Case Of Primary Adrenal Lymphoma Research Articles

A Decade of Insights into Bilateral Adrenal Masses: Epidemiology, Clinical Profiles, and Therapeutic Approaches

Bilateral adrenal masses (BAMs) are rare, accounting for 10-15% of adrenal masses. Their diagnostic and therapeutic approaches differ from those of unilateral masses. This study aimed to analyze the epidemiological, clinical, paraclinical, and therapeutic profiles of BAMs. It is based on a retrospective analysis of 30 patient records from the Endocrinology, Diabetology, and Nutrition Department at CHU Hassan II in Fez, over a 12-year period. The average age of the patients was 53 years, with a male predominance (53.3%). BAMs were symptomatically revealed in 46.7% of cases, incidentally discovered in 23.3%, identified during tumor staging in 20%, and associated with a genetic syndrome in 10% of cases. Clinical examination revealed hypertension in 23.3% of patients, an abdominal mass in 10%, signs of adrenal insufficiency in 10%, hypercortisolism in 6.67%, and hyperandrogenism in 16% of cases. Biological assessment showed elevated urinary or plasma metanephrine derivatives in 9 patients, hypercortisolism in 3 patients, adrenal insufficiency in 3 patients, 11-β-hydroxylase enzyme block in 3 patients, and primary hyperaldosteronism in 1 patient. Adrenal computed tomography (CT) was performed in 29 patients. A staging workup, including PET scan, was requested in 20% of cases. MIBG scintigraphy was performed in 23.3% of patients. The investigations revealed 53% of BAMs were secreting and 47% non-secreting. There were 9 cases of pheochromocytomas, including 5 associated with a syndrome (3 MEN2a, 1 MEN2b, 1 VHL), 8 cases of non-secreting adenomas, 3 cases of congenital adrenal hyperplasia due to 11-β-hydroxylase enzyme block, 2 cases of adrenal metastases, 2 cases of adrenocortical carcinoma, 2 cases of adrenal cysts, 1 case of Conn’s adenoma, 1 case of cortisol-secreting adenoma, 1 case of primary adrenal lymphoma, and 1 case of adrenal hematoma. Treatment included the management of emergencies and preoperative preparation for patients eligible for surgery. Bilateral adrenalectomy was performed in 33.3% of patients, and unilateral adenectomy in 1 case of cortisol-secreting adenoma. Medical treatment was initiated in 16.6% of cases, and palliative treatment in 13.33% of cases. Surveillance alone was implemented in the remaining 33.3%. Prognosis depends on the type of adrenal mass, with outcomes marked by the death of one case of malignant adrenocortical carcinoma and one case of primary adrenal lymphoma. Our study, involving 30 cases of BAMs, highlights their clinical, biological, radiological, therapeutic, and prognostic characteristics. Data in the literature are limited compared to unilateral adrenal masses. Future larger studies are desirable for optimal management.

A Case of Primary Adrenal Lymphoma: How Do We Detect Early Progression to Adrenal Insufficiency?

A Case of Primary Adrenal Lymphoma: How Do We Detect Early Progression to Adrenal Insufficiency?

Severe hypercalcemia as the presenting manifestation of bilateral primary adrenal lymphoma

ABSTRACT Primary adrenal lymphoma (PAL) is a rare tumor with fewer than 300 cases reported to date. In the absence of characteristic imaging features and diagnostic serum markers, a biopsy remains the definitive way to diagnose PAL. Although rituximab-based chemotherapeutic regimens have led to improved outcomes, late presentation, undetected, and untreated adrenal insufficiency result in a dismal prognosis. Although it is well known that hypercalcemia in malignancy carries a grave outcome, well documented cases of hypercalcemia association with PAL is very rarely described in the literature. Here, we describe a case of PAL that predominantly presented with gastrointestinal manifestations of hypercalcemia.

Histopathological Experience of Primary Adrenal Lymphoma From Two Tertiary Hospitals.

