Primary pulmonary synovial sarcoma: A clinicopathological study of 22 cases.

INTRODUCTION: Primary pulmonary synovial sarcoma (PPSS) is a rare mesenchymal tumour with characteristic translocation SS18-SSX1/2/rarely 4 fusion transcripts, and presents most often in adolescents and young adults. According to the World Health Organization (WHO) classification, synovial sarcoma is a malignant tumour of uncertain differentiation. AIMS: To present a case series of PPSS with clinical, pathological and molecular analysis at a rare primary site. SETTING AND DESIGN: Retrospective study conducted in a tertiary care hospital. MATERIALS AND METHODS: Twenty-two cases of PPSSs were retrieved from electronic database between January 2009 to December 2018. Metastatic tumours from soft tissue primaries were excluded. Immunohistochemistry (IHC) and reverse transcription polymerase chain reaction (RT-PCR) were performed. Statistical analysis was performed using Mann-Whitney non-parametric test. RESULTS: Among 22 patients, the male-female ratio was 3.4:1 and the median age was 31.5 years. The tumours were classified as monophasic (90.9%) and biphasic (9.1%) subtypes and graded as grade 2(77.3%) and grade 3(22.7%). IHC demonstrated expression of TLE1 (17/17 cases), Bcl-2 (7/8 cases), focal EMA (16/17 cases), CD99 (10/11 cases), focal pancytokeratin (8/12 cases) and CD56 (14/14 cases). The fusion transcripts included SYT-SSX1(4/11, 36.4%) and SYT-SSX2 (2/11, 18.2%). The remaining five cases were negative for SS18 rearrangement by RT-PCR. Only 8 patients had localised tumour. Surgical excision was performed in 5 patients. The median follow-up period was 6 months and 21 days. CONCLUSIONS: Monophasic SS was the most common subtype. Small core biopsies pose a diagnostic challenge, in such a scenario, a combination of clinical, histomorphological, immunomarkers and genetic studies help confirm the diagnosis of PPSS.

Pathological response and clinical outcomes in operable triple-negative breast cancer with cisplatin added to standard neoadjuvant chemotherapy.

BACKGROUND: Response to neoadjuvant chemotherapy is associated with improved outcomes for patients with triple negative breast cancer (TNBC). Patients with residual disease are at increased risk of relapse and death from breast cancer. In this retrospective study, we aimed to evaluate the efficacy and safety of cisplatin added to standard neoadjuvant chemotherapy for locally advanced TNBC. MATERIALS AND METHODS: All TNBC treated with neoadjuvant cisplatin 60mg/m2 once in 3 weeks with weekly paclitaxel for 12 weeks, following 8 weeks of dose-dense epirubicin 90mg/m2 or doxorubicin 60mg/m2 with cyclophosphamide 600mg/m2 were analyzed retrospectively. The data related to pathological complete response, adherence to planned therapy, disease-free survival and overall survival were collected. RESULTS: Eighty-three patients were included, of whom 80% had stage III disease. Pathological complete response in both breast (T0/Tis) and axilla (N0) was observed in 48.1% of patients. Miller Payne grade 5 pathological response in the breast was seen in 61% of patients. Good partial responses (Miller Payne grades 3,4) were observed in 32.5% of patients. The remaining 6.5% were poor responders. Seventy-seven patients underwent surgery. The disease-free survival at 1 and 3 years for those who had a pathological complete response was 96.7% and 77.6%, respectively, and 92.3% and 62.7% for those who did not, respectively. The predominant adverse events were hematological, with anemia being the most common one. CONCLUSION: The addition of cisplatin to neoadjuvant chemotherapy with anthracycline and taxane in TNBC was tolerable and produced a high rate of pathological complete response. Cisplatin added to standard chemotherapy in patients with locally advanced TNBC could improve clinical outcomes.