Adrenal Lymphoma Research Articles

Outcome of Surgical Resection and Chemotherapy Versus Chemotherapy Alone for the Treatment of Isolated Primary Adrenal Lymphoma: A Retrospective Cohort Study of 16 Consecutive Patients.

Primary adrenal lymphoma (PAL) is rare and aggressive. The aim of this retrospective study was to compare the results of surgery and chemotherapy compared to chemotherapy alone for the treatment of this condition. Sixteen patients, 10 men and 6 women of a median age of 63 years (IQR=56-70.5 years), admitted for the treatment of PAL, were retrospectively reviewed. Six patients (37.5%) underwent surgical resection of the mass followed by CHOP (cyclophosphamide, doxorubicin, vincristine, bleomycin and prednisone) - based chemotherapy (Group A). Ten patients (62.5%) underwent chemotherapy alone, consisting of CHOP alone in one case and Rituximab-CHOP (R-CHOP) in 9 cases (Group B). As primary study endpoints of the study, overall survival (OS) and progression-free survival (PFS) were considered. At two years follow-up, OS was 50% in Group A and 60% in group B (p=0.69). The PFS was 50% in group A and 30% in group B (p=0.42). PAL exhibits overall a dismal prognosis. Chemotherapy remains the most appropriate treatment, although unable to ensure long-term survival. Surgery combined with chemotherapy is ineffective in improving survival and may, at best, have a limited role in relieving the pain related to the local mass effect.

A Novel Diagnostic Model for Primary Adrenal Lymphoma.

ObjectivePrimary adrenal lymphoma (PAL) is easily misdiagnosed as other adrenal masses, such as adrenocortical carcinoma and pheochromocytoma, but patients with PAL benefit little from surgery. The diagnostic method for PAL thus far is limited to adrenal biopsy. In our study, we aimed to develop a quick and efficient diagnostic method for PAL.Methods and ResultsAt the same institution, 505 patients (between 2009 and 2019) and 171 patients (between 2019 and 2020) were separately included in the primary and validation studies. Univariate and multivariate analyses were conducted to evaluate clinical manifestations, laboratory findings, and radiological characteristics. Four determinants (age, bilateral masses, high-density lipoprotein cholesterol, and lactate dehydrogenase) were selected and further incorporated into a regression model to screen PAL. Accordingly, the nomogram was developed for clinical practice. In the primary study, the nomogram showed good discrimination, with an area under the receiver operating characteristic (ROC) curve (AUC) of 95.4% (95% CI, 90.6%–100.0%). Further validation study verified the efficacy of the nomogram, with an AUC of 99.0% (95% CI, 96.9%–100.00%) and 100.0% in all patients and patients with bilateral masses, respectively, and a sensitivity/specificity/positive predictive value (PPV)/negative predictive value (NPV) of 66.67%/99.40%/66.67%/99.40%, 66.67%/100%/100%/92.86%, 50%/99.20%/50%/99.20%, and 100%/100%/100%/100%, in all patients, patients with bilateral adrenal masses, patients with nonfunctional adrenal masses, and patients with positive catecholamine results, respectively. The validation study also revealed a diagnostic specificity of 99.35% and 100% for patients with a unilateral adrenal mass and functional PCC, respectively.ConclusionsThe presented nomogram is the first user-friendly diagnostic model for PAL that simplifies the complex diagnostic process into personalized numeric estimates. We deem that patients who score below 50 are less likely to have PAL. We suggest that clinicians should arrange adrenal biopsy and surgery for patients with nonfunctional tumors and overt catecholamine-secreting tumors, respectively, who receive a score of 50 points or higher to confirm the diagnosis as soon as possible.

Cross-sectional imaging features of unusual adrenal lesions: a radiopathological correlation.

