tracheal hemangioma a case report and literature review

Massive Hemoptysis due to Endotracheal Hemangioma: A Case Report and Literature Review

Affiliations.

• 1 Department of Internal Medicine, Seoul Medical Center, Seoul, Korea.
• 2 Department of Otorhinolaryngology-Head and Neck Surgery, Seoul Medical Center, Seoul, Korea.
• 3 Department of Radiology, Seoul Medical Center, Seoul, Korea.
• 4 Department of Pathology, Seoul Medical Center, Seoul, Korea.
• PMID: 25861344
• PMCID: PMC4388893
• DOI: 10.4046/trd.2015.78.2.106

Tracheal hemangioma is a rare benign vascular tumor in adults. We reported a case of massive hemoptysis caused by a cavernous hemangioma in a 75-year-old man. This is the first report, to our knowledge, of a tracheal cavernous hemangioma that presented with massive hemoptysis. The lesion was removed with a CO2 laser under rigid laryngoscopy. Endovascular tumors, such as tracheobronchial hemangiomas, should be considered a diagnostic option in cases of massive hemoptysis without a significant underlying lung lesion.

Keywords: Hemangioma, Cavernous; Hemoptysis; Trachea.

Pediatric Tracheal Lobular Capillary Hemangioma: A Case Report and Review of the Literature.

Chronic cough is a common complaint in the pediatric population and can have many different etiologies. We present a rare case of a tracheal lobular capillary hemangioma (LCH), also known as pyogenic granuloma, causing chronic cough in a child. In this case, the tracheal LCH was managed successfully with laser ablation. A review of the literature reveals only 2 other reported pediatric cases of tracheal LCH. Laryngoscope, 131:1729-1731, 2021.

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Case report article, successful management of tracheal lobular capillary hemangioma with arterial embolization followed by electrosurgical snaring via flexible bronchoscopy in an 11-year-old boy: a case report and literature review.

• 1 Department of Pulmonology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang, China
• 2 Department of Endoscopy Center, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang, China
• 3 Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, Guangdong, China
• 4 Department of Radiology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang, China
• 5 Department of Respiratory Medicine, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
• 6 Department of Pediatric Intensive Care Unit, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang, China

Lobular capillary hemangioma (LCH), previously known as pyogenic granuloma, is a benign vascular lesion commonly found within the oral and nasal cavities. However, it is rarely encountered within the trachea, especially in pediatric patients, where it manifests as hemoptysis, cough, and wheeze, and is frequently misdiagnosed as bronchitis or asthma. There is limited literature on the presentation, behavior, and management of tracheal LCH. Herein, we describe a rare case of tracheal LCH in an 11-year-old boy with a history of hemoptysis, which was successfully managed with arterial embolization followed by electrocautery loop snaring via flexible bronchoscopy. No complications occurred during and after the procedure. A review of the relevant literature is also provided. Our case is unique, given the therapeutic strategy utilized for pediatric tracheal LCH, and reminds physicians to be aware of tracheal LCH in the differential diagnosis for hemoptysis.

Introduction

Primary tracheal tumors are rare, with an estimated annual incidence of 2.7 new cases per million population ( 1 ). As a benign tumor, lobular capillary hemangioma (LCH) was previously known as pyogenic granuloma and most commonly appears on the lip, nose, oral cavity, or tongue ( 2 , 3 ). The etiology of this lesion remains elusive. There are many theories on pathogenesis revolving around LCH, such as a sequela of local irritation and minor trauma, neovascular response to an angiogenic stimulus with an imbalance of promoters and inhibitors, and bacterial and viral infections ( 3 – 6 ). As the clinical presentations are various and non-specific, LCH is often confused with true hemangiomas and granulation tissue ( 7 , 8 ). Definitive diagnosis relies on histopathologic examination, which demonstrates a distinctive lobular arrangement of variably sized capillaries embedded in a fibromyxoid matrix without atypical mitoses ( 2 , 9 – 11 ). Despite their benign nature, localized recurrence is common, and thus, surgical debulking or excision remains the mainstay of treatment if symptomatic ( 6 , 12 – 14 ). Although there are a few studies of LCH on mucous membranes ( 2 , 3 , 6 , 11 , 15 – 17 ), tracheal origin for LCH is extremely rare among all primary tracheal tumors, with only four cases of children reported in the literature ( 9 , 10 , 18 , 19 ). Thus, there is limited knowledge about the presentation, behavior, and management of tracheal LCH, which represents a diagnostic and therapeutic challenge in pediatric patients. Herein, we report a rare case of an 11-year-old boy with a tracheal LCH managed by arterial embolization, followed by electrosurgical snaring via flexible bronchoscopy.

