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Clinicopathological analysis of primary adrenal diffuse large B-cell lymphoma: effectiveness of rituximab-containing chemotherapy including central nervous system prophylaxis.

Ichikawa S, Fukuhara N, Inoue A, Katsushima H, Ohba R, Katsuoka Y, Onishi Y, Yamamoto J, Sasaki O, Nomura J, Fukuhara O, Ishizawa K, Ichinohasama R, Harigae H - Exp Hematol Oncol (2013)

Bottom Line: Bilateral adrenal involvement was confirmed in five patients.The overall survival rate at two years was 57% (median follow-up; 24.8 months).Primary adrenal DLBCL can be a clinically aggressive disease entity.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Sendai 980-8574, Japan. nfukuhara@med.tohoku.ac.jp.

ABSTRACT

Background: Primary adrenal lymphoma (PAL) is an extremely rare subtype of extranodal non-Hodgkin's lymphoma. Some researchers have reported some of the characteristics of PAL and its association with poor prognosis; however, the clinicopathological features of PAL remain to be elucidated.

Methods: From 2008 to 2011 we experienced seven cases of PAL in our institutions. We retrospectively analyzed the clinical and pathological features of these patients.

Results: The patients ranged in age from 50 to 85 years, with a median of 71 years. The overall male:female ratio was 6:1. All seven patients were diagnosed with diffuse large B-cell lymphoma (DLBCL) pathologically. Bilateral adrenal involvement was confirmed in five patients. The median largest tumor diameter at diagnosis was 58 mm. The Ki-67 index was generally high (>70%). All patients were treated with rituximab-containing chemotherapy, and central nervous system (CNS) prophylaxis was conducted for three patients. One patient with CNS involvement at the time of the diagnosis also received whole-brain radiation. The overall survival rate at two years was 57% (median follow-up; 24.8 months). It is noteworthy that the three patients who received a full course of the rituximab-containing regimen and CNS prophylaxis are currently alive without disease relapse, and that none of the seven patients died due to progression of lymphoma.

Conclusions: Primary adrenal DLBCL can be a clinically aggressive disease entity. Rituximab-containing chemotherapy combined with CNS prophylaxis could be a reasonable option for the treatment of PAL; however, analyses of more PAL cases are needed for the establishment of this strategy.

No MeSH data available.


Related in: MedlinePlus

Representative pathological findings of primary adrenal diffuse large B-cell lymphoma (Case 1). The black lines in the lower right corner of each figure indicate 100 μm. (A, B) The results of hematoxylin and eosin staining. On low-power magnification (A), the lymphoma was located in the zona reticularis (represented by r) of the adrenal cortex. f and g represent the zona fasiculata and zona glomerulosa, respectively. High-power magnification (B) showed medium- to large-sized atypical lymphoid cells with dispersed chromatin. (C) Immunohistochemical staining for CD20 showed that the large lymphoma cells were positive for CD20. (D) Immunohistochemical staining for CD3 showed reactivity in small lymphocytes, but not in large lymphoma cells.
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Figure 1: Representative pathological findings of primary adrenal diffuse large B-cell lymphoma (Case 1). The black lines in the lower right corner of each figure indicate 100 μm. (A, B) The results of hematoxylin and eosin staining. On low-power magnification (A), the lymphoma was located in the zona reticularis (represented by r) of the adrenal cortex. f and g represent the zona fasiculata and zona glomerulosa, respectively. High-power magnification (B) showed medium- to large-sized atypical lymphoid cells with dispersed chromatin. (C) Immunohistochemical staining for CD20 showed that the large lymphoma cells were positive for CD20. (D) Immunohistochemical staining for CD3 showed reactivity in small lymphocytes, but not in large lymphoma cells.

Mentions: The pathological and immunohistochemical findings of all seven cases are shown in Table 2, and representative pathological findings are shown in Figure 1. There were no unique morphological characteristics of the PAL cases. All cases were diagnosed as DLBCL. The tumor cells expressed CD20 (7 of 7), CD79a (6 of 6), BCL2 (5 of 7), BCL6 (4 of 7) and MUM1 (5 of 7). CD10 was negative in all cases. CD5 was positive in two cases. The Ki-67 index was generally high (> 70%). A flow cytometric analysis of the lymphoma cells (data not shown) was available for Cases 6 and 7, and did not conflict with the immunohistochemical data described above. According to Hans’ criteria [10], most of the cases (6 of 7) were classified as the non-germinal center B-cell subtype.


