Key result
Patients with oral-maxillary undifferentiated pleomorphic sarcoma who experienced partial response or stable disease with neoadjuvant chemotherapy (anlotinib) had a significantly improved median overall survival of 17 months compared to 6.25 months in other cases (p=0.0101).
Why the study?
Undifferentiated pleomorphic sarcoma in the oral-maxillary area is rarely reported, leaving its clinical characteristics, treatment strategies, prognosis, and molecular features poorly understood.
Observational (n=10)
No
Absolute Event Rate: 17% vs 6.25%
p-value: p=0.0101
Primary oral-maxillary undifferentiated pleomorphic sarcoma is a highly aggressive malignancy with a poor median overall survival of 7.75 months, though neoadjuvant chemotherapy may offer survival benefits and molecular profiling identified GBP4 and PIK3CA as potential driver mutations.
Characterizes rare oral-maxillary UPS; leaves open optimal treatment and molecular targets pending larger prospective studies.
BackgroundUndifferentiated pleomorphic sarcoma (UPS) in oral-maxillary area is rarely reported. Herein, we aimed to investigate the clinical characteristics, treatment strategies, prognosis, and molecular features of the oral-maxillary UPS. MethodsRetrospectively, we reviewed the UPS patients who were diagnosed and treated in our department. The medical histories, imaging features, histopathological characteristics, treatment strategies, clinical outcomes were summarized and analyzed. Besides, the molecular features were demonstrated by whole exonic sequencing. ResultsTotally, 10 cases with primary oral-maxillary UPS were included. The rapidly progressive UPS can easily develop at advanced and life-threatening stage, especially concerning the complex anatomical structures and spaces in the oral-maxillary area. The finial diagnosis for UPS greatly depends on histological findings and immunohistochemistry under the exclusion of all the possible differential diagnosis. Retrospectively, the treatment strategies for the involved cases still referred to those of oral squamous cell carcinoma. Statistically, the median overall survival (OS) for all the included cases was 7.75 months (range: 5-17 months). Comparatively, 3 cases had improved OS (median survival: 17 months, range: 17-18 months) experienced PR/SD with neoadjuvant chemotherapy (anlotinib). Cancer driver genes detection revealed GBP4 as a candidate driver gene for the oral-maxillary UPS. Additionally, a missense mutation in gene PIK3CA (p.E545K) was also identified. ConclusionOur findings can greatly expand the knowledge about the oral-maxillary UPS, and provide molecular evidences to improve the therapeutic options for the oral-maxillary UPS. Further studies are warranted to validate our discoveries on the oral-maxillary UPS.
No takes yet. Share an insight, caveat, or question.
Xiao et al. (2020) conducted an observational in Primary undifferentiated pleomorphic sarcoma (UPS) in oral-maxillary area (n=10). Neoadjuvant chemotherapy (anlotinib) vs. Other treatments (no neoadjuvant anlotinib with partial response/stable disease) was evaluated on Median overall survival (p=0.0101). Patients with oral-maxillary undifferentiated pleomorphic sarcoma who experienced partial response or stable disease with neoadjuvant chemotherapy (anlotinib) had a significantly improved median overall survival of 17 months compared to 6.25 months in other cases (p=0.0101).
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: