Sarcomatoid Carcinoma Of The Lung Research Articles

PULMONARY SARCOMATOID CARCINOMA WITH EXTENSION INTO THE RIGHT PULMONARY VEIN AND LEFT ATRIUM

PULMONARY SARCOMATOID CARCINOMA WITH EXTENSION INTO THE RIGHT PULMONARY VEIN AND LEFT ATRIUM

Spindle Cell Sarcomatoid Carcinoma of the Lungs in a Nigerian Woman: A Case Report

Spindle cell sarcomatoid carcinoma of the lung is a very rare but highly aggressive tumour. The presentation is usually non-specific and can be confused with bronchogenic carcinoma, even on imaging. Tissue biopsy, with histology and immunohistochemistry, is very helpful in diagnosing and differentiating sarcomatoid cell cancer from bronchogenic carcinoma. Surgical resection is the treatment of choice, and if it is not possible, palliative chemotherapy may be an option. This report is about a rare case of spindle cell sarcomatoid sarcoma of the left lung in a flight attendant who presented with left-sided, dull aching chest pain, associated shortness of breath, and cough that was occasionally productive, haemoptysis and weight loss of one-month duration. The tissue biopsy with histology and immunohistochemistry supported the clinical diagnosis of the tumour. Although rare, spindle cell sarcomas may occur in very few patients. The clinical history, imaging and pathologic findings are essential in preventing misdiagnosis of these patients.

KRAS Mutation in Rare Tumors: A Landscape Analysis of 3453 Chinese Patients.

KRAS is the most commonly mutated oncogene in human cancers. Targeted therapy and immunotherapy for this gene have made remarkable progress in recent years. However, comprehensive molecular landscape analysis of KRAS in rare tumors is lacking. Retrospective analysis was performed on clinical samples from patients with rare tumors collected between September 2015 and September 2021, using hybrid-capture-based next-generation sequencing for genomic profiling and immunohistochemistry assay for PD-L1. Of the 3,453 patients included in analysis, KRAS mutations were identified in 8.7% patients in overall; mutation rate and mutation subtypes varied widely across tumor systems and subtypes. KRAS mutations included 21 missense mutations, of which G12D (29.2%), G12V (24.6%), and G13D (10.8%) were most common. Interestingly, KRAS G12C was observed in 0.6% patients overall, and in 5.7% of sarcomatoid carcinoma of the lung and 5.4% of clear cell ovarian cancer tumors, but none in small-bowel cancer tumors. 31.8% KRAS mutations and 36.4% KRAS G12C mutations co-occurred with other targetable alterations. No significant correlation was observed between TMB-H, MSI-H, PD-L1 status, and KRAS mutation status, which may be related to the high proportion of G12D. This study is the first KRAS mutation landscape study in rare tumors of large sample size in China and worldwide. Our results suggest that targeted therapy and immunotherapy are both feasible, albeit complex, in these patients. This information may have significant impact on the operation of clinical trials for rare tumor patients with KRAS mutations in China.

Analysis of SMARCA4 and SMARCA2 Loss in Lung Sarcomatoid Carcinomas.

Sarcomatoid carcinomas of the lung are a group of aggressive tumors. It has been reported that losses of SMARCA4 and SMARCA2, which play a role in the repair and remodeling of chromatin, contribute to the initiation, progression, and differentiation of neoplasms. The aim of our study was to examine SMARCA4 and SMARCA2 profiles in sarcomatoid carcinomas of the lung. We screened pleomorphic carcinomas (PCs), carcinosarcomas (CSs), and pulmonary blastomas (PBs). The loss of SMARCA4 and SMARCA2 expression in the tumors was evaluated using immunohistochemical methods. The tumors were also examined to determine immunophenotype, histological tumor diagnosis, surgical resection, tumor histological component, largest tumor diameter, and lymph node metastasis status. Sixty-nine cases were screened, of which 84% were PCs, 13% were CSs, and 2.8% were PBs. In PCs components, 84.4% were biphasic and 15.5% were monophasic. The PCs showed the most frequent loss of SMARCA4 (25.8%) and SMARCA2 (44.8%). A loss of SMARCA4 and SMARCA2, respectively, was detected in 14.2% and 24.4% in both components of biphasic PCs; 12.2% and 14.2% in the sarcoma component of biphasic PCs; 0% and 8.1% in the carcinoma component of biphasic PCs; 22.2% and 33.3% in monophasic PCs; 0% and 22.2% in both components of CSs; and 0% and 22.2% in the sarcoma component of CSs. These findings demonstrate a loss of expression of SMARCA4 and SMARCA2 in pulmonary sarcomatoid carcinomas. Loss of the SMARCA complex may be caused by the heterogeneous morphological profile of sarcomatoid carcinomas, independent of tumor histopathological parameters.

