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Case report
DOI: 10.1016/j.bjorl.2021.05.011
Open Access
Available online 17 June 2021
Tracheal glomus tumor misdiagnosed as pulmonary disease: a case report and literature review
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Miao Gao, Sheng-Nan Ye, Chang Lin, Yuan-Teng Xu
Corresponding author
xytent123@163.com

Corresponding author.
Fujian Otorhinolaryngology Institute, The First Affiliated Hospital of Fujian Medical University, Department of Otorhinolaryngology Head and Neck Surgery, Fujian, PR China
Received 11 February 2021. Accepted 22 May 2021
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Table 1. Summary of literature of tracheal glomus tumor.
Table 2. Different parts of the treatment.
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Introduction

Glomus tumors (GTs) are benign tumors formed at the anastomosis of arteries and veins, affecting blood flow and temperature control. The tumor can occur in any part of the body. Owing to little data concerning tracheal glomus tumors (TGTs) of uncertain malignant potential, more accumulated cases are required to clarify its characteristics. In this report, we also review relevant literature and discuss the common features and treatments of GTs.

Case report

A 53-year-old female presented with a one-year history of cough and intermittent shortness of breath without hemoptysis or smoking history. She was admitted to the Respiratory Department due to an initial diagnosis of chronic obstructive pulmonary disease (COPD). No improvement in shortness of breath was observed after receiving long-term treatment for spasm and asthma. Spiral computed tomography (CT) with three-dimensional reconstructions in conjunction with tracheal CT enhancement scan showed enhanced tissue in the initial segment of the trachea, measuring approximately 1.4 × 0.9 × 1.4 cm (Fig. 1). A polypoid mass under the glottis almost completely blocked the lumen and moved in tandem with the patient’s breathing. The patient was referred to Otolaryngology-Head and Neck Surgery Department subsequently. To ensure the airway remained unobstructed, we performed a temporary tracheotomy. During the operation, the tumor of the subglottic area, arising from the posterior wall of the trachea was detected (Fig. 2A). The pedicle was resected, and the residual was completely cauterized with the help of Harmonic scalpel.

Figure 1.

Trachea spiral CT shown a tracheal mass resulting in a 90% obstruction of trachea lumen.

(0.13MB).
Figure 2.

(A) The mass seen during the operation. (B) The histopathology showed well-circumscribed and solid lesion, slightly eosinophilic cytoplasm surrounded by dilated capillary-sized vessels, the cells were without obvious mitotic phase.

(0.66MB).

Postoperative pathology (Fig. 2B) revealed focal eosinophilic round cells arranged like hemangiopericytoma around vessels. These cells were positive for vimentin and smooth muscle actin antibody (SMA), synaptophysin (Syn), CD56, S-100, and the Ki67 proliferation index was around 5%. Cells were negative for chromogranin-A (Cg-A), casein kinase (CK), CD34 and epithelial membrane antigen (EMA). Two weeks after the operation, a tracheal CT scan and electronic laryngoscopy were performed, demonstrating healing and scarring. The patient recovered well, and the tracheostomy tube was removed. After one year, hemoptysis occurred, and the tumor was found to have recurred (Fig. 3), expanded upward along the midline of the original tracheotomy position. The tumor and the partial tracheal sleeve resection were subsequently carried out. The patient recovered quickly after the resection and the patient is continually being monitored (Fig. 4). This study was performed under the approval of the ethics committee of our hospital (nº IEC-FOM-013-2.0).

Figure 3.

The tumor recurred in situ one year later.

(0.19MB).
Figure 4.

Appearance of trachea 3 month after second surgery on electronic laryngoscope.

(0.17MB).
Discussion

Glomus tumors are rare soft tissue neoplasms derived from modified smooth muscle cells.1 GTs were first described by Masson in 1924. The first case was reported in 1950.2 Based on the World Health Organization (WHO) classification of tumors, GTs are commonly classified into three groups including benign GTs, GTs of uncertain malignant potential, and malignant GTs.3 According to the recent WHO classification, the criteria for tumors of uncertain malignant potential is contingent on not fulfilling the criteria for malignancy, and additionally exhibiting at least one atypical feature other than nuclear pleomorphism.4 However, GTs > 2 cm in size with a deep location which were previously diagnosed as malignant are now classified as having uncertain malignant potential.3 In the present case, the tumor was large enough but did not show significant mitotic characteristics or nuclear atypia, thus the diagnosis of uncertain malignant potential.