The detection rates of adrenal masses are increasing with the common and widespread use of cross-sectional imaging. Adrenal adenomas, myelolipomas, metastases, pheochromocytomas, and adrenocortical tumors are well-known and relatively common adrenal tumors. However, there are many less-known neoplastic and nonneoplastic adrenal diseases that might affect the adrenal glands in addition to these common lesions. These rare entities include, but are not limited to, hydatid cysts, congenital adrenal hyperplasia, Wolman disease, adrenal tuberculosis, primary adrenal lymphoma. This article aims to present imaging findings of these unusual lesions in accordance with their pathologic characteristics. We think that the simultaneous presentation of the pathological findings with the imaging features may facilitate the learning process and may potentially enhance the recognition of these entities.

Haemorrhagic shock from solid tumours of the adrenal gland: a case of bleeding primary adrenal lymphoma

Suprarenal or adrenal gland haemorrhage is an uncommon but potentially lethal condition if unrecognised. Adrenal masses rarely present with haemorrhage, but they remain an important differential aetiology for adrenal bleeding. We present a novel case of primary adrenal lymphoma with adrenal haemorrhage in a middle-aged woman who presented with right-sided abdominal pain and class 1 haemorrhagic shock. She was found to have spontaneous unilateral adrenal gland haemorrhage in the absence of any underlying previous pathology. Presenting features, diagnosis and subsequent oncological management are reported.

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.

Systemic vs. intrathecal central nervous system prophylaxis in primary adrenal/renal diffuse large b-cell Lymphoma: A multi-institution retrospective analysis and systematic review

Primary adrenal lymphoma (PAL) and primary renal lymphoma (PRL) are rare extranodal lymphomas, predominantly of diffuse large B-cell lymphoma subtype. Primary adrenal and renal lymphomas (PARL) exhibit a high predilection for the central nervous system (CNS). Therefore, current guidelines support the use of CNS prophylaxis in PARL, particularly in cases of high-risk Central Nervous System International Prognostic Index (CNS-IPI). However, the route of administration (i.e. systemic vs. intrathecal chemotherapy) has not been clearly elucidated. With this in mind, we initiated an international collaboration and literature review to analyze 50 patient cases, 20 of whome received CNS prophylaxis. Based on our analysis, we conclude that PARL may indicate a need for CNS chemo-prophylaxis in the form of systemic high-dose methotrexate (HD-MTX) over intrathecal methotrexate (IT-MTX), although IT-MTX may still have utility in certain cases.

Bilateral Adrenal Lymphoma: A Diagnostic Challenge

Bilateral primary adrenal lymphoma is rare with no specific clinical or imaging features. We report herein the case of a 44-year-old man presenting with massive bilateral adrenal tumors associated with normal adrenal function. Computed tomography findings were nonspecific. Because pheochromocytoma was ruled out and the patient had a normal coagulation test, we performed an ultrasound-guided adrenal biopsy for diagnostic purposes. Pathology confirmed adrenal non-Hodgkin's lymphoma. By reporting our case, we aimed to propose a practical approach for this rare entity and to show that there are still situations in which biopsy is necessary to make a diagnosis.

Pierwotne chłoniaki nadnerczy jako interdyscyplinarny problem endokrynologiczny i hematologiczny — praktyczne wskazówki w zakresie diagnostyki i leczenia

Primary adrenal lymphomas (PAL) are rare neoplasms that should be considered in the differential diagnosis of adrenal insufficiency and incidentally detected focal lesions within the adrenal glands, especially those located bilaterally. PAL are tumors characterized by a rapid growth, an aggressive course and a poor prognosis. The disease usually develops in the elderly, most often in the 6.–7. decade of life, 2–7 times more often in men than in women. The etiopathogenesis of PAL is complex, multifactorial and still not fully understood.. The most commonly reported symptoms include fever of unknown origin, abdominal pain, fatigue, and weight loss. In the case of lesions localized on both sides, symptoms of adrenal insufficiency are relatively common. PAL may also be clinically silent and be detected as incidentalomas. The examination of choice in PAL diagnostics is computed tomography (CT) of the abdominal cavity aimed at the adrenal glands and combined with hormonal assessment. The gold standard in the diagnosis of PAL is percutaneous fine-needle aspiration biopsy under ultrasound or CT guidance, which allows for the final diagnosis. Histopathologically, the vast majority of cases are diffuse large B-cell lymphoma. It has been suggested to combine various methods of treatment, including immunochemotherapy/chemotherapy, surgery with subsequent immunochemotherapy/chemotherapy and/or radiotherapy. The purpose of the following systematic review was to perform an interdisciplinary analysis of the clinical picture, pathogenesis, diagnostic methods and current therapeutic strategies, taking into account the prognosis for individual PAL subtypes.

