Primary Bilateral Adrenal Lymphoma Research Articles

Primary bilateral adrenal lymphoma revealed by hemophagocytic syndrome

Primary adrenal lymphoma is rare. It is often bilateral and in most of the cases of B-cell type. The clinical features are various and not specific. We report a case of a 69-year-old woman who had a diffuse large B-cell lymphoma associated with hemophagocytic syndrome. The abdominal imaging reveals the existence of bilateral adrenal hypertrophy. A CT scan-guided biopsy concluded to a diffuse large B-cell lymphoma CD 20-positive associated EBV. The treatment consisted on “CHOP like” chemotherapy associated with rituximab. Primary adrenal lymphoma has a poor prognosis, even more poorly if associated with hemophagocytic syndrome.

Primary Bilateral Adrenal Non-Hodgkin's Lymphoma Presented with Adrenal Insufficiency: A Case Report

Primary Bilateral Adrenal Non-Hodgkin's Lymphoma Presented with Adrenal Insufficiency: A Case Report

Bilateral primary adrenal lymphoma

A 51-year-old man presented with abdominal pain. Abdominopelvic magnetic resonance imaging (MRI) revealed inhomogeneous tumours involving both adrenal glands, with the right gland measuring about 14 cm and the left 11 cm in maximum diameter (Figure). A percutaneous ultrasound-guided biopsy of the right adrenal gland was performed. Diffuse large B-cell non-Hodgkin lymphoma was diagnosed by histopathological examination. The serum biochemistry profile, adrenocorticotropic hormone (ACTH) stimulation test and urine analysis were normal. The patient received six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy; complete remission was achieved after three cycles. At present, 6 months after chemotherapy, the patient is considered tumour-free. Primary adrenal lymphoma is rare. The majority of the patients are elderly men with bilateral adrenal involvement and the most common histology is diffuse large B-cell lymphoma. The importance of early awareness of the possibility of this diagnosis in patients with bilateral adrenal masses complicated by adrenal insufficiency should be emphasized. Definitive diagnosis is established by image-guided biopsy or by surgical excisional biopsy. On computed tomography and MRI imaging, primary adrenal lymphomas tend to appear as complex masses of variable density, often with areas of necrosis and/or haemorrhage; most tumours are bilateral. An appropriate treatment of this lymphoma is not well established. Therapeutic modalities include multi-agent chemotherapy and surgery followed by chemotherapy and/or radiation therapy. Most of these tumours are highly aggressive and treatment is not satisfactory. Usually, the prognosis is poor with early death occurring during chemotherapy. However, one-third of patients achieve partial or complete remission during chemotherapy.

Primary Bilateral Adrenal Diffuse Large B-cell Lymphoma Demonstrating Adrenal Failure

Primary adrenal lymphoma (PAL) is extremely rare although involvement of malignant lymphoma into adrenals is common. We report a case of a 58-year-old man with bilateral PAL who demonstrated adrenal insufficiency. Primary large B-cell lymphoma was proven by a computed tomography-guided needle biopsy of the adrenal tumor. Although a complete remission was once achieved by combination chemotherapy plus rituximab, a recurrence occurred with brain metastasis leading to his death. We concluded that PAL should be considered as a possible cause of bilateral adrenal incidentalomas with progressive adrenal insufficiency.

Uncertainty and discordance in the staging and prognosis of diffuse large B‐cell lymphoma with isolated bilateral testicular involvement

