Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study

Abstract

<jats:p>Purpose: We sought to refine the clinical picture of primary adrenal lymphoma (PAL), a rare lymphoid malignancy with predominant adrenal manifestation and risk of adrenal insufficiency. </jats:p> <jats:p>Methods: 97 patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017. </jats:p> <jats:p>Results: Of 81 patients with imaging data, 19 (23%) had isolated adrenal involvement (iPAL), while 62 (77%) had additional extra-adrenal involvement (PAL+). Among patients who had both CT and PET scans, 18FDG-PET revealed extra-adrenal involvement not detected by CT scan in 9/18 cases (50%). The most common clinical manifestations were B symptoms (55%), fatigue (45%), and abdominal pain (35%). Endocrinological assessment was often inadequate. With a median follow-up of 41.6 months, 3-year progression-free (PFS) and overall (OS) survival rates in the entire cohort were 35.5% and 39.4%, respectively. The hazard ratios of iPAL for PFS and OS were 40.1 (95% CI: 2.63-613.7, p=0.008) and 2.69 (95% CI: 0.61-11.89, p=0.191), respectively. PFS was much shorter in iPAL versus PAL+ (median 4 months vs. not reached, p=0.006), and OS also appeared to be shorter (median 16 months vs. not reached), but the difference did not reach statistical significance (p=0.16). Isolated PAL was more frequent in females (OR=3.81; P=0.01) and less frequently associated with B symptoms (OR= 0.159; p=0.004). </jats:p> <jats:p>Conclusion: We found unexpected heterogeneity in the clinical spectrum of PAL. Further studies are needed to clarify whether clinical distinction between iPAL and PAL+ is corroborated by differences in molecular biology. </jats:p>

Adrenal Schwannoma in an Elderly Man: A Case Report and Literature Review

“…Primary adrenal lymphoma can be confined to the adrenal gland and was considered a differential diagnosis in our case. Primary adrenal lymphomas have been reported to have a median Hounsfield density of 35 HU on CT, hypointensity on T1-weighted images, and hyperintensity on T2-weighted images, similar to our observed in case ( 16 , 17 ). However, primary adrenal lymphoma was deemed unlikely, as the median largest dimension of primary adrenal lymphomas was 80 mm (range, 40-180 mm), the SUVmax of the tumor was high (median 17.24; range, 9.5-48), and extra-adrenal tumor localization was frequent (range, 68-77%) ( 16 , 17 ).…”

Synchronous gastric cancer and primary lymphoma of right adrenal gland: a case report

“…However, primary adrenal lymphoma is rare, accounting for 3% of all cases of primary extranodal lymphomas [1]. Among primary adrenal lymphomas, the most frequent histology is DLBCL, accounting for 76.3-78.6% of all cases, and common symptoms include B symptoms (fever, night sweats, and weight loss), fatigue, abdominal pain, and anorexia, which are consistent with the patient in this case report [27,32,33]. Primary adrenal DLBCL is a disease of the elderly, with a median age at diagnosis of 71 years, and more likely to affect men, with a male-to-female ratio of 2:1, which are also consistent with the patient in this case report [1].…”

Frequently asked questions and answers (if any) in patients with adrenal incidentaloma

“…Bilateral non-adrenal malignancies (metastasis, adrenal lymphoma [ 149 ]) may lead to adrenal insufficiency due to neoplastic infiltration of the adrenal glands: in selected cases, especially if imaging is not conclusive for lipid-rich AI and ACTH levels are increased, an insufficient cortisol secretion should be reasonably suspected.…”