limitations. For example, the imprecise joining of the V-D-J gene segments leads to random deletion and insertion of nucleotides during recombination events, resulting in sequence diversification at the junctional regions. Moreover, the majority of sequences could be classified to their most closely related reference sequences For IGHV and IGHJ genes (average of 99.8 and 96.1%, respectively). Substantially, fewerIGHDwere identifiable (average of 40.5%) due to the shorter sequence length and potential insertions and deletions within the joining regions between the V-D-J boundaries, which have been noted in previous studies. [11] In our case, time to remission was 2 years and the histopathological properties of the two lymphomas were similar; thus, it could be diagnosed as a relapsed lymphoma. However, we preferred to use the VDJ rearrangement gene analysis technique in this case because both of the lymphomas in our patient were of rare extranodal type. VDJ analysis is a reliable technique to discriminate metachronous NHLs from recurrence.

 > Acknowledgement Top

We thank to the Dr. Christiane Pott, Department of Medicine II, University of Schleswig-Holstein, Kiel, Germany for for performing the VDJ gene analysis.

 > References Top

Levine MS, Rubesin SE, Pantongrag-Brown L, Buck JL, Herlinger H. Non-Hodgkin′s lymphoma of the gastrointestinal tract: Radiographic findings. AJR Am J Roentgenol 1997;168:165-72.  Back to cited text no. 1
Chim CS, Liang R, Chan AC, Kwong YL, Ho FC, Todd D. Primary B cell lymphoma of the mediastinum. Hematol Oncol 1996;14:173-9.  Back to cited text no. 2
Popat U, Przepiork D, Champlin R, Pugh W, Amin K, Mehra R, et al. High - dose chemoterapy for relapsed and refractory diffuse large B-cell lymphoma: Mediastinal localization predicts for a favorable outcome. J Clin Oncol 1998;16:63-9.  Back to cited text no. 3
Economopoulos T, Fountzilas G, Kostourou A, Daniilidis J, Pavlidis N, Andreopoulou H, et al. Primary extranodal non Hodgkin′s lymphoma of the head and neck in adults: A clinicopathological comparison between tonsillar and non tonsillar lymphomas. (Hellenic co-Operative Oncology Group). Anticancer Res 1998;18:4655-60.  Back to cited text no. 4
Paling MR, Williamson BR. Adrenal involvement in non-Hodgkin′s lymphoma. AJR Am J Roentgenol 1983;141:303-5.  Back to cited text no. 5
Rosenberg SA, Diamond HD, Jaslowitz B, Craver LF. Lymphosarcoma: A review of 1269 cases. Medicine (Baltimore) 1961;40:31-84.  Back to cited text no. 6
Coiffier B, Thieblemont C, Felman P, Salles G, Berger F. Indolent nonfollicular lymphomas: Characteristics, treatment, and outcome. Semin Hematol 1999;36:198-208.  Back to cited text no. 7
Shioyama Y, Nakamura K, Kunitake N, Kimura M, Terashima H, Masuda K. Relapsed non-Hodgkin′s lymphoma: Detection and treatment. Radiat Med 2000;18:369-75.  Back to cited text no. 8
Nishiuchi R, Yoshino T, Teramoto N, Sakuma I, Hayashi K, Nakamura S, et al. Clonal analysis by polymerase chain reaction of B- cell lymphoma with late relapse: A report of five cases. Cancer 1996;77:757-62.  Back to cited text no. 9
Libra M, De Re V, Gasparotto D, Gloghini A, Marzotto A, Milan I, et al. Differentiation between non-Hodgkin′s lymphoma recurrence and second primary lymphoma by VDJ rearrangement analysis. Br J Haematol 2002;118:809-12.  Back to cited text no. 10
Libra M, De Re V, Gloghini A, Navolanic PM, Carbone A, Boiocchi M. Second primary lymphoma or recurrence: A dilemma solved by VDJ rearrangement analysis. Leuk Lymphoma 2004;45:1539-43.  Back to cited text no. 11


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