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N. Girard



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    MO03 - Thymic Malignancies (ID 123)

    • Event: WCLC 2013
    • Type: Mini Oral Abstract Session
    • Track: Medical Oncology
    • Presentations: 1
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      MO03.03 - RYTHMIC: a nationwide network for thymic malignancies in France (ID 2631)

      10:30 - 12:00  |  Author(s): N. Girard

      • Abstract
      • Presentation
      • Slides

      Background
      RYTHMIC (Réseau tumeurs THYMiques et Cancer) is a nationwide network for thymic malignancies, which was appointed in 2012 by the French National Cancer Institute, as part of its rare cancer program. The objectives of the network include a territorial coverage by regional expert centers, the dissemination of highest standards for the diagnostic and therapeutic management of patients, and the promotion of collaborative research. Registration in RYTHMIC of all patients diagnosed with thymic malignancy is recommended as part of good clinical practice for oncologists.

      Methods
      Starting January 2012, the management of all patients diagnosed with thymic malignancy in France has been discussed on a real-time basis at a reference national multidisciplinary tumor board (MTB), which is organized twice a month using a web-based conferencing system. Decision-making is based on consensual recommendations, that were originally established using available evidence, and are updated and approved each year by all members of the network. A prospective database of all patients is hosted by the French Thoracic Cancer Intergroup. We report the characteristics and treatment modalities of patients included during the first year.

      Results
      From January to December 2012, 257 patients were enrolled in RYTHMIC. There were 126 (49%) men and 131 (51%) women; mean age at diagnosis was 54.5 years. Among 214 cases, histology was thymoma for 146 (56%) patients (11 (5%) type A, 28 (13%) type AB, 22 (10%) type B1, 35 (16%) type B2, 24 (11%) type B3, 26 (12%) mixed type), and thymic carcinoma for 33 (15%) patients, 8 of which were neuroendocrine carcinomas; other histologies were diagnosed for 35 (16%) patients. Among 144 cases, Masaoka-Koga stage was I, IIA, IIB, III, IVA, and IVB in 34 (24%), 19 (13%), 20 (14%), 22 (15%), 35 (24%), and 14 (10%) patients, respectively. 44 (17%) patients presented with autoimmune disorder, consisting of myasthenia gravis in 28 cases. Surgery was performed for 166 patients, mostly using a median sternotomy approach (52% of cases). Postoperative radiotherapy was delivered to 42 patients; 71 patients received perioperative chemotherapy. Exclusive chemotherapy/radiotherapy was administered to 20 and 4 patients, respectively. Mature data will be presented at the meeting.

      Conclusion
      This first analysis of the RYTHMIC prospective cohort demonstrates the feasibility of a national MTB for thymic malignancies, that, besides ensuring all patients an equal access to highly specialized treatment, provides with a comprehensive tool to monitor dedicated actions to improve the management of patients in the future, increase the quality-of-care, and screen patients for future translational research and clinical trials. Supported by Institut National du Cancer

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    MS16 - ESTS/IASLC Thymic Session (ID 33)

    • Event: WCLC 2013
    • Type: Mini Symposia
    • Track: Thymoma & Other Thoracic Malignancies
    • Presentations: 1
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      MS16.4 - Chemotherapy for Thymic Tumours (ID 533)

