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- W4387248110 abstract "SESSION TITLE: Lung Cancer Case Report Posters 8 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 02:10 pm - 02:55 pm INTRODUCTION: Pleomorphic carcinoma (PC) is a rare variety of non-small cell lung cancer (NSCLC) with reported incidence of only up to 0.4% in the US. It is a subtype of sarcomatoid carcinoma with components of spindle or giant cells. Most cases follow an aggressive course with rapid progression, distant metastases, and a high relapse rate, warranting aggressive management at an early stage. CASE PRESENTATION: 52-year-old male with past medical history of JAK2-positive myeloproliferative neoplasm (MPN), coronary artery disease, HIV, tobacco abuse presented with hemoptysis with blood clots, black stools, and unintentional weight loss. Blood work revealed leukocytosis with left shift, microcytic anemia, and thrombocytosis. Chest imaging showed central right lung mass with near complete post-obstructive atelectasis and mediastinal lymphadenopathy (figure1). PET scan revealed 6 cm necrotic right upper lobe mass consistent with primary lung cancer with mediastinal invasion, metastatic mediastinal lymphadenopathies with central necrosis, the largest subcarinal lymph node measuring 5.2x4.5 cm, distal metastases including intra-abdominal lymph nodes and left adrenal mass, suggestive of stage T4N2M1C. Right bronchial biopsy specimen was significant for invasive pleomorphic carcinoma with giant cell component. Tumor cells were positive for CAM 5.2, AE1/AE3 with high PD-L1 (98%) expression, negative for TTF-1, p63, HER2. Patient was diagnosed with stage IVb pleomorphic carcinoma of lung, initiated on Pembrolizumab and palliative radiation therapy. During a previous visit, he was diagnosed with essential thrombocythemia and found to have JAK2 V617F mutation (9.3%) for which he never received treatment. Patient responded well to treatment for 5 months, then was readmitted for altered mental status and hypernatremia. MRI revealed three new discrete cerebellar masses with 3x2.7x2.9 cm ring-enhancing lesion in right cerebellum with marked vasogenic edema, effacement of inferior aspect of fourth ventricle and mild hydrocephalus (figure2). Pan CT also revealed increase in size of lung mass with new metastatic lymphadenopathy. Due to his HIV status, there was high concern for infectious etiology, but with MR spectroscopy we established that these masses were metastatic and he underwent craniectomy and resection (figure3). Upon discharge, palliative radiation therapy to chest, brain as well as palliative chemotherapy with carboplatin, paclitaxel and pembrolizumab were started. Despite that, he developed new metastatic lesions to sternum, pancreas, right temporal lobe. During another hospitalization for septic shock, owing to poor prognosis, he chose not to be resuscitated and died due to cardiac arrest. DISCUSSION: Association of JAK2 V617F positive MPN to NSCLC has been widely debated but no causal association is established, however, the importance of next-generation sequencing in identifying targeted therapies for these patients has been proven beneficial. Some studies have revealed that, in patients with NSCLC and simultaneous JAK2 mutations, an increased incidence of lymph node metastasis, as well as cancer progression, was noted (1). They not only demonstrated overexpression of PDL-1 in the presence of simultaneous JAK 2 mutations in NSCLC, but also showed a reduction in expression with JAK2 inhibitors (2,3). CONCLUSIONS: Further investigation into the role of JAK 2 inhibitors in combination with immunomodulators is warranted in managing tumor progression and improving survival rates in aggressive cancers. REFERENCE #1: Xu Y, Jin J, Xu J, Shao YW, Fan Y. JAK2 variations and functions in lung adenocarcinoma. Tumour Biol. 2017 Jun;39(6):1010428317711140. doi: 10.1177/1010428317711140. PMID: 28639892. REFERENCE #2: Li SD, Ma M, Li H, Waluszko A, Sidorenko T, Schadt EE, Zhang DY, Chen R, Ye F. Cancer gene profiling in non-small cell lung cancers reveals activating mutations in JAK2 and JAK3 with therapeutic implications. Genome Med. 2017 Oct 30;9(1):89. doi: 10.1186/s13073-017-0478-1. PMID: 29082853; PMCID: PMC5662094. REFERENCE #3: Ikeda S, Okamoto T, Okano S, et al.: PD-L1 Is upregulated by simultaneous amplification of the PD-L1 and JAK2 genes in non-small cell lung cancer. J Thorac Oncol. 2016, 11:62-71. 10.1016/j.jtho.2015.09.010 DISCLOSURES: No relevant relationships by Krishna Desai No relevant relationships by Sabah Iqbal No relevant relationships by Rajesh Thirumaran" @default.
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- W4387248110 date "2023-10-01" @default.
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- W4387248110 title "A RARE CASE OF METASTATIC PLEOMORPHIC CARCINOMA WITH CO-EXISTING JAK2 MPN" @default.
- W4387248110 doi "https://doi.org/10.1016/j.chest.2023.07.2866" @default.
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