Case presentation

An 11-year-old otherwise healthy boy presented to our hospital with hemoptysis for 5 days on 20 July 2022. He had more than 10 episodes of hemoptysis per day, with 5–30 ml of bright red blood mixed with clots each time, over the preceding 5 days. Associated fatigue, dizziness, shortness of breath, chest pain, dysphagia, nasal congestion, runny nose, hematuresis, hematochezia, fever, and rigors were denied. There was no history of foreign body aspiration, trauma, hoarseness, airway intubation, or endoscopy. Initially, he was treated at a local hospital for 5 days, but he was not responding well to medical treatment with antibiotics.

On admission, he was in good condition, and the physical examination showed no abnormalities. Thoracic non-contrast-enhanced computed tomography (CT) imaging revealed a cauliflower-like lesion of 4.9 * 4.6 * 4.6 mm in size affixed to the right wall of the lower trachea ( Figure 1A ), with an average density of 29 Hounsfield unit (HU). Contrast-enhanced CT imaging showed an inhomogeneous enhancement in the lesion with an average density of 200 HU ( Figure 1B ). Flexible bronchoscopy revealed a hyperemic, broad pedunculated tracheal neoplasm, arising from the right wall of the trachea in the lower one-third and projecting into its lumen, approximately 1 cm above the main carina with blood on the surface ( Figure 1C ). Glomus tumor was highly suspected. To avoid potential massive tumor bleeding during the biopsy, a multidisciplinary consultation was scheduled; the boy's parents were also in favor of less invasive surgery, rather than thoracotomy. The multidisciplinary consensus was to proceed with bronchial artery embolization (BAE) for the tracheal neoplasm by interventional radiologists, and then the mass was removed with an electrocautery snare via flexible bronchoscopy by a pulmonologist. Surgical resection (e.g., thoracotomy) was considered as an alternative option by thoracic surgeons if procedures mentioned above failed, and the extracorporeal membrane oxygenation (ECMO) team was also on standby.

Figure 1 . Both lung views of plain chest computed tomography (CT) scan (A) and mediastinal view of contrast-enhanced CT scan (B) demonstrate the tracheal mass. (C) Initial bronchoscopic view of the mass occluding the lower trachea.

In order to minimize the possibility of bleeding during lesion resection, the feeding artery was attempted to be embolized through the right common femoral artery. Digital subtraction angiography (DSA) confirmed the presence of a feeding artery ( Figure 2A ). Under fluoroscopic guidance, endovascular embolization was performed using a 5-Fr catheter and polyvinyl alcohol particles of 300–500 microns and 500–700 microns for distant and proximal branches, respectively. Embolization of the main bronchial artery with gelatin sponge particles was performed simultaneously. Post-embolization DSA revealed a marked reduction of the abnormal vascular blush and occlusion of the feeding artery and its branches ( Figure 2B ).

Figure 2 . Arteriography revealed a pathological artery originating from the bronchial artery (A) (blue arrow: tumor; red arrow: bronchial artery branch) that was selectively embolized (B). (C) Electrocautery loop snaring via flexible bronchoscopy; (D) posttreatment view of the same area.