      10:30 - 12:00  |  Author(s): N. Girard

      • Abstract
      • Presentation
      • Slides

      Abstract
      Thymic epithelial tumors represent a wide range of anatomical, clinical, histological, and molecular malignant entities, which may be aggressive and difficult to treat. The histopathological classification distinguishes thymomas from thymic carcinomas. Chemotherapy may be used in two clinical scenarios in thymic epithelial tumors: 1) chemotherapy may constitute primary part of the multimodal curative-intent treatment of locally-advanced tumors, and is subsequently combined with surgery or radiotherapy; the main objective is to achieve long-term survival with no evidence of tumor recurrence; 2) chemotherapy may be delivered as the sole treatment modality in unresectable, advanced, metastatic, or recurrent tumors; then a palliative-intent treatment, the aim is to improve tumor-related symptoms through achievement of tumor response, while no prolonged survival is expected. Several chemotherapy regimens have been used in the curative-intent setting, mostly consisting of adriamycin- and/or platin-based multi-agent combinations. Usually 2-4 cycles of chemotherapy are administered before imaging reassessment. Aiming at increasing the response rate to primary treatment, and thus complete resection rate, chemotherapy may be combined with radiotherapy; however, retrospective data available do not provide with interpretable figures to compare chemotherapy to chemo-radiotherapy in the pre-operative setting. Response rates to curative-intent chemotherapy ranged from 70% to 80% in the largest studies. Patients for whom R0 resection was thought to be feasible undergo surgery, and complete resection is achieved in about 50% of cases. Postoperative radiotherapy is then frequently delivered. When the patient is not deemed to be a surgical candidate - either because R0 resection is not thought to be achievable or because of poor performance status or co-existent medical condition, definite radiotherapy, as reported above, is delivered. If radiotherapy is not feasible, either because of a large tumor burden that precludes safe delivery of appropriate doses or because of co-morbidities increasing the risks of radiation-induced toxicity, treatment is chemotherapy alone, in a strategy that may ultimately be considered palliative. In the reported literature, 10-21% of patients with locally-advanced thymic tumors receiving upfront chemotherapy did not receive either surgery or radiation therapy or other local treatment. Survival of these patients is frequently limited. Overall, the major challenge in interpreting data about pre-operative chemotherapy in thymic malignancies is the wide variation in the number of patients subsequently treated with surgery, radiotherapy, or chemotherapy alone, which suggests significant heterogeneity in the inclusion criteria among series. Response has been evaluated based on elusive criteria in some studies published before CT scan was largely available. In most studies, thymomas and thymic carcinomas, as well as newly diagnosed and recurrent tumors, were not analyzed separately. Ultimately, the majority of studies are retrospective, with uncontrolled design. Finally, one should consider the potential effect of corticosteroids, that have been known for a long time to have a “lympholytic” effect. Palliative chemotherapy is given as the sole treatment modality for thymic tumors, usually in the setting of stage IV, unresectable, recurrent disease. Prolonged disease control is possible, but tumor eradication is not expected. Several studies - both prospective and retrospective - described several regimens for definite chemotherapy, but because there are no randomized studies, it is unclear which are best; multi-agent combination regimens and anthracycline-based regimens appear to have improved response rates compared to others, especially the etoposide, ifosfamide and cisplatin combination. In general, combination regimen is recommended, for at least 3 and no more than 6 cycles. Overall, response rates are 20-40%, lower than that observed in the preoperative setting. Progression-free and overall survival of patients ranges from 12 to 66 months, and 37 to 72 months, respectively; such variability may be related to the various settings in which chemotherapy was delivered in those studies. In the palliative-intent setting, several consecutive lines of chemotherapy may be administered when the patient presents with tumor progression. It is estimated that 50-70% of patients with thymoma recurrence are eligible to chemotherapy. Strategy may consist of the re-administration of a previously effective regimen, especially in case of previous response, late occurring recurrence, and for anthracyclins, a patient in a good medical condition and not having received cumulative doses precluding the safe delivery of at least 3 additional cycles. In case of recurrence, the strategy may actually primarily consist of a similar multimodal management to that conducted at time of first diagnosis, with surgery and radiotherapy in eligible cases. Complete re-resection remains a major prognostic factor in this setting. In patients not eligible to receive additional chemotherapy, octreotide may represent a valuable option; as a single agent, octreotide produced objective tumor responses rates, and of more relevance in this setting, disease control rates. Novel treatment strategies are needed, especially for refractory, recurrent tumors, and thymic carcinomas, which carry a poor prognosis despite multimodal treatment. Potentially druggable targets are emerging, laying the foundations to implement personalized medicine for patients. Given the currently available targeted agents outside of a clinical trial, the signaling pathways that are relevant in the clinical care of patients, are the KIT and the Vascular Endothelial Growth Factor (VEGF)-R (Receptor) pathways. Promising new targets in thymoma and thymic carcinoma include IGF-1R and histone-deacetylase. Cixutumumab, an IGF1-R directed monoclonal antibody was recently reported to produce a promising 90%-disease control rate in refractory thymomas. Belinostat, a histone deacetylase inhibitor was evaluated in thymic malignancies in a recently completed phase II trial enrolling 41 patients (25 thymomas and 16 thymic carcinomas). Response and 2-year survival rates were 8% and 77% in thymomas. mTOR inhibitors, in the setting of phase I trials, have been reported to produce significant control rates in thymoma and thymic carcinoma. Along with the large variety of questions relative to the treatment strategy, thymic epithelial tumors represent a model of therapeutic implementation and achievement in orphan thoracic oncology, showing how the advent of new results induces new questions, as well as diversifies further clinical research directions; in this setting, regional and international collaborative initiatives are mandatory to progress both in the understanding of the biological mechanisms underlying the development of thymic malignancies, and in the identification and validation of new targets with prognostic and predictive value.