Clinically, symptoms associated with airway irritation are common in bronchial and tracheal GTs, but asymptomatic GT usually occurs in the peripheral pulmonary parenchyma.5 Depending on the pathology, the main differential diagnoses of tracheal glomus tumor are carcinoid tumor and hemangiopericytoma. CT scan and bronchoscopy are the best diagnostic methods for determining the origin of the tumor currently. However, the complete diagnosis depends on the results of a pathological examination. Due to their abundant vessels, tracheal glomus tumors (TGTs) display an obvious enhanced area in CT images.6 Despite this, it is difficult to distinguish GTs from carcinoid or hemangiomas solely based on radiologic findings as they appear to have a well-circumscribed round mass under contrast enhancement.7 Thus, the identification of the cytological and vascular structural characteristics are particularly important for an accurate classification.

While there is currently no consensus on the treatment of TGTs. Masoum et al.8 reported a case of a 21-year-old patient who underwent GT resection via bronchoscopy, which recurred one year later, followed by subsequent open resection. Jin et al.5 preferred open surgery due to the young age of the patient and the sizable nature of the lesion. In that case, the risk of bleeding and high recurrence was reduced. Recently, Suresh et al.9 introduced a novel treatment for thoracic tracheal GTs, named percutaneous trans-tracheal endoscopic approach (PTEA). The procedure has many obvious advantages in the resection of benign lumen lesions of the lower trachea, it is an easy and better controlled, simple, and less morbid procedure, but further studies are needed to determine the practicability and safety of the method. Hartert et al.10 described a patient who was treated by tracheal sleeve resection via a right posterolateral thoracotomy with end-to-end anastomosis, citing a 96 months followup period without recurrence. Sleeve resection with primary reconstruction of the trachea is the treatment of choice for tracheal glomus tumor.1 So in the second operation, we adopted this approach. The patient recovered well.

To the best of our knowledge, there are 77 published articles regarding GT in the English language medical literature (Table 1), most of the relevant articles being case reports. The most common symptoms reported among symptomatic GT patients are cough 54.55% (42/77), dyspnea 54.55% (42/77) and hemoptysis 44.16% (34/77). Patients with a lower frequency of chest pain (7.9%) were more rarely reported and presented chest tightness, fever, and asthma-like symptoms. Only five patients were reported to be asymptomatic. The locations of TGTs were of superior origin in 20.78% (16/77) of cases, middle in 23.38% of cases (18/77), inferior in 35.06% of cases (27/77), and bronchial in 20.78% of cases (16/77) (Table 2). These distributions suggest that incidences of GT in the lower two-thirds of the trachea are commonplace, perhaps due to the numerous mucous glands and vessels. Most of the published GT cases are benign, with only two cases of uncertain malignant potential previously reported.5 The tumors in these cases were located at the lower third of the trachea or bronchus, both removed by open surgery with no recurrence after two-year follow-up. Except for the location of the bronchial section, a majority of tumors were surgically removed. Only four bronchial GT patients underwent open surgery. Meanwhile, two patients underwent partial or total lung resection. A few patients accepted adjuvant treatments after surgery, including radiochemotherapy, cryotherapy, Nd-YAG laser and argon plasma coagulation (Table 2).

Table 1.

Summary of literature of tracheal glomus tumor.

