Abstract Leiomyosarcoma is a rare malignancy affecting the lung, often as a metastasis, with the primary tumor arising from the smooth muscles usually in the hollow organs in the intra-abdominal or pelvic region. Primary pulmonary leiomyosarcoma (PPL) is an exceedingly rare neoplasm comprising of 0.2-0.5% of all primary lung malignancies. It typically originates within the pulmonary parenchyma, bronchus, or pulmonary artery, with the intrabronchial subtype occurring in approximately 20% of PPL cases. While the median age for diagnosis is in the 5th - 6th decade of life, it has been reported previously in children. Since it was first reported in 1950’s, there have been 16 pediatric case reports. Presenting symptoms include cough, dyspnea, chest pain or hemoptysis. Diagnosis is often challenging solely based on imaging, cytology or biopsy, usually necessitating an excision biopsy. Based on case reports, surgical resection of the tumor has been associated with an 87.5% survival rate while improved survival was reported when adjunct chemotherapy was used in a patient with incomplete tumor resection. Recent studies suggest prognosis was related to grade, size, distant stage and surgical resection, emphasizing the importance of early detection. We report and explore a unique case of a 16-year-old female who presented with trivial symptoms of 2 days of cough and fever with an abnormal chest radiograph diagnosed with COVID-19 and discharged home. Persistent symptoms prompted referral to our pulmonary service for additional workup. Further history suggested she had exertional dyspnea and pleuritic chest pain. Chest computed tomography showed a 6x5 cm endobronchial mass with associated left upper lobe collapse. Bronchoscopic evaluation revealed a fungating mass at the take-off of the left upper lobe bronchus and bronchoalveolar lavage showed a negative cytology and inconclusive histology. Further molecular and genetic testing identified a rare mutation, which has been associated with other neoplastic conditions. A Positron emission tomography scan demonstrated a left upper lung para-mediastinal mass with moderate hypermetabolism with mild uptake seen in one left prevascular lymphonde, but no other regions of uptake. She underwent left-sided complete pneumonectomy, and the final pathology confirmed the diagnosis of endobronchial leiomyosarcoma. Based on guidelines for treatment of non-rhabdomyosarcoma soft tissue sarcoma, she started chemotherapy with Ifosfamide/doxorubicin and has been doing well. We aim to present this rare case to alert pediatricians to the utilization of a multidisciplinary approach along with advanced diagnostic techniques to facilitate early diagnosis of rare diseases in traditional presentations with abnormal chest radiograph. This abstract is funded by: None
Dayalan et al. (Fri,) studied this question.