Late Local Recurrence and Metastasis in Soft Tissue Sarcoma of the Extremities and Trunk Wall: Better Outcome After Treatment of Late Events Compared with Early.

BACKGROUND: Approximately 80% of soft tissue sarcoma (STS) recurrences, local and metastatic disease, are diagnosed within the first 3 years after primary diagnosis and treatment. Recurrences, however, can present after a longer period of remission. Our goal was to identify factors that may predict the risk of late recurrence. METHODS: We identified 677 patients with STS of the extremities and trunk wall from a population-based sarcoma register. Of these, 377 patients were alive and event-free at 3 years and were included for analysis of possible risk factors for late recurrence. RESULTS: Fifty-five of 377 (15%) patients developed late recurrence: 23 local recurrence, 21 metastasis, and 11 both manifestations. With R0 wide surgical margin as reference, R0 marginal (hazard ratio [HR] 2.6; p = 0.02) and R1 (HR 5.0; p = 0.005) margins were risk factors for late local recurrence. Malignancy grade (HR 8.3; p = 0.04) and R0 marginal surgical margin (HR 2.3; p = 0.04) were risk factors for late metastasis. We could not find a statistically significant correlation of late recurrence with many of the generally known risk factors for local recurrence and metastasis in STS. Outcome after treatment of late recurrences was better compared with outcome after treatment of early events. CONCLUSIONS: Late recurrences, albeit relatively rare, do occur. Outcome after treatment was good compared with outcome after early events. Long surveillance of all patients with high-grade STS, especially if R0 wide surgical margin is not achieved in the primary treatment, appear to be well justified.

Comparative cytological and histological assessment of 828 primary soft tissue and bone lesions, and proposal for a system for reporting soft tissue cytopathology.

INTRODUCTION: The aim of the study was to evaluate the diagnostic utility of fine needle aspiration (FNA) cytology and core needle biopsies (CNBs) in a series of primary soft tissue and bone lesions and to test a possible system for reporting results of FNA cytology of soft tissue lesion. METHODS: This retrospective study encompassed 828 primary soft tissue and bone lesions, analysed with FNA, CNB and/or surgical specimen in order to perform sensitivity/specificity as well as accuracy analyses. The series was then used to test a system for reporting soft tissue cytopathology with six categories and the risk of malignancy in each category was calculated. RESULTS: With a malignant diagnosis defined as positive test result, FNA and CNB analysis showed sensitivity of 87% and 94%, respectively, and specificity of 89% and 95%, respectively. FNA and CNB analyses identified the correct histopathological entity of the examined lesion in 55% and 66%, respectively. The risk of malignancy within the tested categories was non-diagnostic 42%, non-neoplastic 0%, atypia of unknown significance 46%, neoplasm benign 3%, neoplasm of unknown malignant potential 27%, suspicious for malignancy 72% and malignant 97%. CONCLUSION: FNA cytology is a suitable tool to determine the malignant potential of a sampled soft tissue/bone lesion but is inferior to CNB in defining the correct entity. A standardised reporting system might improve the clinical management of patients with soft tissue tumours examined primarily by FNA cytology.

High Recurrence Rate of Myxofibrosarcoma: The Effect of Radiotherapy Is Not Clear.