American Journal of HematologyVolume 84, Issue 11 p. 762-763 LetterFree Access Uncertainty and discordance in the staging and prognosis of diffuse large B-cell lymphoma with isolated bilateral testicular involvement† Ronald S. Go, Corresponding Author Ronald S. Go [email protected] Center for Cancer and Blood Disorders, Gundersen Lutheran Health System, La Crosse, WisconsinCenter for Cancer and Blood Disorders, Gundersen Lutheran Health System, Mail Stop: EB2-001, 1900 South Avenue, La Crosse 54601, WI 54601Search for more papers by this authorJacob D. Gundrum, Jacob D. Gundrum Department of Medical Research, Gundersen Lutheran Medical Foundation, La Crosse, WisconsinSearch for more papers by this author Ronald S. Go, Corresponding Author Ronald S. Go [email protected] Center for Cancer and Blood Disorders, Gundersen Lutheran Health System, La Crosse, WisconsinCenter for Cancer and Blood Disorders, Gundersen Lutheran Health System, Mail Stop: EB2-001, 1900 South Avenue, La Crosse 54601, WI 54601Search for more papers by this authorJacob D. Gundrum, Jacob D. Gundrum Department of Medical Research, Gundersen Lutheran Medical Foundation, La Crosse, WisconsinSearch for more papers by this author First published: 10 August 2009 https://doi.org/10.1002/ajh.21522Citations: 4 † Conflict of interest: Nothing to report. AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL References 1 Carbone PP,Kaplan HS,Musshoff K, et al. Report of the committee on Hodgkin's disease staging classification. Cancer Res 1971; 31: 1860– 1861. 2 Gospodarowicz M. Individuality versus conformity. Is perfection necessary in staging and prognostic classifications of specific primary extranodal lymphomas? Ann Oncol 1999; 10: 1405– 1407. 3 Armitage JO. Staging non-Hodgkin lymphoma. CA Cancer J Clin 2005; 55: 368– 376. 4 Zucca E,Conconi A,Mughal TI, et al. Patterns of outcome and prognostic factors in primary large-cell lymphoma of the testis in a survey by the international extranodal lymphoma study group. J Clin Oncol 2003; 21: 20– 27. 5 Bentley RC,Devlin B,Kaufman RE, et al. Genotypic divergence precedes clinical dissemination in a case of synchronous bilateral B-cell malignant lymphoma of the testes. Hum Pathol 1993; 24: 675– 678. 6 Salvatore JR,Ross RS. Primary bilateral adrenal lymphoma. Leuk Lymphoma 1999; 34: 111– 117. 7 Ryan G,Martinelli G,Kuper-Hommel M, et al. Primary diffuse large B-cell lymphoma of the breast: Prognostic factors and outcomes of a study by the international extranodal lymphoma study group. Ann Oncol 2008; 19: 233– 241. 8 Hoffman PM,McKelvie P,Hall AJ, et al. Intraocular lymphoma: A series of 14 patients with clinicopathological features and treatment outcomes. Eye 2003; 17: 513– 521. 9 Kojima M,Shimizu K,Nishikawa M, et al. Primary salivary gland lymphoma among Japanese: A clinicopathological study of 30 cases. Leuk Lymphoma 2007; 48: 1793– 1798. 10 Decaudin D,de Cremoux P,Vincent-Salomon A, et al. Ocular adnexal lymphoma: A review of clinicopathologic features and treatment options. Blood 2006; 108: 1451– 1460. 11 Cordier JF,Chailleux E,Lauque D, et al. Primary pulmonary lymphomas. A clinical study of 70 cases in nonimmunocompromised patients. Chest 1993; 103: 201– 208. 12 Cortelazzo S,Mian M,Rossi A, et al. Primary extranodal diffuse large B-cell lymphomas (DLBCL) arising in distinct sites of head and neck have different clinical characteristics and outcome. Haematologica 2007; 92 (s1). Abstract 0863. 13 Rohatiner A,d'Amore F,Coiffier B, et al. Report on a workshop convened to discuss the pathological and staging classifications of gastrointestinal tract lymphoma. Ann Oncol 1994; 5: 397– 400. 14Surveillance, Epidemiology, and End Results. Available at: http://seer.cancer.gov/Accessed on July 2, 2009. Citing Literature Volume84, Issue11November 2009Pages 762-763 ReferencesRelatedInformation

Primary Bilateral Adrenal Non-Hodgkin's Lymphoma Associated With Normal Adrenal Function

Primary bilateral adrenal non-Hodgkin's lymphoma is rare. Adrenal insufficiency or adrenal failure as a result of tumor destruction is the main pathophysiological change of most cases. Normal adrenal function despite bulky bilateral adrenal masses is extremely rare. We present a case of primary bilateral adrenal non-Hodgkin's lymphoma associated with normal adrenal function. Positron emission tomography-computed tomography is helpful to the diagnosis.

