Primary Adrenal Non-Hodgkin's Lymphoma Research Articles

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.

Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature

BackgroundLymphomas are cancers that arise from the white blood cells and have been traditionally divided into two large subtypes: Hodgkin and non-Hodgkin lymphoma. B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma; almost 85% of patients with lymphoma have this variant. Lymphomas can potentially arise from any lymphoid tissue located in the body; however, primary adrenal non-Hodgkin lymphoma is extremely rare. We report the history, examination findings, and laboratory results of a 50-year-old man diagnosed with a primary left adrenal diffuse large B-cell lymphoma.Case presentationA 50-year-old Pakistani man presented to our hospital with progressively increasing pain and fullness in the left upper quadrant of his abdomen, generalized weakness, easy fatigability, and decreased appetite of 1.5 months’ duration. On examination, he had a blood pressure of 140/80 mmHg with no postural drop, a pulse rate of 106 beats/minute, and no fever. His past medical history was significant for pulmonary tuberculosis 2 years earlier, for which he received antituberculous therapy. Computed tomography revealed a heterogeneous enhancing soft tissue density mass in the left adrenal gland. It measured 7.1 × 5.6 × 9.5 cm. Further laboratory workup revealed the following levels: sodium 135 mEq/L, potassium 4.5 mEq/L, lactate dehydrogenase 905 IU/L, renin 364 IU/ml, aldosterone 5.79 ng/dl, dehydroepiandrosterone sulfate 79.20 μg/dl, urinary vanillylmandelic acid 6.4 mg/24 hours, and a low-dose overnight dexamethasone suppression test result of 3.20 μg/dl. The patient underwent left adrenalectomy. Histopathological test results showed a diffuse large B-cell lymphoma. Immunohistochemical stains were strongly positive for CD20 and negative for CD3, CD5, CD10, and cyclin D1. The patient’s Ki-67 (Mib-1) index was approximately 80%. He received a total of six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy (rituximab was not given, owing to financial constraints) and was routinely followed pre- and postchemotherapy at our hematology clinic with complete blood count and serum lactate dehydrogenase evaluations. The patient responded to chemotherapy and is currently doing well.ConclusionsPrimary adrenal lymphoma is an extremely rare but rapidly progressive disease. It generally carries a poor prognosis, partly because an optimal treatment protocol has not yet been established. Further studies with larger sample sizes are needed to establish the best treatment option and increase overall survival.

Central nervous system involvement in primary adrenal non-hodgkin lymphoma.

Central nervous system involvement in primary adrenal non-hodgkin lymphoma.

Bilateral primary adrenal non-hodgkin lymphoma.

Bilateral primary adrenal non-hodgkin lymphoma.

Primary adrenal lymphoma: Differential involvement with varying adrenal function

Primary adrenal Non-Hodgkin's lymphoma is rare. The symptoms of the disease and response to treatment are variable depending on the type of lymphoma, tumor size, and presence of adrenal insufficiency. We report two cases of primary adrenal lymphoma who had varied presentations. One presenting with abdominal pain and weight loss was documented to have unilateral disease without any adrenal insufficiency and showed a good response to combination chemotherapy, while the second one had bilateral adrenal involvement with adrenal insufficiency and died after second chemotherapy. Functional adrenal involvement in lymphoma depends on the extent of involvement; patients with bilateral involvement almost always have adrenal insufficiency.

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.

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.

A Primary Adrenal Non-Hodgkin's Lymphoma Presenting as an Incidental Adrenal Mass

The primary adrenal localization of a non-Hodgkin's lymphoma (NHL) is a rare event. We report the case of a 70-yr-old woman, who was admitted at our Institute for a hormonal evaluation after the incidental discovery of a right adrenal mass during ultrasonography (US) performed for cardiovascular disease. At the physical examination, no sign of adrenal hyperfunction was present. She showed only an androgenetic alopecia and her blood pressure was 180/70 mm Hg, with an arrhythmic heart rate of 100 beats/min. No alterations in hormonal and biochemical data were observed. US studies showed a right adrenal mass (major diameter 16 mm), and an abdominal computed tomography (CT) scan confirmed this solid lesion (major diameter 15 mm) with a high density. [75Se] methylnorcholesterol adrenal scintigraphy exhibited a normal symmetrical radiotracer uptake. After 8 months of follow-up, an abdominal CT scan demonstrated a significant increase of the right adrenal mass (major diameter: 40 mm), with a solid tissue density and enhancement after i.v. contrast. [75Se] methylnorcholesterol adrenal scintigraphy showed an absent uptake on the right side versus the contralateral side. The hematological, hormonal and radiological evaluation did not reveal any sign of malignancy. Owing to the mass enlargement and the modification of scintigraphic pattern, the patient underwent unilateral adrenalectomy. Histological examination revealed a primary diffuse large B-cell NHL (REAL classification) of the adrenal gland. After surgery, she underwent a combined polychemotherapy (cyclophospamide, adriamycin, vincristine and prednisone) and subsequently one cycle of radiotherapy. At present, the patient is in good conditions and there are no signs or symptoms of recurrent disease.

Successful Treatment of Primary Adrenal Non-Hodgkin's Lymphoma Associated with Adrenal Insufficiency

We report a case of primary adrenal NHL associated with adrenal insufficiency which was successfully treated with steroid replacement and chemotherapy. A 69-year-old woman hospitalized with fatigue and weight loss developed shock and recovered with steroid therapy. Adrenal insufficiency was confirmed by an elevated plasma adrenocorticotropic hormone level and low cortisol. Computed tomography revealed large bilateral adrenal masses. Needle biopsy showed a diffuse, mixed B cell lymphoma. CHOP therapy accompanied by steroid replacement was begun, and she achieved a complete remission after 4 cycles. She received additional 4 cycles of chemotherapy. Although adrenal insufficiency was irreversible, she has continued in complete remission for 50 months at this reporting.

PRIMARY ADRENAL NONHODGKIN'S LYMPHOMA: LONG-TERM SURVIVAL

Primary nonHodgkin's lymphoma of the adrenal gland is a rare neoplasm, with only 27 cases reported in the world literature, and maximum survival is 15 months. To our knowledge we report the first case of long-term survival (8 years) of a patient with primary adrenal nonHodgkin's lymphoma. In contrast to the cases reported the treatment of our patient consisted of adrenalectomy and adjuvant radiation therapy. CASE REPORT A 59-year-old patient presented with a painless mass above the right kidney incidentally discovered on abdominal ultrasound. Abdominal sonography and computerized tomography (CT) showed a right 8 X 4 X 4 cm. inhomogeneous suprarenal mass adherent to the vena cava with contrast enhancement ranging from 60 to 80 Hounsfield units (fig. 1). No other abdominal mass or retroDentoneal1vmDhadenooathv was identified. Excretory urograp6y demonstra& no pa&ological findings. Endocrinological examination was normal. The adrenal tumor was removed via a thoracoabdominal approach above the 10th rib. Because of its adherence the mass had to be dissected sharply from the vena cava. Histological examination, including immunohistochemical analysis, showed malignant centroblastic T cell nonHodgkin's lymphoma (Eel Classification, fig. 2). Postoperative staging procedures, including gastroscopy, barium enema, bone marrow biopsy and chest x-ray, revealed no evidence of metastatic disease. Intraoperative and histological findings did not confirm total extirpation. Therefore, 59 Gy. adjuvant radiotherapy were administered in 4 weeks. At 8-year followup the patient has no evidence of disease. DISCUSSION Secondary involvement of the adrenal gland is found in 25% of autopsy cases of nonHodgkin's lymphoma.' primary adrenal