Tracheal airway obstruction induced by a large glomangioma: discussion of management and literature review

Summary

Glomus tumours (GTs) are rare benign neoplasms arising from modified smooth muscle cells (SMCs) surrounding arteriovenous anastomosis. Typically, these tumours are found in the distal portion of the digits, especially under the fingernails. A GTs originating from the trachea is extremely rare. We are presenting the case of a Caucasian man in his early 80s, presenting with upper airway obstruction and massive bleeding caused by a large tracheal tumour to which final diagnosis of glomangioma of the trachea was retained. Methods of diagnostics, management and follow-up are documented. The incidence of GTs accounts about 1.6% of soft tissue tumours, and they are mainly located in dermal and subcutaneous tissue but can be equally find throughout the body. However, tracheal glomus tumours are the most clinically significant as they can be malignant and cause life-threatening condition through central airway obstruction. Histological analysis provides certainty of diagnosis, and surgical resection is the main treatment option.

Background

Glomus apparatus is a specialised form of arteriovenous shunt involved in thermoregulation GTs originate from these glomus bodies.¹ These tumours are typically seen in the deep dermis or subcutis of the extremities, with ≤65% occurring in the subungual area. They also can occur in sites where normal glomus bodies are absent or even may be sparse, such as bone, stomach, chest wall, colon, nerve, eyelid, nose, mediastinum and trachea.²

Histologically, the GTs have varying proportion of vascular structures, glomus cells and smooth muscles. According to the relative proportion, these tumours have been divided into three subtypes: classical glomus tumours, glomangiomas and glomangiomyomas, with the last type accounting for less than 10% of all glomus tumours.²

Glomus tumours presenting in airways are extremely rare, they usually occur in the distal part of the respiratory tree and the majority of these tumours are benign.³ GTs are usually asymptomatic. However, the upper airway obstruction provoked by these tumours often lead to an acute respiratory syndrome that can be life threating, without mentioning the risk of massive haemoptysis due to the vascular nidus nature of the tumour.⁴

We are reporting a case of glomangioma subtype of the trachea in a patient presenting with acute respiratory disorders with massive haemoptysis. Clinical manifestations, radiological and endoscopic findings, histological characteristics, management and follow-up are documented.

Case presentation

A man in his early 80s was admitted to the emergency department with respiratory distress and massive haemoptysis. He presented a few days earlier with self-resolving episodes of bloody sputum. His patient history showed an arterial hypertension, gastro-oesophageal reflux disease, active smoking and moderate alcohol consumption. There was no history of respiratory pathologies, haemorrhagic manifestations of the upper aero digestive tract, coagulopathies, anticoagulants or antiplatelet aggregation therapy.

Emergency upper airway stabilisation and haemostasis were immediately performed in the operating room. The initial endoscopic examination showed an active and significant bleed emerging from a large tumour in the trachea. The patient was therefore intubated with a size five endotracheal tube to insure ventilation.

Taking into account the seriousness of the situation allows surgical tracheotomy between the third and fourth tracheal rings given the location and the lack of knowledge as to the extent of the tumour. A non-fenestrated cuffed tracheal cannula was then introduced.

After securing the airway, an endoscopic examination of the trachea revealed bulging, quite fleshy mass located on the posterior tracheal wall, just below the vocal folds, obstructing approximately 90% of the tracheal lumen and extending 2.5 cm distally (figure 1A). The mass was actively and diffusively bleeding. Haemostasis by electrical bipolar cauterisation was performed, allowing us to control the bleeding; biopsies of the tracheal mass were then performed. After stabilisation, the patient was transferred to the intensive care unit for further investigations and management.

Figure 1. (A) Endoscopic view shows the tumour with highly narrowing of the tracheal luminal. (B) Axial CT scan with arterial phase shows the hypervascularity of the tumour. (C) Sagittal CT scan shows the distal extension of the tracheal tumour. (D) Post radiation therapy axial CT scan shows significant reduction of the tumour size.

Preventive intravenous antibiotic with co-amoxicillin 1.2 g, three times daily introduced and local care of the tracheal cannula was carried out regularly. Feeding was done through a nasogastric tube with oral feeding being gradually resumed.

Our institutional tumour board recommended surgical treatment with tracheal resection; the patient refused any form of surgical approach including an endoscopic one. Therefore, an alternative treatment of radiation therapy alone was initiated. The patient presented a second episode of massive bleeding from the tracheal tumour urging surgical haemostasis endoscopically and through the tracheotomy using bipolar cauterisation. The bleeding was controlled without compromising the airway.

Investigations

Radiological workup by CT showed a 25 mm posterior parietal mass of the posterior sub cricoid portion of the trachea uptaking contrast in the arterial phase as well as infiltration of the intertracheaoesophageal fat with mass effect on the oesophagus (figure 1B and C). There was neither pulmonary nor abdominal lesions nor any evidence of suspicious lymph nodes. A full blood workup did not show any abnormalities.

Histological analysis showed proliferation of the chorion in the form of small nests with relatively uniform, medium-sized cells and rounded to polyhedral cells containing homogenous, ovoid nuclei with fine chromatin. It was associated with numerous thin-walled vascular structures, lined by endothelial cells without atypia. The tumour cells containing a cytoplasmic frame well defined by reticulin were positive for actin and caldesmone with low expression for CD99. There was no expression for pancytokeratins, CD34, chromogranin, synaptophysin, S100 protein, SOX-10, desmin or inhibin. There also was no signs of malignancy. These histological findings were compatible with glomangioma subtype of glomus tumour (figure 2A–E).