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

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.

A Case of Primary Adrenal Diffuse Large B-cell Lymphoma Achieving Complete Remission with Rituximab-CHOP Chemotherapy

Primary adrenal lymphoma is a very rare extranodal lymphoma; its clinical features consist of a high incidence of bilateral adrenal involvement and diffuse large B-cell lymphoma. We report a patient with primary bilateral adrenal diffuse large B-cell lyphoma who achieved complete remission with R-CHOP (rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. A 52-yr-old man presented with fever and progressive fatigue for 3 months. Computed tomography (CT) scans of the abdomen and pelvis demonstrated large bilateral adrenal masses, and a needle biopsy of the left adrenal mass revealed diffuse large B-cell lymphoma. After 6 cycles of R-CHOP chemotherapy, CT scans showed no residual disease. To our knowledge, this is the second report to date of a patient with primary bilateral adrenal diffuse large B-cell lymphoma who achieved complete remission using R-CHOP chemotherapy.

Lymphome surrénalien primitif bilatéral de phénotype T. Un cas clinique beaucoup plus rare que le lymphome B

Primary adrenal lymphoma is a rare condition. Only 70 cases were described in the literature. Adrenal lymphoma is often bilateral and in most of the cases of B-cell type. T-cell lymphoma is exceptional. The prognosis is bad and patient can die early because of acute adrenal insufficiency. We report a case of a 70-year-old man who was admitted for acute adrenal insufficiency due to primary bilateral adrenal T-cell lymphoma. He had corticotherapy and surgical exploration for intra-abdominal sepsis. He died because of multivisceral deficiency. Clinical features and imaging are not specific. 18F-FDG PET Scan is an excellent mean to detect malignant tumor of adrenal gland. Percutaneous needle biopsy is useful to determine histology. The standard treatment is chemotherapy.

Bilateral Primary Adrenal Non-Hodgkin's Lymphoma and Primary Adrenocortical Carcinoma - Review of the Literature Preoperative Differentiation of Adrenal Tumors

Most of the adrenal tumors that are incidentally detected are benign adenomas. The incidence of malignant adrenal tumors including adrenocortical carcinoma (ACC) and primary adrenal lymphoma (PAL) is rather low. As many patients with ACC and PAL are diagnosed at an advanced stage of disease, the overall survival time of both entities remains poor. The therapeutic strategies for both entities differ. Thus an early differentiation between ACC and PAL is necessary. Unfortunately hitherto preoperative diagnosis of potentially malignant adrenal masses is still a main problem in the treatment of adrenal tumors. We present the case of a 57-year-old male patient with ACC and the case of an 87-year-old male patient with PAL and provide a systematic comparison of the clinical and pathological features of both entities. In both cases clinical and radiological features resulted in an initially false diagnosis. Primary surgical therapy was performed in both patients. The patient with PAL died five months after initial surgery. The patient with ACC showed tumor progression with local and systemic recurrence despite adjuvant therapy with mitotane and additional surgical therapy. Prognosis of patients with ACC and PAL seems to be dependant on the ability to start accurate treatment without any time delay. We propose some guidelines for diagnosis and surgical management of adrenal tumors.

Primary Bilateral Adrenal Non-Hodgkin's Burkitt-Like Lymphoma: A Rare Cause of Primary Adrenal Insufficiency. Case Report and Literature Review