Primary adrenal lymphoma (PAL) is a rare tumor. The aim of this study was to demonstrate the histopathological features of PAL at two tertiary hospitals. All PALs diagnosed between January 2003 and February 2023 were retrieved. Pathology and immunohistochemistry slides were reviewed. Additional immunohistochemical markers were done in selected cases.Follow-up data were obtained. There were 7 cases of PAL. The age range of the patients was 52 to 73 years (median 64 years; mean 63.3 years). There were 4 males (57.1%) and 3 females (42.9%). The clinical manifestations included abdominal pain nausea, vomiting, and loss of weight. There were 4 cases of diffuse large B-cell lymphoma (DLBCL), 2 cases of high-grade B-cell lymphomas, and 1 case of follicular lymphoma. There were 5 cases that were unilateral and 2 cases that were bilateral, and both were high-grade B-cell lymphoma. During follow-up, the 1-year and 2-year overall survival rates were 50% and 33%, respectively. PAL is a disease of the elderly, and DLBCL is the most common pathological type. The prognosis is generally poor.Further reporting of PAL cases might help in understanding this disease and could lead to improvement in its management.

An unusual cause of adrenal insufficiency with elevation of 17-hydroxyprogesterone: case report

BackgroundWe present an intriguing case of primary adrenal lymphoma, with associated primary adrenal insufficiency (PAI), in a patient presenting a transitory partial 21-hydroxylase deficiency during the active phase of the adrenal disease.Case presentationAn 85-years old woman was referred because of worsening asthenia, lumbar pain, generalized myalgia and arthralgia. During investigations a computed tomography (CT) scan evidenced two large bilateral adrenal masses, highly suspicious for primary adrenal tumor. The hormonal assessment revealed very low levels of morning plasma cortisol and 24-h urinary cortisol, elevated ACTH levels with low plasma concentration of aldosterone, pointing to the diagnosis of PAI. After diagnosis of PAI our patient started glucocorticoid and mineralcorticoid replacement therapy with clinical benefit. In order to further characterize the adrenal lesions, adrenal biopsy, was performed. The histology revealed a high grade non-Hodgkin lymphoma with an immunophenotype consistent with intermediate aspects between diffuse large B-cell and Burkitt lymphoma, with a high proliferation index (KI-67 > 90%). The patient received chemotherapy with epirubicin, vincristine, cyclophosphamide, and rituximab, associated with methylprednisolone that resulted in a complete clinical and radiological remission within one year. After 2 years from the diagnosis and a total of 6 cycles of rituximab, the patient was in good clinical condition and was taking only the replacement therapy for PAI. The patient initially presented also a slight increase of 17-hydroxyprogesterone (17-OHP) for age that normalize after resolution of lymphoproliferative disease.ConclusionsIn the presence of bilateral adrenal disease and/or in the presence of signs and symptoms of PAI clinicians must exclude the presence of PAL. The evidence of elevated ACTH-stimulated 17-OHP levels also in patients with other adrenal masses, together with the detection of elevated basal 17-OHP levels in our patient make it more plausible, in our view, an effect of the lesion on the “healthy” adrenal tissue residue than a direct secretory activity by the adrenal tumor.

Abstract #1177083: Acute Adrenal Insufficiency with Hypercalcemia: An unusual initial presentation of Primary Adrenal Lymphoma

Primary Adrenal Lymphoma (PAL) is a very rare malignancy accounting for < 1% of non-Hodgkin's Lymphoma. We report, to our knowledge, the first case of PAL associated with adrenal insufficiency (AI) and hypercalcemia.

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.

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.

Asymptomatic Unilateral Primary Adrenal Lymphoma Mimicking a NonFunctioning Adenoma in Adult Male with a Good Outcome

Primary adrenal lymphoma (PAL) is a type of extranodal lymphoma that is rarely encountered in clinical settings. It is regarded as a high-grade malignancy with a very poor prognosis. Most PALs are bilateral and hardly unilateral. Nevertheless, a rise in the number of cases of bilateral and unilateral PALs has been noted in the past few years, with a wide range of presenting symptoms from fatigue to adrenal insufficiency. Many were found incidentally via radiology imaging or on postmortem examinations. This paper aims to report an unusual case of a unilateral primary adrenal non-Hodgkin’s lymphoma in a previously healthy 53-yearold Bahraini male, who presented with vague yet persistent right flank pain of a few months’ duration. Imaging modalities revealed a large right adrenal mass measuring 11x10x9 cm which was initially thought to be an adenoma on CT scan, but post laparoscopic surgical excision was diagnosed histologically as Diffuse Large B Cell Lymphoma (DLBCL). Cycles of Rituximab-CHOP chemotherapy was given, and on regular follow up for two and a half years, the patient remains disease-free with no signs of recurrence.

