SESSION TITLE: Metastatic and Other Primary Lung Tumors SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: In 2007, Chang et al., described the first case reports of pneumocytic adenomyoepithelioma. We present a case of pneumocytic adenomyoepithelioma and describe the available literature regarding these rare tumors. CASE PRESENTATION: A 63 year-old female was seen for evaluation of bilateral pulmonary nodules. She was a lifelong nonsmoker and did not have any known occupational exposures. She had also been seen in our clinic eight years prior for bilateral pulmonary nodules which were felt to be benign. CT imaging in 2016 demonstrated a dominant slowly growing 14 mm right apical nodule. Bronchoscopy revealed an endobronchial lesion in the right upper lobe. Biopsy revealed a low-grade spindle cell neoplasm. Biopsied lymph nodes were negative for malignancy or infection. Her PET scan showed active hilar nodes. Video-assisted thoracoscopy with right upper lobectomy and lymph node dissection was pursued. Her pathology report revealed a 14 mm pneumocytic adenomyoepithelioma with an inner epithelial cell layer, which was surfactant A positive, alongside surrounding spindle cells which stained positive for mesenchymal markers. On follow-up imaging 2 months later, her bilateral pulmonary nodules were stable without evidence of interval change and she felt well overall. DISCUSSION: In 2007, Chang et al., described a new subset of 5 adenomyoepitheliomas of pneumocyte origin, which they termed pneumocytic adenomyoepithelioma1.Since this initial report, only one additional report has been described2. The initial report by Chang et al., encompassed cases from five hospitals, and only 3 had data regarding their presentation. Of these patients, 2 presented with incidentally discovered lung nodules and the third presented with pleuritic chest pain. In one subsequent case, the patient presented with a history of reported recurrent submandibular pleomorphic adenoma. All of these patients were female between 52-63 years of age and lesions ranging from 0.8-2.6 cm in size. The clinical course of the patients by Chang et al., was largely favorable without evidence of recurrence 13-78 months later. Our patient was unusual in that we had imaging data from 8 years prior, suggesting an indolent clinical course. Her long-term prognosis is likely quite good, but the drivers behind this unique and extraordinarily rare tumor have yet to be elucidated. CONCLUSIONS: This rare and poorly described lesion appears to portend to favorable clinical outcomes, but with only a very small subset of patients being described. Future studies are needed in order to better characterize and understand this unusual pulmonary tumor. Reference #1: Chang T, Husain AN, Colby T, et al. Pneumocytic adenomyoepithelioma: A distinctive lung tumor with epithelial, myoepithelial, and pneumocytic differentiation. Am J Surg Pathol 2007;31:562-8. Reference #2: Krishnamurthy A, Vaidhyanathan A, Majhi U. Pneumocytic adenomyoepithelioma in a case of myoepithelial carcinoma of the submandibular gland. Indian J Cancer 2015;52:216-U188. DISCLOSURE: The following authors have nothing to disclose: Brandon Nokes, Anjuli Brighton, Staci Beamer, Brandon Larsen, Prasad Panse, Rodrigo Cartin-Ceba, Karen Swanson No Product/Research Disclosure Information
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