The value of multidisciplinary team (MDT) management in the diagnosis and treatment of primary intrathoracic synovial sarcomas: a single-center experience

Abstract

Background: Synovial sarcoma (SS) is a rare malignant soft tissue tumor. Primary intrathoracic SS is extremely rare, with limited diagnosis and treatment experiences. The aim of our study was to retrospectively study the clinicopathological characteristics, treatment and prognosis of primary intrathoracic SS and the impact of multidisciplinary team (MDT) management in diagnosis and treatment on patient prognosis. Methods: The clinical and pathological characteristics, treatment, survival and prognosis of patients with primary intrathoracic SS admitted to the National Cancer Center from January 1999 to December 2018, as well as MDT intervention during diagnosis and treatment, were retrospectively analyzed. Results: Thirteen patients were enrolled, including 7 (53.8%) males and 6 (46.3%) females, with primary intrathoracic SS in the lung (8/13, 61.5%), mediastinum (4/13, 30.8%) and pleura (1/13, 7.7%) as confirmed by morphological observation, immunohistochemical (IHC) staining and fluorescence in situ hybridization (FISH). Overall, 10/13 (76.9%) patients underwent surgery, and 6/10 (60.0%) received postoperative adjuvant therapy. Only 23.1% of patients received nonsurgical therapy. The MDT discussed and managed seven patients before and/or after surgery and one patient who did not undergo surgery. The estimated 3and 5-year overall survival (OS) rates were 50.0% and 30.0%, respectively. Patients who were managed by an MDT had a longer median OS time than those who were not (46.0 vs. 18.0 months). Age (P=0.018), tumor location (P=0.029), and Ki-67 (P=0.020) were found to be significantly related to OS.Conclusions: Monophasic morphology and fusion gene characteristics are the main features for the diagnosis of primary intrathoracic SS. MDT management can help obtain accurate diagnoses and provide reasonable therapeutic options.

Feasibility and long-term outcomes of surgery for primary thoracic synovial sarcoma

“…The importance of MDT management has been widely established, notably for rare malignancies [ 21 ]. Recently, He et al [ 11 ] published a study on the impact of MDT management for PTSS. Although MDT discussion was not independently associated with outcomes, the median OS of patients managed by an MDT was longer than that of patients who were not (46.0 vs 18.0 months).…”

“…Five-year overall survival (OS) of patients with SS arising from any site ranges from 52% to 66% [ 10 ]. However, location within the thorax is associated with a far poorer prognosis, with only 30% of patients being alive 5 years after surgery [ 3 , 11 ].…”

Anterior Endoscopy Combining with Modified Total En Block Spondylectomy for Synovial Sarcoma in Thoracic Paraspine Causing Neurological Deficits: Case Report and Literature Review

“… 10 The biphasic synovial sarcomas are only composed of epithelial or spindle, but the monophasic synovial sarcomas are characterized by epithelial cells mixed with spindle cells. 11 …”

Rare lung cancers—Primary pulmonary leiomyosarcoma: A case report

“…Interestingly, smoking seems to be less common in patients with PPS than in malignant epithelial lung tumours. In the published case series consistently less than 50% of the patients had a history of (heavy) smoking [1,2,6,9], which is clearly less than compared to historical data on lung tumours [10]. Concerning further risk factors, like for other sarcomas, cases of involved field sarcomas after prior radiotherapy are reported [1].…”

“…Interdisciplinary therapy decisions by a tumour board are highly recommended for all sarcomas and have been shown to improve outcomes of patients. For PPS the positive influence of the management by a multidisciplinary team was documented for primary intrathoracic synovial sarcoma [9]. The main pillar of therapy for PPS is without doubt surgery.…”