First author  Year  Age (years)  Sex  Symptoms  Duration of symptoms before treatment  Tumor site (S/M/I/B)  Size (cm)  Treatment  Follow-up 
Hussarek  1950  43  Dyspnea  Not stated  Bean-sized  Tracheal resection  Not stated 
Fabich  1980  63  Cough  2 years  2.5 × 2.0 × 1.0  Sleeve resection  Died of complications on the 10th post-op day 
Warter  1980  69  Dyspnea, hemoptysis  Not stated  2.3 × 1.5 × 1.5  Segmental resection  Unremarkable 
Heard  1982  50  Dyspnea  Not stated  2.5 × 1.5 × 1.0  Sleeve resection  Sepsis, died on the 15th post-op day 
Ito  1988  51  Hemoptysis  9 months  1.5 × 1.2 × 1.0  Segmental resection  2 years 
Sheffield  1988  74  Cough, dyspnea  <1 month  2.2  Endoscopic resection  7 months 
Kim  1989  54  Cough, dyspnea, hemoptysis  3 years (cough)  1.5 × 1.2  Segmental resection  13 months 
Shin  1990  47  Cough, hemoptysis  3 years  1.5 × 1.0 × 1.0  Wedge resection  Not stated 
GarciaPrats  1991  58  Cough, dyspnea, hemoptysis  Several years  2.5 × 1.8  Segmental resection  8 months 
Haraguchi  1991  61  Asymptomatic  Asymptomatic  1.2  Sleeve resection  Not stated 
Arapantoni  1995  65  Dyspnea, hemoptysis  3 months (dyspnea), 3 days (hemoptysis)  4.5 × 3.0  Endoscopic resection and Nd-YAG  1 year 
Koskinen  1998  66  Asymptomatic  Not stated  2.0 × 3.0  Endoscopic resection, Nd-YAG and external radiotherapy  Not stated 
Watanabe  1998  43  Hoarseness  Not stated  2.0 × 1.6 × 1.4  Sleeve resection  20 months 
First author  Year  Age (years)  Sex  Symptoms  Duration of symptoms before treatment  Tumor site (S/M/I/B)  Size (cm)  Treatment  Follow-up 
Menaissy  2000  34  Hemoptysis  2 months  2.4 × 2.1 × 1.6  Tracheal resection  4 months 
Lange  2000  20  Asthma-like symptoms  < 1 month  1.4 × 1.3 × 0.6  Bronchial sleeve resection  9 months 
Oizumi  2000  48  Hemoptysis  Not stated  0.7  Bronchial resection  3 months 
Gowan  2001  73  Chest pain, dyspnea, hemoptysis  5 weeks  1.6 × 0.3 × 0.6  Segmental resection  6 years 
Chien  2003  50  Cough, dyspnea, hemoptysis  8 years (cough and dyspnea), 1 day (hemoptysis)  2.5 × 2.5 × 2.0  Segmental resection  1 year 
Vailati  2004  40  Dyspnea, cough, fever  6 months  5.0 × 1.5  Endoscopic resection  1 month 
De Weerdt  2004  37  Dyspnea, cough, fever  2 months  Not stated  Endoscopic resection + cryotherapy + Nd-YAG laser  3 months 
Nadrous  2004  39  Hemoptysis  30 months  2.0 × 1.5 × 1.5  Sleeve resection  3 months 
Ren  2005  29  Cough, dyspnea  2 years (cough), 2 months (dyspnea)  1.7 × 2.0 × 1.7  Segmental resection  Not stated 
Takahashi  2005  67  Cough  Not stated  0.8  Bronchial resection  Not stated 
Altinok  2006  83  Dyspnea, hemoptysis  3 months  2.0 × 1.5 × 1.2  Partial sleeve resection  1 year 
Haver  2008  10  Dyspnea, chest pain, cough  3 weeks  M to I  1.8 × 1.3 × 1.3  Tracheal resection  2 years 
Colaut  2008  70  Dyspnea, wheezing  2 months  2.0 × 1.0 × 1.0  Endoscopic resection and Nd-YAG  2 years 
Akata  2008  39  Cough  <1 month  2.5 × 2.5 × 2.0  Endoscopic resection  6 years 
First author  Year  Age (years)  Sex  Symptoms  Duration of symptoms before treatment  Tumor site (S/M/I/B)  Size (cm)  Treatment  Follow-up 
Shang  2010  59  Chest pain, dyspnea cough  10 years  2.0 × 1.0 × 0.5  Endoscopic resection + electrocautery  1 year 
Shang  2010  22  Cough, hemoptysis dyspnea  1 year  1.8 × 1.5 × 1.4  Endoscopic resection + electrocautery  1 year 