Case Report: Pulmonary sarcomatoid carcinoma in a female patient from Nepal

Sarcomatoid carcinoma of the lung is an uncommon subtype of non-small-cell lung cancer (NSCLC). Even in the early stages, pulmonary sarcomatoid carcinoma (PSC) has a dismal prognosis when compared to other kinds of NSCLC with a mean survival of 9-12 months and a five-year survival rate of around 20%. We present the case of a 68-year-old woman with a two-month history of shortness of breath and cough. Initial computed tomography (CT) scan showed features of interstitial lung disease with chronic obstructive airway changes. After 34 months, the patient's condition worsened with newer complaints of sore throat and hemoptysis. A repeat CT scan showed a ∼49x38x59mm size lesion in the superior segment of the left lower lobe. A core needle biopsy was performed, which revealed tumor cells consisting of irregular tubules and sarcomatoid components. The patient was started on chemotherapy. Unfortunately, she succumbed to her disease. Our case highlights the aggressiveness of PSC.

CONCURRENT PULMONARY AND JEJUNAL SARCOMATOID CARCINOMA WITH BRAIN METASTASES

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary sarcomatoid carcinoma (SCA) is a poorly differentiated, aggressive type of non-small cell lung cancer accounting for 0.1-0.3% of all lung cancers1-3. It is commonly seen in male smokers in their sixties. We present a rare case of concurrent pulmonary and jejunal SCA with brain metastases. CASE PRESENTATION: 75-year-old male with 40-pack year smoking history presents to the hospital with two weeks of nausea, vomiting, weight loss and dyspnea. CXR revealed a 10.1 x 7.4 cm right perihilar mass. Subsequent chest CT showed a 7.8 x 6.6 x 8.7 cm mass involving the posterior segment of right upper lobe, right middle lobe, and right lower lobe. PET scan showed avid lesions involving jejunum, brain, and adrenal glands. Endobronchial biopsy of the lung mass obstructing the lumen of posterior segment of right upper lobe revealed SCA with focal pleomorphism. Immunohistochemical staining was positive for Keratin AE1/AE3 and vimentin. A subsequent jejunal mass biopsy was also consistent with SCA with staining positive for keratin CAM5.2 and ß-hCG. Patient underwent whole brain radiotherapy as well as palliative pembrolizumab therapy. DISCUSSION: SCA is an aggressive cancer with average survival of 8.8 months and 13 months when involving small intestine and lung respectively. 5,7 Immunohistochemical staining is crucial in differentiating metastases versus primary SCA. In our case, TTF-1, Napsin-A, and CK7 staining of the jejunal mass were negative, which contradicts lung derivation. 4 This is a rare case of primary pulmonary SCA and concurrent primary jejunal SCA with brain metastases. Most SCA tumors involving small intestine are polypoid with central ulceration and frequently necrotic and hemorrhagic. 6,7 In contrast, pulmonary SCA usually arises as a solitary central airway mass with an average size of 7 cm.3 Symptoms in pulmonary SCA are non-specific such as chest pain, dyspnea and weight loss.1,4 This makes early diagnosis challenging. Patients often present in advanced stages with extra-thoracic metastases.4 Findings of brain metastasis is rare due to aggressive course of disease with low survival time.3 Both pulmonary and jejunal SCA are resistant to traditional therapeutic methods. Novel therapies like immunotherapy appears promising. CONCLUSIONS: This case is unique as it has two concurrent primaries for SCA, lung and jejunum. It is imperative to perform a complete oncological evaluation to assess for metastases. Early diagnosis and proper adjuvant therapy could prolong survival and improve quality of life. Reference #1: Arshad, H., Dudekula, R., Niazi, M., Malik, S., and Khaja, M. (2017). A Rare Case of Sarcomatoid Carcinoma of the Lung with Spine Metastasis, Including a Literature Review. Am J Case Rep. 2017; 18: 760-765. Beasley, M., Brambilla, E., and Travis, W. (2005). The 2004 World Health Organization classification of lung tumors. Semin Roentgenol. 2005 Apr, 40 (2): 90-7. Reference #2: Oliveira MF, Watanabe SC, Andrade MP, Rotta JM, Pinto FC. Sarcomatoid carcinoma of the lung with brain metastases. J Bras Pneumol. 2013;39(6):753-756. doi:10.1590/S1806-37132013000600016 Jia J, Ren J, Gu J, Di L, Song G. Predominant sarcomatoid carcinoma of the lung concurrent with jejunal metastasis and leukocytosis. Rare Tumors. 2010;2(3):e44. Published 2010 Sep 30. doi:10.4081/rt.2010.e44 Reference #3: Huang, S., Shen, S., and Li, X. (2013). Pulmonary sarcomatoid carcinoma: a clinicopathologic study and prognostic analysis of 51 cases. World J Surg Oncol 2013; 11: 252. Kwok CM. Sarcomatoid carcinoma of the jejunum with gastric metastases: A case report and review of the literature. Int J Surg Case Rep. 2016;28:161-164. doi:10.1016/j.ijscr.2016.09.046 Zhang B, Cheng BO, Wang L, Zhao KE, Zhuo GZ, Ding JH. Primary sarcomatoid carcinoma of the jejunum with massive intra-abdominal hemorrhage: A case report and review of the literature. Mol Clin Oncol. 2016;4(5):811-816. doi:10.3892/mco.2016.809 DISCLOSURES: No relevant relationships by Kushagra Gupta, source=Web Response No relevant relationships by Anthony Loschner, source=Web Response No relevant relationships by Elizabeth McCaskey, source=Web Response No relevant relationships by Toribiong Uchel, source=Web Response