Primary bilateral adrenal non-Hodgkin's lymphoma is an extremely rare entity. Only 44 cases have been reported in the literature. The most common presenting symptoms are abdominal pain, fever, asthenia, constipation, weight loss or typical symptoms of adrenal insufficiency, hypertension, darkening of skin, orthostatic hypotension or an addisonian crisis. The case is presented of a 57-year-old man suffering from primary bilateral adrenal lymphoma with symptoms of adrenal insufficiency syndrome associated with bilateral, stabbing lumbar pain and a palpable mass on the left side. Laboratory tests revealed a considerable increase in lactate dehydrogenase levels, adrenal insufficiency, and high corticotropin levels. Abdominal CT scan showed two large adrenal masses. A CT-guided fine needle aspiration biopsy revealed a large B-cell non-Hodgkin's lymphoma. Combination chemotherapy according to the CHOP protocol with cyclophosphamide, doxorubicin, vincristine and prednisolone was initiated, which caused a slight reduction in size of the two adrenal masses. The patient underwent a bilateral adrenalectomy with almost complete excision of the tumors. Replacement therapy with cortisone acetate was initiated postoperatively. Adjuvant combination chemotherapy according to the CHOP protocol was started but renal failure gradually emerged and the treatment could not be completed. The patient died seven months after surgery due to acute bronchial pneumonia and progression of disease. Primary bilateral adrenal non-Hodgkin's lymphoma mainly affects adult men. Diagnosis is based on histological examination. Whether associated or not with radiotherapy, chemotherapy is the most recommended treatment. Surgery, where possible, seems to lead to an increase in survival rates, but it is not possible to draw any definite conclusions on its effectiveness as yet.

Rituximab in the treatment of primary bilateral adrenal lymphoma with adrenal crisis

Primary adrenal lymphoma (PAL) is a rare extranodal non-Hodgkin's lymphoma. The majority of the patients are elderly men with bilateral adrenal involvements. The optimal treatment of PAL has not been well established currently. We herein report on a patient with primary bilateral adrenal lymphoma and adrenal crisis that treated with rituximab. Our case does demonstrate that rituximab could be administered without significant toxicities, and resulted in clinical improvement with disease stabilization in this critically ill patient.

Lymphome malin non hodgkinien primitif de la surrénale. À propos d'un cas

Bilateral primary non-Hodgkin's lymphoma (NHL) of the adrenals is uncommon. Less than 70 cases have been described in the literature. The symptoms of disease are variable and depend on the tumor size and the presence of adrenal insufficiency .We report a case of large diffuse B-cell adrenal lymphoma discovered in a 40-year-old woman presenting bilateral lumbar pain. Hormonal exploration demonstrated adrenal insufficiency. Imaging explorations showed a large and bilateral adrenal mass. Percutaneous CT-guided biopsy of the adrenal and search for extension revealed primary bilateral adrenal lymphoma. After glucocorticoid substitution, treatment was based on a CHOP regimen chemotherapy; outcome was unfavourable after the second cure; the patient died from septic shock. The diagnosis of primary adrenal non-Hodgkin lymphoma should be investigated in patients with a rapidly growing bilateral adrenal mass associated with adrenal insufficiency.

Imaging of primary adrenal lymphoma: case report and literature review

Primary adrenal lymphoma is very rare, and correct preoperative diagnosis is difficult because of its nonspecific imaging findings. We report a case of bilateral primary adrenal lymphoma, with the largest dimension of 18 cm, which is the largest one reported so far, to our knowledge. Computed tomography (CT) and magnetic resonance (MR) findings were not specific enough to establish a correct diagnosis, but positron emission tomography/CT (PET/CT) showed high fluorodeoxyglucose (FDG) uptake, indicating its malignant nature. We describe the imaging findings and review the related literature to highlight this uncommon entity.

Primary Bilateral Adrenal Lymphoma

Primary adrenal lymphoma is extremely rare. We describe 2 patients who presented with adrenal insufficiency or fever 'of unknown origin. Conventional imaging studies demonstrated bilateral bulky adrenal masses, and whole-body FDG positron emission tomography- computed tomography showed that the masses were the unique manifestation. Pathological diagnosis was non-Hodgkin lymphoma. The present cases indicated that primary adrenal lymphoma should be included in the differential diagnosis of bilateral adrenal masses.