Then, the tumor was debulked by electrosurgical snaring with a flexible bronchoscope under general anesthesia. In detail, the electrocautery snare was used in a blend mode at 40 W to cut and coagulate the base of the mass ( Figure 2C ), which was resected without significant bleeding, and tracheal patency was achieved ( Figure 2D ). Histological examination revealed numerous capillaries arranged in a lobular pattern, separated by an edematous fibrous stroma, accompanied by mild inflammatory changes ( Figure 3A ) with fungal hyphae inside it ( Figure 3B ), which was suggestive of polypoid LCH. Immunohistochemistry revealed SMA(+), LYVB1(partial +), D2-40(-), CD34(+), WT1(+), Glut1(-), and CD31(+), further supporting the diagnosis. Although pathology suggested fungal infection, the patient did not have any typical symptoms of fungal infection throughout the course of the disease. Thus, antifungal therapy was not given during the hospitalization. The patient's postoperative recovery was uneventful, and he was discharged on the third postoperative day.

Figure 3 . Histological examination of the resected specimen revealed capillary hemangioma (A) covered by the squamous epithelium and with fungal hyphae inside it (B) .

In the present case, the lesion was discovered while searching for causes of non-specific clinical symptoms, such as hemoptysis. It is known that primary tracheal tumors account for <2% of all upper respiratory tract tumors ( 20 ), which are usually malignant in the adult population. The most common benign tumors in the trachea include papilloma, chondroma, and fibroma, with <10% being vascular in origin ( 21 ), and LCH commonly appears on the lip, nose, oral cavity, and tongue ( 2 , 3 ). These data indicate that the occurrence of LCH within the tracheobronchial tree is extremely rare and infrequently described in medical literature, especially in children. Based on CT findings, bronchoscopy was indicated and led us to the final diagnosis of tracheal LCH. To the best of our knowledge, only four pediatric cases of tracheal LCH have been reported in the literature ( 9 , 10 , 18 , 19 ), further implying the rarity of tracheal LCH in the pediatric population. However, it should be kept in mind for the differential diagnosis of causes of hemoptysis.

Lobular capillary hemangioma (LCH), as benign vascular tumors characterized microscopically by a distinctive lobular arrangement of capillaries ( 2 , 9 , 22 ), was previously known as “pyogenic granulomas” or “granulomatous hemangiomas.” The traditional term “pyogenic granuloma” is inaccurate since the tumor neither contains purulent material nor is a true granuloma ( 11 ). However, the mechanism triggering the evolution of LCH is unknown, prior trauma, hormonal imbalances, infection, drug adverse effects, and genetic abnormalities associated with the nitric oxide pathway, angiogenesis and vascular injury have been proposed as predisposing factors ( 3 – 5 ). The possible explanation for fungal infection showed by pathology is that BAE caused local tumor tissue necrosis, and then, the necrotic material combined with the previous bleeding on the tumor surface led to the attachment of fungal hyphae to the tumor surface. Our patient had no prior history of foreign body aspiration, severe respiratory infection, signs of fungal infection, significant abnormalities, or traumatic surgery, thus, the most likely cause of our case was underlying microscopic arteriovenous malformations.

The most common clinical symptoms of tracheal LCH are cough, hemoptysis ranging from minor to massive, and expectoration. Giant masses may narrow the airway, causing dyspnea and breathlessness. The most frequent causes of hemoptysis are infectious diseases, tuberculosis, malignant tumors, cardiovascular disorders, and other inflammatory diseases. Only one of the five kids with tracheal LCH presented without hemoptysis ( 18 ) or a chronic cough. Qiu et al. summarized 12 tracheal LCH cases in the adult population and found that all of them had hemoptysis ( 12 ). Altogether, tracheal LCH should be considered a possible benign cause of hemoptysis and cough despite its rarity. Although it is a benign disease, tracheal LCH has a tremendous bleeding tendency even life-threatening. Therefore, tracheal LCH should be managed in a well-prepared manner.

The diagnosis of tracheal LCH relies on chest CT and bronchoscopy, while X-ray may show no abnormality. In detail, chest CT, especially contrast-enhanced CT, identifies the occupying lesions and reveals the nature of the mass with a plentiful blood supply, while bronchoscopy with biopsy plays a key role in achieving the final diagnosis of tracheal LCH and provides additional opportunities for therapeutic intervention. Endoscopic appearances are non-specific and sometimes may be confused with granulation tissue, carcinoid tumor, Kaposi sarcoma, angioendothelioma, paraganglioma, adenoma, angiosarcoma, intravascular angiomatosis, and carcinoma ( 7 , 8 ). Hence, histopathological findings are crucial for a definite diagnosis of tracheal LCHs.