SOLITARY PULMONARY NODULE PRESENTING AS A SARCOMATOID CARCINOMA IN A PREGNANT WOMAN: A DIAGNOSTIC DILEMMA

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: The majority of lung cancers are made up of epithelial tumors with adenocarcinoma being the most common. Sarcomatoid carcinoma of the lung is rare and is present in only 0.52% of all diagnosed cases of NSCLC. The WHO has classified mesenchymal tumors of the lung into vascular, vessel-associated tumors and nonvascular spindle cell tumors. The etiology of sarcomas is usually genetic in nature, a result of environmental toxin exposures, or due to a mutation. CASE PRESENTATION: A 37 year-old female who was six months pregnant, presented with one episode of hemoptysis in the setting of a chronic cough. No history of lung disease or other health conditions. No prior smoking or vaping history. Initial chest X-ray revealed a right lung nodule within the superior segment of the right lower lobe, initially concerning for a pulmonary hamartoma or carcinoid. CT chest was delayed due to her pregnancy, and ultimately deferred until after delivery three months later which showed an enlarging heterogeneous, 3.5 cm x 3.4 cm right lower lobe mass. She refused CT/PET as she was breastfeeding her newborn. She eventually agreed to surgery and underwent video-assisted thoracoscopic surgery (VATS) with mediastinal and regional lymphadenectomy and RLL lobectomy. Pathology revealed a low-grade sarcoma, with multiple analyses concerning for low-grade endometrial stromal sarcoma (ESS) vs. vascular neoplasm. Further molecular analysis with FISH confirmed low-grade vascular sarcoma with regional lymph node involvement (ESS was negative). Her PET scan following surgery was negative for any disease. DISCUSSION: Our patient had a RLL mass showing a malignant spindle cell neoplasm with a positive right hilar lymph node favored to be a low-grade sarcoma. Given its morphology, the differential diagnoses were low-grade endometrial stromal sarcoma, malignant peripheral nerve sheath tumor (MPNST), solitary fibrous tumor (SFT), inflammatory myofibroblastic tumor, PEComa, and synovial sarcoma. A definitive diagnosis is of utmost importance as certain types of sarcoma confer a poorer prognosis. For example, primary pulmonary synovial sarcoma is usually extremely aggressive with a 5-year survival rate of only 50%. CONCLUSIONS: This case is very unique in a few ways. First, it is an uncommon cause for a solitary pulmonary nodule along with being an uncommon malignancy. Second, it presents a complicated diagnostic dilemma in a pregnant patient who was reluctant to have diagnostic imaging and procedures that would have aided in the diagnosis. Reference #1: Hashimoto H, Tsugeno Y, Sugita K, Inamura K (2019) Mesenchymal tumors of the lung: diagnostic pathology, molecular pathogenesis, and identified biomarkers. J Thorac Dis 11:S9–S24 Reference #2: Rahouma M, Kamel M, Narula N, Nasar A, Harrison S, Lee B, Stiles B, Altorki NK, Port JL (2018) Pulmonary sarcomatoid carcinoma: an analysis of a rare cancer from the Surveillance, Epidemiology, and End Results database. Eur J Cardiothorac Surg 53:828–834 DISCLOSURES: No relevant relationships by Bhavik Gupta, source=Web Response No relevant relationships by Bryan Stone, source=Web Response No relevant relationships by Christopher Wyckoff, source=Web Response

