Key result
Primary adrenal lymphoma presents with major symptoms and carries ~62% two-year survival despite polychemotherapy.
Why the study?
Primary adrenal lymphoma (PAL) is a rare disease with unclear clinical, radiological, and prognostic features that complicate accurate diagnosis and management.
Observational (n=28)
Yes
Primary adrenal lymphoma presents with major general symptoms and frequent bilateral involvement, carrying a poor prognosis despite polychemotherapy.
Answers are grounded in this paper and cite their sources.
Alerts clinicians to aggressive primary adrenal lymphoma course; leaves open optimal therapies pending prospective validation.
AIM: This study aimed to identify the clinical, radiological and prognostic features of primary adrenal lymphoma (PAL) in order to diagnose the disease more accurately. MATERIALS AND METHODS: A retrospective multi-centre study was conducted on the clinical, biological and radiological features as well as the treatment and overall survival outcomes in PAL. RESULTS: Between 1994 and 2014, 28 patients from five regions of eastern France were diagnosed with primary adrenal lymphoma. The revealing symptoms were a worsening general state (77%), weight loss (77%) and abdominal pain (42%). Biological features of PAL were almost omnipresent: increased LDH, β2 microglobulin, CRP or ferritinaemia levels. The PAL was bilateral in 20 cases (71%), adrenal insufficiency was searched for in 11 patients and found in eight (73%). CT scans showed masses of various sizes measuring up to 180 mm. On MRI, the lesions were hypointense in T1 and hyperintense in T2. When done, positron emission tomography with fluorodeoxyglucose (FDG-PET) showed locations not seen on the CT and revealed extra-adrenal locations in 70% of examinations. Adrenalectomy brought no benefit. The overall survival rate was poor (61.9% at 2 years) despite polychemotherapy. CONCLUSION: The clinical presentation of PAL comprised major general symptoms. Adrenal insufficiency was very common in patients with bilateral involvement but was not systematically tested. PET was an efficient examination to visualize extra-adrenal locations. The preliminary results of MRI to distinguish between PAL and adrenocortical carcinoma should be confirmed. Further studies are needed to establish an optimal strategy for the management of these primary adrenal lymphomas.
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Laurent et al. (2016) conducted an observational in Primary adrenal lymphoma (n=28). Primary adrenal lymphoma was evaluated on Overall survival at 2 years. Primary adrenal lymphoma presented with major general symptoms and had a poor overall survival rate of 61.9% at 2 years despite polychemotherapy.
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