Due to the limited number of tracheal LCH cases, the preferred treatment for it has not yet been established, and therapy methods vary considerably among different studies ( 12 , 13 ). Generally speaking, tracheal LCH can be treated with interventional bronchoscopy or surgical excision. Endoscopic excision, laser therapy, cryoprobe, electrocautery, brachytherapy, surgical debulking, argon plasma coagulation (APC), and cryotherapy were performed in a separate or combined way in adult tracheal LCH cases ( 12 ). The treatment experience is relatively limited in children when compared with adults because only four cases reported in the literature. The previously reported interventions for pediatric tracheal LCH include electrocauterization, cryoablation, potassium-titanyl-phosphate laser ablation, and cylindrical resection ( 9 , 10 , 18 , 19 ), while a tracheal LCH utilizing DSA/BAE followed by electrosurgical snaring via flexible bronchoscopy in an 11-year-old boy was successfully removed in the present case. Collectively, although no adequate guidelines were provided and tracheal LCH is amenable to various techniques, the extent and size of the lesion, as well as patient age and comorbidities require consideration prior to any therapeutic intervention for tracheal LCH.

Although our department has rich experience in removing transbronchial tracheal tumors ( 23 , 24 ), there is a substantial risk of intraoperative hemorrhage dealing with the tracheal LCH. We confront the following challenges: ( 1 ) the large size of the tumor and the rich blood supply did not allow the resection with biopsy forceps; ( 2 ) the lesion was attached to the trachea wall, and the resection with laser or cryotherapy may be associated with a high risk of perforation [6]. In the present case, DSA/BAE was employed to prevent massive intra-procedural bleeding. Of note, bronchial artery embolization ahead of tracheal LCH removal is of great importance for our case. For instance, without scheduling BAE before surgery, Dabó et al. described significant hemorrhage during tracheal LCH removal by rigid forceps via rigid bronchoscopy, and the instillation of cold saline and epinephrine failed to control the massive bleeding was described [20]. Inversely, in another case of BAE followed by lumpectomy under bronchoscopy, the amount of intraoperative blood loss was dramatically decreased [17]. This evidence further emphasizes that BAE was the procedure of choice in order to achieve better control of bleeding.

Meanwhile, electrocautery loop snaring was used in our case due to its ability to cut and cauterize simultaneously. The specimens obtained by electrosurgical loop snaring for diagnostic purposes are much larger and of excellent tissue quality compared with specimens extracted with forceps. Moreover, alternative surgical options are available. If a flexible bronchoscope fails to remove the tumor, a rigid bronchoscope will be employed. The endotracheal intubation should adjust the depth of endotracheal intubation if the patient loses a substantial amount of blood during the procedure, and balloon compression should be used to try to stop the bleeding. In the event that the endobronchial treatment cannot be performed as intended, the thoracic surgeon will perform a thoracotomy. If vital signs become unstable, the extracorporeal membrane oxygenation team is prepared to provide emergency care. In other words, the ECMO team and thoracic surgeons were on standby as a precautionary measure to ensure the removal of the lesion successfully. This information demonstrates the safe, successful, effective, and less invasive removal of the challenging tracheal LCH without discernible bleeding depends on close interdisciplinary cooperation and elaborate preparation.

Since the degree of tumor infiltration into the tracheal wall cannot be evaluated during bronchoscopy, individuals who have undergone bronchoscopy for tumor resection may have residual and incomplete tumor removal, and some patients may experience tumor recurrence. Future monitoring of this patient is required. In other words, it might be necessary to use bronchoscopy during the follow-up period because there is a risk of local recurrence ( 1 ).