BACKGROUND: Myxofibrosarcoma (MFS) is one of the more common types of soft-tissue sarcoma (STS) in patients over 60 years of age. Local recurrence (LR) rates have been reported to be higher compared to other STS types. PATIENTS AND METHODS: Using a population-based series from the southern Sweden health care region, 56 consecutive patients with MFS and localized disease at diagnosis were analyzed with respect to LR and distant metastases after surgery ± adjuvant treatment. RESULTS: The overall local recurrence (n = 15) and metastasis (n = 13) rates were 27% and 21%, respectively; 6 patients had both. Surgical margin was the only statistically significant prognostic factor for LR. Patients operated with a marginal margin had an HR of 4.5 (CI 1.3-15.1, p=0.02) and those operated with an intralesional margin 9.4 (CI 2.0-43.5, p=0.004) compared to those operated with a wide surgical margin. There was no difference in the LR rate depending on radiotherapy or not, although the latter group had smaller and more superficial tumors. 23 patients received radiotherapy, 9 of whom developed LR, all within the irradiated field. A tumor size >5 cm and intralesional surgical margin were shown to be risk factors for distant metastases. CONCLUSIONS: The rate of LR for patients with myxofibrosarcoma was high. The impact of RT on local tumor control was unclear. The surgical margin was important for both local and distant tumor control. Large tumor size was a risk factor for distant metastasis.

Comprehensive genetic analysis of a paediatric pleomorphic myxoid liposarcoma reveals near-haploidization and loss of the RB1 gene.

AIMS: Pleomorphic myxoid liposarcoma (PML) is an exceptionally rare and poorly studied subtype of liposarcoma, occurring typically in children and adolescents. The few previous genetic studies have shown that PML lacks the gene fusions and amplifications that characterize myxoid liposarcoma, atypical lipomatous tumour and de-differentiated liposarcoma. To learn more about its pathogenesis, we performed a comprehensive genetic analysis, including chromosome banding, fluorescence in-situ hybridization, single nucleotide polymorphism (SNP) array analysis, deep sequencing of the exome (WES) complemented by targeted sequencing of hot-spot regions of selected cancer-associated genes and transcriptome sequencing (RNA-seq) of a PML in a 10-year-old boy. METHODS AND RESULTS: Banding analysis revealed a hyperdiploid/hypotriploid karyotype that at SNP array analysis could be shown to derive from a near-haploid ancestral clone. Structural imbalances were few, but included homozygous loss of the RB1 locus; no fusion transcripts were identified at RNA-seq, no somatic mutations were seen at gene panel analysis and the most interesting mutation detected at WES involved KMT2D. CONCLUSION: The results support the notion that PML is a distinct type of liposarcoma, associated with a spectrum of somatic mutations that is different from that in other liposarcoma subtypes. The findings in the present case, combined with previous data, suggest that PML develops through combinations of numerical chromosome aberrations, possibly initialized by haploidization. The results also suggest that inactivation of RB1 is pathogenetically important.

The prevalence and severity of low back pain and associated symptoms in 3,009 old men.

PURPOSE: The aim of this study is to evaluate the prevalence and severity of low back pain (LBP) and the influence of sciatica and neurological deficits in old men. MATERIAL AND METHOD: Mister osteoporosis Sweden includes 3,014 community-dwelling men aged 69-81 years. At study start 3,009 participants answered questions on LBP, low back pain and sciatica (LBP + SCI) or low back pain and sciatica with associated neurological deficits (LBP + SCI + NEU) during the preceding 12 months. Data are presented as proportions or medians with mid-quartile ranges. Differences between groups were tested by χ(2) test and Kruskall-Wallis test. RESULTS: 24% had experienced LBP without SCI, 8% LBP + SCI and 14% LBP + SCI + NEU. 10% of the men with LBP, 22% of those with LBP + SCI, and 36% of those with LBP + SCI + NEU rated the pain as severe (p < 0.001). 23% of the men with LBP, 31% of those with LBP + SCI and 50% of those with LBP + SCI + NEU reported limitation in activity of daily living (ADL) (p < 0.001). Men with only LBP had to restrict their activities for 7 days (3-14), those with LBP + SCI 6 days (2-14) and those with LBP + SCI + NEU 10 days (3-30) (p < 0.05). CONCLUSIONS: The 1-year prevalence of LBP in community living men aged 69-81 years was close to 50% but for individuals with LBP or LBP + SCI the morbidity was low with more than two-thirds having no limitations in ADL. In men with LBP + SCI + NEU more than one-third rated the pain as severe and close to half had limitations in ADL.