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CASE REPORT HUANG ET AL PEA FOR CTEPH FROM SEPTIC EMBOLI

A Malignant Glomus Tumor in the Upper Trachea Cheng Huang, MD,* Qiao-fei Liu, MD,* Xing-ming Chen, MD, Li Li, MD, Zhi-jun Han, MD, Xiao-yun Zhou, MD, Lei Liu, MD, and Shan-qing Li, MD Thoracic Surgery Department and Otorhinolaryngological Surgery Department, Peking Union Medical College Hospital, Chinese Academy of Medicine, Beijing, China

Malignant glomus tumors are extremely rare, and a malignant glomus tumor in the trachea has not been described previously. In this report, we present the first known case of a malignant glomus tumor originating in the trachea. (Ann Thorac Surg 2015;99:1812–4) Ó 2015 by The Society of Thoracic Surgeons

FEATURE ARTICLES

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lomus tumors account for less than 2% of all soft tissue tumors, and less than 1% of glomus tumors are malignant [1]. Approximately 30 cases of glomus tumors in the trachea or bronchi have been reported, most of which were benign, with the exception of one with uncertain malignant potential [2, 3]. To our knowledge, no malignant glomus tumors originating in the trachea have previously been reported. Documented accounts of the treatment of malignant glomus tumors are limited, and therefore, this report will add to our knowledge. A 39-year-old woman was admitted to our hospital because of dyspnea and audible stridor. A high-resolution computed tomography scan revealed a 2.3-  1.0-cm mass on the left posterior wall of the upper trachea. The mass protruded into the tracheal lumen and most likely invaded the adjacent esophagus (Fig 1A). The mass was significantly enhanced on an intravenous contrast computed tomography scan (Fig 1B). Under general anesthesia, flexible bronchoscopy was performed, and a fleshy mass was found approximately 5 cm inferior to the vocal cords. It originated from the left posterior membranous portion of the trachea and occupied more than 80% of the tracheal lumen (Fig 1C). A cervical collar incision was performed. After mobilization of the diseased trachea, the tumor was found to invade through the tracheal wall and adhere to the esophagus. A 4-cm-long tracheal resection and end-to-end tracheal anastomosis were performed. Both recurrent laryngeal nerves were well

Accepted for publication May 27, 2014. *Drs Huang and Liu contributed equally and are both first authors. Address correspondence to Dr SQ Li, Thoracic Surgery Department, Peking Union Medical College Hospital, Chinese Academy of Medicine, Beijing, China, 100730; e-mail: [email protected].

Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier

Ann Thorac Surg 2015;99:1812–4

protected. The fresh tumor measured 2.5  1.2 cm in diameter. It was an R0 resection with a microscopically negative margin. Histologically, the tumor was composed of groups of small, uniform, rounded cells with centrally placed round nuclei and amphophilic to lightly eosinophilic cytoplasm. The tumor was rich in small blood vessels, and the tumor cells grew invasively, invading the membranous wall and reaching the adventitia of the esophagus. Mild nuclear atypia and mitotic activity were present, and the Ki-67 index was 2% (Figs 2A, 2B). However, atypical mitotic figures and marked nuclear atypia were absent. Immunohistochemically, the tumor was positive for smooth muscle actin, desmin, and Syn and negative for AE1/AE3, CD56 (NK-1), CK34be, CgA, HMB45, S-100, and p63 (Figs 2C–2E). The patient recovered well, and the postoperative course was uneventful. One week after operation, the patient was discharged. Before discharge, another highresolution computed tomography showed no stenosis or leakage from the reconstructed trachea. During the 1-month follow-up, the patient had no medical complaints.

