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M.K. Riaz
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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
- Moderators:
- Coordinates: 9/09/2015, 09:30 - 17:00, Exhibit Hall (Hall B+C)
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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 - 09:30 | Author(s): M.K. Riaz
- Abstract
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.