Tags

Type your tag names separated by a space and hit enter

Extraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions.
Front Oncol. 2020; 10:828.FO

Abstract

Background:

Extraskeletal myxoid chondrosarcoma (EMC) is a rare subtype of STS, which usually arises in extremities. It carries reciprocal translocations involving the NR4A3 gene. It displays an indolent behavior, but studies with long follow-up showed a high proportion of local and distant recurrences. For patients with progressing metastatic disease anthracycline-based chemotherapy is the standard front-line regimen, though has limited activity. There is some evidence on possible activity of antiangiogenetics.

Methods:

This is a retrospective study conducted at Istituto Oncologico Veneto and at Institut Gustave Roussy. All patients with a confirmed diagnosis of EMC from January 1980 to December 2018 were extracted from a prospectively maintained database.

Results:

59 patients were identified, 37 male (62.7%) and 22 female (37.3%) with a M/F ratio of 1.7/1. We performed molecular analysis in 23 cases, all carried a EWSR1-NR4A3. Out of 49 patients treated with curative intent, 28.6% developed local recurrence and 40.8% patients developed metastases. In patients who had been radically resected (R0) local recurrence occurred in 7.6% of cases and metastases occurred in 15.4% of cases; in patients treated with R1 surgery, rates of relapse were higher. Twenty patients received chemotherapy for metastatic disease; best response was partial response with clinical benefit in 50% of patients. Fourteen patients received a second line of chemotherapy, with 46.1% disease control rate. A drug holiday was proposed to 8 patients with a mean duration of 22.8 months. Median overall survival was 180 months for the study population and 76 months for metastatic patients. No significant prognostic role was found for all studied variables, yet a trend of better survival for complete surgery, location in extremities of primary tumor and solitary lung metastases was observed. Chemotherapy for metastatic disease was negatively associated with survival.

Conclusion:

In this large retrospective cohort of patients with ECM, location of primary tumor and solitary lung metastases seem to be associated with better survival. Chemotherapy did not impact survival in unselected patients. Further research is necessary in order to identify more active regimens and to provide clinical and molecular factors to select patients that could delay systemic treatment for metastatic disease.

Authors+Show Affiliations

Medical Oncology 1, Istituto Oncologico Veneto IRCCS, Padova, Italy.Medical Oncology, Insitut Gustave Roussy, Villejuif, Ile-de-France, France.Surgical Oncology, Istituto Oncologico Veneto IRCCS, Padova, Italy.Medical Oncology 1, Istituto Oncologico Veneto IRCCS, Padova, Italy.Medical Oncology 1, Istituto Oncologico Veneto IRCCS, Padova, Italy.Department of Pathology, University of Padua, Padova, Italy.Surgical Oncology, Istituto Oncologico Veneto IRCCS, Padova, Italy.Accreditation and Aknowledgment Unit, Istituto Oncologico Veneto IRCCS, Padova, Italy.Department of Pathology, University of Padua, Padova, Italy.Department of Biology and Medical Pathology, Institut Gustave Roussy, Villejuif, France.Department of Orthopedics and Orthopedic Oncology, University of Padua, Padova, Italy.Department of Pathology, University of Padua, Padova, Italy.Surgical Oncology, Istituto Oncologico Veneto IRCCS, Padova, Italy. Department of Surgery, Oncology and Gastroenterology (DISCOG), University of Padova, Padova, Italy.Medical Oncology 1, Istituto Oncologico Veneto IRCCS, Padova, Italy.Medical Oncology 1, Istituto Oncologico Veneto IRCCS, Padova, Italy.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32612944