A case of primary adrenal diffuse large B cell lymphoma presenting with severe hyponatremia.

We herein report a case of primary adrenal lymphoma with severe hyponatremia. The patient was admitted for an evaluation of severe hyponatremia and an enlarged bilateral adrenal mass, which were found in a previous examination for causes of general fatigue and anorexia. Laboratory data, including the serum levels of sodium (115mEq/L), osmolality (239mOsm/kgH2O), ADH (5.8pg/mL), cortisol (11.6μg/dL), free T3 (2.42pg/mL), urinary Na (117mEq/L) and urine osmolality (490mOsm/kgH2O), fulfilled the diagnostic criteria for the syndrome of inappropriate secretion of ADH (SIADH). An abdominal computed tomography scan revealed a large bilateral adrenal mass. A biopsy of the enlarged left adrenal mass revealed diffuse large B cell lymphoma, which was negative for ADH protein. Hydrocortisone treatment normalized the patient's body temperature and serum sodium concentration. In this case, hyponatremia developed when both adrenal glands were involved and was normalized with hydrocortisone. These findings suggest that adrenal insufficiency was the cause of hyponatremia, although the basal serum cortisol was normal. The current case suggests that the administration of hydrocortisone is recommended if suspicious clinical signs or symptoms are found in severe hyponatremia, even if hyponatremia is associated with a normal serum cortisol level and fulfills the diagnostic criteria for SIADH.

Primary adrenal lymphoma: three case reports and review of Japanese cases

Abstract Although primary adrenal lymphoma (PAL) is thought to be extremely rare, its prognosis is much worse than that of other extranodal malignant lymphomas. There are so far about 130 reported cases in the English literature, while 186 cases have been reported in the Japanese literature. Thus, the incidence of PAL in the Japanese population may be higher than in other races. The typical characteristics of PAL in Japanese patients are similar to those previously reported in Western literature: it predominantly occurs in male and elderly patients and involves bilateral involvement of the adrenal glands, which frequently leads to adrenal insufficiency. Here, we review three recent cases of PAL at our hospital, and analyze data from our institution regarding patients with PAL from 2002 to 2014. On biochemical analysis, median levels of sIL2R (5027.5 U/mL) and LDH (1111.46 U/L) were elevated in Japanese PAL patients compared to other adrenal tumors. It is critical that clinicians be familiar with the traits of PAL, especially for its differential diagnosis from adrenal large tumors.

Primary adrenal lymphoma: a systematic review

Fewer than 200 cases of primary adrenal lymphoma (PAL) have been reported. We have systematically reviewed all 187 cases of PAL reported in the English literature until June 2013, from which we drew the following conclusions: PAL is typically a highly symptomatic and aggressive, metabolically hyperactive, hypovascular, hypoechoic (and heterogeneous on ultrasound), hypodense (with slight to moderate enhancement on computed tomography), high-grade lymphoma, primarily affecting elderly males and presenting with large bilateral adrenal masses. Most cases have adrenal insufficiency, B-symptoms, and elevated lactate dehydrogenase. Hepatosplenomegaly, lymphadenopathy, concurrent or prior immune dysregulation, and bone marrow involvement are uncommon. Epstein-Barr virus positivity is observed in more than half of cases and the disease is disseminated at presentation in 18 % of cases. The two most common WHO 2008-defined PAL subtypes are diffuse large B cell lymphoma (78 %) and peripheral T cell lymphoma (7 %). The prognosis of PAL has improved with the advent of rituximab-containing chemotherapeutic regimens. According to our results, administration of chemotherapy and adrenal insufficiency are significant independent predictors of prognosis.