FDG-PET Imaging in Primary Bilateral Adrenal Lymphoma: A Case Report and Review of the Literature

Primary adrenal lymphoma is an extremely rare entity. Only 70 cases have been reported in the English literature. Most of the patients are elderly men with bilateral adrenal masses without extraadrenal involvement. The most common presenting symptoms are fever, weight loss, lumbar pain, and/or symptoms of adrenal insufficiency. Of the cases reported, CT, ultrasound, and MRI were the imaging modalities used to describe the lesions. FDG PET has been used successfully to differentiate benign and malignant adrenal masses. The authors report a 67-year-old man diagnosed as having primary bilateral adrenal lymphoma and having no evidence of extraadrenal spread who was evaluated from the time of diagnosis to complete remission with FDG PET scanning. The literature of this unusual neoplasm is reviewed in detail.

Primary adrenal lymphoma with choroidal metastases

PURPOSE: To describe a 71-year-old man with bilateral primary adrenal lymphoma with metastases to the choroid. DESIGN: Interventional case report. METHODS: A 71-year-old man presented with decreased visual acuity in his left eye secondary to an exudative retinal detachment. Abdominal computed tomography and percutaneous biopsy revealed non-Hodgkin large-cell lymphoma involving both adrenal glands. RESULTS: Ocular and adrenal lesions resolved after six cycles of chemotherapy with cytoxan, adriamycin, vincristine, and prednisone. Seven years after his initial diagnosis, the patient reported no recurrence of either ocular or systemic lymphoma. CONCLUSIONS: To our knowledge, this is the first case report of bilateral primary adrenal lymphoma with apparent metastases exclusively to the eye.

Primary Bilateral Adrenal Lymphoma Relapsing as a Solid Cerebral Mass After Complete Clinical Remission

Primary adrenal lymphoma is extremely rare. Only 75 cases have been reported in the medical literature. A case of non-Hodgkin's lymphoma originating in both adrenal glands is presented. Combination chemotherapy apparently produced complete disappearance of the primary lymphomatous lesions, but subsequently a cerebral relapse was discovered 6 months later, in the form of a solid brain mass. Cranial extension of primary adrenal lymphoma is extremely unusual, and the presentation as a solid mass seems to be unique.

Primary Bilateral Adrenal Lymphoma

Primary adrenal lymphoma is a rare primary neoplastic disease of the adrenal glands, with up to 65 cases reported in the literature over the past 40 years. The increasing use and sophistication of medical diagnostic imaging has allowed this disease to be diagnosed more frequently premortem, presenting more opportunity for treatment. The true incidence of these neoplasms is not known, nor is it clear why historical autopsy series have not reported this neoplasm more frequently, and why its existence has been documented more recently with advanced diagnostic imaging. This review has presented our new case, and reviewed 5 more in the literature, bringing the total number of cases to over 70. Accumulation of more cases and the experience treating these cases is needed to develop a better picture of diagnostic procedures and treatment regimens that have maximum efficiacy.

Primary Bilateral Adrenal Lymphoma Associated with Idiopathic Thrombocytopenic Purpura

Autoimmune disorders are occasionally associated with malignant lymphoma. To date only one case of primary adrenal lymphoma associated with idiopathic thrombocytopenic purpura (ITP) has ever been reported. This paper reports the case of a 63-year-old man with bilateral adrenal masses whose laboratory data showed decreased platelet count. Despite normal blood pressure, the adrenal tumors endocrinologically appeared to be pheochromocytoma. Core needle biopsy was not done due to thrombocytopenia attributed to concurrent ITP. After intravenous immunoglobulin treatment, splenectomy and bilateral adrenalectomy were performed since the first pathological findings of the frozen specimen suggested the possibility of a poorly-differentiated carcinoma. Immunohistochemical study finally showed the tumors to be diffuse large B-cell lymphoma. The patient underwent a subsequent course of combination chemotherapy and survived 6 years recurrence-free without any need for further treatment other than steroid replacement. The coincidence of adrenal lymphoma and ITP should be considered even if another kind of tumor is suspected, and core needle biopsy should be performed prior to operation, since the specific kind of tumor found alters the therapeutical strategy adopted.