Our case is unique as it highlights the successful use of DSA/BAE followed by electrosurgical loop snaring via flexible bronchoscopy for the diagnosis and treatment of pediatric tracheal LCH with an abundant blood supply. Although exceeding rare, tracheal LCH should be considered a cause of recurrent hemoptysis in children. However, our experience should be validated in further large studies and further rigorous follow-up is needed for the possibility of local recurrence.

Data availability statement

The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.

Ethics statement

Written informed consent was obtained from the minor(s)' legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.

Author contributions

XT and LW collected the data and drafted and edited the manuscript. SL, YWu, CL, EC, ZC, and GJ revised the manuscript. YWa supervised this study. All authors critically reviewed, revised, and approved the final manuscript and agreed to be responsible for all aspects of the study.

This study was supported by a grant from the Zhejiang Provincial Natural Science Foundation (LQ20H190006).

Acknowledgments

The authors appreciate the support from the boy enrolled in this study and his guardians. Moreover, the authors gratefully thank all the doctors and nurses who were involved in the treatment.

Conflict of interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Publisher's note

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Keywords: lobular capillary hemangioma, tracheal, arterial embolization, electrocautery loop snaring, flexible bronchoscopy, case report

Citation: Tao X, Wu L, Li S, Wu Y, Lai C, Chen E, Chen Z, Jin G and Wang Y (2023) Successful management of tracheal lobular capillary hemangioma with arterial embolization followed by electrosurgical snaring via flexible bronchoscopy in an 11-year-old boy: A case report and literature review. Front. Med. 10:1088815. doi: 10.3389/fmed.2023.1088815

Received: 03 November 2022; Accepted: 20 February 2023; Published: 20 March 2023.

Reviewed by:

Copyright © 2023 Tao, Wu, Li, Wu, Lai, Chen, Chen, Jin and Wang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

*Correspondence: Yingshuo Wang, wangyingshuo@zju.edu.cn

† These authors have contributed equally to this work

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Tracheal hemangioma: a case report and literature review.

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• Amirfakhrian H 1
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• Sadidi H | 0000-0001-5812-5939

Annals of Medicine and Surgery (2012) , 19 May 2023 , 85(7): 3615-3622 https://doi.org/10.1097/ms9.0000000000000850   PMID: 37427188  PMCID: PMC10328570

Abstract 

• Case presentation

Clinical discussion

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Tracheal hemangioma: a case report and literature review

Hossein sadidi.

a Thoracic Surgery Department of Ghaem Hospital, Mashhad University of Medical Sciences

Pegah Bahrami Taqanaki

b Mashhad University of Medical Sciences

Hamed Amirfakhrian

Reza rezaei.

c Endoscopic and Minimally Invasive Surgery Research Center, Mashhad University of Medical Sciences, Mashhad, Iran

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All the required data are available in the manuscript.

• Introduction and importance:

Hemangiomas are the most common vascular tumors in children. Although hemangiomas are common, they are rarely seen in areas like the trachea and larynx.The most common clinical manifestations in tracheal hemangiomas are stridor and respiratory distress. The main diagnostic method is bronchoscopy. Other imaging techniques like computed tomography scans and MRIs are also helpful. Various treatment options are now used for treating the disease, including beta blockers like propranolol, local and systemic steroids, and surgical resection.

Case presentation:

An 8-year-old boy with the chief complaint of severe progressive dyspnea and a history of neonatal postbreastfeeding cyanosis was admitted. On physical examination, he had tachypnea, and stridor was heard upon auscultation. There was no history of fever, chest pain, or coughing. He underwent a rigid bronchoscopy followed by a neck computed tomography scan. The results indicated a soft tissue mass with a vascular nature. An MRI of the neck confirmed the diagnosis of tracheal hemangioma. The mass was not resectable during surgery; hence, angioembolization was carried out. Treatment was successful and there was no recurrence on the follow-up.

Clinical discussion:

Based on the findings in this literature review tracheal hemangiomas present with stridor, progressive respiratory distress, dyspnea, hemoptysis, and chronic coughs. Advanced tracheal hemangiomas commonly do not reduce in size by themselves and need treatment. A close follow-up ranging from 3 months to 1 year is recommended.