A UNIQUE PRESENTATION OF SARCOMATOID CARCINOMA

SESSION TITLE: Fellows Lung Cancer Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Sarcomatoid carcinoma is a rare group of non-small cell lung cancers with a prevalence of less than 1% and high mortality, typically presenting with locally advanced or metastatic disease. CASE PRESENTATION: A 70 year old male with a history of coronary artery disease, 1 pack-per-day tobacco abuse (and declined lung cancer screening in the past) and COPD presented to the emergency room with 1 month of progressive shortness of breath and chest pain. Upon evaluation, the patient was in significant distress with a lactate elevation to 7mmol/L. Bedside ultrasound showed a large pericardial effusion with tamponade. Patient was emergently taken for pericardial window placement with 700cc bloody fluid drainage. CT Chest was performed and showed a large mediastinal mass resulting in mass effect and complete compression of right mainstem bronchus without any distal aeration. Patient underwent endobronchial ultrasound. It was noted that the right main bronchus was completely obstructed and mass appeared to be coming from the anteromedial wall of the right mainstem bronchus. Argon plasma coagulation was used to coagulate the tumor and obtain biopsies; cryotherapy used for larger samples. RUL was completely recanalized, however bronchus intermedius remained occluded by extrinsic compression.Cytology from pericardial fluid was positive for markedly atypical, poorly differentiated malignant cells. Final pathology of mass showed spindle cells with extensive necrosis, most consistent with sarcomatoid carcinoma. Tumor was also positive for AE1/AE3, CK7; negative for PDL-1. DISCUSSION: Due to the low prevalence of sarcomatoid carcinoma, there is a limited data regarding clinical manifestations of disease. According to the 2015 World Health Organization Classification of Lung Tumors, sarcomatoid carcinoma includes 5 subtypes (including spindle cell carcinoma) and suggests molecular testing to assess for genetic abnormalities, which can help direct treatment options. Typically, immunohistory chemistry for spindle cell carcinoma is positive for cytokeritin and vimentin, but negative for desmin, CD34, and S100 protein. Most are also positive for PDL-1 which opens the possibility for immunotherapy. Our patient was positive for AE1/AE3, which is of epithelial origin and negative for PDL-1. While surgical resection is the mainstay of treatment for local disease, there is minimal data on the treatment of metastatic disease and are usually treated similarly to non-small cell lung cancer. CONCLUSIONS: Given the rarity of sarcomatoid carcinoma of the lung, there is minimal literature to guide treatment in these patients. More data is necessary to help treatment of widespread disease. Reference #1: Arshad, Hafiza Sobia, et al. “A Rare Case of Sarcomatoid Carcinoma of the Lung with Spine Metastasis, Including a Literature Review.” American Journal of Case Reports, vol. 18, 2017, pp. 760–765., doi:10.12659/ajcr.904584. Reference #2: Travis WD, Brambilla E, Nicholson AG, et al. The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification. J Thorac Oncol. 2015;10(9):1243-1260. doi:10.1097/JTO.0000000000000630 Reference #3: Ouziane, Imane, Saber Boutayeb, Hind Mrabti, Issam Lalya, Mouna Rimani, and Hassan Errihani. “Sarcomatoid Carcinoma of the Lung: A Model of Resistance of Chemotherapy.” North American Journal of Medical Sciences 6.7 (2014): 342. Print. DISCLOSURES: No relevant relationships by Jennifer Fung, source=Web Response No relevant relationships by Janvi Paralkar, source=Web Response

Prognostic factors in patients undergoing pulmonary resection for sarcomatoid carcinomas of the lung.

