ABSTRACT
BACKGROUND: A randomised phase II trial demonstrated that the addition of olaratumab to doxorubicin significantly increased overall survival (OS) in patients with advanced soft tissue sarcomas (STS) compared to doxorubicin alone. The recently presented phase III study of doxorubicin and olaratumab in advanced soft tissue sarcoma was discordant with this finding. METHODS: We performed a retrospective analysis of adult patients with advanced-/metastatic STS treated with at least two cycles of doxorubicin and olaratumab at eight sarcoma units across England and Northern Ireland between May 2017 and March 2019. RESULTS: 172 patients were evaluable and 40 patients (23.3%) had died at the time of analysis. Median ECOG performance status (PS) was 1. Median progression free survival (PFS) was 6.8 months (95% CI 5.9-7.7 months). Leiomyosarcoma was the most common histological subtype (75 patients, 43.6%), followed by liposarcomas (19, 11.0%). The mean number of cycles was 5 (doxorubicin range 2-6; olaratumab range 2-23). Two patients (1.2%) had a complete response and 34 (19.8%) had a partial response. 79 (45.9%) had stable and 58 (33.7%) progressive disease. 57 patients (33.1%) experienced grade ≥ 3 neutropenia and 7 patients (4.1%) grade ≥ 3 febrile neutropenia. Grade ≥ 3 anaemia was seen in 21 patients (12.2%). Grade ≥ 3 non-haematological toxicities were seen in 35 patients (20.3%). A clinically significant drop in left ventricular ejection fraction was seen in 6 patients (3.5%). 48 patients (27.9%) required a dose reduction. Overall survival (OS) is pending. CONCLUSIONS: Our results are in keeping with the phase III study findings: response rate, PFS and OS were similar to those reported in the phase III ANNOUNCE trial.
ABSTRACT
The occurrence of multiple primary sarcomas in one individual is very uncommon and the development of osteosarcoma as a second tumor following a soft tissue sarcoma is extremely rare. We report a case of a 62-year-old man who developed 2 histologically distinct sarcomas: a soft tissue myxofibrosarcoma and vertebral osteosarcoma. This unusual case highlights the critical role of careful histopathological evaluation in distinguishing synchronous or metachronous neoplasia from metastatic tumor. To the best of the authors' knowledge, this is the first reported case of metachronous soft tissue myxofibrosarcoma and osteosarcoma.
Subject(s)
Fibrosarcoma/pathology , Neoplasms, Second Primary/pathology , Osteosarcoma/pathology , Soft Tissue Neoplasms/pathology , Spinal Neoplasms/pathology , Forearm/pathology , Humans , Male , Middle Aged , Thoracic Vertebrae/pathologyABSTRACT
BACKGROUND: Desmoid tumours/aggressive fibromatosis (DT/AF) are infrequent soft-tissue neoplasms. They usually behave as indolent diseases. However, they may grow locally infiltrating or compressing adjacent structures. The role of local treatment is limited and only a few drugs have shown activity. CASES PRESENTATION: We report the outcome of two patients affected by progressive DT/AF treated with the angiogenesis inhibitor pazopanib in two different institutions. Both patients achieved dramatic improvement in their symptoms and radiological signs of response. The clinical benefit lasted for more than 1 year and it is still ongoing. CONCLUSIONS: Pazopanib is an active treatment in DT/AF. It is the first time this has been reported.