Citation

Chiusole, Benedetta, et al. "Extraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions." Frontiers in Oncology, vol. 10, 2020, p. 828.
Chiusole B, Le Cesne A, Rastrelli M, et al. Extraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions. Front Oncol. 2020;10:828.
Chiusole, B., Le Cesne, A., Rastrelli, M., Maruzzo, M., Lorenzi, M., Cappellesso, R., Del Fiore, P., Imbevaro, S., Sbaraglia, M., Terrier, P., Ruggieri, P., Dei Tos, A. P., Rossi, C. R., Zagonel, V., & Brunello, A. (2020). Extraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions. Frontiers in Oncology, 10, 828. https://doi.org/10.3389/fonc.2020.00828
Chiusole B, et al. Extraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions. Front Oncol. 2020;10:828. PubMed PMID: 32612944.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Extraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions. AU - Chiusole,Benedetta, AU - Le Cesne,Axel, AU - Rastrelli,Marco, AU - Maruzzo,Marco, AU - Lorenzi,Martina, AU - Cappellesso,Rocco, AU - Del Fiore,Paolo, AU - Imbevaro,Silvia, AU - Sbaraglia,Marta, AU - Terrier,Philippe, AU - Ruggieri,Pietro, AU - Dei Tos,Angelo Paolo, AU - Rossi,Carlo Riccardo, AU - Zagonel,Vittorina, AU - Brunello,Antonella, Y1 - 2020/06/16/ PY - 2020/01/30/received PY - 2020/04/28/accepted PY - 2020/7/3/entrez PY - 2020/7/3/pubmed PY - 2020/7/3/medline KW - NR4A3 KW - anthracycline KW - chemotherapy (CHT) KW - drug holiday KW - extrascheletal myxoid chondrosarcoma KW - surgery KW - trabectedin SP - 828 EP - 828 JF - Frontiers in oncology JO - Front Oncol VL - 10 N2 - Background: Extraskeletal myxoid chondrosarcoma (EMC) is a rare subtype of STS, which usually arises in extremities. It carries reciprocal translocations involving the NR4A3 gene. It displays an indolent behavior, but studies with long follow-up showed a high proportion of local and distant recurrences. For patients with progressing metastatic disease anthracycline-based chemotherapy is the standard front-line regimen, though has limited activity. There is some evidence on possible activity of antiangiogenetics. Methods: This is a retrospective study conducted at Istituto Oncologico Veneto and at Institut Gustave Roussy. All patients with a confirmed diagnosis of EMC from January 1980 to December 2018 were extracted from a prospectively maintained database. Results: 59 patients were identified, 37 male (62.7%) and 22 female (37.3%) with a M/F ratio of 1.7/1. We performed molecular analysis in 23 cases, all carried a EWSR1-NR4A3. Out of 49 patients treated with curative intent, 28.6% developed local recurrence and 40.8% patients developed metastases. In patients who had been radically resected (R0) local recurrence occurred in 7.6% of cases and metastases occurred in 15.4% of cases; in patients treated with R1 surgery, rates of relapse were higher. Twenty patients received chemotherapy for metastatic disease; best response was partial response with clinical benefit in 50% of patients. Fourteen patients received a second line of chemotherapy, with 46.1% disease control rate. A drug holiday was proposed to 8 patients with a mean duration of 22.8 months. Median overall survival was 180 months for the study population and 76 months for metastatic patients. No significant prognostic role was found for all studied variables, yet a trend of better survival for complete surgery, location in extremities of primary tumor and solitary lung metastases was observed. Chemotherapy for metastatic disease was negatively associated with survival. Conclusion: In this large retrospective cohort of patients with ECM, location of primary tumor and solitary lung metastases seem to be associated with better survival. Chemotherapy did not impact survival in unselected patients. Further research is necessary in order to identify more active regimens and to provide clinical and molecular factors to select patients that could delay systemic treatment for metastatic disease. SN - 2234-943X UR - https://www.unboundmedicine.com/medline/citation/32612944/Extraskeletal_Myxoid_Chondrosarcoma:_Clinical_and_Molecular_Characteristics_and_Outcomes_of_Patients_Treated_at_Two_Institutions L2 - https://doi.org/10.3389/fonc.2020.00828 DB - PRIME DP - Unbound Medicine ER -
Try the Free App:
Prime PubMed app for iOS iPhone iPad
Prime PubMed app for Android
Prime PubMed is provided
free to individuals by:
Unbound Medicine.