BackgroundPulmonary sarcomatoid carcinomas are very rare lung neoplasms, and no consensus exists regarding their optimal treatment. The prognosis of sarcomatoid carcinomas has been reported to be unfavorable compared with non-small-cell lung cancers; however, prognostic factors in patients undergoing surgery for sarcomatoid carcinomas remain unclear.AimsTo analyze clinicopathologic prognostic factors and survival outcomes in patients who underwent surgery for pulmonary sarcomatoid carcinoma.Study DesignRetrospective cross-sectional study.MethodsWe designed a retrospective cross-sectional study in patients who underwent surgery for pulmonary sarcomatoid carcinomas and statistically analyzed the prognostic factors regarding clinicopathologic features with respect to survival outcomes.ResultsWe had a total of 44 patients with sarcomatoid carcinoma who had pulmonary resection. Sex distribution was 34 (77%) males and 10 (22.7%) females, which was determined by declaration. The mean age of patients was 57.3 ± 16 years with a median of 60 years. The 5-year progression-free survival and overall survival rates were 59% and 58%, respectively. The 5-year survival rates were significantly different for tumors > 5 cm (P = 0.044), tumorstatus (T) (P=0.016), lymph node status (N) (P=0.005), and pathologic tumor-node-metastasis (TNM) stage (P = 0.0001 ). However, histologic subtype (P = 0.628) and adjuvant treatment (P = 0.804) did not have any significant effect on survival. Similarly, the significant prognostic factors in univariate analysis were tumor size (P = 0.085), T status (P = 0.005), N status (P = 0.028), and pathologic TNM stage (P = 0.0001). Multivariate analysis showed only T status (P = 0.058), N status (P = 0.018), and pathologic TNM stage (P = 0.019) as independent prognostic factors, with statistical power of 87%, 43.1% and 21.2%.ConclusionSurgery appears to be an optimal treatment with favorable outcomes for patients with pulmonary sarcomatoid carcinoma. Patients with small tumors at earlier stages are very likely to benefit from surgery, regardless of histologic subtype.

The prevalence of targetable genomic alterations is substantially high in rare tumors.

e13539 Background: Comprehensive Genomic Profiling may be informative for novel treatment strategies and to improve outcome for patients with rare solid tumors. This study aims to discover opportunities for use of targeted therapies currently in routine practice in patients with rare tumors. Methods: Solid tumors with an incidence lower than 2.5/100,000 per year was defined as rare tumors in China after comprehensive analysis based on epidemiology data and current availability of standardized treatment. Genomic data of rare tumors from the public database cBioPortal was compared with that of Chinese population for targetable genomic alterations (TGAs). TGAs was defined as mutations of ALK, ATM, BRAF, BRCA1, BRCA2, CDKN2A, EGFR, ERBB2, FGFR1,2,3, KIT, MET, NF1, NTRK1,2,3, PIK3CA, PTEN, RET, and ROS1 with level 1 to 4 of evidence according to OncoKB knowledge database. Results: Genomic data of 4901 patients covering 63 subtypes of rare tumor from cBioportal was obtained as the western cohort. Data of next generation sequencing (NGS) of 1312 patients from across China covering 67 subtypes was summarized as the Chinese cohort. Forty-one subtypes were overlapped between the two cohorts. The accumulative prevalence of TGAs was 20.40% (1000/4901) in cBioportal cohort, and 53.43% (701/1312) in Chinese cohort (p < 0.001). Among those 41 overlapping subtypes, it was still significantly higher in Chinese cohort compared with cBioportal cohort (54.1%% vs. 26.1%, p < 0.001). Generally, targetable mutations in BRAF, BRCA2, CDKN2A, EGFR, ERBB2, KIT, MET, NF1, ROS1 were ≥3 times more frequent in Chinese cohort compared with that of cBioportal cohort. Cancer of unknown primary tumor type, gastrointestinal stromal tumor, gallbladder cancer, intrahepatic cholangiocarcinoma, and sarcomatoid carcinoma of the lung were the top 5 tumor types with highest TGAs. Conclusions: The incidence of TGAs in rare tumors was substantially high worldwide and was even higher in Chinese rare tumor population. Comprehensive genomic profiling may offer novel treatment paradigms to address the limited options for patients with rare tumors.

Comprehensive Genomic Profiling of Rare Tumors: Routes to Targeted Therapies.

