SESSION TYPE: Cancer Case Report Posters II
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Epithelial myoepithelial carcinomas are rare tumors primarily of salivary gland origin. We report a case of a tracheal epithelial myoepithelial tumor incidentally discovered during bronchoscopy performed for suspected sarcoidosis. To our knowledge, this is the first reported case of sarcoidosis associated with epithelial myoepithelial carcinoma.
CASE PRESENTATION: A 61 year old Hispanic male presented with several weeks of altered mental status, weight loss, and malaise. Initial evaluation was notable for hypercalcemia (14.0 mg/dL), slightly elevated parathyroid hormone related protein (34 pg/mL), undetectable parathyroid hormone (<2.5 pg/dL), normal Vitamin D 1,25(OH)2 (27.5 ng/mL), and an elevated angiotensin converting enzyme level (108 U/L). Position emission tomography and computerized tomography revealed diffuse intrathoracic and intra-abdominal lymphadenopathy with mild to moderate (F-18) 2-Fluoro-2-deoxy-D-glucose uptake. His hypercalcemia was treated with normal saline, calcitonin and pamidronate with clinical improvement. On flexible fiberoptic bronchoscopy a 0.7 cm tracheal nodule was biopsied. Pathology demonstrated a tumor composed of glands with a double layer of lining cells. The inner layer was composed of eosinophilic cells. The outer layer was composed of clear cells. Immunohistochemistry demonstrated the inner cell layer was immunoreactive with CK7. The outer cell layer was immunoreactive with SMA and p63. Both cell layers were negative for CK20, TTF-1, PSA, and PSAP. The tumor was classified as an epithelial myoepithelial carcinoma. Bronchoscopy was otherwise non-diagnostic. Lymph nodes sampled by mediastinoscopy demonstrated noncaseating granulomas consistent with stage I sarcoidosis. There was no evidence of malignancy or infection. Three ring tracheal resection with primary reconstruction of the trachea was performed. The patient was clinically stable with a normal calcium level at his one month outpatient follow-up.
DISCUSSION: Sarcoidosis has been associated with a variety of malignancies including lung, breast, lymphoma, germ cell, and melanoma. It is unclear if there is a true relationship or if this is simply an incidental finding. Bronchial epithelial myoepithelial carcinoma is exceedingly rare with 28 reported cases in the literature. Treatment generally entails complete resection with lobectomy or pneumonectomy. In our case, three ring tracheal resection with primary tracheal reconstruction was performed.
CONCLUSIONS: To our knowledge, this is the first report of a pulmonary epithelial myoepithelial carcinoma associated with sarcoidosis. Surgical resection of the malignancy is considered curative for local disease and is generally recommended given its potential for metastatic disease.
1) Chao TY, Lin AS, Lie CH, Chung YH, Lin JW, Lin MC. Bronchial epithelial-myoepithelial carcinoma. Ann Thorac Surg. 2007 Feb;83(2):689-91.
DISCLOSURE: The following authors have nothing to disclose: George Girgis, Jaime Betancourt, Michael Lewis, Robert Cameron, Scott Oh
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