Clinicopathological features, treatment and survival outcomes of synovial sarcoma

Siva Prasad Kuruva; Stalin Bala; Meher Lakshmi Konatam; Ashok Kumar Karnam; Lakshmi Srinivas Maddali; Sadashuivudu Gundeti

doi:10.4103/sajc.sajc_269_17

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ORIGINAL ARTICLE: Sarcomas

07 (

); 270-272

doi:

10.4103/sajc.sajc_269_17

Clinicopathological features, treatment and survival outcomes of synovial sarcoma

Siva Prasad Kuruva¹, Stalin Bala^1,, Meher Lakshmi Konatam¹, Ashok Kumar Karnam¹, Lakshmi Srinivas Maddali¹, Sadashuivudu Gundeti¹

1 Department of Medical Oncology, Nizam’s Institute of Medical Sciences, Hyderabad, Telangana, India

*Corresponding author: Dr. Stalin Bala. stalinchowdarybala@gmail.com

Published: 2020-12-21

MedIntel Services Pvt Ltd. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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Abstract

Introduction: Synovial sarcoma (SS) is a malignant mesenchymal tumor. It is most common among children and adults. The data on SS from India are scarce. In this study, we analyzed the clinicopathological treatment parameters and survival outcomes of SS patients. Materials and Methods: A total of 57 histologically proven SS diagnosed from 2010 to 2016 were retrospectively analyzed. Results: The median age was 23 years with a male-to-female ratio of 1.28:1. Localized disease was seen in 44 patients (77%) and 13 patients (23%) had metastasis. The primary sites of involvement such as lower limb, upper limb, thorax, and abdomen were seen in 60%, 28%, 7%, and 5% patients, respectively. Surgery was done in 39 patients and 18 patients had unresectable disease. Adjuvant chemotherapy with doxorubicin-based regimen was given in 30 patients and adjuvant radiotherapy in 21 patients. Palliative chemotherapy with anthracycline-based or gemcitabine-based regimen was used in 17 and 2 patients, respectively. The median event-free survival (EFS) was 30 months with 3 years and EFS rate was 36%; median progression-free survival (PFS) was 11.5 months and 1 year; and PFS rate was 38%. On univariate analysis, resection and performance status were significantly associated with survival. There is no impact of grade and size of the tumor on survival. In metastatic patients, the lung is the most common site. Conclusion: SS is the most common soft-tissue sarcoma among adults. Resectability and performance status were impacting the survival.

Keywords

Acknowledgment

Department of Pathology, Radiology and Surgical Oncology, Nizam's Institute of Medical Sciences, Hyderabad.

References

Fisher C, de Bruijn DR, Geurts van Kessel A. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Soft Tissue and Bone. Tumours of Uncertain Differentiation Synovial Sarcoma. Ch. 9. International Agency for Research on Cancer Press. 2006.
Ramaswamy A, Rekhi B, Bakhshi S, Hingmire S, Agarwal M. Indian data on bone and soft tissue sarcomas: A summary of published study results. South Asian J Cancer 2016;5:138-45.
Iqbal N, Shukla NK, Deo SV, Agarwala S, Sharma DN, Sharma MC, et al. Prognostic factors affecting survival in metastatic soft tissue sarcoma: An analysis of 110 patients. Clin Transl Oncol 2016;18:310-6.
Haldar M, Randall RL, Capecchi MR. Synovial sarcoma: From genetics to genetic-based animal modeling. Clin Orthop Relat Res 2008;466:2156-67.
Ladanyi M. Fusions of the SYT and SSX genes in synovial sarcoma. Oncogene 2001;20:5755-62.
Lagarde P, Przybyl J, Brulard C, Pérot G, Pierron G, Delattre O, et al. Chromosome instability accounts for reverse metastatic outcomes of pediatric and adult synovial sarcomas. J Clin Oncol 2013;31:608-15.
Sultan I, Rodriguez-Galindo C, Saab R, Yasir S, Casanova M, Ferrari A, et al. Comparing children and adults with synovial sarcoma in the surveillance, epidemiology, and end results program, 1983 to 2005: An analysis of 1268 patients. Cancer 2009;115:3537-47.
Ferrari A, De Salvo GL, Brennan B, van Noesel MM, De Paoli A, Casanova M, et al. Synovial sarcoma in children and adolescents: The European Pediatric Soft Tissue Sarcoma Study Group Prospective Trial (EpSSG NRSTS 2005). Ann Oncol 2015;26:567-72.
Ates O, Aksoy S, Yeter H, Sunar V, Kertmen N, Dizdar O, et al. Prognostic factors and treatment of patients with advanced synovial sarcoma: A single-center experience. Indian J Cancer 2017;54:321-5.
Pack GT, Ariel IM. Synovial sarcoma (malignant synovioma); a report of 60 cases. Surgery 1950;28:1047-84.
Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 1996;14:1679-89.
Lewis JJ, Antonescu CR, Leung DH, Blumberg D, Healey JH, Woodruff JM, et al. Synovial sarcoma: A multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity. J Clin Oncol 2000;18:2087-94.
Su L, Sampaio AV, Jones KB, Pacheco M, Goytain A, Lin S, et al. Deconstruction of the SS18-SSX fusion oncoprotein complex: Insights into disease etiology and therapeutics. Cancer Cell 2012;21:333-47.
Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg 1993;217:72-7.
Setsu N, Kohashi K, Fushimi F, Endo M, Yamamoto H, Takahashi Y, et al. Prognostic impact of the activation status of the Akt/mTOR pathway in synovial sarcoma. Cancer 2013;119:3504-13.