Suspected Primary Adrenal Lymphoma (PAL) Associated With Hemophagocytic Lymphohistiocytosis (HLH).

Adrenal incidentalomas, masses noted on imaging performed for other purposes, are common, with 10% to 15% presenting as bilateral adrenal masses. These cases can be challenging as the differential diagnosis is broad, including metastatic disease, primary adrenal lymphoma (PAL), or infection, and often requiring a biopsy if initial biochemical workup is unrevealing. We present here a relevant case description, laboratory and radiologic imaging studies, and discussion of literature. A 62-year-old Korean woman presented with altered mental status and fevers. She was found to have bilateral adrenal incidentalomas and retained acupuncture needles. Adrenal workup did not show biochemical evidence of hormonal excess. Infectious workup was unrevealing, as was a metal/toxin workup due to retained acupuncture needles. Fevers and episodes of hypotension persisted which prevented the patient from obtaining an adrenal biopsy. Bone marrow biopsy was obtained for pancytopenia and revealed B-cell lymphoma with large cell morphology and few histiocytes with hemophagocytosis, raising concern for lymphoma-induced hemophagocytic lymphohistiocytosis (HLH). PAL associated with HLH was highly suspected in our patient, given the large (7 cm) bilateral adrenal masses and bone marrow biopsy findings of lymphoma. The patient was treated for diffuse large B-cell lymphoma, with clinical improvement. PAL is a rare but aggressive lymphoma with few reported cases. It should be considered in the differential for both unilateral and bilateral adrenal masses. An early diagnosis is crucial as the main treatment is chemotherapy rather than surgery and it confers a significant survival benefit.

CME Sonography 94/Answers: Adrenal Glands

CME Sonography 94/Answers: Adrenal Glands Abstract. Adrenal glands are often not examined in abdominal ultrasound because it is thought that the adrenal glands cannot be visualized. The normal right adrenal gland can be represented in most cases and the left one very often. Sonography makes an important contribution to the diagnosis of adrenal diseases. In addition to bleeding and tuberculosis, there are benign tumors (adrenal adenomas), pheochromocytomas, and adrenal carcinomas. There are also adrenal metastases and lymphomas.

Clinical Features of 50 Patients With Primary Adrenal Lymphoma.

Background and Objective: Primary adrenal lymphoma is a rare, progressive, easily misdiagnosed adrenal tumor with a poor prognosis. There are limited data on its clinical characteristics, and these have been derived from small sample studies. This study aimed to identify the clinical characteristics and prognosis of primary adrenal lymphoma.Methods: This single-center study retrospectively analyzed data of 50 primary adrenal lymphoma patients treated between January 2008 and January 2018. Demographic information, biochemical indexes, computed tomography images, pathological findings, treatment regimens, and prognostic factors were analyzed.Results: The median age of onset was 60.3 years, and 30 (60.0%) of 50 patients were male. Abdominal pain was the most common symptom, followed by incidentaloma and B symptoms. On average, patients presented with elevated lactate dehydrogenase (348 IU/L, normal range 110–220 IU/L) and hydroxybutyrate dehydrogenase levels (287 IU/L, normal range 72–182 IU/L) and decreased high-density lipoprotein cholesterol levels (0.88 mmol/L, normal range > 0.9 mmol/L). Bilateral lesions in the adrenal glands were observed in 30 (60.0%) patients. Computed tomography showed that 42 (84%) patients had signs of infiltration. Diffuse large B-cell lymphoma was present in 44 (88%) patients. Immunohistochemistry revealed that 70.6% (12/17), 89.5% (17/19), 92.0% (23/25), and 68.8% (11/16) of patients were positive for MYC, p53, BCL2, and both MYC and BCL2, respectively. Combined chemotherapy was associated with a good prognosis.Conclusions: Early diagnosis of primary adrenal lymphoma depends on a combination of biochemical examination, imaging studies, and pathological biopsy, and combined chemotherapy may lead to a better prognosis.

