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Tracheobronchial Airway Necrosis: An Atypical Presentation of Recurrent Osteosarcoma

Tunsupon, Pichapong MD; Harris, Kassem MD, FCCP; Bower, Jessie MBA, CT, MB (ASCP); Alraiyes, Abdul Hamid MD, FCCP

Journal of Bronchology & Interventional Pulmonology: January 2017 - Volume 24 - Issue 1 - p 59–62
doi: 10.1097/LBR.0000000000000327
Images in Interventional Pulmonology

*Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University at Buffalo

Roswell Park Cancer Institute, Buffalo, NY

P.T., K.H., J.B., and A.H.A. prepared the manuscript or revised it critically for important intellectual content.

Disclosure: There is no conflict of interest or other disclosures.

Reprints: Pichapong Tunsupon, MD, Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University at Buffalo, Western New York Veterans Administration Healthcare System, 3495 Bailey Avenue, Buffalo, NY 14215 (e-mail:

Received March 6, 2016

Accepted July 26, 2016

Osteosarcoma is the most common primary bone malignancy that has a bimodal age distribution. The overall incidence rate in adolescent and childhood is approximately 4 to 5 cases per year per million.1 The first peak occurs in adolescents from 10 to 14 years of age, and the second occurs in adulthood older than 65 years of age, predominantly in male sex.1 Recurrent osteosarcoma occurs approximately in 30% to 40% of patients initially diagnosed with nonmetastatic disease, despite surgical treatment and the advent of novel chemotherapeutic agents.2 The incidence of intrathoracic metastasis is higher than extrathoracic metastasis. The most common site of recurrent osteosarcoma is the lung, where it typically presents as calcified pulmonary nodules. Atypical manifestations of intrathoracic osteosarcoma such as mediastinal lymph nodes metastasis, esophagomediastinal fistula, lymphangitic carcinomatosis, pulmonary artery tumor emboli, and ribs metastasis have been reported in the literature.3 The endobronchial invasion as a consequence of recurrent osteosarcoma is a rare condition. We herein report a unique manifestation of chemotherapy-resistant recurrent osteosarcoma involving the mediastina lymph node, causing extensive endotracheal necrosis.

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A 47-year-old man was referred for evaluation of intermittent hemoptysis. His past medical history was significant for the left distal femur osteosarcoma treated with left above-knee amputation. He completed a course of adjuvant Adriamycin, Cisplatin chemotherapy alternating with high-dose Methotrexate 5 years earlier. During the posttreatment surveillance period, serial computed tomography of the chest demonstrated a new right upper lobe mass and right paratracheal lymphadenopathy. Cytologic examination of the right paratracheal lymph node obtained by endobronchial ultrasound–guided transbronchial needle aspiration (EBUS-TBNA) was consistent with recurrent osteosarcoma. Although he received treatment with 2 courses of salvaged chemotherapy, Gemcitabine/Docetaxel and Ifosfamide/Etoposide, he reported intermittent hemoptysis in the amount of a teaspoon, 2 to 3 times per day. Physical examination revealed audible bilateral expiratory wheeze. Follow-up chest computed tomography revealed progression of the mediastinal tumor compressing the main carina, pulmonary artery, superior vena cava, and bilateral main stem bronchi (Fig. 1). Osteosarcoma is not traditionally responsive to radiation therapy; in addition, the involvement of the pericardium and major blood vessels precluded palliative radiation. Flexible bronchoscopy was performed to evaluate the new-onset intermittent hemoptysis that demonstrated an extensive endotracheal necrosis involving the distal trachea, carina, and both main stem bronchi (Fig. A). Y-silicone stent deployment was a possible treatment option even though the carina was anatomically distorted. However, in the absence of tracheobronchial wall and extensive mucosa necrosis, the deployment of any type of airway stents was not possible. The placement of a stent in such cases could lead to migration of the stent in to the mediastinum and result in airway perforation or life-threatening hemorrhage. Thus, our clinical judgment did not justify the deployment of the airway stent. The patient was referred to hospice for conservative management. He passed away a few weeks later comfortably.





