Adrenal Diffuse Large B-cell Lymphoma Research Articles

Predictive value of clinical characteristics and baseline 18F-FDG PET/CT quantization parameters in primary adrenal diffuse large B-cell lymphoma: a preliminary study

Predictive value of clinical characteristics and baseline 18F-FDG PET/CT quantization parameters in primary adrenal diffuse large B-cell lymphoma: a preliminary study

Primary adrenal diffuse large B cell Lymphoma with Tumor thrombus in central adrenal vein: a case report and literature review

BackgroundPrimary adrenal lymphoma (PAL) is a rare disease confined wholly or chiefly to extramural involvement. Tumor thrombus in the central adrenal vein, renal vein, and inferior vena cava has been reported in adrenal pheochromocytoma, adrenocortical carcinoma, adrenal metastasis carcinoma, and adrenal leiomyosarcoma. Primary adrenal diffuse large B cell lymphoma with tumor thrombus in the central adrenal vein has rarely been reported in the current study. ( We searched in PubMed, Web of Science databases, Embase, and Medline in the English language from 1970 to December 2022. The keywords used were “Primary adrenal lymphoma " and " tumor thrombus”.)Case presentationIn this report, we discuss the case of a 57-year-old woman who complained of abdominal discomfort following cold stimulation, low back pain, anorexia, fatigue, and weight loss for 1 year. Contrast-enhanced spiral computed tomography (CT) showed mild-to-moderate enhancement of the bilateral masses and central adrenal vein tumor thrombus. After an exhaustive study, the patient was diagnosed with primary adrenal diffuse large B-cell lymphoma. In the diagnosis of PAL, the possibility of a tumor embolism in the central adrenal vein, renal vein, or inferior vena cava should be considered, although this is rare.

Epstein-Barr Virus-Positive Adrenal Diffuse Large B-Cell Lymphoma after Treatment for Angioimmunoblastic T-Cell Lymphoma.

We report a case of EBV-positive B-cell lymphoma of the adrenal gland after treatment for angioimmunoblastic T-cell lymphoma (AITL)When patients present with signs and symptoms suggestive of relapse after AITL treatment, another biopsy should be performed for the accurate determination of whether these lesions indicate AITL relapse or new-onset of EBV-positive B-cell lymphoma.The involvement of extranodal sites may indicate a poor prognosis of EBV-positive B-cell lymphoma after AITL treatment.

Clinicopathologic Analysis of Primary Adrenal Diffuse Large B-Cell Lymphoma: A Reappraisal of 23 Japanese Patients Based on EBV Association and PD-L1 Expression in Tumor Cells.

Primary adrenal diffuse large B-cell lymphoma (PA-DLBCL) is rare. We investigate 23 Japanese patients with PA-DLBCL to understand the clinicopathologic features and biological behavior of this disease. The 17 males and 6 females had a median age of 74 years (range: 40 to 86 y). Tumor cells harbored Epstein-Barr virus-encoded small RNA (EBER) in 9 (39%) samples, including samples from the 2 patients with methotrexate-associated B-cell lymphoproliferative disorder. Programmed cell death ligand 1 (PD-L1) expression was detected in tumor cells of 6 (26%) samples, including 1 EBER+ and 5 EBER- samples. Four (17%) patients exhibited an intravascular proliferating pattern, and all 4 patient samples showed positive staining for PD-L1 in tumor cells. Among those patients, 3 showed intravascular proliferating pattern accompanied by a diffuse extravascular proliferation of tumor cells, and 1 patient was diagnosed with intravascular large B-cell lymphoma. We divided the 23 patients into 3 groups: EBER+ (n=9, 39%), EBER-PD-L1+ (n=5, 22%), and EBER-PD-L1- (n=9, 39%). A comparison of the outcomes among the 3 groups showed significant differences in overall survival (P=0.034). The EBER+ group had the worst prognosis, and the EBER-PD-L1- group had the best prognosis. We also compared the outcomes among the 3 groups that received rituximab-containing chemotherapies. Both the overall survival and progression-free survival were significantly different among these groups (P<0.001 and P=0.002, respectively). In conclusion, we evaluated 3 types of PA-DLBCL and found that each had unique clinical, pathologic, and prognostic features. Our results suggested that immune senescence, iatrogenic immunodeficiency, and immune evasion contribute to the development of PA-DLBCL.