Comprehensive Genomic Profiling may be informative for novel treatment strategies and to improve outcomes for patients with rare tumors. This study aims to discover opportunities for use of targeted therapies already approved for routine use in patients with rare tumors. Solid tumors with an incidence lower than 2.5/100,000 per year was defined as rare tumors in China after comprehensive analysis based on epidemiological data and current availability of standardized treatment. Genomic data of rare tumors from the public database cBioPortal were compared with that of the Chinese population for targetable genomic alterations (TGAs). TGAs were defined as mutations of ALK, ATM, BRAF, BRCA1, BRCA2, CDKN2A, EGFR, ERBB2, FGFR1,2,3, KIT, MET, NF1, NTRK1,2,3, PIK3CA, PTEN, RET, and ROS1 with level 1 to 4 of evidence according to the OncoKB knowledge database. Genomic data of 4,901 patients covering 63 subtypes of rare tumor from cBioPortal were used as the western cohort. The Chinese cohort was comprised of next generation sequencing (NGS) data of 1,312 patients from across China covering 67 subtypes. Forty-one subtypes were common between the two cohorts. The accumulative prevalence of TGAs was 20.40% (1000/4901) in cBioPortal cohort, and 53.43% (701/1312) in Chinese cohort (p < 0.001). Among those 41 overlapping subtypes, it was still significantly higher in Chinese cohort compared with cBioPortal cohort (54.1%% vs. 26.1%, p < 0.001). Generally, targetable mutations in BRAF, BRCA2, CDKN2A, EGFR, ERBB2, KIT, MET, NF1, ROS1 were ≥3 times more frequent in Chinese cohort compared with that of the cBioPortal cohort. Cancer of unknown primary tumor type, gastrointestinal stromal tumor, gallbladder cancer, intrahepatic cholangiocarcinoma, and sarcomatoid carcinoma of the lung were the top 5 tumor types with the highest number of TGAs per tumor. The incidence of TGAs in rare tumors was substantial worldwide and was even higher in our Chinese rare tumor population. Comprehensive genomic profiling may offer novel treatment paradigms to address the limited options for patients with rare tumors.

Favorable response to pembrolizumab after durvalumab failure in a stage III sarcomatoid carcinoma of the lung: a case report

BackgroundPulmonary sarcomatoid carcinoma is a rare non-small-cell lung cancer (NSCLC) subtype with a poor prognosis. In the phase III PACIFIC study, durvalumab significantly improved progression-free survival and overall survival versus placebo, in patients with stage III NSCLC who do not have disease progression after concurrent chemoradiotherapy. However, treatments for patients who discontinue durvalumab due to disease progression, are unknown.Case presentationWe report a case of favorable response to pembrolizumab in a patient with disease progression during durvalumab consolidation therapy after chemoradiotherapy for stage III pulmonary sarcomatoid carcinoma with high programmed cell death ligand 1 (PD-L1) and PD-L2 expression.ConclusionHere, we present what, to the best of our knowledge, is the first reported case in which durvarumab resistance after definitive chemoradiotherapy in a patient with stage III pulmonary sarcomatoid carcinoma was overcome by pembrolizumab.

Underestimating the Burden of Disease in Sarcomatoid Carcinoma of The Lung: A Case Report and Literature Review

Acute appendicitis is one of the most common emergency surgical procedure, yet atypical presentation sometimes can be challenging for clinician. I present a case of 19-year-old gentleman that initially presented with 1day history of bilateral testicular pain and lower abdominal pain. His past history includes a positive sexual history. Initial ultrasound of the testis showed bilateral orchitis and an equivocal appendix. With a significantly raised inflammatory marker and highly suspicious for appendicitis, a CT scan was obtained which showed perforated appendicitis and the patient underwent laparoscopic appendicectomy with resolution of symptoms after that. We encourage clinician to be aware of this clinical pitfall as patient can sometimes be managed in other department to minimise any delayed diagnosis or any unnecessary procedure

The Prognostic Significance Of JMJD3 In Primary Sarcomatoid Carcinoma Of The Lung, A Rare Subtype Of Lung Cancer.

IntroductionPrimary sarcomatoid carcinoma of the lung (PSC) is a rare subtype of non-small cell lung cancer, which has a bad prognosis and lacks biomarkers for its diagnosis and prognosis. Recent studies suggested that KDM6B (lysine demethylase 6B), also known as Jumonji domain-containing protein D3 (JMJD3), plays an oncogenic role in various human cancers. However, abnormalities of JMJD3 in sarcomatoid carcinoma of the lung and its clinical prognostic significance have not been determined. Therefore, the present study aimed to ascertain the relationship between JMJD3 and PSC.Materials and methodsIn this study, immunohistochemistry (IHC) was performed to examine the expression of JMJD3 in a tissue microarray (TMA) containing 96 cases of PSC.ResultOverexpression of JMJD3 was observed in nuclei of the PSC cells. Further analyses indicated that the overexpression of JMJD3 was significantly associated with tumor size, pN stage, and clinical stage. By univariate survival analysis, positive expression of JMJD3 was significantly correlated with shortened patient survival. More importantly, multivariate analysis identified JMJD3 as an independent prognostic factor for sarcomatoid carcinoma of the lung.ConclusionThese findings provide evidence that JMJD3 protein levels, as examined by IHC, may act as a novel prognostic biomarker for patients with primary sarcomatoid carcinoma of the lung.