TCL-297: A Rare Case of Bilateral Adrenal T Cell Lymphoma

Introduction: Primary adrenal lymphoma is a very rare and aggressive disease. B cell lymphoma is considered to be more common than T cell lymphoma, which is extremely rare. Very few cases were reported in the literature. The disease process usually affects bilateral glands. Case Presentation: A 53-year-old female, experienced typical signs of weight loss, dyspnea, and diffuse abdominal pain. Following an extended workup, she was reported having significant pericardial effusion, left pleural effusion, and bilateral heterogeneous adrenal masses of 90×56 mm and 100×67 mm at the right and left adrenal glands, respectively. An emergent pericardiocentesis was performed that showed atypical cells compatible with lymphoma, with immunochemistry positive for anti-LCA antibodies (anti-CD45) compatible with lymphoid origin. Subsequently, she underwent a PET-CT scan that showed increase signal in both adrenal glands with excessive retroperitoneal and mediastinal lymph nodes in addition to the suspected areas of involvement of the pericardium and pleural spaces previously described. The findings were compatible with lymphoma. The definitive diagnosis was made by an image guided percutaneous biopsy of the adrenal mass that confirmed peripheral T cell lymphoma of bilateral adrenal glands and treatment with CHOEP was planned (cyclophosphamide, oncovin, etoposide). Doxorubicin was not given due to her coronary artery disease and heart failure. Following her first cycle, she was admitted to the intensive care unit due to general status alteration and febrile neutropenia for which she received the proper treatment and was discharged home few days later. The patient then received her second cycle of CHOEP without any complications as of now. Conclusions: Although extremely rare, lymphoma of the adrenal glands should be considered in patients presenting with adrenal masses, mainly when multiple systems are involved due to metastatic spread. PET-CT is an excellent tool for diagnosis and follow up, while biopsy is useful to determine histology.

Primary adrenal lymphoma

Primary adrenal lymphoma

Primary adrenal insufficiency secondary to bilateral adrenal lymphoma

Involvement of adrenal glands in lymphoma is rare; the patient may have variable presentations. We report a case of a 62-year-old Saudi female who presented to our center with a few-week history of fatigue, weight loss, subjective fever, and a recent change in skin color. On examination, she looked dehydrated and had a drop in blood pressure with postural change and hyperpigmentation of the skin of the face and hands. Morning cortisol levels were low, and adrenocorticotropic hormone levels were high, which indicated primary adrenal insufficiency (PAI). An adrenal computed tomography (CT) scan revealed bilateral adrenal enlargement. After excluding pheochromocytoma, a CT scan-guided trucut biopsy of the adrenals confirmed non-Hodgkin lymphoma. The patient was started on steroid replacement therapy, and after stabilization, a plan was made to initiate chemotherapy for the treatment of lymphoma; unfortunately, the patient died shortly after diagnosis because of rapid progression of the disease. Adrenal lymphoma can present with PAI and should be considered in the presence of bilateral adrenal enlargement; it is an aggressive tumor and carries poor prognosis if the treatment is delayed.

Primary adrenal failure and central nervous system lesions: a rare case report of primary adrenal lymphoma

Primary adrenal failure and central nervous system lesions: a rare case report of primary adrenal lymphoma

Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study.

