No abstract

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.

number 16_136). CPRD collects deidentified patient data from a network of general practitioner practices across the UK. Primary care data are linked to a range of other health-related data to provide a longitudinal, representative UK population health dataset. The data encompass 50 million patients, including 14 million currently registered patients. Additional methods and results are available upon direct request. In our cohort there were 12 057, 17 614 and 80 854 patients in the whole of the UK living with MM, SCC or BCC, respectively. The prevalence of MM was 0Á3%, SCC had a prevalence of 0Á4%, and BCC had the highest prevalence at 1Á8%. Over the 10-year study period, there was a 36% increase in the age-adjusted incidence of MM, a 34% increase in SCC and a 21% increase of BCC. Sex-stratified incidence per 100 000 person years (PYs) was age-adjusted (SEM) for 2004 and then 2014 [for MM, 15Á8 (0Á9) to 26Á4 (1Á0) for men and 22Á4 (1Á1) to 25Á5 (1Á0) for women; for SCC, 48Á2 (1Á4) to 66Á0 (1Á6) for men and 25Á4 (1Á2) to 32Á6 (1Á2) for women; and for BCC, 161Á2 (2Á6) to 201Á1 (2Á7) for men and 124Á0 (2Á5) to 143Á8 (2Á5) for women].

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. Methods: 97 patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017. 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). 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.

No abstract