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    MTE19 - Biology and Treatment of Thymoma / Thymic Carcinoma (ID 63)

    • Event: WCLC 2013
    • Type: Meet the Expert (ticketed session)
    • Track: Medical Oncology
    • Presentations: 1
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      MTE19.1 - Biology and Treatment of Thymoma / Thymic Carcinoma (ID 616)

      07:00 - 08:00  |  Author(s): N. Girard

      • Abstract
      • Presentation
      • Slides

      Abstract
      Thymic epithelial tumors represent a wide range of anatomical, clinical, histological, and molecular malignant entities, which may be aggressive and difficult to treat. The histopathological classification distinguishes thymomas from thymic carcinomas. Our current understanding of the carcinogenesis of these tumors remains limited; only few well-characterized preclinical models - mostly consisting of thymic carcinoma cell lines -, have been established. Several biomarkers studies have been reported; while tumor stage, completion of surgical resection, and - to a lesser extent – histology have been shown to significantly predict the outcome of patients, the expression of EGFR, KIT, or IGF1-R by immunohistochemistry failed to consistently demonstrate a prognostic value on the survival of patients. More recently, expression signatures have been reported, both for thymomas and thymic carcinomas, to correlate with metastasis-free survival; these results remain to be validated in separate cohorts, while prospective study to understand the clinical significance of these results will be required. The management of thymic epithelial tumors is a paradigm of cooperation between clinicians, surgeons, and pathologists from establishing the diagnosis to organizing the multimodal therapeutic strategy. Chemotherapy may be used in two clinical scenarios in thymic epithelial tumors: 1) chemotherapy may constitute primary part of the multimodal curative-intent treatment of locally-advanced tumors, and is subsequently combined with surgery or radiotherapy; the main objective is to achieve long-term survival with no evidence of tumor recurrence; 2) chemotherapy may be delivered as the sole treatment modality in unresectable, advanced, metastatic, or recurrent tumors; then a palliative-intent treatment, the aim is to improve tumor-related symptoms through achievement of tumor response, while no prolonged survival is expected. Novel treatment strategies are needed, especially for refractory, recurrent tumors, and thymic carcinomas, which carry a poor prognosis despite multimodal treatment. Potentially druggable targets are emerging, laying the foundations to implement personalized medicine for patients. Given the currently available targeted agents outside of a clinical trial, the signaling pathways that are relevant in the clinical care of patients are the KIT and the Vascular Endothelial Growth Factor (VEGF)-R (Receptor) pathways. Several tyrosine kinase inhibitors targeting KIT have been developed - including imatinib (Novartis, Basel, Switzerland), sunitinib (Pfizer, New-York, NY), and sorafenib (Bayer, West Haven, CT) - , most of which also potently inhibiting other kinases, including VEGFRs and Platelet-Derived Growth Factor Receptors. Collectively, KIT is overexpressed in 80% of thymic carcinomas, while KIT gene mutations are found only in 9% of cases. Clinically, KIT-mutant thymic carcinomas then represent a small molecular subset of thymic tumors. The clinical relevance of KIT mutations is more limited in thymic carcinoma than in other cancers like gastro-intestinal stromal tumor (GIST), as 1) KIT mutations are far less frequent; 2) KIT expression does not correlate with the presence of KIT mutation; and 3) non-pretreated KIT mutants are not uniformly sensitive to imatinib, based on the clinical and/or the preclinical evidence in thymic carcinoma and/or other KIT-mutant tumors. These findings may explain why the 2 reported phase II trials with imatinib, where patients were not selected, or selected based upon histologic type (B3 thymomas and thymic carcinomas) or KIT staining by immunohistochemistry, and not upon KIT genotyping, were negative. Multi-kinase inhibitors may also be of interest to target neoangiogenesis. The most potent pro-angiogenic molecules are those of the VEGF/VEGFR signaling pathway. VEGF-A and VEGFR-1 and -2 are overexpressed in thymomas and thymic carcinomas. Micro-vessel density and VEGF expression levels have been shown to correlate with tumor invasion, aggressive histology and clinical stage. In a phase II trial, bevacizumab was tested in combination with erlotinib in thymomas and thymic carcinomas. No tumor response was observed. Interestingly, despite the large tumor burden of thymic tumors and the frequent abutment to mediastinal vascular structures, no hemorrhagic side effect has been reported with the use of these drugs in these studies. Beyond the inhibition of KIT, sunitinib and sorafenib also inhibit VEGFR-1, VEGFR-2, VEGFR-3 at the nanomolar range. The effect of these drugs, especially in KIT-wild-type thymic carcinoma tumors may then be partially related to an anti-angiogenic effect. Promising new targets in thymoma and thymic carcinoma include IGF-1R and histone-deacetylase. Cixutumumab, an IGF1-R directed monoclonal antibody was recently reported to produce a promising 90%-disease control rate in refractory thymomas. Belinostat, a histone deacetylase inhibitor was evaluated in thymic malignancies in a recently completed phase II trial enrolling 41 patients (25 thymomas and 16 thymic carcinomas). Response and 2-year survival rates were 8% and 77% in thymomas. Given the rarity of the tumor, translation of pre-clinical findings to the clinic may be quick; several strategies have been implemented. A pragmatic approach is the recommendation for KIT genotyping in clinical practice, what represents a model of n-of-one trial approach in the field. Another approach to validate the concept of personalized medicine in thymic malignancies includes the development of open-label multicentric phase II trials, using high throughput genome analysis (CGH array, next generation sequencing) as a therapeutic decision tool, to compare a medical treatment administered according to the identified molecular anomaly of the tumor with a medical treatment administered without considering the genome analysis; thymic tumors have been integrated in some ongoing trials using such methodology. A third approach is to promote the enrollment of patients with refractory thymic tumor in phase I trials, what may lead to identify new molecular pathways of therapeutic interest; mTOR is emerging as a potential target, following tumor responses observed in phase I trials, with recent data from several groups. Along with the large variety of questions relative to the treatment strategy, thymic epithelial tumors represent a model of therapeutic implementation and achievement; in this setting, regional and international collaborative initiatives are mandatory to progress both in the understanding of the biological mechanisms underlying the development of thymic malignancies, and in the identification and validation of new targets with prognostic and predictive value.