Primary adrenal lymphoma with paraneoplastic syndrome

Context:The adrenal gland is a common site for neoplastic diseases and primary adrenal lymphoma (PAL) is a rare tumor with around 120 cases reported so far.Case Report:We present a rare case of 76-year-old male who presented with headache, confusion, inappropriate body movements and abdominal pain. Adrenal biopsy revealed PAL and he has had an excellent neurologic outcome to date with chemotherapy and involved field radiation.Conclusion:The majority of cases of PAL are B cell lymphomas with diffuse large cell in 70% of cases. Clinical symptoms are variable and patients may present with abdominal pain, fever, anorexia, weight loss, fatigue or symptoms of adrenal insufficiency. Therapeutic modalities for PAL include surgery, chemotherapy and radiotherapy and corticosteroid replacement. With this case report, we hope to raise awareness about this rare disease and to include lymphoma in the differential of adrenal masses.

Primary adrenal non-Hodgkin’s lymphoma: Report of two cases

Primary adrenal lymphoma is very rare. It is characterized by a high incidence of bilateral adrenal involvement of diffuse large B-cell lymphoma. It can be diagnosed with endocrine evaluation, imaging studies and histopathological examination. We present two cases of primary adrenal lymphoma. One is a 74-year-old female patient with right primary adrenal lymphoma and the other is a 62-year-old male patient with bilateral primary adrenal lymphoma associated with normal adrenal function. In both cases, radiological features led to an initial misdiagnosis. The surgical exploration demonstrated masses invading the retroperitoneal space, and the biopsy revealed diffuse large B-cell lymphoma. In conclusion, primary adrenal lymphoma should be kept in mind in the differential diagnosis of adrenal masses. In cases of suspicious primary adrenal lymphoma, percutaneous computerized tomography or ultrasonography-guided needle biopsy can help to avoid unnecessary surgeries.

Bilateral Primary Adrenal Lymphoma Accompanying Hypertension

Primary adrenal lymphoma (PAL) accompanied by hypertension is extremely rare. We present a case of PAL with hypertension, whom was treated with bilateral adrenalectomy and a combination of the modified Appleby operation and chemotherapy. Computed tomography and biopsy is helpful to aid diagnosis.

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.

Adrenal Lymphoma: Case Report and Review of Literature

ABSTRACT Adrenal lymphoma is a rare diagnosis in subject presenting with bilateral adrenal masses. Adrenal insufficiency is a common complication of this disease. Most cases of primary adrenal lymphoma (PAL) present with adrenal insufficiency and huge bilateral adrenal masses. These are very aggressive tumors with poor prognosis. We, hereby, report two subjects of adrenal lymphoma presenting with bilateral adrenal masses. First case is a 50 years old male presented with features of adrenal insufficiency like anorexia, weight loss, nausea, vomiting, and generalized hyperpigmentation. Routine investigations revealed hyponatremia, normal serum K; serum lactate dehydrogenase was moderately elevated. Primary adrenal insufficiency was confirmed by low basal and cosyntrophin stimulated cortisol. CT abdomen revealed huge bilateral heterogeneous enlargement of adrenal glands. Lymphoma was confirmed by histopathology and immunohistochemistry, diagnosis of PAL was made after further staging. He was treated with Rituximab-CHOP regimen. Second case, 50 years old lady presented with fever, anorexia, weight loss. She had generalized lymphadenopathy. On imaging found to have bilateral adrenal mass, and histopathology from lymph node, breast mass and adrenal mass was suggestive of non-Hodgkin's lymphoma.

Most primary adrenal lymphomas are diffuse large B-cell lymphomas with non-germinal center B-cell phenotype, BCL6 gene rearrangement and poor prognosis

Primary adrenal lymphoma is extremely rare, accounting for <1% of non-Hodgkin lymphomas, and lymphoma-associated chromosomal translocations have yet to be reported in this entity. We performed a retrospective study of 10 cases in immunocompetent patients including 4 males and 6 females with a median age of 68 years. The most common presenting symptoms were abdominal pain and fever; unexpectedly, clinically evident adrenal insufficiency was detected only in one patient. The mean tumor size at diagnosis was 8.5 cm. Half of the patients had bilateral involvement. All cases presented with stage IE disease without regional nodal involvement. Histologically, eight cases were diffuse large B-cell lymphoma, all of which carried a non-germinal center B-cell phenotype. Fluorescence in situ hybridization revealed BCL6 gene rearrangement in 5 (83%) of 6 diffuse large B-cell lymphomas investigated. The remaining cases were one case each of plasmablastic lymphoma and extranodal NK/T-cell lymphoma, nasal type, the first and third case of primary adrenal lymphoma of these particular lymphoma subtypes in the English literature, respectively. At a median follow-up of 4.5 months, 7 patients died of lymphoma, 1 died of an unrelated disease, 1 was alive with disease, and 1 was alive without disease. The prognosis of these patients was poor as compared with those with nodal diffuse large B-cell lymphoma. We speculate that the poor outcome of primary adrenal lymphoma might be related to the bulky tumor size at presentation, non-germinal center B-cell phenotype, and frequent BCL-6 gene rearrangement.

