Persistent cough with left lower lobe infiltrate in a patient with history of granular cell tumors

SESSION TITLE: The Airway Under a Microscope SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: GCT are rare and can occur in almost any organ. We report here a unique case of multifocal recurrent benign granular cell tumor (GCT) found in the bronchi of the lung. CASE PRESENTATION: 42-year-old Black female presented with persistent non-productive cough for five months. She had a history of recurring granular cell tumor (GCT) at multiple sites (tongue, abdominal wall, left hip) which were all surgically resected within the last 18 years. The patient had received several rounds of antibiotics, bronchodilator therapy and corticosteroids without relief. Chest radiograph showed left lower lobe opacities. A non-contrast computerized tomography (CT) scan of the chest revealed a nodular soft tissue lesion in the left lower lobe (LLL) bronchus along with consolidation of the LLL concerning for post obstructive pneumonia. Bronchoscopy demonstrated LLL endobronchial mass completely occluding the LLL bronchus. The lesion was debrided with cryotherapy and argon plasma coagulation. Pathology of the tumor showed proliferating sheets of eosinophilic granular cells with indistinct cell border and syncytial growth pattern. Immunostaining with CD 68 and 100 was diffusely positive while negative for desmin. PAS and PASD show positive cytoplasmic granules. These findings were consistent with benign granular cell tumor. DISCUSSION: GCT are mostly benign tumors. They may occur synchronously in multiple organs or metachronous in a single organ. Our patient has a rare presentation of GCT with multifocal occurrences but never having a recurrence in the same organ. Incidence of GCT affecting the tracheobronchial tree is unknown, and only about one hundred cases have been reported. GCT originates from Schwann cells and are distinguished based on histological features. These commonly present between the fourth to sixth decade with no specific sex predilection. African Americans appear to be more commonly affected. Ninety percent of pulmonary GCT are endobronchial. Symptoms range depending on the size of the tumor and its location. Patients may present with chest pain, cough, dyspnea, hemoptysis, or stridor. The chest x-ray may show normal findings, or it can show signs of pneumonia, atelectasis, mucoid impaction, and bronchiectasis as well as apparent tumor. CT usually shows a soft tissue mass located in the endobronchial region. Definite diagnosis of PGCT is established by pathology. Most common diagnostic procedures include fine needle aspiration, bronchial brushing, and endobronchial biopsy. Treatment options for benign GCT are either conservative treatment or surgical resection depending on the size of the tumor. Surgical resection is curative, but tumor recurrence has been reported. Follow up once a year for a minimum of 5 years is recommended. For the rare malignant variant of GCT, more aggressive and uniform clinical treatments are needed as chemotherapy and radiotherapy treatments are not expected to be effective, with surgical resection being the primary option. CONCLUSIONS: Pulmonary GCT is an extremely rare tumor with predilection for endobronchial disease. Intervention is determined by size and symptoms, and resection yields good prognosis. Close follow up for recurrence is advised. REFERENCE #1: 1. K. R, "Myoblastoma of the bronchus," vol. 48, ed: Ann Otol Rhinol Lawngol, 1939, pp. 1083-1086 REFERENCE #2: 2. Goel G., Singh N., Gupta R., Jain S. Recurrent benign cutaneous granular cell tumor: A case report. Journal of Cytology. 2013, October;30(4):287–288. doi: 10.4103/0970-9371.126675. REFERENCE #3: 3. M. A. Meyer J. M. Becker W. Quinones Endobronchial granular cell tumor: a case report. J. Radiol. Case Rep. Retrieved November 3, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3303395/#b8-jrcr-4-8-29. DISCLOSURES: No relevant relationships by Kha Dinh No relevant relationships by Pushan Jani No relevant relationships by Khalid Mohamed Ahmed No relevant relationships by Bilal Zafar