Spectrum of imaging findings of primary bone lymphoma in pediatric patients.

Primary bone lymphoma, a rare oncologic entity, may initially present with minimal symptoms. Presenting symptoms range from local pain and mild systemic symptoms to large palpable masses and pathologic fractures. The term "primary bone lymphoma" indicates the finding of bone involvement without other organ sites for at least 6 months. Although some radiological features may raise suspicion about this tumor form, there are no pathognomonic imaging findings, and the diagnosis will likely be delayed for a long time. The most critical radiological feature is soft tissue involvement associated with a preserved cortical layer, much more than expected for an infiltrating lesion. Anyway, very different radiological findings may be displayed in patients with primary bone lymphoma. Although these radiological features of primary bone lymphoma have been discussed in the literature by various authors, there is little data concerning imaging in pediatric patients. This paper aims to depict the possible spectrum of imaging features of primary bone lymphoma in the pediatric age, providing an exemplification pictorial essay extracted from a single institution experience in the year range period 2006-2022.

Extraskeletal Ewing Sarcoma of the Extremities and Trunk: A Retrospective Analysis of a Mono-Institutional Series.

INTRODUCTION: Extraskeletal Ewing sarcoma (EEwS) is a rare malignant tumor, and current international recommendations indicate systemic and local treatment like bone Ewing sarcoma (BEwS); to the best of our knowledge, very few studies tried to explore the clinical and genetic characteristics of this tumor, and the most appropriate treatment strategy remains uncertain. METHODS: We reviewed 35 EEwS cases enrolled at Rizzoli Orthopedic Institute in Bologna, Italy, between 1988-2022. We performed RNA sequencing in 18 Ewing sarcoma cases, including 12 BEwSs and 6 EEwSs. We analyzed overall survival (OS), local relapse-free survival (LRFS), and metastasis-free survival (MFS) and the risk factors associated to survival. RESULTS: Unsupervised hierarchical clustering showed no differences in the transcriptional profile between EEwS and BEwS. Five-year OS was 67% (95% confidence interval [CI]: 47-80), 5-year LRFS was 61% (95% CI: 43-75), and 5-year MFS was 55% (95% CI: 38-70). Recurrent tumors, larger than 8 cm, and elevated lactate dehydrogenase (LDH) serum value resulted to be negative prognostic factors. CONCLUSIONS: The finding/detection of a genetic profile that is indistinguishable between EEwS and BEwS confirms the view that the two subgroups belong to the same tumor entity and supports the use of a single therapeutic approach for Ewing sarcoma, regardless of the site of origin. Statistical evaluation showed that size bigger than 8 cm, elevated LDH, and recurrent tumors had a worse prognosis, suggesting a risk-stratification method for identifying patients for specific therapy treatment. However, larger, multicenter, prospective trials are called for to validate our findings.

Sensitivity, Specificity, and Predictive Values of Tru-Cut® Biopsy in Grading Primary Localized Myxoid Liposarcomas of the Extremities.

(1) Background: Histological diagnosis and tumor grading are major prognostic and predictive factors in soft tissue sarcomas (STS), as they dictate the treatment strategies with a direct impact on patient survival. This study aims to investigate the grading accuracy, sensitivity, and specificity of Tru-Cut® biopsy (TCB) in primary localized myxoid liposarcomas (MLs) of the extremities and its impact on patient prognosis. (2) Methods: Patients with ML undergoing TCB and a subsequent tumor resection between 2007 and 2021 were evaluated. Concordance between the preoperative assessment and definitive histology was calculated with a weighted Cohen's kappa coefficient. Sensitivity, specificity, and diagnostic accuracy were calculated. (3) Results: Of 144 biopsies, the histological grade concordance rate was 63% (Kappa 0.2819). Neoadjuvant chemotherapy and/or radiotherapy impacted concordance with a downgrading effect in high-grade tumors. Among forty patients not treated in neoadjuvant settings, the sensitivity of TCB was 57%, the specificity was 100%, and the overall predictive values of positive and negative TCB were 100% and 50%, respectively. Misdiagnosis did not impact overall survival. (4) Conclusions: TCB may underestimate ML grading due to tumor heterogeneity. Neoadjuvant ChT and/or radiotherapy are associated with pathological downgrading; however, discordance in diagnosis does not modify patient prognosis because systemic treatment decision-making also includes other variables.

Total hip arthroplasty in hereditary multiple exostosis patients: literature review and evaluation of 10 cases.

BACKGROUND: Acquired hip deformities in patients affected by hereditary multiple exostosis (HME) may incur in early hip osteoarthritis and functional limitation requiring primary total hip arthroplasty (THA). Characteristic coxo-femoral joint dysmorphisms in HME may pose a challenge for the orthopaedic surgeon. Here we report our experience in a series of patients with HME treated in our hospital with THA. METHODS: With a mean follow-up of 5 years, 10 primary THAs were reviewed; proximal femur deformities, acetabular dysplasia and joint osteoarthritis has been assessed through x-rays and CT-scan evaluation. In all cases hemispheric press-fit cups were used; 4 stem had metaphyseal engagement, 5 had proximal diaphyseal engagement and 1, with anatomical geometry, had metaphyseal fixation. 2 cases required stem cementation, 3 modular neck and 1 lateralised. The clinical data, complications and clinical outcomes, were recorded and analysed. RESULTS: The mean Harris Hip Score (HHS) increased from 34 preoperative to 86 postoperative; preoperative mean neck shaft angle (NSA) was 150°, head/neck ratio 0.6, offset 31 mm; Wiberg angle 28°, Sharp angle 38°, 1 patient had subluxation grade 4 according to Crowe, 8 hips showed osteoarthritis (Tönnis grade ⩾2 ); 5 femurs were classified as Dorr type C, 2 as type B and 3 as type A. Perioperative complications were not observed. CONCLUSIONS: Primary THA in HME significantly improved clinical and functional outcomes. Press-fit cup fixation together with metaphyseal and proximal diaphyseal stem engagement on reliable bone quality femur, represents a valid option in HME patients with normal acetabular morphology, wide broaden neck and valgus NSA.

Primary High-Grade Myxoid Liposarcoma of the Extremities: Prognostic Factors and Metastatic Pattern.

(1) Background: This retrospective study aimed to analyze the history and treatment outcomes of localized, high-grade MLS of the extremities. (2) Methods: We retrospectively reviewed 82 patients with primary high-grade MLS of the extremities. OS, LRFS, MFS, PRS, and DFS were analyzed. (3) Results: Five-year OS and LRS were 96% (95% CI: 86-98) and 94% (95% CI: 85-98), respectively. Statistical analysis indicated no risk factors for OS and LFRS. MFS was 77% (65-85) at 5-year follow-up. Size (p = 0.0337) was the only risk factor statistically significant for MFS (HR = 0.248, 95% CI: 0.07-0.84). Median PRS after distant metastasis was 34 months (range: 1-127 months). Five-year PRS was 79% (48-93). Overall, the 5-year DFS was 76% (65-85). (4) Conclusions: Patients with MLS were found to have a good prognosis. In high-grade deep-seated tumors, common risk factors for MLS do not correlate with survival. Tumor size appears to be the only predictor of long-term DSF and MSF.