SPINDLE CELL CARCINOMA OF THE LUNG: A RARE, AGGRESSIVE TUMOR ELUSIVE TO TYPICAL DIAGNOSTIC METHODS

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Spindle cell carcinoma is a subtype of sarcomatoid carcinoma, which encompasses a rare group of non-small cell lung carcinomas (NSCLC) that contain true sarcoma or sarcoma-like elements. Less than one percent of all lung cancers are sarcomatoid carcinomas. Here we present a case of spindle cell carcinoma that proved difficult to diagnose in the absence of surgical lobectomy. CASE PRESENTATION: A previously healthy 73-year-old female non-smoker presented to her primary care physician for a persistent non-productive cough. A chest radiograph revealed a large left upper lobe (LUL) mass. A computed tomography (CT) scan of the chest was performed, which demonstrated a 7.5cm LUL mass with a necrotic-appearing center and mediastinal lymphadenopathy. A subsequent positron emission tomography (PET)-CT scan showed uptake around the rim of the mass, sparing the center. She underwent bronchoscopy with endobronchial ultrasound-transbronchial needle aspirations of the mediastinal lymph nodes as well as radial probe ultrasound-guided transbronchial biopsies of the mass, with pathology showing inflammation and no evidence of malignancy. Shortly after the bronchoscopy, she became febrile with shaking chills. She was started on amoxicillin-clavulanate for possible lung abscess. A three-week follow-up CT scan showed no improvement in the size of the mass, so she underwent a CT-guided core biopsy, the pathology from which revealed extensive necrosis but was again negative for malignancy. A thoracentesis was also performed for a small left pleural effusion, during which 470ml of hemorrhagic fluid was drained. Fluid cytology and cultures were negative. Antibiotics were discontinued and she was referred for a lobectomy for definitive diagnosis. She underwent a left upper lobectomy, with pathology revealing an 8.5cm spindle cell carcinoma with visceral pleural invasion and involvement of the internal mammary and levels 5 and 6 lymph nodes, consistent with stage IV disease. A PET-CT performed three weeks post-operatively was highly concerning for residual or recurrent neoplasm in the left anterior chest wall. Radiation therapy was initiated. Genetic testing revealed a MET exon 14 deletion as well as a high expression of PD-L1. The patient is planning to start chemotherapy with pembrolizumab and crizotinib in the near future. DISCUSSION: This case highlights a rare form of lung cancer that was very difficult to diagnose, with false negative bronchoscopic biopsies and CT-guided percutaneous core needle biopsy. Such biopsy methods may not be capable of detecting epithelial and sarcomatous components, which could lead to a missed diagnosis. CONCLUSIONS: Given the high clinical suspicion for malignancy in this patient with a persistent and enlarging lung mass, a surgical biopsy was necessary to obtain a definitive diagnosis. Reference #1: Franks TJ, Galvin JR. Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis. Arch Pathol Lab Med. 2010;134(1):49-54. Reference #2: Girard N, Cadranel J, Brambilla E, Cordier J-F. Rare primary lung tumors. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. United States: Elsevier; 2016:965-980. Reference #3: Tazelaar, HD. Pathology of lung malignancies. UpToDate. https://www.uptodate.com/contents/pathology-of-lung-malignancies?search=sarcomatoid%20carcinoma&source=search_result&selectedTitle=1∼29&usage_type=default&display_rank=1. Updated November 16, 2018. Accessed March 13, 2019. DISCLOSURES: No relevant relationships by Stephanie Hart, source=Web Response No relevant relationships by Leah Lande, source=Web Response No relevant relationships by Kriti Pathak, source=Web Response

EP1.09-21 Sarcomatoid Carcinoma of the Lung. Analysis of the Tumor Immune-Microenvironment and Survival of a Case Series

EP1.09-21 Sarcomatoid Carcinoma of the Lung. Analysis of the Tumor Immune-Microenvironment and Survival of a Case Series

459P - Clinical outcomes and treatment strategies of sarcomatoid carcinoma of the lung

459P - Clinical outcomes and treatment strategies of sarcomatoid carcinoma of the lung

P2.16-46 Clinical Outcomes and Treatment Strategies of Sarcomatoid Carcinoma of the Lung