Primary adrenal lymphoma: a case series study

Dear Editor, Non-Hodgkin’s lymphoma affects the adrenal gland, usually in the advanced stage of the disease. In 30% to 50% of patients with primary adrenal lymphoma (PAL), there are signs compatible with subclinical adrenal insufficiency only detected by laboratory tests [2, 3]. Autopsy series show that 25% of patients present gland involvement, in some degree, during disease course [1]. PAL is a rare form of primary adrenal neoplasia, and the Englishlanguage medical literature has reported 70 cases of PAL; the Chinese has only 30 cases [3]. We had three cases, and they will be presented below. Case 1: An 80-year-old man, reporting abdominal pain, nausea, and loss of weight for 5 months. Physical examination: pale, hyperpigmentation of the skin, and cardiac arrhythmia. Abdominal magnetic resonance imaging: ovalshaped bilateral mass of the adrenal gland, swollen on its left side (5.5 cm). Laboratory tests: Table 1. The patient started glucocorticoid and mineralocorticoid therapy and went through CHOP chemotherapy regimen (cyclophosphamide, Adriamycin or hydroxydoxorubicin, vincristine, and prednisone) associated to rituximab. The clinical outcome was the patient’s death due to respiratory failure 2 months after the diagnosis. Case 2: A 62-year-old man presenting a history of asthenia, anorexia, loss of weight, postural hypotension for 6 months, associated with severe abdominal pain. Physical examination: blood pressure 110×80 mmHg (seating) and 80×70 mmHg (standing); ungual hyperpigmentation. Insulin resistance test with no cortisol enhancement; abdominal tomography: bilateral mass lesions of the adrenal gland, swollen on its right side (10 cm), with renal capsule invasion. Laboratory tests: Table 1. He started chemotherapy but died 6 months after due to pulmonary thromboembolism. Case 3: A 73-year-old woman, with diabetes, reporting hypoglycemia and decreased consciousness, using chlorpropramide. After having discontinued medication, she presented hyponatremia and hypoglycemia over 7 days (drug half-life). Physical examination showed disorientation and obnubilation. Laboratory exams disclosed bicytopenia (Table 1). Under suspicion of hematological neoplasia, she had an abdominal tomography that disclosed bilateral diffuse adrenal hyperplasia. She started chemotherapy associated with corticotherapy, making the full endocrine investigation impossible. The glycemic level and the natremia were normalized. Currently, the patient is under chemotherapy regimen (CHOP and rituximab) with a 14-month survival. Literature indicates that adrenal insufficiency is the most common clinical manifestation of PAL cases [5, 6] identified in the first two reported cases. Regarding case 3, unfortunately, there has not been enough time to make a more detailed investigation. Case 1 presented elevated serum levels of adrenocorticotropic hormone (ACTH), upholding the presence of skin hyperpigmentation. All the studied cases presented low levels of dehydroepiandrosterones (DHEAs), not confirming adrenal carcinoma. The radiological investigation of the three cases pointed out bilateral and heterogeneous images, which are the most typical forms described in literature. The fine-needle aspiraAnn Hematol (2008) 87:859–861 DOI 10.1007/s00277-008-0492-x

Lymphome primitif de la surrénale de phénotype B, diagnostiqué à la ponction—biopsie percutanée

The authors report a case of primary adrenal lymphoma in a 30-year old-female who complained of lumbar pain and was in poor general condition. Ultrasonography and CT scan revealed a heterogeneous mass with necrosis in the left adrenal gland. Non-Hodgkin's lymphoma of B-cell origin was determined by ultrasound-guided aspiration biopsy of the adrenal mass. Taking this case and the findings in the literature into consideration, the features of this disease have been reviewed and the problem of diagnosis, treatment and prognosis have been examined.