Synchronous gastric cancer and primary lymphoma of right adrenal gland: a case report

This report presents an extremely rare case of synchronous gastric cancer and primary adrenal diffuse large B-cell lymphoma (DLBCL). An 82-year-old man underwent computed tomography, which revealed a heterogeneous appearing and hypodense adrenal mass and a gastric mass with no enlarged lymph nodes in the neck, mediastinum, abdomen, and inguinal region. Upper gastrointestinal endoscopy revealed a protruding gastric tumor. The specimens obtained from endoscopic biopsy were histologically confirmed to be adenocarcinoma. The hormonal findings eliminated functional adrenal tumor. The patient underwent distal gastrectomy with regional lymph node resection for gastric cancer and incisional biopsy of the adrenal mass. Based on the pathological findings, diagnoses of mixed mucinous and tubular adenocarcinomas of the stomach and adrenal DLBCL were confirmed. Postoperation, the patient received rituximab combined with low-dose doxorubicin, cyclophosphamide, vincristine, and prednisone (R-miniCHOP). Six courses of R-miniCHOP were planned, but were completed in only one course at the patient’s request. The patient died 2 months after surgery.

Unusual bilateral adrenal tumors: Primary adrenal diffuse large B-cell lymphoma

Primary adrenal non-Hodgkin lymphoma is an extremely rare disease confined wholly or chiefly to extranodal involvement. We report the case of an old woman who presented with progressive malaise and was diagnosed with primary adrenal diffuse large B-cell lymphoma pathology using laparoscopic adrenalectomy. Enhanced computed tomography and positron emission tomography revealed bilateral adrenal involvement.

SAT-233 Primary Adrenal Diffuse Large B-Cell Lymphoma in HIV Patient: A Rare Case Report

Introduction: Primary adrenal lymphoma is remarkably rare type of extra-nodal lymphomas, accounting for <1% of non-Hodgkin lymphomas. For unclear reasons, HIV population appear to have an increased risk of developing non-Hodgkin lymphoma. We are presenting a rare case of bilateral primary adrenal non-Hodgkin lymphoma in HIV patient. Case Presentation: A 65-year-old man with history of HIV, presents with nausea and generalized weakness for the past month. Other symptoms include, fever and unintentional weight loss of 10 kg also reported.On admission, patient was found to have low sodium value of 124 mmol/L. He was worked up for hyponatremia; Labs were suggestive of primary adrenal insufficiency. Other endocrinology labs including; TSH, metanephrines, renin, and aldosterone were within normal values.CT of the abdomen showed bilateral adrenal masses. The right lesion measured 10.6 cm. The left lesion measured 10.1 cm. Fungal work up was negative. Epstein Barr virus (EBV) panel was suggestive of convalescent versus reactivated disease.CT guided biopsy of the adrenal mass confirmed Diffuse Large B-cell Lymphoma, activated B Cell Phenotype. Bone marrow biopsy showed no evidence of lymphoma.Following oncology recommendations, patient was started on R-CHOP therapy. Currently, patient received two cycles of chemotherapy which he is tolerating well. Discussion: Primary adrenal lymphoma is a rare type of non-Hodgkin lymphomas (NHL). Diffuse large B cell lymphoma (DLBCL) is the most common histologic subtype of NHL that present in the adrenal glands. Approximately one hundred cases reported of primary adrenal DLBCL in the literature with only two previously reported cases in HIV patients. It is considered an AIDS defining malignancy. HIV population are at higher risk of developing NHL than general population. The pathogenesis of the development of NHL in adrenal glands, which lack lymphoid tissue, particularly in HIV patients is yet to be determined, but impaired cellular immunity and chronic B cell stimulation and T cell immunodeficiency leading to loss of control of transforming viruses, particularly EBV are thought to play essential roles.Because of the rarity of this tumor, diagnosis can be challenging. As exemplified in our patient, most cases have nonspecific presentation, including; fever, weight loss, night sweats, nausea, vomiting, adrenal insufficiency, and hyprecalcemia.The recommended treatment for primary adrenal DLBCL is chemotherapy, with R-CHOP being the typical treatment. This disease appears to have a poor prognosis, and the median survival is approximately 1 year. Young age, good performance status, and early diagnosis and treatment can improve the outcome. Conclusion: Primary adrenal DLBCL are rare tumors that has been associated in the literature with impaired cellular immunity. Our patient is the third case reported of primary adrenal DLBCL in HIV positive patient.