We sought to refine the clinical picture of primary adrenal lymphoma (PAL), a rare lymphoid malignancy with predominant adrenal manifestation and risk of adrenal insufficiency. Ninety-seven patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017. Of the 81 patients with imaging data, 19 (23%) had isolated adrenal involvement (iPAL), while 62 (77%) had additional extra-adrenal involvement (PAL+). Among patients who had both CT and PET scans, 18FDG-PET revealed extra-adrenal involvement not detected by CT scan in 9/18 cases (50%). The most common clinical manifestations were B symptoms (55%), fatigue (45%), and abdominal pain (35%). Endocrinological assessment was often inadequate. With a median follow-up of 41.6 months, 3-year progression-free (PFS) and overall (OS) survival rates in the entire cohort were 35.5% and 39.4%, respectively. The hazard ratios of iPAL for PFS and OS were 40.1 (95% CI: 2.63-613.7, P = 0.008) and 2.69 (95% CI: 0.61-11.89, P = 0.191), respectively. PFS was much shorter in iPAL vs PAL+ (median 4 months vs not reached, P = 0.006), and OS also appeared to be shorter (median 16 months vs not reached), but the difference did not reach statistical significance (P = 0.16). Isolated PAL was more frequent in females (OR = 3.81; P = 0.01) and less frequently associated with B symptoms (OR = 0.159; P = 0.004). We found unexpected heterogeneity in the clinical spectrum of PAL. Further studies are needed to clarify whether clinical distinction between iPAL and PAL+ is corroborated by differences in molecular biology.

SAT-187 Altered Mental Status in a Patient with Bilateral Adrenal Masses

IntroductionPrimary adrenal lymphoma (PAL) is a very rare and aggressive form of Non-Hodgkin’s lymphoma originating from the adrenal glands and with no prior history of lymphoma. It can present with symptoms related to adrenal insufficiency due to tumoral destruction of normal adrenal tissue. Hypercalcemia has also been previously reported as a less common presentation of PAL. We describe here a case of PAL presenting with severe hypercalcemia and adrenal insufficiency.Clinical Case75 year male presented with 50 pound weight loss, fatigue and anorexia for several months. On admission, patient had altered mental status. Further investigation revealed severe hypercalcemia (corrected calcium for albumin =15mg/dl) and AKI (serum creatine=3.45mg/dl, blood urea nitrogen=89). He was started on IV fluids for the hypercalcemia and AKI. Additional work up revealed suppressed parathyroid hormone (PTH) levels of 9 (12 - 88 pg/mL), high normal PTH-related peptide 22 (14-27), low 25-OHD 7.34 (20-120 ng/ml), high normal 1,25-(OH)2-D 63.0 (19.9-79 pg/mL). His ACTH level was elevated ~159 (7.2-63.3 pg/ml) with low normal morning cortisol levels ~10 mcg/dl. Patient was started on IV stress dose steroids for concerns of adrenal insufficiency and calcitonin was also initiated for the treatment of hypercalcemia. CT of the abdomen showed bilateral adrenal masses, measuring 9.8 x 6.4 x 10.8 cm on the right and 9.3 x 5.2 x 9.6 cm on the left with marked retroperitoneal lymphadenopathy. After ruling out pheochromocytoma with negative plasma and urine metanephrines, biopsy of the adrenal mass revealed a highly aggressive diffuse B large cell lymphoma, with BCL2 and MYC overexpression. Concurrent flow cytometric analysis also identified an abnormal population of medium to large B cells expressing bright CD20, CD19, CD38, CD45 without CD10, CD5, CD23 or overt kappa or lambda expression. His calcium levels improved with hydration and steroids. Chemotherapy was later initiated.ConclusionPrimary adrenal lymphoma is rare and aggressive type of NHL. While adrenal insufficiency is expected in this clinical scenario, hypercalcemia from 1,25-(OH)2-D excess is a relatively uncommon presentation. Adrenal insufficiency should be ruled out in bilateral infiltrative adrenal masses. Likely etiologies for hypercalcemia in our case appeared to be secondary to elevated 1,25-(OH)2-D production, humoral hypercalcemia of malignancy and worsened by untreated clinically evident adrenal insufficiency.References(1) Laurent C, Casasnovas O, Martin L, et al. Adrenal lymphoma: presentation, management and prognosis. QJM. 2017 Feb 1;110(2):103-109. doi: 10.1093/qjmed/hcw174(2) Masood A, Tumyan A, Nussenzveig D R., Wakefield D N., Barb D, Ghayee H K., Maalouf N M. The Diverse Clinical Presentations of Adrenal Lymphoma. AACE Clinical Case Reports: Autumn 2017, Vol. 3, No. 4, pp. e307-e312. doi: 10.4158/EP161595.CR

