Primary anatomical site as a prognostic factor for pleomorphic liposarcoma.

BACKGROUND: Pleomorphic liposarcomas (PLS) is an aggressive, high-grade subtype of soft tissue sarcoma representing < 15% of liposarcomas. It most commonly arises in the retroperitoneum and proximal upper extremities. Current prognostic factors are centered around staging, which accounts for the grade, size, and location of the tumor in relation to the superficial fascia. METHODS: 750 patients diagnosed with pleomorphic liposarcoma from the National Cancer Database were analyzed. Kaplan-Meier survival tables, log-rank tests, and Cox proportional hazards analysis were utilized to compare survival between groups within variables. RESULTS: The most common primary anatomical site was the lower limb/hip. The head/neck primary anatomical site demonstrated the highest 10-year overall survival probability, while the retroperitoneum/abdomen had the lowest (50% and 18.4%). Compared to the thorax/lung site, the following sites demonstrated a decreased risk of death: lower limb/hip (HR = 0.54; 95% CI: 0.35-0.82, p = 0.004), pelvis (HR = 0.49; 95% CI: 0.28-0.84, p = 0.010), and the retroperitoneum/abdomen (HR = 0.54; 95% CI: 0.33-0.89, p = 0.015). Both adjuvant radiation (HR = 0.64; 95% CI: 0.48-0.85, p = 0.002) and neoadjuvant radiation (HR = 0.70; 95% CI: 0.49-1.00, p = 0.049) provided a survival benefit to patients. There was an increased risk of death for every 10-year increment in age (HR = 1.31; 95% CI: 1.12-1.45, p < 0.001). CONCLUSION: Statistically significant prognostic factors for PLS include primary anatomical site, age, Charlson-Deyo Comorbidity Index Scores and the use of neoadjuvant and adjuvant radiation.

Clonality of neutrophilia associated with plasma cell neoplasms: report of a SETBP1 mutation and analysis of a single institution series.

A rare but well-known association between plasma cell neoplasms and neutrophilia is known to exist. Whether the neutrophilia is secondary to the plasma cell neoplasm or this convergence represents two independent clonal disorders is unclear. We reviewed five consecutive cases from a single institution over a 3-year period, applying molecular, cytogenetic and cytokine-profiling approaches to determine whether neutrophilia associated with plasma cell neoplasms represents a reactive or clonal process. We report, for the first time, the occurrence of a SETBP1 mutation in two cases, as well as changes in G-CSF and IL-6 in SETBP1 wild type vs. mutated patients that are supportive of a hypothesis that neutrophilia associated with plasma cell neoplasms may sometimes be reactive and may sometimes represent a second clonal entity. Finally, using an ex vivo drug screening platform we report the potential efficacy of the multi-kinase inhibitor dasatinib in select patients.