Nakajima  2010  30  Hemoptysis  6 months  1.5 × 1.3  Bronchial resection  10 months 
Parker  2010  43  Dyspnea, chest pain, cough  6 months  2.0 × 1.6 × 1.5  Tracheal resection  11 months 
Baek  2011  54  Dyspnea, cough  3 months  1.3 × 1.2  Tracheal resection  2 years 
Mogi  2011  56  Cough, dyspnea  7 months  1.3 × 1.2 × 1.1  Tracheal sleeve resection  9 months 
Ravenna  2011  79  Dyspnea, cough  3 months  Not stated  Endoscopic resection + Nd-YAG laser  5 years 
Sakr  2011  66  Cough, dyspnea  2 months (cough), 10 days (dyspnea)  1.2 × 0.8 × 2.0  Endoscopic resection + tracheal sleeve resection  21 months 
Okereke  2011  58  Dyspnea  Long term  1.1  Tracheal resection  6 months 
Norder  2012  49  Cough, dyspnea  3 years  1.2 × 1.1 × 1.1  Endoscopic resection  Not stated 
Lange Lazdunki  2012  62  Cough, dyspnea  Not stated  1.6  Left upper lung resection  Not stated 
Cukurova  2012  50  Cough, dyspnea, hemoptysis  Not stated  Not stated  Endoscopic resection  3 years 
Ariizumi  2012  43  Asymptomatic  3 months  Not stated  Tracheal resection  6 months 
First author  Year  Age (years)  Sex  Symptoms  Duration of symptoms before treatment  Tumor site (S/M/I/B)  Size (cm)  Treatment  Follow-up 
Zhu  2013  30  Dyspnea, hemoptysis  1 year  4.0 × 0.5 × 0.5  Tracheal resection  18 days 
Fan  2013  15  Cough, dyspnea, hemoptysis  3 months  2.0 × 2.5  Tracheal resection  1 year 
Ghigna  2013  70  Hemoptysis  Not stated  1.6  Tracheal resection  Not stated 
Ghigna  2013  40  Hemoptysis  Not stated  1.0  Tracheal resection  Not stated 
Chang  2013  76  Fever  1 week  Not stated  Endoscopic resection  Not stated 
Singh  2013  65  Cough  3 months  1.2 × 0.4 × 0.5  Endoscopic resection  Not stated 
Wei  2013  39  Cough, hemoptysis  1 year  1.9 × 1.4 × 0.8  Tracheal resection  26 months 
Wei  2013  43  Dyspnea  3 years  2.0 × 1.5  Tracheal resection  19 months 
Choi  2014  52  Asymptomatic  Asymptomatic  1.6  Resection of carina and both main bronchi  3 months 
Choi  2014  64  Asymptomatic  Asymptomatic  2.6  Tracheal resection  2 years 
Xiong  2014  48  Cough, dyspnea  6 years  1.2 × 1.0 × 0.8  Bronchoscopic cryoablation and argon plasma coagulation (APC)  6 months 
Xiong  2014  55  Cough, dyspnea; chest pain  13 days (Hemoptysis); 5 months (Cough and chest pain)  0.5 × 0.3 × 0.3  Bronchoscopic cryoablation and argon plasma coagulation (APC)  6 months 
Wu  2014  58  Hemoptysis  Not stated  2.2 × 2.2  Tangential resection with spiral tracheoplasty  2 years 
Zhang  2014  54  Cough, hemoptysis  4 years  2.5 × 1.5 × 1.0  Right total lung resection  6 months 
First author  Year  Age (years)  Sex  Symptoms  Duration of symptoms before treatment  Tumor site (S/M/I/B)  Size (cm)  Treatment  Follow-up 
Zhang  2014  48  Cough  1 year  Not stated  Right upper lung lesion resection  7 months 
Huang  2015  39  Dyspnea  More than 1 year  2.5 × 1.2  Segmental resection  1 month 
Liu  2015  39  Dyspnea  More than 1 year  2.5 × 1.2  Segmental resection  1 month 
Li  2015  15  Cough, hemoptysis  2 months  1.2 × 1.0 × 1.0  High-frequency electrocautery and APC  3 months 
Tan  2015  44  Cough, dyspnea, hemoptysis  2 months  3.0 × 2.5 × 1.0  Tracheal resection  20 months 
Masoum  2015  21  Cough, hemoptysis  Several months  Not stated  Endoscopic resection + tracheal resection  1 year 