Figure 2. Microscopic slides: (A) Solid proliferation of monotonous small round glomus cells with round central uniform nuclei. (B) Palely eosinophilic cytoplasm with sharply defined cell borders. (C) Distribution of cells around open vascular lumina and edematous surrounding stroma. (D and E) Immunopositivity for smooth muscle markers (alpha-actin and caldesmon), but not for pancytokeratin, neuroendocrine marker or S11 protein.

Differential diagnosis

Based on the radiologic findings, the main differential diagnosis of tracheal glomus tumour include carcinoid tumour and haemangiopericytoma. All three usually appear as well-circumscribed round masses that are relatively well enhanced on contrast images.⁵ The immunohistochemical findings allow contributing differential diagnosis⁶ with GTs being usually positive for smooth muscle actin and vimentin, but negative for neuroendocrine and epithelial markers, including S-100 protein, chromogranin, desmin, cytokeratins and factor VIII.

Treatment

The patient underwent volumetric intensity by arc therapy radiation at a total dose of 53 Gy over 25 sessions, with a frequency of five sessions per week. He was kept hospitalised during this period. The treatment was well tolerated without side effects or complications. He remained stable without either recurrence of bleeding or respiratory distress.

He was then followed on an outpatient basis with a favourable outcome and he was symptom free. The tracheostomy was removed 2 months after the end of the treatment. The various endoscopic and radiologic checks carried out at 3, 6, 9, 12, and 18 months after radiation therapy demonstrated a regression of more than 80% of the tracheal tumour with stability of the minor residual lesion (figure 1D). The patient remained asymptomatic and resumed his day to day activities. His follow-up is still ongoing.

Outcome and follow-up

The initial emergent management in the operating room allowed to secure the airway by an orotracheal intubation with small tube followed by surgical tracheotomy and control of bleeding.

The second episode of bleeding from the tracheal tumour required management in the operating room.

The diagnosis of tracheal glomangioma was retained.

Radiation therapy alone was initiated as the first line of treatment because the patient refused the surgery or any other endoscopic approach.

No side effects or complications related to radiation therapy were observed.

Control at 18 months post-treatment showed a significant reduction in the tumour size, stability of the minor residual tumour and no recurrence of symptoms. Follow-up is still ongoing.

Discussion

Glomus tumours (GTs), histologically, are aggregates, nests and masses of specialised glomus cells associated with branching vascular channels, all surrounded by connective tissue matrix. They are biologically benign, but often painful tumours⁷ and macroscopically characterised by round, slightly elevated, red-blue, firm nodules. GTs were first described by Masson in 1924, and the first case was reported in 1950.

The aetiology of these tumours remains unclear, and certain individuals attribute GTs to endocrine disorder, trauma or autosomal dominant inheritance. In addition to the classic three subtypes already mentioned, an oncocytic variant was reported and first described in 1990. Proper glomus tumours account for about three quarters of all glomus tumours, Glomangiomas constitute about one-fifth of them, glomangiomyomas account for less than 10% and the oncocytic sub-type consisted of the least common of them. The main histological characteristic of glomangiomas is that the glomus cell clusters are arranged around dilated venous vessels.⁸ Otherwise, for glomangiomyomas, there is a gradual transition from glomus cells to elongated, mature smooth muscle cells.

The WHO also classified glomus tumours into three categories: benign glomus tumours, glomus tumours of uncertain malignant potential and malignant glomus tumours, with the majority of reported glomus tumours being benign, a small proportion are histologically malignant. Malignant GTs are characterised by marked nuclear atypia and any level of mitotic activity; otherwise, uncertain malignant potential tumours do not fulfil the criteria for malignancy but have one or more atypical features other than nuclear polymorphisms. These features include a deep site location and a size greater than 2.0 cm.

Tracheal GTs are hypervascular tumours that grow concentrically, and to our knowledge, there have been approximately 70 cases reported to date, with half of these occurring in the past decade. They often occur in the fourth and fifth decades of life with a 2:1 male predominance ratio.⁹ Up to 10% of tracheal GTs are found incidentally, but patients are usually symptomatic with cough, dyspnoea and/or haemoptysis.¹⁰ Due to the abundant vascularity of these tumours, they appear on CT images as masses with avid contrast enhancement.

Management of tracheal GTs varies, and several adequate methods are found to be suitable; tracheal resection is the definitive curative treatment of choice, requires no further treatment and exhibits favourable prognosis, tracheal stenting through bronchoscopy or laser resection with or without adjuvant radiotherapy. Endoscopic intervention alone was also carried out in a limited number of cases, which filled rigorous indications, but the possibility of tumour recurrence after endoscopic removal is higher than that of complete surgical removal.¹¹ The main cause of mortality reported in the literature following surgery has not been attributed to the primary tumour or to the procedure but to complications.

Glomangioma of the trachea is extremely rare, and to our knowledge, only five cases have been previously reported. In our case, the tumour was located in the upper part of the trachea, which is unusual because these tumours have always previously been located in the distal part of the trachea. The initial emergency management was extremely difficult and challenging considering the respiratory distress combined with the massive bleeding. Radiation therapy alone allowed a significant reduction in the tumour with stability of the minor residual lesion as well as a full control of the symptoms at the 18 month follow-up.

The surgical resection represents the main and curative treatment for tracheal GTs, and to our knowledge, radiation therapy alone has never been initiated before as an initial and first line of management. This therapy could be an alternative in elderly patients or those who refuse surgery. Even if this treatment is not curative, it allows significant reduction in the tumour with long-term and sufficient control of symptoms.

Learning points.

• Tracheal glomus tumour is an extremely rare entity.

• Airways obstruction associated with bleeding causes a challenging and life-threatening condition.

• Early diagnosis and adequate management are crucial for prognosis.

• Surgical removal of the tumour is the first choice of treatment.

• Radiation therapy could be an alternative but not as curative course of treatment.