Comment In 2001, Folpoe and colleagues [4] proposed a new set of criteria for malignant glomus tumors: (1) size greater than 2 cm with a subfascial or visceral location, (2) atypical mitotic figures, or (3) marked nuclear atypia and any level of mitotic activity. In this case, the tumor grew invasively, invaded through the membranous wall, and reached the adjacent adventitia of the esophagus. Its fresh size was 2.5  1.2 cm in diameter, and mild nuclear atypia and low levels of mitotic activity were present. Thus, it reached the diagnostic standard of a malignant glomus tumor. In the largest previous study [4], immunohistochemistry showed that all of the cases expressed smooth muscle actin, vimentin, and collagen type IV, 19% expressed CD34, 6% expressed desmin, rare cases expressed Syn, and no cases expressed S-100 or cytokeratins. In this case, the tumor cells diffusely expressed smooth muscle actin, densely expressed desmin, sparsely expressed Syn, and did not express CD34, S-100, or cytokeratins. Metastasis is the main cause of death owing to malignant glomus tumors. Before 2001, the literature reported that of the 21 malignant glomus tumors described, 7 underwent metastasis [4]. After 2001, 16 cases of malignant glomus tumors were reported, 5 of which metastasized. Many of the malignant glomus tumors were found to be metastatic at the time of the initial diagnosis. Metastasis usually occurred 3 to 4 years after radical resection [4]. Whether adjunctive radiochemotherapy could reduce the risk of metastasis or recurrence is less clear because related reports are rare. For squamous cancer and adenoid cystic carcinoma in the trachea, routine adjunctive radiochemotherapy is not recommended after radical resection, but for palliative resection, adjunctive radiochemotherapy will benefit the patients 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.05.102

Ann Thorac Surg 2015;99:1812–4

CASE REPORT HUANG ET AL PEA FOR CTEPH FROM SEPTIC EMBOLI

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Fig 1. (A) The sagittal plane of a computed tomography scan indicating a soft tissue mass (arrow) originating in the upper trachea. (B) Intravenous contrast computed tomography scan revealing the significant enhancement of the mass (arrow). (C) Flexible bronchoscopy indicating a reddish mass (arrow) occupying greater than 80% of the tracheal lumen.

[5]. In the present case, the tumor was R0 resected, and because atypical mitotic figures and marked nuclear atypia were absent, the risk of metastasis was theoretically relatively low. Meanwhile, cervical radiochemotherapy may cause stenosis of the reconstructed trachea and fibrosis of the cervical

muscles, which would significantly reduce quality of life. Moreover, radiotherapy may increase the risks of thyroid cancer and lymphoma. After we explained the situations and risks, the patient decided not to receive the adjunctive radiochemotherapy. We will continue to follow up with the patient. FEATURE ARTICLES

Fig 2. The pathologic and immunohistochemical characteristics of the tissue mass. (A) Mild nuclear atypia and mitotic activity were present (hematoxylin and eosin staining, 200). (B) The Ki-67 index was 2% (100). Immunohistochemically, the smooth muscle actin (C) was diffusely positive, the desmin (D) was densely positive, and the intracellular CD34 (E) was negative (all 100).

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CASE REPORT CROSLAND ET AL PEA FOR CTEPH FROM SEPTIC EMBOLI

We express our deep thanks to Dr Cui Quan-Cai and Dr Shi Jie of the Pathological Department of Peking Union Medical College Hospital for their extraordinary pathological work.

References 1. Zhang Y, Li H, Zhang WQ. Malignant glomus tumor of the esophagus with mediastinal lymph node metastases. Ann Thorac Surg 2013;96:1464–6. 2. Zhu YZ, Li WP, Wang ZY, et al. Glomus tumor of uncertain malignant potential arising in the bronchus. J Cardiothorac Surg 2013;8:146. 3. Urdaneta AI, Yu JB, Wilson LD. Population based cancer registry analysis of primary tracheal carcinoma. Am J Clin Oncol 2011;34:32–7. 4. Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25:1–12. 5. Honings J, van Dijck JA, Verhagen AF, van der Heijden HF, Marres HA. Incidence and treatment of tracheal cancer: a nationwide study in the Netherlands. Ann Surg Oncol 2007;14: 968–76.