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    P2.06 - Poster Session 2 - Prognostic and Predictive Biomarkers (ID 165)

    • Event: WCLC 2013
    • Type: Poster Session
    • Track: Biology
    • Presentations: 1
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      P2.06-031 - Mutational profiling of synchronous bone metastases from lung adenocarcinoma: feasibility and results in a prospective cohort of 46 patients (POUMOS study) (ID 2471)

      09:30 - 16:30  |  Author(s): N. Girard

      • Abstract

      Background
      Mutational profiling for targetable oncogenic drivers is systematically recommended in the pretherapeutic workup of metastatic lung adenocarcinoma. Pathological and molecular diagnoses may be performed on specimens from metastatic lesions, when the primary pulmonary tumor is not accessible, either because of proximal location increasing the risk of transthoracic procedures, or when the material collected is insufficient in size. The feasibility of performing molecular diagnoses on small specimens from bone metastases has been questioned over time. To prospectively study patients with bone metastases from lung cancer, we set up a multidisciplinary group at the Hospices Civils de Lyon, including rheumatologists, pulmonologists, oncologists, interventional radiologists, pathologists and molecular biologists.

      Methods
      POUMOS was a prospective observational study aiming at evaluating the feasibility of routine percutaneous biopsy of synchronous bone metastases from lung adenocarcinoma, to perform pathological diagnosis and mutational profiling on the bone lesion. Results were correlated with that obtained on specimens from the primary tumor, if available. Technically, bone metastasis specimens, usually multiple, were sent fresh for immediate formalin-fixation, and, if possible, snap-freezing. Decalcification of bone was performed using EDTA for a better preservation of cell morphology and DNA. Mutational profiling of EGFR, KRAS, HER2, BRAF, and PIK3CA, as well as testing for ALK rearrangements, was conducted as recommended by the French National Cancer Institute (INCa) for all adenocarcinoma cases. DNA extraction was performed after laser microdissection of cancer cells; genotyping was based on direct sequencing and/or SNaPshot. Complete description of the process will be presented at the meeting.

