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Primary bilateral adrenal B-cell lymphoma associated with EBV and JCV infection.

Barzon L, Trevisan M, Marino F, Guzzardo V, Palù G - Infect. Agents Cancer (2009)

Bottom Line: The pathogenesis is unknown, but detection of Epstein Barr virus (EBV) genome sequences and gene expression in some cases of primary adrenal lymphomas suggested the virus might be a causative agent of the malignancy.While investigating the presence of genome sequences of oncogenic viruses in a large series of adrenal tumors, both EBV and JC polyomavirus (JCV) DNA sequences were detected in a diffuse large primary bilateral B-cell non-Hodgkin lymphoma of the adrenal gland, which was diagnosed only at postmortem examination in a 77 year-old woman with incidentally discovered adrenal masses and primary adrenal insufficiency.The presence of both EBV and JCV genome sequences suggests the relevance of EBV and JCV coinfection in the pathogenesis of this rare form of B-cell lymphoma.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Histology, Microbiology and Medical Biotechnologies, University of Padova, I-35121 Padova, Italy. luisa.barzon@unipd.it

ABSTRACT
Primary lymphoma of the adrenal gland is a rare and highly aggressive disease, with only a few reports in the literature. The pathogenesis is unknown, but detection of Epstein Barr virus (EBV) genome sequences and gene expression in some cases of primary adrenal lymphomas suggested the virus might be a causative agent of the malignancy. While investigating the presence of genome sequences of oncogenic viruses in a large series of adrenal tumors, both EBV and JC polyomavirus (JCV) DNA sequences were detected in a diffuse large primary bilateral B-cell non-Hodgkin lymphoma of the adrenal gland, which was diagnosed only at postmortem examination in a 77 year-old woman with incidentally discovered adrenal masses and primary adrenal insufficiency. The presence of both EBV and JCV genome sequences suggests the relevance of EBV and JCV coinfection in the pathogenesis of this rare form of B-cell lymphoma.

No MeSH data available.


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a) Non-Hodgkin lymphoma and glandular structures (H & E; original magnification 400×); b) immunostaining showing neoplastic cells positive for CD20 (original magnification 400×).
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Figure 1: a) Non-Hodgkin lymphoma and glandular structures (H & E; original magnification 400×); b) immunostaining showing neoplastic cells positive for CD20 (original magnification 400×).

Mentions: The patient was a 77-yr woman with bilateral adrenal masses which were incidentally discovered at abdominal ultrasonography performed for dispepsia and weigth loss. The past history of the patients was unremarkable except hypertension. Computed tomography (CT)-scan confirmed the presence of bilateral (right adrenal mass maximum diameter, 5 cm; left adrenal mass maximum diameter, 7 cm) solid and heterogeneous adrenal masses which probably infiltrated the liver. Brain and bone CT-scans were negative. Laboratory evaluation demonstrated the presence of anemia, increased erythrosedimentation rate, and the presence of monoclonal G immunoglobulins, while autoantibodies testing was negative. Endocrine evaluation demonstrated primary adrenal insufficiency, since all adrenal steroids were low and adrenocorticotropin levels were markedly elevated. Replacement therapy with cortisone was started, resulting in immediate improvement of symptoms. Repeated chest-abdominal CT-scan performed after 3 months showed increased mass size with areas of colliquation and the presence of left pleural effusion. Bilateral nonvisualization at adrenal scintiscan suggested the presence of malignant or space-occupying adrenal lesions [8]. Fine needle aspiration biopsy was not performed because bilateral adrenocortical carcinoma or metastases were suspected based on CT-scan and scintigraphic appearance. At 5 months from diagnosis, the patient died because of advanced disease. Postmortem examination revealed bilateral involvement of adrenal glands by encapsulated masses with a solid, friable, grayish-white cut surface and extensive necrosis. Microscopic features consisted of large transformed lymphoid cells with pleomorphic vesicular nuclei with prominent nucleoli (Fig. 1a). Upon immunohistochemical analysis, the neoplastic cells expressed the B cell marker CD20 (Fig. 1b). A diagnosis of diffuse large B-cell non-Hodgkin lymphoma was made. To investigate whether oncogenic viruses were involved in the disease, both left and right adrenal masses were examined for the presence of all human herpesviruses and polyomaviruses by quantitative real-time PCR, as reported [7]. EBV DNA was present at high titre (about 100 genome copies/cell) and JCV DNA at lower titre (about 0.1 genome copy/cell) in both adrenal masses. EBV genotyping by PCR amplification of the EBNA-2 and EBNA-3B genes [9] demonstrated EBV type 1, while sequencing of the JCV VP1 gene [10] classified JCV as type 1B (Fig. 2), which are the genotypes most commonly found in our country. Moreover, PCR-amplification of both large T antigen (TAg) and the VP1 sequences suggested that the entire JCV genome was present. EBV and JCV DNA was not detected in other tissues (lymph nodes, spleen, liver, kidney). Both immunohistochemical staining and western blot analysis of JCV TAg expression in lymphoma samples, which were performed with an anti-SV40 TAg cross-reacting antibody (Calbiochem, Anti-SV40 T Antigen Ab-2, Pab416) as reported [7], gave negative results. It cannot however be excluded that lack of JCV TAg detection was due to the relatively low number of JCV-positive cells and to the poor quality of tissue samples.