Morphologic Patterns and the Correlation With MYD88 L265P, CD79B Mutations in Primary Adrenal Diffuse Large B-Cell Lymphoma.

Primary adrenal diffuse large B-cell lymphoma (PA-DLBCL) is a rare subtype of extranodal DLBCL. Because of the rarity of this disease, its morphologic and genetic features are not comprehensively studied. Here, we systematically reviewed the clinicopathologic features of 42 cases of PA-DLBCL from our institution and investigated the frequency of MYD88 L265P and CD79B (exon 5) mutation in 29 eligible cases using Sanger sequencing. Clinically, PA-DLBCL was predominant in elderly male patients with advanced clinical stage and poor outcomes. Morphologically, the tumors often showed a sinusoidal and/or cohesive pattern with condensed chromatin and inconspicuous nucleolus which mimicked neuroendocrine carcinoma. Moreover, increased Reed-Sternberg-like cells were observed frequently. These confounding morphologic manifestations may lead to misdiagnosis. Genetically, PA-DLBCL harbored a high prevalence of MYD88 L265P (24%) and CD79B mutations (52%) which may be involved in lymphomagenesis. The CD79B mutation was significantly associated with a worse prognosis. A novel Histo-Molecular Classification system (4 categories) was proposed based on correlation with genetic changes. Generally, the neuroendocrine carcinoma-like type was associated with CD79B mutation, whereas the RS-like cell type indicated MYD88 L265P. The biphasic type was correlated with coexisting mutations of MYD88 and CD79B, whereas the common type implied no mutation. Furthermore, the common type showed significantly better survival. In conclusion, the proposed new category system could indicate the genetic changes as well as facilitate risk stratification to guide treatment and predict prognosis. Although this study augmented our understanding of PA-DLBCL, further analysis is required to validate our results and extend them to extranodal DLBCL at other sites.

Adrenal insufficiency secondary to bilateral adrenal diffuse large B-cell lymphoma: a case report

Adrenal insufficiency secondary to bilateral adrenal diffuse large B-cell lymphoma: a case report

MON-342 Calcitriol-Mediated Hypercalcemia in Adrenal Diffuse Large B-Cell Lymphoma

Background: Hypercalcemia of malignancy can present with a suppressed intact PTH level in response to various etiologies including elevated PTH-rP, increased calcitriol production via activation of the enzyme 1-alpha hydroxylase, or osteolytic bone lesions. PTH-independent hypercalcemia with elevated 1,25-dihydroxyvitamin D (calcitriol) levels should prompt evaluation for granulomatous disorders including sarcoidosis, tuberculosis, and lymphoma. Here we present a case of calcitriol-mediated hypercalcemia secondary to a rare, adrenal diffuse large B-cell lymphoma. Clinical Case: A 61-year-old female presented with fatigue, myalgias, and generalized weakness. On admission, calcium was 14.1 mg/dL (8.9-10.3), phosphorus 2.4 mg/dL (2.3-4) and iPTH was suppressed to 3.6 pg/mL (8.5-75). CT scan revealed a 30-35 Houndsfield unit, 14 x 9 x 13 cm left adrenal mass. Workup included PTH-rP 0.3 pmol/L (<2.0) and calcitriol 109 pg/mL (18-78). Serum and urine electrophoresis were negative for monoclonal protein spikes. Further hormonal evaluation revealed normal serum and urine metanephrines, 24-hour urine cortisol 64 mcg/24h (3.5-45), 8 AM cortisol 1.9 ug/dL following 1 mg dexamethasone suppression (<1.8), midnight salivary cortisol 87 ng/dL (<100), DHEA-S 16.2 mcg/dL (<15-157), and 11-deoxycortisol 24 ng/dL (10-79). Angiotensin-converting enzyme level was normal at 10 U/L (8-53). Her hypercalcemia improved with intravenous fluids and calcitonin. She was not a surgical candidate as PET/CT revealed multiple para-aortic and mesenteric hypermetabolic lymphadenopathy, left breast soft tissue density, and a lesion in the right posterior pelvis likely indicative of metastatic disease. Nuclear bone scan ruled out bone metastases. Para-aortic biopsy was considered technically difficult in this patient so she underwent CT-guided core needle biopsy of the adrenal mass which confirmed high-grade CD5-positive diffuse large B-cell lymphoma. Immunohistochemical staining was positive for CD20, BCL2, BCL6, MUM-1, and Ki-67 90%. She completed 6 cycles of chemotherapy with rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisone (R-CHOP). Repeat PET/CT following completion of chemotherapy showed no residual disease however one month later, she presented with acute encephalopathy with MRI brain showing a large frontal lobe tumor concerning for relapse. She received 1 cycle of intrathecal methotrexate and cytarabine however these were discontinued due to severe deconditioning. She now remains on oral ibrutinib. Conclusions: Primary adrenal lymphomas are a rare cause of non-Hodgkin lymphomas with less than 150 cases noted in the literature. Lymphoma should be included in the differential of patients presenting with PTH-independent hypercalcemia given the aggressive nature of the disease and associated high mortality.