Fernandez-Bu  2015  48  Hemoptysis and cough  3 months  2.0 × 2.0  Endoscopic resection  2 years 
Brzezinski  2015  38  Dyspnea  1 year  1.6 × 1.8 × 0.8  Tracheal resection  Not stated 
Rashid  2015  52  Hemoptysis  3 months  Not stated  Endoscopic resection  6 months 
Xiong  2016  52  Dyspnea, cough  6 months  2.0 × 1.0 × 1.0  High-frequency electrocautery and APC  9 months 
Aryan  2016  50  Hemoptysis, cough, dyspnea  1 week  Not stated  Endoscopic resection  Not stated 
Wang  2016  63  Hemoptysis  1 week  0.5 × 0.3  High-frequency electrocautery and APC  15 months 
Wang  2016  44  Hemoptysis, cough  1 week  1.0 × 1.5  Endoscopic resection + tracheal resection  8 months 
Venegas  2017  51  Dysphagia, hemoptysis dyspnea  Several weeks  2.6 × 2.3 × 1.7  Tracheal resection;  12 months 
First author  Year  Age (years)  Sex  Symptoms  Duration of symptoms before treatment  Tumor site (S/M/I/B)  Size (cm)  Treatment  Follow-up 
Huang  2017  38  Cough, hematemesis  20 days  2.4 × 2.2 × 2.7  The video-assisted transthoracic surgery (VATS)  3 months 
Suresh  2018  43  Dyspnea  Not stated  Not stated  Percutaneous trans-tracheal endoscopic approach (PTEA)  Not stated 
Gou  2018  30  Cough, expectoration  1 month  2.0  High frequency electroexcision  1 year 
Gou  2018  47  Hemoptysis  3 years  1.8  Tracheal resection, and anastomosis  1 year 
Jin  2019  51  Cough  4 months  2.0  Segmental tracheal resection  2 years 
Hartert  2019  66  Cough, hemoptysis dyspnea  3 months  1.1 × 2.2  Thoracotomy with end-to-end anastomosis  96 months 
Shao  2020  41  Chest tightness; chest pain; hemoptysis  1 month (chest tightness; chest pain); hemoptysis (2 weeks)  1.0 × 1.2 × 1.2  Endoscopic resection  Not stated 
Present case  2020  53  Cough, dyspnea  1 year  1.4 × 1.4 × 0.9  Harmonic scalpel with Video endoscope  11 months 
Table 2.

Different parts of the treatment.

Location  Number  Endoscopic  Surgery  High frequency eletrocautery  Others (like APC Nd-YAG laser) 
16  20.78 
18  23.38  14 
27  35.06  18 
16  20.78  11 

S, superior; M, medium; I, inferior; B, bronchi.

We suggest that as respiratory symptoms cannot be effectively resolved, it may be necessary to perfect examinations for rare diseases conscientiously, especially GTs can be easily missed and misdiagnosed. Although there is no cure in this case, initial bronchoscopy intervention plays a key role in timely and effectively restoring the airway of patients with severe symptoms and providing preoperative diagnostic information. The bronchoscopy biopsy should be avoided due to the tumor’s rich vasculature. Complete resection of the tumor remains the basic procedure of treatment. Long-term followup of tracheal conditions after surgery is certainly necessary.

Conclusion

Tracheal GTs of uncertain malignant potential, while uncommon, currently have no uniform standards for the surgical treatment of GTs and can be easily mistaken for a pulmonary disease if the symptoms are atypical, we should pay attention to it. Radical resection is still worthy of consideration because of the possibility of recurrence in the clinic. Additionally, tracheal stenosis is the most likely complication of concern.

Conflicts of interest

The authors declare no conflicts of interest.

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