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A 53-year-old woman presented with a growing mass in the left lumbar region. The patient had become casually aware of it 10 years ago. Since then she performed radiological and clinical follow-up; she refused surgical resection. In 2009, a cytological examination had displayed mesenchymal cells without nuclear atypia. In the last year, the tumor had been growing and had become symptomatic with pain during physical activity. Clinical examination revealed an unmovable swelling on the left lumbar region with intact skin. MRI showed a 9 3 435 cm T1 hypointense and T2 hyperintense mass, with a strong and heterogeneous enhancement after contrast infusion and a hypointense central core. It extended from the body of the second to the fifth lumbar vertebra. CT scan of the chest also revealed four pulmonary nodules enhancing with contrast, two of which were hypermetabolic during the whole-body PET-CT scan (max SUV 2.7 and 2.4). The patient underwent surgical resection of the lumbar lesion which was easily removed with sharp dissection from the surrounding muscle fibers. PATHOLOGICAL FINDINGSAt gross examination, the mass was encapsulated and its cut section was multinodular, glistening, mainly white and firm, with central areas of bone consistency ( Figure 1A). Histopathological examination of hematoxylin and eosin stain showed a partially circumscribed tumor, with biphasic appearance. The main aspect was a proliferation of atypical spindle cells arranged in fascicles, mitotically active (7 mitoses/10 HPF) and focally embedded in a myxoid matrix ( Figure 1B,C); several foci of necrosis were detected. The second aspect was more differentiated and made of elongated bundles, interweaving fascicles and loose whorls of cells with wavy nuclei, without pleomorphism and in absence of mitoses ( Figure 1D,E). Bone metaplasia was present between these two areas. Immunohistochemical staining revealed diffuse reactivity to GLUT1 ( Figure 1F), CD10 ( Figure 1G), EMA ( Figure 1H) and Bcl2 in the most differentiated area but absence of reactivity in the most atypical areas. Both of them were focally reactive to CD34. Negative were S100 and CD99.Ultrastructural examination revealed the presence of spindle cells containing prominent pinocytic vesicles and surrounded by a focally discontinuous external lamina ( Figure 1I). What is your diagnosis? Figure 1.

Introduction PCSK9 inhibitors (PCSK9i) significantly decrease LDL cholesterol (LDL-C), either as monotherapy or in addition to the maximally tolerated dose of statin and/or ezetimibe. Yet, few data are available on efficacy and background lipid-lowering therapy (LLT) adjustment in patients treated with PCSK9i in real-world observations. Purpose AT-TARGET-IT is an Italian multicenter registry involving 9 Italian centers, designed to assess efficacy, adherence, and persistence of PCSK9i, as well as prescribing doctors' behavior in patients with atherosclerotic cardiovascular disease (ASCVD) or familial hypercholesterolemia (FH). The aim of the present analysis was to assess efficacy and changes in background LLT therapy in patients on PCSK9i in a real-world single country observation. Methods From June through November 2021, we enrolled patients with PCSK9i first prescription from 6 months before inclusion through starting of PCSK9i use. Clinical and demographic characteristics, concomitant therapies, blood chemistry, were recorded at the time of first prescription and at the latest observation preceding inclusion in the study. Background therapy was assessed at baseline and during follow-up, evaluating treatment withdrawal, reduction of doses, or changes from statin-ezetimibe association to single drug therapy. Results We enrolled 798 patients (27% with FH) receiving either alirocumab or evolocumab and followed for a median time of 19.3 months. At the time of PCSK9i first prescription LDL-C was 147.6 mg/dl and reached 51.5 mg/dl at the time of latest observation (64% reduction), and 129 patients (16%) were not receiving any LLT, 669 patients received background LLT, of them 246 (31%) were taking ezetimibe alone and 423 (53%) were taking statins with or without ezetimibe. At the end of the observation period, 785 patients (98%) were still receiving PCSK9i and 550 (69%) did not change background LLT. Of 248 patients changing background LLT, 116 (47%) withdrew therapy, 132 (53%) changed dose or type of LLT. After stratification by achievement of LDL-C target according CV risk class, 483 patients achieved the target (60%). Target was achieved at the end of the observation period in 63% of patients taking triple therapy, 65% patients receiving PCSK9i plus statins, 62% of patients receiving PCSK9i plus ezetimibe and 55% receiving PCSK9i alone (Figure 1). No significant differences in terms of percentage of patients changing background LLT during PCSK9i treatment were found between patients at target for LDL-C and those not at target. Conclusion AT-TARGET-IT study shows that PCSK9i therapy is effective in reaching LDL-C target in the majority of patients, yet a sizable number of them (40%) remains undertreated. LLT background therapy is either reduced or withdrawn in 31% of patients, being responsible for not reaching target. Reasons for inappropriate LLT changes in patients receiving PCSK9i should be identified and removed to optimize lipid control. Funding Acknowledgement Type of funding sources: None.

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