Primary adrenal diffuse large B cell lymphoma: a clinicopathological and molecular study from China.

Primary adrenal lymphoma is a rare entity that, in most cases, is derived from B cells. The most commonly seen primary adrenal lymphoma is diffuse large B cell lymphoma (DLBCL). To better understand the clinicopathological and molecular features of these tumors, we studied 14 Chinese patients with DLBCL who initially presented with an adrenal tumor. The clinicopathological features of the 14 primary adrenal DLBCL cases were retrospectively reviewed using immunohistochemistry, immunoglobulin gene rearrangement analysis, evaluation of Epstein-Barr virus status, and fluorescence in situ hybridization. Patient age ranged from 43 to 69years, with a mean age of 58years. The patients most commonly presented with abdominal pain and adrenal mass. Ten patients exhibited bilateral adrenal masses, and four had unilateral adrenal masses (three left, one right). Thirteen of 14 DLBCLs were DLBCL not otherwise specified, and one was an intravascular large B cell lymphoma. According to the algorithm of Hans et al. (Blood 103:275-282, 2004), 13 and 1 cases were classified as the non-germinal center B-cell-like subtype and the germinal center B-cell-like subtype, respectively. The Ki-67 index ranged from 35 to 80%. Epstein-Barr virus-encoded RNA was detected by in situ hybridization in 6 of the 12 available cases. Two patients showed BCL-6 rearrangements. The follow-up period ranged from 1 to 87months. During the follow-up period, four patients died of the disease, five were alive with the disease, four were alive without disease, and one was lost during the follow-up period. In summary, most primary adrenal lymphomas are non-germinal-center B-cell-like subtype DLBCLs, which have high proliferative activity and a poor prognosis.

Bilateral Primary Adrenal Lymphoma in a 59- year-old Female

Occasionally, lymphoma involves the adrenal glands; however, primary adrenal lymphoma (PAL) is rare and only few cases have been reported. We report a case of a 59-year-old female with primary adrenal diffuse large B-cell lymphoma (DLBCL) manifested by weakness, fatigue, anorexia, and hyper pigmentation of skin. The patient initially responded to intravenous hydrocortisone in large doses by total remission of the symptoms. An abdominal computed tomography scan showed a hypodense mass in the right subdiaphragmatic space, which was suggestive of an adrenal gland tumor with adjacent liver involvement. Additionally, a smaller lesion with similar characteristics was found in the left adrenal gland. The results obtained from adrenal gland needle biopsy confirmed the diagnosis of DLBCL. Moreover, primary laboratory findings demonstrated hyponatremia, hyperkalemia, fasting blood sugar level of 153 mg/dl, and alkaline phosphatase level of 663 U/L. The mentioned symptoms and laboratory findings were indicative of adrenal insufficiency. After about 2 months, her level of consciousness decreased and urinary and fecal incontinence occurred. Therefore, brain involvement was suspected, and magnetic resonance imaging of the brain showed heterogeneous enhancement (24.8 mm) in the posterior aspect of the left temporal lobe together with patchy foci of enhancement in around the ventricular areas of the brain that showed metastatic lesions of PAL. This case should remind clinicians that PAL may be a cause of adrenal incidentaloma, especially when the patient presents with the symptoms of adrenal insufficiency.