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Pulmonary Endarterectomy for Pulmonary Hypertension From Septic Emboli William Crosland, MD, Mustafa Ahmed, MD, Julian Booker, MD, Keith Wille, MD, Satinder Singh, MD, FCCP, Thomas Winokur, MD, and David McGiffin, MBBS, FRACS Departments of Medicine, Radiology, and Pathology, The University of Alabama at Birmingham, Birmingham, Alabama; and Department of Cardiothoracic Surgery and Transplantation, The Alfred Hospital and Monash University, Melbourne, Victoria, Australia

Pulmonary endarterectomy (PEA) can significantly increase long-term survival in patients with chronic thromboembolic pulmonary hypertension; however, the role of PEA for chronic thromboembolic pulmonary hypertension due to pulmonary valve endocarditis is controversial. A critically ill 61-year-old man with intractable right ventricular heart failure was found to have chronic thromboembolic pulmonary hypertension due to Streptococcus bovis pulmonary valve endocarditis and underwent successful pulmonary valve replacement and PEA. The successful outcome in this case suggests that PEA should be considered in patients with this condition. (Ann Thorac Surg 2015;99:1814–6) Ó 2015 by The Society of Thoracic Surgeons

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hronic thromboembolic pulmonary hypertension (CTEPH) develops in 3.8% of all patients diagnosed with a pulmonary embolus [1]. This is a chronic progressive Accepted for publication June 11, 2014. Address correspondence to Mr McGiffin, The Alfred Hospital, 55 Commercial Rd, Melbourne VIC 3004 Australia; e-mail: d.mcgiffin@alfred. org.au.

Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier

Ann Thorac Surg 2015;99:1814–6

disease characterized by fibrotic stenosis of the pulmonary vasculature, remodeling of the unobstructed distal pulmonary arteries (PAs), and subsequent right-sided heart failure [1]. Medical therapy is largely ineffective. Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH because surgical intervention has clearly demonstrated substantial survival benefit over the natural history of the disease [2–5]. CTEPH due to rightsided infective endocarditis typically occurs in chronically debilitated patients with protracted hospital courses, and surgical intervention is inherently high risk. Right-sided endocarditis predominantly involves the tricuspid valve in more than 80% of cases, typically due to intravenous drug use or indwelling catheter placement [6]. The incidence of septic embolism from tricuspid or pulmonary valve endocarditis is largely unknown, but it seems the systolic shear stress that develops across the infected pulmonary valve could increase the risk of septic emboli. The benefit of PEA for CTEPH due to right-sided infective endocarditis is unclear, with some opinion recommending against the procedure [7]. We present a case from the University of Alabama at Birmingham of a patient with CTEPH due to septic embolization from pulmonary valve endocarditis who underwent successful combined pulmonary valve replacement and PEA [3–4]. A 61-year-old man presented to the University of Alabama at Birmingham for management of bilateral pulmonary thromboemboli associated with pulmonary valve vegetations. The patient had had multiple admissions for pneumonia during the past year. Streptococcus bovis bacteremia had developed 3 months before presentation. The patient was critically ill with intractable right ventricular heart failure. His clinical condition remained unresponsive to multiple administrations of tissue plasminogen activator, and his fever and leukocytosis persisted despite prolonged antibiotic therapy. Echocardiography on admission noted a severely dilated right ventricle with reduced systolic function and pulmonary valve vegetations with severe pulmonary valvular regurgitation (Fig 1). Three-dimensional computed tomography revealed a severely dilated PA, complete occlusion of the right PA, and multiple occlusive defects within the left lung, including the left lower lobe segmental, left superior lingular, and left inferior lingular arteries (Fig 2). Right heart catheterization noted elevated right atrial pressures of 22/13 (mean 19) mm Hg and elevated right ventricular pressures of 56/12 (mean 22) mm Hg. The PA was not entered due to concern for further embolization and was measured directly in the operating room, noting elevated PA pressures of 53/18 (30) mm Hg. PEA was performed through a median sternotomy using the usual methods of cardiopulmonary bypass and myocardial protection. Deep hypothermia to 20 C and circulatory arrest facilitated visualization during the endarterectomy. Cerebral protection was augmented by intermittent cerebral perfusion. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.06.109

A malignant glomus tumor in the upper trachea.

Malignant glomus tumors are extremely rare, and a malignant glomus tumor in the trachea has not been described previously. In this report, we present ...
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