HIGH GRADE SARCOMA WITH MYXOID FEATURES

Abstract

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Primary pulmonary sarcomas (PPS) are a rare tumor entity in general and tend to be low grade tumors (1). Most sarcomas diagnosed in the lung are due to metastasis and follow an aggressive course (2,3,4,5). This case presents an aggressive, high grade, primary lung spindle cell sarcoma with myxoid features. CASE PRESENTATION: An 84-year-old female presented with chest pain and worsening dyspnea on exertion for two weeks. Imaging revealed a left posterior perihilar mass in her lung. The patient was stabilized and discharged with planned outpatient bronchoscopy with endobronchial ultrasound with transbronchial needle biopsy (EBUS-TBNA). Biopsies of left hilar adenopathy and the lower lobe mass were positive for malignant cells. Histologic analysis, including immunohistochemistry, revealed the tumor to be most consistent with Primary Spindle Cell Sarcoma of the lung not otherwise specified (NOS). The patient presented two weeks after the bronchoscopy with worsening dyspnea and cough. Repeat imaging of her chest revealed significant progression of the mass-like consolidation. The patient was admitted for further evaluation. She became hemodynamically unstable and required intubation with mechanical ventilation and vasopressor support. The patient’s malignancy was determined staged as IVA, M1a. The patient's clinical status continued to rapidly deteriorate. The patient and her family decided to pursue comfort cares and the patient died shortly thereafter. DISCUSSION: Primary Pulmonary Myxoid Sarcoma (PPMS) is a similar tumor entity to PPS. Histologically, PPMS is characterized by polygonal tumor or spindle cells in a myxoid stroma with some atypia (4,6). The time of symptom onset to expiration was 61 days. This case describes a tumor with both aggressive and non-aggressive characteristics. Tissue analysis of this tumor revealed no cytological atypia or necrosis. Feng et al found that tumors in the respiratory system have a poorer prognosis. Tumor size is a prognostic indicator in PPS and tumors over 5 cm had a poorer overall survival (8). This tumor at symptom onset was 8 cm. The size and location of this tumor may have contributed to its aggressiveness and prognosis. Surgical resection is the treatment of choice for PPS and PPMS (3,7,8,9). CONCLUSIONS: There have been few case reports of this type of tumor being an aggressive and high-grade tumor. Given the aggressive nature, physicians need to be aware of this entity so appropriate guidance to patients and families can be provided. Furthermore, more research needs to be done in order to better classify this type of primary sarcoma of the lung. Reference #1: 1.Prieto-Granada CN, Ganim RB, Zhang L, Antonescu C, Mueller J. Primary Pulmonary Myxoid Sarcoma: A Newly Described Entity—Report of a Case and Review of the Literature. International Journal of Surgical Pathology. 2017;25(6):518-525. doi:10.1177/1066896917706413. Reference #2: 2.Koelsche C, Tavernar L, Neumann O, et al. Primary pulmonary myxoid sarcoma with an unusual gene fusion between exon 7 of EWSR1 and exon 5 of CREB1. Virchows Archiv. 2019. doi:10.1007/s00428-019-02716-4. Reference #3: 3.Billingsley KG, Burt ME, Jara E, et al. Pulmonary Metastases From Soft Tissue Sarcoma. Annals of Surgery. 1999;229(5):602. doi:10.1097/00000658-199905000-00002. 4.Jeon YK, Moon KC, Park S-H, Chung DH. Primary pulmonary myxoid sarcomas with EWSR1-CREB1 translocation might originate from primitive peribronchial mesenchymal cells undergoing (myo)fibroblastic differentiation. Virchows Archiv. 2014;465(4):453-461. doi:10.1007/s00428-014-1645-z. 5.Von Mehren M, Randall L, Benjamin RS et al. Soft tissue sarcoma version 2.2018. The Journal of the National Comprehensive Cancer Network. 2018;16(5): 536-563. doi: 10.6004/jnccn.2018.0025 6.Thway K, Nicholson AG, Lawson K, et al. Primary Pulmonary Myxoid Sarcoma With EWSR1-CREB1 Fusion. The American Journal of Surgical Pathology. 2011;35(11):1722-1732. doi:10.1097/pas.0b013e318227e4d2. 7.Feng L, Wang M, Yibulayin F, et al. Spindle cell sarcoma: a SEER population-based analysis. Scientific Reports. 2018;8(1). doi:10.1038/s41598-018-23145-4. 8.Spraker MB, Bair E, Bair R, Connell PP, Mahmood U, Koshy M. An Analysis of Patient Characteristics and Clinical Outcomes in Primary Pulmonary Sarcoma. Journal of Thoracic Oncology. 2013;8(2):147-151. doi:10.1097/jto.0b013e318277401f. 9.Bacha EA, Wright CD, Grillo HC, et al. Surgical treatment of primary pulmonary sarcomas1. European Journal of Cardio-Thoracic Surgery. 1999;15(4):456-460. doi:10.1016/s1010-7940(99)00045-7. DISCLOSURES: No relevant relationships by Jared Alberts, source=Web Response No relevant relationships by Alheli Arce Gastelum, source=Web Response No relevant relationships by Zachary DePew, source=Web Response No relevant relationships by Paul Millner, source=Web Response No relevant relationships by Kenneth Mueller, source=Web Response