Primary Adrenal Diffuse Large B-cell Lymphoma: A Mini Review

ABSTRACT Background Primary adrenal diffuse large B-cell lymphoma are aggressive high grade lymphomas affecting elderly gentlemen and mostly present with bilateral enlarged adrenal masses associated with B symptoms, adrenal insufficiency and elevated lactate dehydrogenase. When imaged with CT or ultrasound, these lesions usually appear as heterogeneous complex large masses with low density. They appear metabolically active on PET scan. Confirmatory diagnosis is established by image guided biopsy or surgical excision. Treatment is usually with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) or CHOP like regimes. Surgery has very limited role, if any in the management of this condition. The prognosis is generally poor with only about a third of patients achieving partial or complete remission following treatment. A concise review of the literature (PubMed database; 1990–2014) on the clinical management of primary adrenal lymphoma along with a case example is discussed. How to cite this article Parameswaran R, Chan DKH, Michelle PLM, Wang S. Primary Adrenal Diffuse Large B-cell Lymphoma: A Mini Review. World J Endoc Surg 2015;7(3):65-68.

Pituitary and adrenal involvement in diffuse large B-cell lymphoma, with recovery of their function after chemotherapy

BackgroundDiffuse large B-cell lymphoma sometimes involves the endocrine organs, but involvement of both the pituitary and adrenal glands is extremely rare. Involvement of these structures can lead to hypopituitarism and adrenal insufficiency, and subsequent recovery of their function is rarely seen. The present report describes an extremely rare case of pituitary and adrenal diffuse large B-cell lymphoma presenting with hypopituitarism and adrenal insufficiency with subsequent recovery of pituitary and adrenal function after successful treatment of the lymphoma.Case presentationA 63-year-old Japanese man was referred to our hospital due to miosis, ptosis, hypohidrosis of his left face, polydipsia and polyuria. 18F-fluorodeoxy glucose positron emission tomography / computed tomography revealed hotspots in the pituitary gland, bilateral adrenal glands and the apex of his left lung. Surgical biopsy from the pituitary lesion confirmed the diagnosis of diffuse large B-cell lymphoma, with lymphoma cells replacing normal pituitary tissue. Endocrine function tests revealed adrenal insufficiency and panhypopituitarism, including a possible affection of the posterior pituitary. Hormone replacement therapy with desmopressin and hydrocortisone was started. Chemotherapy consisted of six courses of R-CHOP (rituximab, cyclophosphamide, vincristine, doxorubicin and prednisolone) and two courses of high-dose methotrexate followed by autologous hematopoietic stem cell transplantation. Subsequently, his pituitary and bilateral adrenal lesions resolved, and serial endocrine function tests showed gradual improvement in pituitary and adrenal function.ConclusionsThe present report describes an extremely rare case of diffuse large B-cell lymphoma with involvement of both the pituitary and bilateral adrenal glands. R-CHOP and high-dose methotrexate therapy followed by autologous hematopoietic stem cell transplantation was quite effective, and panhypopituitarism and adrenal insufficiency improved to almost normal values after successful treatment of the lymphoma with chemotherapy.

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

BackgroundPrimary 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.MethodsFrom 2008 to 2011 we experienced seven cases of PAL in our institutions. We retrospectively analyzed the clinical and pathological features of these patients.ResultsThe 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.ConclusionsPrimary 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.

