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J. Wang



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    P2.01 - Poster Session/ Treatment of Advanced Diseases – NSCLC (ID 207)

    • Event: WCLC 2015
    • Type: Poster
    • Track: Treatment of Advanced Diseases - NSCLC
    • Presentations: 1
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      P2.01-053 - The Role of Systemic Therapy in Sarcomatoid Carcinoma of the Lung (ID 890)

      09:30 - 17:00  |  Author(s): J. Wang

      • Abstract

      Background:
      Primary sarcomatoid carcinoma (PSC) accounts for 2% to 3% of all lung cancers. Stage-for-stage, PSC carries a poorer prognosis compared to the more common types of lung cancer. It typically occurs in older heavy smoking men and has a predilection for upper lobe involvement. PSC of the lung was initially described by Virchow in 1865 as a “biphasic” lesion of adenocarcinomatous or squamous cell components along with spindle cell or giant cell elements forming at least 10% of the tumor mass. This description fulfills the current WHO criteria for the diagnosis of PSC. Mutational analysis has revealed a common origin of both elements and it is thought that epithelial-mesenchymal transition (EMT) is the mechanism of that gives rise to this tumor, with the epithelial elements (adenocarcinoma or squamous component) that has undergone a transition to a poorly-differentiated mesenchymal type (sarcomatoid) with the expression of mesenchymal proteins such as vimentin. Efforts to study PSC has been hindered by the rarity of this variant. Aim of the study: To assess the impact of surgery and various systemic therapies on patients with PSC of the lung at the University of Cincinnati Medical Center (UCMC).

      Methods:
      This retrospective study included all patients identified with a pathologically confirmed diagnosis of PSC of the lung treated at UCMC between the years 2000-2014. Death was considered as the study endpoint. Kaplan-Meier analysis was used to calculate median overall survival (OS) and 95% confidence intervals (CI). Cox model was used to test the chemotherapy effect adjusted for age, sex and surgery, and determine hazard ratios (HR). Data was analyzed using SAS® Version 9.4.

      Results:
      We identified 21 patients with a diagnosis of PSC of the lung that were eligible for chart review and analysis. The 14 men and 7 women had a median age of 59 (range, 31-84 years). Treatment with systemic chemotherapy showed a trend in improvement in outcome among all stages of disease (p=0.08 and HR 0.04) but chemotherapy was most often used in advanced stages. Female gender demonstrated a trend for improved OS (p=0.1), and older patients demonstrated a better OS (HR=0.849; p=0.041) by a one-year increase in age. The median OS of the patients with PSC treated with systemic chemotherapy was 375 days (95% CI 114-600 days). Patients with early stage disease who were eligible for surgical resection, with or without the addition of systemic chemotherapy had a median survival of 457.5 days (95% CI 206.-1187 days), only slightly different from patients with advanced disease that received systemic chemotherapy. Patients who did not receive systemic chemotherapy had a lower median OS of 256 days (95% CI 98-999 days). Two patients demonstrated EML-4/ALK translocations. The patient with the longest OS of about three years was treated with systemic therapies including cisplatin, gemcitabine, docetaxel and crizotinib.

      Conclusion:
      Patients with PSC of the lung may benefit from systemic therapy. Larger prospective studies are needed to confirm this benefit especially if used as an adjuvant therapy in early stage disease.

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    P3.03 - Poster Session/ Treatment of Locoregional Disease – NSCLC (ID 214)

    • Event: WCLC 2015
    • Type: Poster
    • Track: Treatment of Locoregional Disease – NSCLC
    • Presentations: 1
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      P3.03-035 - Unclear Clinical Course and Treatment Options for Clear Cell Lung Cancer: A Case Series (ID 2486)

      09:30 - 17:00  |  Author(s): J. Wang

      • Abstract
      • Slides

      Background:
      Clear cell lung cancer (CCLC) is an extremely rare variant of lung tumors which was first described by Liebow and Castleman in 1963. It has vague natural history with slight female predominance and is most often seen in elderly. The clear appearance results from intracellular accumulation of glycogen in absence of mucus production. Because of the rarity of this disease, clinical course and treatment options are not well established. Here we describe a case series of 4 patients of clear cell adenocarcinoma and highlight these aspects.

      Methods:
      We reviewed the charts, pathological diagnosis and imaging studies of 4 rare cases of clear cell adenocarcinoma of the lung that were encountered over the past 5 years at the University of Cincinnati Medical Center. Case1: 61 yo F smoker presented with new onset hemoptysis and unintentional weight loss. CT scan revealed 7.2cm lung mass with endobronchial extension. This was followed by biopsy and the pathology revealed poorly differentiated CCLC. She was started on definitive chemoradiotherapy but, just after a month of completing treatment, she developed brain metastasis. Case2: 52 yo F smoker had workup for unintentional weight loss and CT scan revealed 1.4cm spiculated nodule. She underwent Video-assisted thoracoscopic surgery (VATS) and pathology confirmed high grade CCLC without lymphovascular or perineural invasion. She was managed conservatively. Case3: 71 yo F smoker had CT chest which showed a 2.2cm nodule. Because FNA was equivocal she underwent VATS and pathology showed clear cell adenocarcinoma without evidence of metastasis. No adjuvant therapy was offered. Case4: 74 yo F with history of Orthotopic heart transplantation had a CT scan which incidentally showed 4cm nodule which turned out to be an adenocarcinoma on biopsy. She underwent VATS and pathology was consistent with clear cell adenocarcinoma without evidence of infiltration. Her post-op course was complicated by infection and she passed away.

      Results:
      Clear cell features are cytologic changes that occur in association with adenocarcinoma or large cell carcinoma per WHO classification. Our 4 cases were all clear cell adenocarcinoma of the lung confirmed by immunohistochemistry. Immunostains were positive for CAM5.2, TTF-1 and CK7 and negative for CK20, p63, S-100 and PAX-8. Case1 and case2 highlights the lack of established treatment guidelines for CCLC. Currently, same chemotherapy regimens are used as for non-clear cell adenocarcinoma. One study reported KRAS as driver mutation, which, if established, could lead to future therapeutic options. Case3 showed that small biopsies could be insufficient for diagnosis of CCLC. Case4 questions if these lesions could be followed without surgery in high-risk patients to avoid unnecessary morbidity and mortality. Finally, clear cell carcinoma of renal, endometrium and ovarian origin should be considered before making the final diagnosis of CCLC.

      Conclusion:
      CCLC is a rare tumor and more studies are needed to establish management and guidelines. Genetic mutations could be potential therapeutic targets. Risks and benefits of treatment should be considered in high-risk patients.

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