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

Ram, Nanik; Rashid, Owais; Farooq, Saba; Ulhaq, Imran; Islam, Najmul

doi:10.1186/s13256-017-1271-x

Cited by 19 publications

(14 citation statements)

References 7 publications

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“…Age, tumor size, adrenal insufficiency, lactate dehydrogenase level, and performance status of the patient can significantly influence prognosis. [ 8 , 10 , 11 ] Interestingly, we also note that PAL could relapse with secondary CNS lymphoma, but only 6 cases of PAL relapse with CNS lymphoma have been reported. We still do not know if there is a link between CNS and adrenal lymphoma because of the limited number of cases reported.…”

Section: Discussionmentioning

confidence: 77%

Adrenal relapse of primary central nervous system diffuse large B-cell lymphoma

Sen

Jug

et al. 2018

Medicine

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Rationale:Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma with a dismal outcome. Most patients relapse in intracranial sites and <5% of patients relapse in extracranial sites. Here, we present the first case of PCNSL with an adrenal relapse.Patient concerns:A 72-year-old woman, first presented 7 years ago with complaints of headache and dizziness.Diagnoses:Enhanced magnetic resonance imaging revealed the mass within the splenium of the corpus callosum. On histological examination, there was a diffuse growth pattern of neoplastic cells in the brain biopsy. Immunohistochemistry and flow cytometric analysis demonstrated that the neoplastic cells were of B-cell lineage.Interventions:The patient underwent methotrexate-based chemotherapy and whole-brain radiotherapy after the initial diagnosis of primary central nervous system-large B-cell lymphoma (CNS-DLBCL).Outcomes:After 4 years of clinical remission, the patient was diagnosed with endometrial cancer. Interestingly, a radiological study following the treatment of endometrial cancer demonstrated a right adrenal mass, which was suspicious for malignancy. Morphologic examination and immunohistochemistry studies confirmed the diagnosis of diffuse large B-cell lymphoma. A fluorescent in situ hybridization panel for lymphoma showed rearrangement of Immunoglobulin heavy chain (IGH) and B-cell lymphoma 6 (BCL6), respectively, suggesting fusion of BCL6/IGH. Immunoglobulin kappa analysis demonstrated a common origin for the brain and adrenal lesions, which led to the final diagnosis of an adrenal relapse of CNS-DLBCL.Lessons:PCNSL is a highly infiltrative neoplasm, particularly at relapse. To the best of our knowledge, this is the first case of CNS-DLBCL with adrenal relapse. Considering the poor outcome of CNS-DLBCL, molecular genetic studies should be done to identify a common origin for the primary and secondary lesion.

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Section: Discussionmentioning

confidence: 77%

Adrenal relapse of primary central nervous system diffuse large B-cell lymphoma

Sen

Jug

et al. 2018

Medicine

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“…The clinical and imaging characteristics of the case were consistent with the reported PAL cases. [ 1 , 3 , 9 ]…”

Section: Discussionmentioning

confidence: 99%

“…PAL is based on a histologically confirmed lymphoma involving 1 or 2 adrenal glands, with no previous history of lymphoma, and if other tissues or lymph nodes are involved in addition to the adrenal glands, adrenal lesions must be significantly dominant. [ 1 – 3 ]…”