両側性副腎原発悪性リンパ腫の1例

両側性副腎原発悪性リンパ腫の1例

Prognostic factors in primary diffuse large B-cell lymphoma of adrenal gland treated with rituximab-CHOP chemotherapy from the Consortium for Improving Survival of Lymphoma (CISL)

BackgroundThe objective of this study was to identify prognostic factors for survival in patients with primary diffuse large B-cell lymphoma (DLBCL) of the adrenal gland.MethodsThirty one patients diagnosed with primary adrenal DLBCL from 14 Korean institutions and treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) were analyzed.ResultsComplete remission (CR) and overall response rate after R-CHOP chemotherapy were 54.8% and 87.0%. The 2-year estimates of overall survival (OS) and progression-free survival (PFS) were 68.3% and 51.1%. In patients achieving CR, significant prolongations of OS (P = 0.029) and PFS (P = 0.005) were observed. Ann Arbor stage had no influence on OS. There was no significant difference in OS between patients with unilateral involvement of adrenal gland and those with bilateral involvement. When staging was modified to include bilateral adrenal involvement as one extranodal site, early stage (I or II) significantly correlated with longer OS (P = 0.021) and PFS (P <0.001).ConclusionsContrary to prior reports, our data suggests that outcomes of primary adrenal DLBCL are encouraging using a regimen of R-CHOP, and that achieving CR after R-CHOP is predictive of survival. Likewise, our modified staging system may have prognostic value.

Clinical analysis of 10 AIDS patients with malignant lymphoma.

ObjectiveThis work summarizes the clinical features and treatment of 10 AIDS patients with malignant lymphoma.MethodsA total of 10 AIDS patients with malignant lymphoma seen in Beijing Ditan Hospital since 2009 were enrolled. Clinical manifestations, pathological examinations, immunity levels, Epstein-Barr virus antibody examinations, complications, treatments, and outcomes were retrospectively analyzed.ResultsThe main clinical manifestations of these patients included intermittent fever in 2 cases, neck masses and fever in 3 cases, auxiliary lymph node enlargement in 2 cases, and abdominal pain and bloating with fever in 3 cases. Up to 7 patients were pathologically diagnosed with diffuse large B cell lymphoma (DLBCL), and 3 patients were pathologically diagnosed with Burkitt’s lymphoma. Up to 8 patients had CD4 cell counts below 200/µL, and 2 patients had a level of more than 200/µL. Up to 7 patients were negative for EBV-IgM antibodies and 3 patients were not examined. Six patients underwent different chemotherapy and their prognoses were different. One patient with Burkitt’s lymphoma alternatively took CODOXM and IVAC for 3 turns after VP chemotherapy; 1 patient with liver metastasis took R-CHOP 5 times, then changed therapy regimen to R-MINE and MINE. One patient with adrenal DLBCL took CHOP 6 times. Three patients with DLBCL took CHOP 1 or 2 times. Four patients gave up treatment. Various infections and side effects occurred, including bone marrow suppression, gastrointestinal bleeding, and renal dysfunction during chemotherapy. Six patients took HAART, and 4 did not. Six patients died, whereas 3 patients got improved; and 1 patient was discharged.ConclusionsAIDS patients with malignant lymphoma had various clinical manifestations, were immunocompromised, and had multiple metastases when they were admitted; they were already in the interim or late stage of lymphoma. Chemotherapy was not effective, and additional complications occurred. HAART failed to improve patient prognosis, and the overall prognosis was poor.

Complete remission of rapidly-progressing, unilateral, primary adrenal diffuse large B-cell lymphoma with surgery and rituximab-chop chemotherapy

Complete remission of rapidly-progressing, unilateral, primary adrenal diffuse large B-cell lymphoma with surgery and rituximab-chop chemotherapy

Complete remission of primary bilateral adrenal lymphoma achieved by Rituximab-CHOP chemotherapy

AbstractPrimary adrenal lymphoma (PAL) is an exceedingly rare disease. Because of its rareness, uncertain etiology, variable duration of survival, unremarkable clinical presentations and unsatisfied therapeutic strategies, its treatment has always been unsatisfactory. Here we report a 50-year-old male patient with primary bilateral adrenal diffuse large B-cell lymphoma. He was treated with surgery followed by combined chemotherapy using R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone). He achieved complete remission after 3 cycles of chemotherapy. At present, he is considered free of tumor at 3 years after chemotherapy. Our case report demonstrates that patients with primary bilateral adrenal diffuse large B-cell lymphoma may achieve a good outcome using R-CHOP chemotherapy. To the best of our knowledge, our patient who achieved complete remission after R-CHOP chemotherapy has had the longest survival as published in the literature.