Clinicopathological analysis of primary adrenal diffuse large B-cell lymphoma: effectiveness of rituximab-containing chemotherapy including central nervous system prophylaxis.

Ichikawa S, Fukuhara N, Inoue A, Katsushima H, Ohba R, Katsuoka Y, Onishi Y, Yamamoto J, Sasaki O, Nomura J, Fukuhara O, Ishizawa K, Ichinohasama R, Harigae H - Exp Hematol Oncol (2013)

Representative pathological findings of primary adrenal diffuse large B-cell lymphoma (Case 1). The black lines in the lower right corner of each figure indicate 100 μm. (A, B) The results of hematoxylin and eosin staining. On low-power magnification (A), the lymphoma was located in the zona reticularis (represented by r) of the adrenal cortex. f and g represent the zona fasiculata and zona glomerulosa, respectively. High-power magnification (B) showed medium- to large-sized atypical lymphoid cells with dispersed chromatin. (C) Immunohistochemical staining for CD20 showed that the large lymphoma cells were positive for CD20. (D) Immunohistochemical staining for CD3 showed reactivity in small lymphocytes, but not in large lymphoma cells.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3750298&req=5

Figure 1: Representative pathological findings of primary adrenal diffuse large B-cell lymphoma (Case 1). The black lines in the lower right corner of each figure indicate 100 μm. (A, B) The results of hematoxylin and eosin staining. On low-power magnification (A), the lymphoma was located in the zona reticularis (represented by r) of the adrenal cortex. f and g represent the zona fasiculata and zona glomerulosa, respectively. High-power magnification (B) showed medium- to large-sized atypical lymphoid cells with dispersed chromatin. (C) Immunohistochemical staining for CD20 showed that the large lymphoma cells were positive for CD20. (D) Immunohistochemical staining for CD3 showed reactivity in small lymphocytes, but not in large lymphoma cells.
Mentions: The pathological and immunohistochemical findings of all seven cases are shown in Table 2, and representative pathological findings are shown in Figure 1. There were no unique morphological characteristics of the PAL cases. All cases were diagnosed as DLBCL. The tumor cells expressed CD20 (7 of 7), CD79a (6 of 6), BCL2 (5 of 7), BCL6 (4 of 7) and MUM1 (5 of 7). CD10 was negative in all cases. CD5 was positive in two cases. The Ki-67 index was generally high (> 70%). A flow cytometric analysis of the lymphoma cells (data not shown) was available for Cases 6 and 7, and did not conflict with the immunohistochemical data described above. According to Hans’ criteria [10], most of the cases (6 of 7) were classified as the non-germinal center B-cell subtype.

Bottom Line: Bilateral adrenal involvement was confirmed in five patients.The overall survival rate at two years was 57% (median follow-up; 24.8 months).Primary adrenal DLBCL can be a clinically aggressive disease entity.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Sendai 980-8574, Japan. nfukuhara@med.tohoku.ac.jp.

ABSTRACT

Background: Primary adrenal lymphoma (PAL) is an extremely rare subtype of extranodal non-Hodgkin's lymphoma. Some researchers have reported some of the characteristics of PAL and its association with poor prognosis; however, the clinicopathological features of PAL remain to be elucidated.

Methods: From 2008 to 2011 we experienced seven cases of PAL in our institutions. We retrospectively analyzed the clinical and pathological features of these patients.

Results: The patients ranged in age from 50 to 85 years, with a median of 71 years. The overall male:female ratio was 6:1. All seven patients were diagnosed with diffuse large B-cell lymphoma (DLBCL) pathologically. Bilateral adrenal involvement was confirmed in five patients. The median largest tumor diameter at diagnosis was 58 mm. The Ki-67 index was generally high (>70%). All patients were treated with rituximab-containing chemotherapy, and central nervous system (CNS) prophylaxis was conducted for three patients. One patient with CNS involvement at the time of the diagnosis also received whole-brain radiation. The overall survival rate at two years was 57% (median follow-up; 24.8 months). It is noteworthy that the three patients who received a full course of the rituximab-containing regimen and CNS prophylaxis are currently alive without disease relapse, and that none of the seven patients died due to progression of lymphoma.

Conclusions: Primary adrenal DLBCL can be a clinically aggressive disease entity. Rituximab-containing chemotherapy combined with CNS prophylaxis could be a reasonable option for the treatment of PAL; however, analyses of more PAL cases are needed for the establishment of this strategy.

No MeSH data available.


Related in: MedlinePlus