Conclusion:

Although tracheal hemangiomas are rare they should be considered in the differential diagnosis of severe dyspnea and stridor.

• Introduction

Hemangiomas are the most common benign soft tissue tumors in children. Even though their most common sites are the head and neck, they are rarely seen in the trachea and larynx.

Tracheal or laryngeal hemangiomas can significantly affect patients’ lives, and even cause respiratory distress and arrest. Hence, proper diagnosis and treatment of these diseases are crucial. Tracheal hemangioma’s most common signs and symptoms are stridor, respiratory distress, progressive dyspnea, chronic cough, and hemoptysis.

The main diagnostic tool for this disease is bronchoscopy.

Treatment options vary from beta blockers like propranolol, local and systemic steroids, to surgical resection.

Vascular diseases in children and infants include vascular tumors and malformations. Hemangiomas are the most common among vascular tumors and also the most common benign soft tissue tumor in infants 1 , 2 . Infantile hemangiomas occur in nearly 4–5% of infants, which may be present at birth or shown later in life 3 . Hemangiomas go through an involution stage once they have reached their complete growth near one year of age and it may take from months to years; however, a small percentage of hemangiomas never go through this stage and do not become smaller in size 3 , 4 . The most common areas of involvement are the head and neck with the subglottic region being the most involved 4 . Although hemangiomas usually do not require special intervention, factors such as hemangioma size, growth rate, and location can change this 5 .

Hemangiomas residing in the larynx or trachea, although rare, need close monitoring and intervention as they may lead to irreparable and life-threatening consequences. Tracheal hemangiomas present in various ways in children and adults. Dyspnea, stridor, chronic cough, and obstructive respiratory disease are the most common signs and symptoms of tracheal hemangiomas in children. The main diagnostic tool for tracheal hemangiomas is bronchoscopy 6 . Several different approaches have been suggested over time for the treatment of tracheal hemangiomas including steroid injections, endoscopic and open excisions, and medications like systemic steroids and vincristine 5 .

Here, we present a case of an 8-year-old with intraluminal tracheal hemangioma presenting with respiratory distress to an academic tertiary center, which was managed with three sessions of angioembolization.

This manuscript was prepared following the Surgical CAse REport (SCARE) guidelines 7 .

An 8-year-old boy without any documented medical or surgical history was admitted to a tertiary academic center with the chief complaint of progressive dyspnea. His parents mentioned that he used to suffer from postbreastfeeding cyanosis in infancy and also after crying. In the years after infancy and before school age the patient’s symptoms were described as exertional dyspnea becoming worse by exercising and walking accompanied by fast heartbeats and increased shallow breathes. As the patient had reached the school age, his dyspnea had become more severe with stridor heard while breathing. His dyspnea and stridor had progressed in the last 3 months. He had not experienced fever, chest pain, weight loss, and coughing in the course of his illness.

In general appearance, he was a child with a height of 125 cm and a weight of 27 kg, and physical examination revealed tachycardia (heart rate: 120), tachypnea (respiratory rate: 30), mild hypotension ( blood pressure: 90/70), and low oxygen saturation (Spo 2 : 85%). Laboratory results were normal and no anemia or leukocytosis was seen.

The patient underwent a rigid bronchoscopy. Bronchoscopy findings included normal tracheal mucosa and a soft tissue mass that could be compressed with the bronchoscope. Afterward, a neck computed tomography (CT) scan was performed revealing a vascular mass behind the larynx and cervical trachea (Figs. ​ (Figs.1 1 – 3 ). Subsequently, surgery is performed for investigating the mass in the patient’s neck. It was reported during the operation that the mass is of a vascular nature but resection could not be done. A tracheostomy was performed by the surgeon in the same session.

Demonstrate the neck CT scan of the patient demonstrating a vascular mass behind the larynx and cervical trachea.