      Results
      Starting April 2011, 46 patients with lung adenocarcinoma and synchronous bone metastasis were enrolled. No grade 3-4 adverse effects were reported after the bone biopsy. Mutational profiling was obtained in 45 (98%) cases; one specimen did not provide with sufficiently good quality DNA for the analysis. EGFR mutation was observed in 6 (13%) patients, KRAS mutation in 14 (30%) patients, HER2, BRAF and PIK3CA mutations in 1 (2%) patient each. Updated results, especially correlations between the mutational profiles of primary lung tumors and bone metastases, will be reported at the meeting.

      Conclusion
      Our data demonstrate the feasibility of percutaneous biopsy of synchronous bone metastasis from lung adenocarcinoma to conduct mutational profiling for common oncogenic alterations. The establishment of multidisciplinary teams to ensure the coordination between clinicians, radiologists and pathologists, makes routine pathological and molecular diagnosis on bone metastasis specimens a fast, reliable and safe procedure.

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    P2.24 - Poster Session 2 - Supportive Care (ID 157)

    • Event: WCLC 2013
    • Type: Poster Session
    • Track: Supportive Care
    • Presentations: 2
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      P2.24-037 - Feasibility of creative art therapy as a supportive care modality for patients with metastatic lung cancer treated with chemotherapy (ID 2465)

      09:30 - 16:30  |  Author(s): N. Girard

      • Abstract

      Background
      Creative art therapy (CAT) consists in the use of artistic activities to help patients manage physical and emotional problems in a therapeutic setting. In oncology, CAT has mostly been practiced in palliative-care units. CAT has not been used in lung cancer patients so far, especially when chemotherapy treatment is still delivered. Our objectives were to prospectively assess the feasibility of integrating CAT in the management of patients with metastatic lung cancer treated with chemotherapy.

      Methods
      From 2011/11 to 2013/05, CAT was offered to in- and out-patients who received chemotherapy for metastatic lung cancer in our department. Creative activities included the production of paintings, drawings, and/or sculptures. Patients were assessed by a trained art therapist for anxiety levels, self-awareness and satisfaction, before and after each art session.

      Results
      84 patients were included in the study, among whom 41 (55%) accepted CAT and received a mean number of 3 sessions. In this cohort of 13 men and 28 women, 31 (75%) and 17 (41%) patients reported improvements in anxiety levels and cancer-related symptoms after the art session, respectively. CAT gave satisfaction to 37 (90%) patients. These benefits were transient in all cases. Main reasons for refusing CAT in the remaining 33 patients included fatigue and lack of interest for arts. Painting and drawings from lung cancer patients along the disease management will be presented at the meeting.

      Conclusion
      Our study reports on the feasibility of CAT in lung cancer patients receiving chemotherapy. CAT may be considered part of the multimodal supportive care management of lung cancer patients. This study was supported by an unrestricted grant from Hoffmann-La Roche.

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      P2.24-038 - Lung cancer and combined pulmonary fibrosis and emphysema syndrome in Western patients: a series of 47 patients (ID 2844)

      09:30 - 16:30  |  Author(s): N. Girard

      • Abstract

      Background
      The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterized by imaging features consisting of the association of centrilobular and/or paraseptal emphysema and pulmonary fibrosis, associated with subnormal spirometry, contrasting with severe impairment of gas exchange with strong decrease in carbon monoxide diffusing capacity, and hypoxaemia at exercise. Virtually all patients presenting with CPFE are smokers and may be at high-risk to develop lung cancer; limited data have been made available on such association, mostly from Japanese cohorts.

      Methods
      This retrospective multicentre study was conducted by the Groupe d’Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM”O”P), a collaborative group of about 200 French physicians dedicated to the study of rare pulmonary diseases. Patients presenting with CPFE syndrome and lung cancer at the referring centers from 2003 to 2012, were included. The clinical, pathological, and therapeutic features, as well as the outcome of patients, were collected and analyzed.