SAT-185 Primary Adrenal Lymphoma Presenting with Symptomatic Hypercalcaemia

Introduction: Primary adrenal lymphoma (PAL) is a rare cause of adrenal enlargement with approximately 200 cases reported in the literature to date. It tends to affect elderly men and has a high incidence of bilateral involvement at diagnosis. We report the case of a 66 year old man, whose PAL manifested with symptomatic hypercalcaemia.A 66 year old male, originally from the Philippines, was referred to the emergency department with nausea, vomiting, weight loss and right flank pain. His past medical history was significant for hypertension, gout and stage 3b chronic kidney disease. His medications were amlodipine, losartan and febuxostat. His family history was significant for hypertension. On examination he was hypertensive (blood pressure, 160/100 mmHg) and hyperpigmented. His laboratory investigations revealed; corrected calcium of 3.79 mmol/l, undetectable PTH, vitamin D 49 nmol/l. He was treated with intravenous (IV) 0.9% saline and IV zoledronic acid and his calcium levels improved.To investigate causes of non-PTH mediated hypercalcaemia, computerised tomography of the thorax, abdomen and pelvis (CT TAP) as well as a positron emission tomography (PET) scan were performed. These demonstrated bilateral, large, metabolically active adrenal masses with no evidence of extra-adrenal disease. Differential diagnosis at this point included bilateral adrenal hyperplasia, metastases, lymphoma or adrenal TB. There were no radiological features of adrenocortical carcinoma (ACC) or phaeochromocytoma and subsequent biochemical investigations confirmed no evidence of cortisol, androgen or catecholamine excess. Adrenocorticotrophic hormone (ACTH) levels were elevated however, and a synacthen test revealed inadequate adrenal reserve (peak cortisol 214 nmol/l). The patient was commenced on maintenance steroids and with stress dose steroid cover, proceeded to adrenal biopsy. Histology confirmed diffuse large B cell non-Hodgkin’s lymphoma. Haematology became involved in his care and he commenced polychemotherapy in the form of R-CHOP, 1 week post confirmation of the diagnosis. His treatment is ongoing and he has tolerated it well with minimal side effects, except a flare of gout.Learning points: PAL should be considered in the differential diagnosis in patients with bilateral adrenal masses. Image guided adrenal biopsy is the gold standard for diagnosis, though caution must be exercised and an ACC or phaeochromocytoma should be excluded prior to biopsy. The prognosis of PAL is poor and therefore early diagnosis and prompt initiation of treatment are required to improve outcomes.

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 lymphoma: a case of hiccups.

Approximately 250 cases of primary adrenal lymphoma have been reported. We describe an unusual presentation of this condition. Our patient is a 65-year-old male whom presented to the emergency department with 4 days of severe persistent hiccups. He had lost 26 kg in weight in the preceding 4 months. Computed tomography abdomen revealed large invasive bilateral adrenal masses. Biochemical evaluation confirmed adrenal insufficiency. Hiccups resolved within 24 h of steroid replacement. Adrenal biopsy confirmed a diffuse large B-cell lymphoma. Hypotheses for the aetiology of his hiccups include diaphragmatic irritation and primary adrenal insufficiency. This case is interesting for its rarity involving bilateral adrenal glands, Addison’s disease from the primary adrenal tumour and rapid resolution of hiccups with corticosteroid therapy.