Adrenal lymphoma: presentation, management and prognosis

Laurent, C; Casasnovas, Olivier; Martin, L.; Chauchet, Adrien; Ghesquières, Hervé; Aussedat, Guillaume; Lm, Fornecker; Bologna, Serge; Borot, Sophie; Laurent, Karine; Bouillet, Benjamin; Vergès, Bruno; Petit, J

doi:10.1093/qjmed/hcw174

Cited by 40 publications

(62 citation statements)

References 22 publications

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“…The overall survival was 61.6%; however, it was 100% for those who received autologous stem cell transplants, which suggests that this may prolong survival [6]. The Ki-67 index was high in our patient (80%).…”

Section: Discussionmentioning

confidence: 77%

“…Surprisingly, no difference was found in overall survival between unilateral and bilateral NHL of the adrenal gland [8]. Our patient also underwent adrenalectomy; however, the two largest studies to date showed no survival benefit in patients who underwent adrenalectomy as compared with those who were treated with chemotherapy alone [6, 8]. …”

Section: Discussionmentioning

confidence: 84%

“…Most commonly, this lymphoma is a nongerminal center-type diffuse large B-cell lymphoma (DLBCL), which is present in 70% to 80% of the patients [6]. Patients present with abdominal or lumbar pain; fever; weight loss; and signs of adrenal insufficiency such as hypotension, hyponatremia, fatigue, skin hyperpigmentation, and vomiting [7].…”

Section: Introductionmentioning

confidence: 99%

“…Several etiological factors, such as Epstein-Barr virus infection, genetic defects in p53 and c-kit, and immune dysregulation, have been implicated in the pathogenesis of this disease [5, 7]. Laboratory investigations often show elevated lactate dehydrogenase (LDH), β 2 -microglobulin, C-reactive protein, and ferritinemia, which signify high levels of inflammation associated with PAL [6]. …”

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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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.

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Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 84%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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“…The clinical course of the disease is aggressive, with most cases associated with a poor prognosis . Additional metastasis of the primary, is further associated with a worse prognosis.…”

Section: Discussionmentioning

confidence: 99%

Cytological diagnosis of bilateral primary adrenal lymphoma with cutaneous involvement

Rakheja

Handa

Kaur

et al. 2020

Diagnostic Cytopathology

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Primary adrenal lymphoma (PAL) is an extremely rare condition. We describe here, a case of bilateral adrenal lymphoma in a 62‐year‐old man. He later developed subcutaneous masses on the hand and the leg. Fine‐needle aspiration cytology from the adrenals and the soft tissue swellings led to a diagnosis of non‐Hodgkin's lymphoma (NHL). Histopathological examination from the lesion on the leg, confirmed the diagnosis to be B‐cell NHL. The case highlights the cytomorphological findings of this unusual case. Awareness of this entity is essential to differentiate it from other common causes of adrenal enlargement and formulate an appropriate treatment.

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Primary extranodal diffuse large B‐cell lymphoma: Molecular features, treatment, and prognosis

Chen

et al. 2022

Aging and Cancer

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Diffuse large B‐cell lymphoma (DLBCL) is the most common subtype of non‐Hodgkin's lymphoma and represents a heterogeneous entity. One‐third of DLBCL arises from extranodal organs and its prognosis often varies with regard to the sites involved. Molecular features are important to elucidate the differences in clinical features, predict the disease prognosis, and improve effective targeted therapeutic strategies. Extranodal DLBCLs originated from the breast, skin, uterus, immune‐privileged sites such as the central nervous system and testes, often show a high proportion of non‐germinal center B‐cell‐like (non‐GCB) phenotypes, with a high frequency of MYD88/CD79B mutations. In contrast, extranodal DLBCLs originated from the thyroid gland and stomach show a relatively low proportion of non‐GCB phenotype, with a considerably excellent prognosis. Immunochemotherapy with rituximab is the standard of care in both nodal and extranodal DLBCLs. However, approximately 40% of the patients experience treatment failure. It is critical to optimize the treatment strategy, including radiotherapy, autologous stem cell transplantation and targeted therapy according to the clinical characteristics and molecular heterogeneity. In this review, we present an overview of the key molecular pathways, prognosis assessment and innovative therapies in primary extranodal DLBCLs.

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Adrenal lymphoma: presentation, management and prognosis

Cited by 40 publications

References 22 publications

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

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

Cytological diagnosis of bilateral primary adrenal lymphoma with cutaneous involvement

Primary extranodal diffuse large B‐cell lymphoma: Molecular features, treatment, and prognosis

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