"Spin the Wheel to Spindle": A Rare Case of Inflammatory Myofibroblastic Tumor.

SESSION TITLE: Bronchology/Interventional Procedures Cases IISESSION TYPE: Affiliate Case Report SlidePRESENTED ON: Monday, October 27, 2014 at 11:00 AM - 12:00 PMINTRODUCTION: Inflammatory Myofibroblastic tumors (IMT) were previously included in the loose term "Inflammatory Pseudotumor." However, the rarity of these tumors makes diagnosis and management fairly challenging. We present an interesting presentation of an endobronchial presentation of IMT.CASE PRESENTATION: An 18-year-old non-smoking female presented with cough and intermittent hemoptysis over a few weeks. CT chest ruled out a pulmonary embolism but revealed an endobronchial lesion at the takeoff of the right mainstem bronchus (figure I). An endobronchial carcinoid seemed to be the most obvious diagnosis and rigid bronchosocpy for diagnosis and tumor debulking was scheduled. A non-obstructing medium sized exophytic mass was found at the distal trachea extending down the RMS bronchus (figure II). Endobronchial biopsies were taken and a station 7 lymph node was sampled. On cytology, the endobronchial needle aspiration from the mass showed sheets of spindle cells interspersed with inflammatory infiltrate consisting of lymphocytes. The histopathologic tissue biopsy revealed neoplastic spindle cells with abundant eosinophilic cytoplasm and low grade cytologic atypia. This was diffusely positive ALK-1, weakly positive AE1/AE3, but negative for EWSR1 supporting the diagnosis of an inflammatory myofibroblastic tumor. With the lack of data and highly variable clinical course, a PET/CT and MRI brain1 were done in preparation for a surgical cure. The patient had a right thoracotomy and distal tracheal resection using a biologic prosthesis and intercostal muscle flap. Her post-operative course was uncomplicated.DISCUSSION: IMTs are rare, and the course of theses tumors is highly variable. Morphologically, these tumors are similar to gastro-intestinal stromal tumors (GIST) which benefit from imatinib given the c-kit positivity. The benefit of chemotherapy in ALK positive IMTs remains to be defined and therefore surgical cure, when possible, is recommended.2CONCLUSIONS: IMT is a clinical rarity that is important to pathologically distinguish and differentiate from malignant neoplasms such as spindle cell carcinoma and fibrosarcoma. Treatment must be tailored to the location of tumor and prevention of tumor recurrence.Reference #1: Siminovich M, Pediatr Dev Pathol. 2012;15:179-86.Reference #2: Thistlethwaite P, et al. Ann Thor Surg. 2011;91:367-72.DISCLOSURE: The following authors have nothing to disclose: Tanmay Panchabhai, Debabrata Bandyopadhyay, Siva Raja, Sudhish Murthy, Vamsidhar Velcheti, Sanjay Mukhopadhyay, Joseph Cicenia, Sonali SethiNo Product/Research Disclosure Information.