He was transferred to the radiology department for an MRI with contrast. At MRI, a multiloculated nodular lesion in the intraluminal part of the trachea and also partly in the infra-glottic area, measuring 12×8 mm and taking up half of the tracheal lumen, was found. The lesion was hypo-signal in the T1 sequence, hyper-signal in the T2 sequence, and heterogeneous after the contrast injection (Figs. ​ (Figs.4 4 and ​ and5). 5 ). MRI findings confirmed the diagnosis of hemangioma.

Present the MRI images which reveal a multiloculated nodular lesion in the intraluminal part of the trachea and also partly in the infra-glottic area, measuring 12×8 mm and taking up half of the tracheal lumen.

Three sessions of angioembolization were carried out for the patient, and the patient’s progress was controlled with MRI. Finally, after hemangioma atrophy tracheostomy was extracted.

He was given guaifenesin every 8 h and discharged from the hospital. At 3 month follow-up, the patient’s symptoms were significantly improved and repeated bronchoscopy did not demonstrate any recurrence.

Hemangioma is a soft tissue tumor caused by hyperplasia of the endothelial cells. Hemangiomas are either congenital or infantile. Infantile hemangiomas appear in the first 8 weeks of birth and grow rapidly until 6–12 months of age, and then they usually shrink in a natural and physiological way and disappear completely. Congenital hemangiomas; however, are fully developed lesions at the time of birth 8 . Hemangiomas are classified by the extent of tissue involvement as focal, segmental, and indeterminate, and by the depth of the tissue affected as deep, superficial, and mixed 8 .

Tumors of the tracheobronchial tree are a rare concept in children and are divided in two groups of benign and malignant lesions. The most common benign tumors of the tracheobronchial tree in children are hemangiomas and squamous papilloma 9 . Various factors may lead to the formation of these hemangiomas consisting of hormonal imbalance in pregnancy, infections, arteriovenous malformations, and vascular growth factors alteration 10 .

The low prevalence of this disease causes a delay in its diagnosis, as seen in this case. The presence of a mass in the trachea can cause symptoms such as stridor, cough, shortness of breath exacerbated by exercise, and hemoptysis (in the case of hemangiomas) 11 . A tracheal hemangioma can present as intractable asthma and may lead to respiratory failure and even death in severe cases.

As the signs and symptoms of a tracheal hemangioma are not specific to the disease the initial diagnostic tool used for finding the etiology is a chest radiograph as in all the respiratory diseases. CXR is commonly not diagnostic for the disease but may exclude other differential diagnoses. Further diagnostic tools that have been proven to have appropriate sensitivity and specificity in diagnosing the lesion are the CT scan, MRI, and bronchoscopy 9 . Rigid bronchoscopy and flexible endoscopy are used to retrieve samples and histopathologic assessments confirm the diagnosis; however, some studies start the treatment with imaging findings and do not use pathologic samples for confirmation 9 .

The case presented in the current study is a display of a tracheal mass presenting itself with respiratory distress and severe dyspnea.

We used three databases of PubMed, Scopus, and Web of Science to conduct a literature review. Keywords used were ‘Tracheal Hemangioma’ and ‘Hemangioma of Trachea’ and all studies from 1 January 2013 to 5 March 2023 were included initially. The initial number of articles included was 368, which was then reduced to 216 after deleting the duplicates. After reviewing the remaining articles, irrelevant articles (articles discussing nontracheal hemangiomas, nonhemangioma tumors mimicking hemangiomas, subglottic hemangiomas not radiating to the trachea, cancer treatments, and articles not reporting cases of the issue) were excluded. The remaining articles were assessed for eligibility to be included in this study and 20 articles were excluded due to the age of the patients, as only pediatric case series or case repots are included in the current study. Although one study was conducted on patients with subglottic hemangiomas with reported tracheal stenosis, with the aim of investigating their perioperative airway managements, it did not specify the exact location of the hemangioma and the number of patients with tracheal hemangioma was not reported, hence the study was excluded from this review 12 . Finally, 15 studies were included in the review including 12 case reports and 3 case series. The summary of the studies are displayed in Table ​ Table1 1 .