Primary bilateral adrenal B-cell lymphoma associated with EBV and JCV infection.

Barzon L, Trevisan M, Marino F, Guzzardo V, Palù G - Infect. Agents Cancer (2009)

a) Non-Hodgkin lymphoma and glandular structures (H & E; original magnification 400×); b) immunostaining showing neoplastic cells positive for CD20 (original magnification 400×).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2630304&req=5

Figure 1: a) Non-Hodgkin lymphoma and glandular structures (H & E; original magnification 400×); b) immunostaining showing neoplastic cells positive for CD20 (original magnification 400×).
Mentions: The patient was a 77-yr woman with bilateral adrenal masses which were incidentally discovered at abdominal ultrasonography performed for dispepsia and weigth loss. The past history of the patients was unremarkable except hypertension. Computed tomography (CT)-scan confirmed the presence of bilateral (right adrenal mass maximum diameter, 5 cm; left adrenal mass maximum diameter, 7 cm) solid and heterogeneous adrenal masses which probably infiltrated the liver. Brain and bone CT-scans were negative. Laboratory evaluation demonstrated the presence of anemia, increased erythrosedimentation rate, and the presence of monoclonal G immunoglobulins, while autoantibodies testing was negative. Endocrine evaluation demonstrated primary adrenal insufficiency, since all adrenal steroids were low and adrenocorticotropin levels were markedly elevated. Replacement therapy with cortisone was started, resulting in immediate improvement of symptoms. Repeated chest-abdominal CT-scan performed after 3 months showed increased mass size with areas of colliquation and the presence of left pleural effusion. Bilateral nonvisualization at adrenal scintiscan suggested the presence of malignant or space-occupying adrenal lesions [8]. Fine needle aspiration biopsy was not performed because bilateral adrenocortical carcinoma or metastases were suspected based on CT-scan and scintigraphic appearance. At 5 months from diagnosis, the patient died because of advanced disease. Postmortem examination revealed bilateral involvement of adrenal glands by encapsulated masses with a solid, friable, grayish-white cut surface and extensive necrosis. Microscopic features consisted of large transformed lymphoid cells with pleomorphic vesicular nuclei with prominent nucleoli (Fig. 1a). Upon immunohistochemical analysis, the neoplastic cells expressed the B cell marker CD20 (Fig. 1b). A diagnosis of diffuse large B-cell non-Hodgkin lymphoma was made. To investigate whether oncogenic viruses were involved in the disease, both left and right adrenal masses were examined for the presence of all human herpesviruses and polyomaviruses by quantitative real-time PCR, as reported [7]. EBV DNA was present at high titre (about 100 genome copies/cell) and JCV DNA at lower titre (about 0.1 genome copy/cell) in both adrenal masses. EBV genotyping by PCR amplification of the EBNA-2 and EBNA-3B genes [9] demonstrated EBV type 1, while sequencing of the JCV VP1 gene [10] classified JCV as type 1B (Fig. 2), which are the genotypes most commonly found in our country. Moreover, PCR-amplification of both large T antigen (TAg) and the VP1 sequences suggested that the entire JCV genome was present. EBV and JCV DNA was not detected in other tissues (lymph nodes, spleen, liver, kidney). Both immunohistochemical staining and western blot analysis of JCV TAg expression in lymphoma samples, which were performed with an anti-SV40 TAg cross-reacting antibody (Calbiochem, Anti-SV40 T Antigen Ab-2, Pab416) as reported [7], gave negative results. It cannot however be excluded that lack of JCV TAg detection was due to the relatively low number of JCV-positive cells and to the poor quality of tissue samples.

Bottom Line: The pathogenesis is unknown, but detection of Epstein Barr virus (EBV) genome sequences and gene expression in some cases of primary adrenal lymphomas suggested the virus might be a causative agent of the malignancy.While investigating the presence of genome sequences of oncogenic viruses in a large series of adrenal tumors, both EBV and JC polyomavirus (JCV) DNA sequences were detected in a diffuse large primary bilateral B-cell non-Hodgkin lymphoma of the adrenal gland, which was diagnosed only at postmortem examination in a 77 year-old woman with incidentally discovered adrenal masses and primary adrenal insufficiency.The presence of both EBV and JCV genome sequences suggests the relevance of EBV and JCV coinfection in the pathogenesis of this rare form of B-cell lymphoma.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Histology, Microbiology and Medical Biotechnologies, University of Padova, I-35121 Padova, Italy. luisa.barzon@unipd.it

ABSTRACT
Primary lymphoma of the adrenal gland is a rare and highly aggressive disease, with only a few reports in the literature. The pathogenesis is unknown, but detection of Epstein Barr virus (EBV) genome sequences and gene expression in some cases of primary adrenal lymphomas suggested the virus might be a causative agent of the malignancy. While investigating the presence of genome sequences of oncogenic viruses in a large series of adrenal tumors, both EBV and JC polyomavirus (JCV) DNA sequences were detected in a diffuse large primary bilateral B-cell non-Hodgkin lymphoma of the adrenal gland, which was diagnosed only at postmortem examination in a 77 year-old woman with incidentally discovered adrenal masses and primary adrenal insufficiency. The presence of both EBV and JCV genome sequences suggests the relevance of EBV and JCV coinfection in the pathogenesis of this rare form of B-cell lymphoma.

No MeSH data available.


Related in: MedlinePlus