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[1] Fisher C, de Bruijn DR, Geurts van Kessel A. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Soft Tissue and Bone. Tumours of Uncertain Differentiation Synovial Sarcoma. Ch. 9. International Agency for Research on Cancer Press. 2006.

[2] Ramaswamy A, Rekhi B, Bakhshi S, Hingmire S, Agarwal M. Indian data on bone and soft tissue sarcomas: A summary of published study results. South Asian J Cancer 2016;5:138-45.

[3] Iqbal N, Shukla NK, Deo SV, Agarwala S, Sharma DN, Sharma MC, et al. Prognostic factors affecting survival in metastatic soft tissue sarcoma: An analysis of 110 patients. Clin Transl Oncol 2016;18:310-6.

[4] Haldar M, Randall RL, Capecchi MR. Synovial sarcoma: From genetics to genetic-based animal modeling. Clin Orthop Relat Res 2008;466:2156-67.

[5] Ladanyi M. Fusions of the SYT and SSX genes in synovial sarcoma. Oncogene 2001;20:5755-62.

[6] Lagarde P, Przybyl J, Brulard C, Pérot G, Pierron G, Delattre O, et al. Chromosome instability accounts for reverse metastatic outcomes of pediatric and adult synovial sarcomas. J Clin Oncol 2013;31:608-15.

[7] Sultan I, Rodriguez-Galindo C, Saab R, Yasir S, Casanova M, Ferrari A, et al. Comparing children and adults with synovial sarcoma in the surveillance, epidemiology, and end results program, 1983 to 2005: An analysis of 1268 patients. Cancer 2009;115:3537-47.

[8] Ferrari A, De Salvo GL, Brennan B, van Noesel MM, De Paoli A, Casanova M, et al. Synovial sarcoma in children and adolescents: The European Pediatric Soft Tissue Sarcoma Study Group Prospective Trial (EpSSG NRSTS 2005). Ann Oncol 2015;26:567-72.

[9] Ates O, Aksoy S, Yeter H, Sunar V, Kertmen N, Dizdar O, et al. Prognostic factors and treatment of patients with advanced synovial sarcoma: A single-center experience. Indian J Cancer 2017;54:321-5.

[10] Pack GT, Ariel IM. Synovial sarcoma (malignant synovioma); a report of 60 cases. Surgery 1950;28:1047-84.

[11] Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 1996;14:1679-89.

[12] Lewis JJ, Antonescu CR, Leung DH, Blumberg D, Healey JH, Woodruff JM, et al. Synovial sarcoma: A multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity. J Clin Oncol 2000;18:2087-94.

[13] Su L, Sampaio AV, Jones KB, Pacheco M, Goytain A, Lin S, et al. Deconstruction of the SS18-SSX fusion oncoprotein complex: Insights into disease etiology and therapeutics. Cancer Cell 2012;21:333-47.

[14] Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg 1993;217:72-7.

[15] Setsu N, Kohashi K, Fushimi F, Endo M, Yamamoto H, Takahashi Y, et al. Prognostic impact of the activation status of the Akt/mTOR pathway in synovial sarcoma. Cancer 2013;119:3504-13.

Clinicopathological features, treatment and survival outcomes of synovial sarcoma

Abstract

Abstract

Keywords

Acknowledgment

References

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