Sarcomatoid carcinoma of the lung is characterized by worse prognosis, and generally felt to be chemo-refractory compared with other non-small cell lung cancer. We conduct this retrospective study to investigate the clinical characteristics of patients with sarcomatoid carcinoma of the lung and determine the optimal treatment strategies. We reviewed the medical records of 8176 patients with resected lung cancer in a single high-volume institution between 2008 and 2015. All patients with pathologically diagnosed sarcomatoid carcinoma were evaluated. Clinicopathologic data were analyzed using Kaplan-Meier analysis and Cox regression analysis. Subgroups stratified by pathological stage were analysed to determine the optimal treatment modality. We also conducted subgroup analysis of overall survival among pulmonary sarcomatoid carcinoma and other NSCLC patients. Kaplan-Meier and Cox regression analyses showed pathological stage (8th edition) is the independent prognostic factor(P=0.001, HR=2.601, 95%CI(1.447-4.675)) for pulmonary sarcomatoid carcinoma. Overall survival favored other NSCLC over PSC across subgroups. Pathological stage (8th edition) is independent prognostic factor for sarcomatoid carcinoma of the lung. Surgery followed by adjuvant chemotherapy should be considered for stage I pulmonary sarcomatoid carcinoma. Further prospective studies are needed to confirm these results.

STAGE IV PULMONARY SARCOMATOID CARCINOMA MASQUERADING AS A PACEMAKER

SESSION TITLE: Lung Cancer 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Pulmonary sarcomatoid carcinoma (PSC) is a broad term used to describe poorly differentiated non-small cell lung cancers (NSCLCs) with sarcoma or sarcoma-like (spindle and/or giant cell) features. PSC constitutes less than 1% of all lung cancers, but it is the most common pulmonary neoplasm composed of spindled or pleomorphic tumor cells. We present a case of a stage IV PSC with a unique presentation. CASE PRESENTATION: A 75 year-old male presented with progressive worsening of generalized weakness, loss of appetite, subjective weight loss, and increased number of falls over a three-month period. He had a history of Addison’s disease with chronic hyponatremia and a 100 pack-year smoking history. On exam, the patient had a raised area on the left anterior chest wall, initially suspected to be a pacemaker given its shape and location. However, the patient denied ever having any cardiac procedures. Chest x-ray followed by Computed Tomography (CT) of the chest demonstrated a 13.1 x 9.2 x 10.7 cm mass occupying the left hemithorax that involved the mediastinum, touched the aortic arch and pulmonary artery, eroded through the second left anterior rib, and extended into the pectoralis muscles. The percutaneous biopsy specimen showed a malignant spindle cell neoplasm with marked pleomorphism and necrosis. It stained positively for p63, GATA3, and SMA; weakly/equivocally for pancytokeratin; and negatively for CK5/6, CD34, Desmin, SOX10, CK7, EMA, calretinin, and CK20. The patient was deemed too weak to tolerate aggressive systemic chemotherapy. Palliative radiation was initiated. DISCUSSION: PSC carries a significantly worse prognosis than other major types of NSCLCs. Patients are predominantly male smokers, with a mean age of 65 at time of diagnosis. Definitive diagnosis typically requires a resection specimen, which is obtained concomitantly as part of treatment, or not pursued in advanced stages. The main treatments for early-stage PSC are surgical resection and perioperative chemotherapy. No standard targeted or immunotherapy treatments are available. CONCLUSIONS: PSCs are extremely rare, and as such, clinical presentations can be unique. In the case of this patient, one cannot underestimate the importance of a thorough physical exam and history. Reference #1: Franks TJ, Galvin JR. Sarcomatoid Carcinoma of the Lung: Histologic Criteria and Common Lesions in the Differential Diagnosis. Arch Pathol Lab Med. 2010; 134(1):49-54. Reference #2: Maneenil K, Xue Z, Liu M, et al. Sarcomatoid Carcinoma of the Lung: The Mayo Clinic Experience in 127 Patients. Clin Lung Cancer. In press. https://doi.org/10.1016/j.cllc.2017.12.008. Reference #3: Travis WD. Sarcomatoid neoplasms of the lung and pleura. Arch Pathol Lab Med. 2010; 134(11):1645-58. DISCLOSURES: No relevant relationships by Muhammad Habib, source=Web Response No relevant relationships by Manish Patel, source=Web Response No relevant relationships by Thien Vo, source=Web Response No relevant relationships by Grace Wang, source=Web Response

1362P - Clinical outcomes and treatment strategies of sarcomatoid carcinoma of the lung

1362P - Clinical outcomes and treatment strategies of sarcomatoid carcinoma of the lung