The summary of the studies included in the review

To summarize, a total of 19 children were reported to have tracheal hemangiomas or other subglottal lesions protruding to the trachea. The country distribution of the cases were as follows: USA 42.1% (8/19), Canada 15.7% (3/19), Turkey 10.5% (2/19), Tunisia 10.5% (2/19), Japan 5.2% (1/19), China 5.2% (1/19), and Korea 5.2% (1/19) . Four studies (eight patients) did not mention the age of the patients and the average age in other studies was 55.4 months (from 6 weeks to 15 years) with 54.54% of the cases being 1 year old or younger. There was a slight prevalence of the disease in female with the male to female ration being 4:7. The reported comorbidities and past medical histories included prematurity 5.2% (1/19), hyperbilirubinemia 5.2% (1/19), laryngomalacia 5.2% (1/19), respiratory distress early in life 15.7% (3/19), PHACE syndrome 36.8% (7/19), and a history of adenotonsillectomy 5.2% (1/19). It should be noted that one case suffered from severe prematurity and respiratory distress syndrome at birth, hyperbilirubinemia, and laryngomalacia at the same time. Two studies did not mention the past medical history of the patients and six patients did not have any comorbidities or prior medical conditions.

Among 13 patients whose signs and symptoms were recorded, the most common signs and symptoms were stridor 69.23% ( 9/13), respiratory distress 38.46% (5/13), and hemoptysis 23.07% (3/13). Other complaints consisted of dyspnea, chronic coughing, recurrent respiratory infections, and sporadic fascial and cervical hemangiomas. The diagnostic tools used in all studies were mainly a CT scan, an MRI, bronchoscopy, and histopathologic assessments for confirmation. One study confirmed the accuracy of the 3d-CT/bronchoscopy diagnosis for the tumor.

Only one case series did not specify the treatment option used for the patient with tracheal hemangioma. The main treatment option observed in the studies was the use of Propranolol 61.11 (11/18), which in 10 patients led to improvement of the signs, symptoms, and the size of the tumor. There was a failure of the propranolol regimen in one study, which led to electrical excision of the lesion. Laser ablation, steroids therapy, and surgical removal of the lesion were reported in 27.77% ( 5/18), 16.66 (3/18), and 16.66 (3/18), respectively. Natural shrinkage of the tumor was reported in one case.

There was no report of a tracheal hemangioma from Iran in the past 10 years, therefore making this case report the only reported tracheal hemangioma in the past 10 years in Iran.

The study searching was conducted by two separate individuals; hence, improving the accuracy of the search results. All included studies were obtained in full-text form and were in English language; hence, no exclusion was needed in this part.

The limitations of this literature review include a publication bias as all studies are case reports and series. The results of this study depend on the quality of the literature search.

Tracheal hemangiomas are a rare concept, especially in children. These tumors present with stridor, progressive respiratory distress, dyspnea, hemoptysis, and chronic coughs. Propranolol has been reported to have great efficacy in improving the symptoms and reducing the size of the lesion. Systemic and localized steroids, surgical removal, and laser ablation are other successful methods for treatment. It is not common for a tracheal hemangioma presenting with severe symptoms to shrink naturally. Although there is not sufficient literature, based on this literature review, recurrence is not a common finding after the mentioned treatments as none of the cases reported showed recurrence. A close follow-up ranging from 3 months to 1 year is recommended.

• Ethical approval

No personal information of patients have been revealed in the study.

• Sources of funding
• Author contribution

R.R., H.S.: study concept or design; H.S., P.B.T., H.A.: data collection; R.R.: supervision; H.S., P.B.T., H.A.: data analysis or interpretation; P.B.T.R., R.R., H.A.: writing the paper.

• Conflicts of interest
• Research registration unique identifying number (UIN)

Reza Rezaei.

• Data availability statement
• Provenance and peer review

Not commissioned, externally peer reviewed. Tracheal hemangioma: a case report and literature review.

• Assistance with the study
• Acknowledgements

The authors thank their colleagues and co-authors for their expertise and assistance throughout all aspects of our study and for their help in writing the manuscript.

Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article

Published online 19 May 2023

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