      Results
      A total of 47 patients presenting with lung cancer and CPFE syndrome were identified. All patients but one were men, with a mean age of 68 years. All patients were smokers, with a mean of 47 pack-years. The CPFE syndrome was diagnosed synchronously with lung cancer in 27 (57%) patients. Detection of lung cancer was incidental in 22 (47%) patients. The tumour was diagnosed at an early-stage, i.e. stage I-II, in 55% of cases. A pathological diagnosis of lung cancer was obtained for only 38 (81%) patients. Histological type was squamous cell carcinoma in 17 (36%) patients, adenocarcinoma in 14 (30%) patients, non-small cell lung cancer not otherwise specified in 3 (6%) patients, small cell lung cancer in 3 (6%) patients, and sarcomatoid carcinoma in one (2%) patient. There was no significant relation between tumor histology and location in the lung parenchyma (p=0.32). Overall, 20 of the 47 patients could not receive standard-of-care treatment for lung cancer, as per international recommendations and guidelines; this limitation was considered to be directly related to the CPFE syndrome in 8 (40%) cases. After a mean follow-up of 17 months, 35 patients were dead, and 12 patients were alive. Causes of death included locoregional or systemic tumor progression in 5 (14%) and 17 (49%) patients, respectively, respiratory failure in 8 (23%) patients, treatment-induced toxicity in 4 (11%) patients, and post-operative exacerbation of pulmonary fibrosis in 1 (3%) patient.

      Conclusion
      Lung cancer in patients with CPFE syndrome represents a specific entity characterized by very strong association with tobacco-smoking and male gender, peculiar histological-radiological presentation, poor prognosis due to major limitations and risks to conduct standard-of-care diagnostic and therapeutic interventions in a significant proportion of patients, and possible interest of screening. Lung cancer in CPFE syndrome further represents the most characteristic and severe model of tobacco-related disease.

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    P3.14 - Poster Session 3 - Mesothelioma (ID 197)

    • Event: WCLC 2013
    • Type: Poster Session
    • Track: Mesothelioma
    • Presentations: 1
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      P3.14-001 - Assessing the role of chemotherapy for solitary fibrous tumors of the pleura in a routine practice setting (ID 306)

      09:30 - 16:30  |  Author(s): N. Girard

      • Abstract

      Background
      Solitary Fibrous Tumors of the Pleura (SFTP) refer as to a heterogeneous group of mesenchymal malignancies with various anatomic and histologic features. Upfront surgical resection is the standard approach, but the outcome of patients is unpredictable. Recurrences may be aggressive and difficult to treat.The most widely accepted staging system has been proposed by De Perrot et al., and is based on the anatomy of the tumor implantation (sessile/pedunculated), and the presence of histologic signs of aggressiveness, including cellularity with crowding and overlapping of nuclei, cellular pleomorphism, high mitotic count, necrosis, or stromal/vascular invasion. Given the rarity of the tumor, limited evidence is available about the role and the modalities of perioperative and definite chemotherapy for SFTP.

      Methods
      Multicenter retrospective study of patients (pts) with histologically-proven SFTP with complete follow-up from surgical diagnostic to tumor recurrence and death.

      Results
      68 pts (28 males/40 females) were included. Median age at diagnosis was 62 year-old. Tumor stage according to the De Perrot system was 0/I for 29 pts, II for 23 pts, III for 7 pts, and IV for 4 pts. Adjuvant chemotherapy was given to 7 patients, mostly with stage III/IV SFTP, consisting of doxorubicin-based regimen. Recurrence rate and median time-to-progression (TTP) after surgery were 3%, 52%, 71%, and 75% (p<0.001), and 107, 70, 29, 11 months (p=0.006) for stage 0/I, II, III, and IV tumors, respectively. Besides tumor stage, predictors of shorter TTP were incomplete resection (p<0.001) and a higher number of histologic signs of malignancy (p=0.009). At time of tumor recurrence, 12 pts received chemotherapy. Highest disease control rates were observed with trabectedine (7/9 pts; Disease Control Rate (DCR): 78%; median TTP: 3,4 months), and gemcitabine-dacarbazine combination (2/3 pts, DCR: 66%; median TTP: 1,9 months). Median overall survival of the whole cohort was 56 months.

      Conclusion
      This study 1) confirms the prognostic value of the De Perrot staging system, 2) indicates a high recurrence rate in patients with stage II tumors, for which perioperative chemotherapy may be considered, and 3) suggests an interest for trabectedine in the setting of recurrent tumors. Besides clinical data, further molecular characterization, including recently identified specific gene fusions, may help to better predict the outcome of patients with SFTP.