Section: Introductionmentioning

confidence: 99%

A case report of primary adrenal lymphoma

Yang¹,

Xie

Ren³

et al. 2020

Medicine

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Rationale: Primary adrenal lymphoma (PAL) is an extremely rare and highly invasive malignant disease. Imaging examination usually shows bilateral adrenal involvement with large tumor masses and local infiltration. However, it is unclear how lymphoma dynamically develops into huge tumor masses in the adrenal glands. The overall survival rate of PAL is generally poor, and the underlying mechanism might be related to prooncogenic mutation but not fully elucidated. Patient concerns: A 52-year-old woman complaining of a large mass in the left adrenal region for 1 month was admitted to our department. Diagnosis: Computed tomography firstly showed a huge mass (8.9 × 7.5 cm) in the left adrenal gland and diffusely enlarged right adrenal gland. A month later, the mass in the left adrenal gland further enlarged (9.5x7.5 cm) with infiltration of the left renal artery and retroperitoneal lymphadenopathy, and the right adrenal gland rapidly progressed into a huge mass (8.0x4.7 cm). Additionally, her chest computed tomography revealed mediastinal and bilateral hilar lymphadenopathy. Then an adrenal biopsy confirmed the diagnosis of diffuse large B-cell lymphoma, nongerminal center B-cell type, stage IV by Ann Arbor staging system. Immunohistochemistry showed positivity for Ki-67 (approximately 90%), BCL2 (approximately 80%) and MYC (approximately 70%) double-expressor lymphoma. Interventions: The patient's condition progressed rapidly, there was no opportunity to use pathology-based chemotherapy. Dexamethasone was given intravenously by thoracic and intraperitoneal injection; antibiotics and supporting treatment were also given. Outcomes: The patient's condition progressed rapidly, with the development of malignant chest and abdominal cavity fluid and lung infection, and eventually developed septic shock and respiratory failure. She responded poorly to treatment regimens, and eventually died 8 days after the diagnosis of PAL. Lessons: PAL grows progressively throughout the adrenal glands, high Ki-67 positivity and BCL2/ MYC co-expression predict rapid progress and poor prognosis.

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“…There was still no consensus on the treatment of primary adrenal lymphoma. Some authors had used a combination of surgery and chemotherapy [29] . While ensuring multiple variables comparable with propensity score methodology, patients who had undergone surgery had a better prognosis than the non-surgical group in the present study.…”

Section: Discussionmentioning

confidence: 99%

Summary of clinical characteristics of common primary adrenal malignancies in adults

zhang¹,

wang²,

Li³

et al. 2020

Preprint

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Background To summary the clinical characteristics of common primary adrenal malignancies in adults, the survival time of the patients, and the prognostic factors associated with these rare malignancies. Methods The Surveillance, Epidemiology, and End Results database (1975–2016) was queried for all patients who were diagnosed with primary adrenal malignancies, including adrenocortical carcinoma(ACC), pheochromocytoma and paraganglioma (PPGL), lymphoma, and sarcoma (SA). The clinical characteristics, overall survival (OS) and cancer-specific survival (CSS) were analyzed, in which propensity score methodology, logistic regression modeling and Kaplan-Meier survival curves were used. The Multivariate Cox proportional hazard model was used to identify factors affecting the prognosis of patients with ACC. Results 3204 patients with primary adrenal malignancies were identified, including 2180 with ACC, 593 with PPGL, 307 with lymphoma, and 124 with SA. Generally, PPGL had a better prognosis than others. For ACC and PPGL, the non-metastatic group had better OS and CSS than the metastatic group; for SA, the non-metastatic group had better OS, however, there was no significant difference in CSS between the two groups. Furthermore, For ACC, the single metastasis group had a better OS than the multiple metastasis group. After tumors metastasis, the surgery group, surgery of primary adrenal lesions, had better OS and CSS than the non-surgery group for ACC and PPGL. For lymphoma, the chemotherapy group had better OS and CSS; for ACC and PPGL, the chemotherapy group had better CSS and worse OS; for SA, the survival showed no significant difference between the two groups. Radiotherapy had the same effect on patients' survival as chemotherapy, except for the lymphoma and the ACC. As for lymphoma, the radiotherapy group had better CSS, whereas the survival showed no significant difference in OS between the two groups. For ACC, the non- radiotherapy group had better CSS and OS. More importantly, for lymphoma, under the condition that both cohorts were the same basically, the surgical group had a better prognosis. The prognosis of ACC was related to sex, age, seer historic stage, surgery, chemotherapy. Conclusion Understanding the clinical characteristics of these tumors and factors affecting prognosis can facilitate the selection of more appropriate clinical treatment options.

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

Cited by 19 publications

References 7 publications

Adrenal relapse of primary central nervous system diffuse large B-cell lymphoma

Adrenal relapse of primary central nervous system diffuse large B-cell lymphoma

A case report of primary adrenal lymphoma

Summary of clinical characteristics of common primary adrenal malignancies in adults

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