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Eighty percent of patients after complete surgical resection of the primary osteosarcoma develop recurrent metastatic disease due to subtle hematogenous metastasis. Novel chemotherapeutic agents help prevent recurrent disease from subclinical hematogenous spread. The 5-year relapse-free survival rate is approximately 60% to 65% in patients with diagnosis of primary osteosarcoma that is treated with presurgical (neoadjuvant) or postsurgical (adjuvant) chemotherapy.4 Our patient developed recurrent metastatic disease 5 years postsurgical and adjuvant chemotherapy.

Intrathoracic metastatic osteosarcoma could present with various radiologic manifestations.3 The incidence of osteosarcoma metastasis to the regional lymph nodes is <3% and portends poor prognosis.5 The diagnosis of recurrent disease in the mediastinum in our case is confirmed by the presence of atypical spindle cells and collagenous matrix obtained from the EBUS-FNA of the right paratracheal lymph nodes that match the histology of the left femur osteosarcoma diagnosed 5 years earlier (Fig. 2). The recurrent disease is apparently not responsive to salvage chemotherapy because the mediastinal lymph nodes expand and mechanically compress the central airway leading to extensive necrosis of the tracheal bifurcation. This is a rare circumstance of circumferential necrosis as a result of recurrent osteosarcoma in the mediastinum. An obstruction of the blood flow leading to coagulation necrosis in the center of rapidly growing tumors has been described in the literature.6 We speculate that the enlarging recurrent osteosarcoma in the mediastinum mechanically damages the cartilaginous rings, and induces central hypoperfusion or infraction at the tracheal bifurcation located in the center of the growing tumor.



A case of tracheomediastinal fistula from a large-cell lymphoma causing total destruction of the carina that was successfully treated with self-expanding metallic stents to maintain the structural integrity and promote granulation tissue formation while being treated with chemotherapy has been reported.7 We did not justify the deployment of self-expanding metallic stents because the recurrent disease was progressing despite treatment with salvage chemotherapy. The deployment of tracheal or bronchial silicone stent, such as the Y-shaped tracheal stent, was not feasible given the destruction of the cartilaginous tracheobronchial walls with extensive necrosis and loss of structural integrity.7

It is important for pulmonologists to recognize the possible differential etiologies of endotracheal necrosis. Infections that potentially can cause endobronchial necrosis in immunocompromised patients are Aspergillus fumigatus 8 and Mycobacterium tuberculosis infection.9 Case series of endobronchial necrosis in squamous cell carcinoma of the lung treated with external-beam radiation (5000to 6400 Gy) as well as radiation-induced stump necrosis in postlobectomy or postpneumonectomy cases have been described in the literature.8,10 Bevacizumab (Avastin) is an antiangiogenesis monoclonal antibody indicated in patients with advanced cancer such as non–small cell lung cancer, metastatic colorectal cancer, renal cell carcinoma, breast cancer, ovarian cancer, or primary peritoneal cancer.11 The main side effects of Bevacizumab are bleeding (hemoptysis, gastrointestinal bleeding, intracranial hemorrhage), gastrointestinal perforations, delayed surgical wound healing, and wound dehiscence. Hemoptysis could occur as a consequence of endobronchial and vascular necrosis after treatment with antiangiogenesis chemotherapy, which was precluded in our case. In lung transplant recipients, donor endobronchial ischemia and necrosis is a known complication secondary to the absence of bronchial artery revascularization. The endobronchial mucosa viability relies on the retrograde blood flow from the poorly oxygenated pulmonary circulation.12 The bronchial artery collateral circulation takes up to 4 weeks after the lung transplant. Therefore, during this time period there is a considerable risk of airway ischemia and necrosis beyond the anastomotic area.13,14

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Hemoptysis secondary to endotracheal necrosis in recurrent osteosarcoma is a rare clinical entity. Pulmonologists should be cognizant of the pathophysiology of central necrosis in a rapidly growing malignant tumor, and other etiologies including iatrogenic complications that potentially lead to circumferential endotracheal necrosis. The deployment of stent in the absence of cartilages to maintain the structural integrity of the main carina is considered a relative contraindication in such case because of the greater risk of stents migration into the mediastinum.

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recurrent osteosarcoma; mediastinal lymph node metastasis; central airway necrosis; endotracheal bifurcation necrosis

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