Primary adrenal lymphoma: a case series study

Schreiber, C.; Sakon, J. R.; Simião, F. P. C.; Tomarchio, Marcello; Huayllas, Martha Katherine Paniagua; Pereira, LG; Stella, Lenira Cristina; Santomauro, A.; Bueno, S. S. S.; Fraige, Fadlo

doi:10.1007/s00277-008-0492-x

Cited by 14 publications

(18 citation statements)

References 6 publications

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“…[1][2][3][4][5] Up to half of the small number of reported patients with primary adrenal lymphoma were associated with adrenal insufficiency and there is a high rate of bilateral adrenal involvement. [1][2][3][4][5] However, these data might be publication bias due to the rarity of this tumor. Pathologically, the great majority of cases were diffuse large B-cell lymphomas, 2,3 and when immunophenotyping were performed, these tumors usually expressed bcl-2 with a non-germinal center B-cell phenotype.…”

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

Mozos

Chuang

et al. 2009

Modern Pathology

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Primary adrenal lymphoma is extremely rare, accounting for o1% of non-Hodgkin lymphomas, and lymphomaassociated 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 nongerminal 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. Keywords: adrenal insufficiency; diffuse large B-cell lymphoma; NK/T-cell lymphoma; plasmablastic lymphoma; primary adrenal lymphoma; Taiwan Although secondary involvement of the adrenal glands by non-Hodgkin's lymphoma is not uncommon, primary adrenal lymphoma is extremely rare and accounts for o1% of all non-Hodgkin's lymphoma cases. 1 In the English literature, most of the papers on primary adrenal lymphomas are singlecase reports and literature reviews based on a small number of cases except two large series. [1][2][3][4][5] Up to half of the small number of reported patients with primary adrenal lymphoma were associated with adrenal insufficiency and there is a high rate of

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“…1 In the English literature, most of the papers on primary adrenal lymphomas are singlecase reports and literature reviews based on a small number of cases except two large series. [1][2][3][4][5] Up to half of the small number of reported patients with primary adrenal lymphoma were associated with adrenal insufficiency and there is a high rate of …”

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confidence: 99%

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

Mozos

Chuang

et al. 2009

Modern Pathology

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“…PAL is sometimes diagnosed radiologically as an ‘incidentaloma’ during an examination for abdominal pain 14. The radiological features of PAL on CT scans are variable, and this disease should be differentiated from tuberculosis, non-functional adrenal adenoma, adrenal carcinoma, pheochromocytoma and secondary metastasis.…”

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Bilateral primary adrenal lymphoma with adrenal insufficiency

Ekhzaimy¹,

Mujamammi

2016

BMJ Case Reports

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Primary adrenal lymphoma is an extremely rare condition. We describe a case of bilateral adrenal lymphoma in a man aged 55 years who was admitted to our hospital. He had a 3-month history of left flank pain, nausea and vomiting with weight loss. A CT scan at a private hospital revealed bilateral large adrenal masses; the patient was referred to our centre based on these findings. He was evaluated for pheochromocytoma by an endocrinology team; however, all findings were negative. In addition, a cosyntropin stimulation test indicated adrenal insufficiency. A Trucut biopsy of the adrenal gland revealed diffuse large B-cell lymphoma of the adrenal glands, and the patient responded extremely well to chemotherapy.

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“…Complete remission of disease after initiation of chemotherapy have been described in a few patients, with a followup of 12 months in one patient without remission of disease and a followup of 7 years in another patient with no signs of remission [10–12]. …”

Section: Discussionmentioning

confidence: 99%

Bilateral Primary Adrenal Lymphoma Presenting with Adrenal Insufficiency

Holm

Breum

Stenfeldt

et al. 2012

Case Reports in Endocrinology

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Lymphoma may occasionally involve the adrenal glands, but primary adrenal lymphoma (PAL) is very rare and only few cases have been reported. We present a case of a 60-year-old, otherwise healthy, woman, with bilateral PAL presenting with adrenal insufficiency. The patient responded initially upon administration of large doses of intravenously hydrocortisone with total remission of symptoms. An abdominal computerized tomography scan demonstrated bilateral adrenal lesions but did not demonstrate any other pathology. Since metastatic malignant disease was suspected a positron-emission-tomography scan was performed only showing significant uptake in the adrenal glands. Endocrine evaluation did not reveal abnormal function of any hormonal system and the patient was scheduled for bilateral adrenalectomy. However the clinical condition deteriorated rapidly and the patient was readmitted to hospital before surgery was performed. A new computerized tomography scan showed rapid progression of disease with further enlargement of the adrenal masses and both pulmonary and hepatic metastasis. Needle biopsy was performed but the patient refused further treatment and died before a diagnosis was obtained. The immuneohistochemical diagnosis was large B-cell lymphoma. This case should remind clinicians that PAL may be a cause of bilateral adrenal incidentaloma especially if the patient presents with adrenal insufficiency.

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Primary adrenal lymphoma: a case series study

Cited by 14 publications

References 6 publications

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

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

Bilateral primary adrenal lymphoma with adrenal insufficiency

Bilateral Primary Adrenal Lymphoma Presenting with Adrenal Insufficiency

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