CASE REPORT |
https://doi.org/10.5005/jp-journals-10023-1257 |
Inflammatory Myoblastic Tumor of Larynx and Trachea: A Case Report
1,2,5Department of Laryngology, Ananthapuri Hospitals and Research Institute (AHRI), Thiruvananthapuram, Kerala, India
3,4Department of Pathology, Ananthapuri Hospitals and Research Institute (AHRI), Thiruvananthapuram, Kerala, India
Corresponding Author: Vishnu Vinayakumar, Department of Laryngology, Ananthapuri Hospitals and Research Institute (AHRI), Thiruvananthapuram, Kerala, India, Phone: +91 9539391560, e-mail: vvk1290ent@gmail.com
Received on: 23 April 2024; Accepted on: 18 June 2024; Published on: 15 November 2024
ABSTRACT
Inflammatory pseudotumor is a benign, tumor-like lesion of reactive nature. Its basic morphologic characteristic is spindle cell (myoblasts and fibroblasts) proliferation with a variable number and type of inflammatory cells. The newer terminology is inflammatory myoblastic tumor. Although they are mostly reported in the lung parenchyma, tracheal involvement is not unknown. In the past, it was considered benign, but with recent evidence of local recurrence after surgical resection, cases of metastatic spread, and advances in cytogenetic analysis, they are now categorized as tumors with malignant potential. This paper aims to describe a rare case of laryngotracheal inflammatory myoblastic pseudotumor in a young female who had to undergo tracheostomy due to acute airway obstruction, after which the patient underwent complete excision of the tumor along with expansion laryngoplasty.
How to cite this article: Vinayakumar V, Issac ME, Bahuleyan A, et al. Inflammatory Myoblastic Tumor of Larynx and Trachea: A Case Report. Int J Phonosurg Laryngol 2024;14(2):37-39.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.
Keywords: Benign tumors of trachea, Case report, Histopathology, Hypopharynx, Inflammatory myoblastic tumor, Inflammatory pseudotumor, Larynx.
INTRODUCTION
Inflammatory myofibroblastic tumor (IMT) was first reported in the lungs. Later, it was reported in various sites outside the lungs as well.1 In the head and neck, it is more commonly seen in the maxillary and ethmoid sinuses and has also been described in the orbital tissue, faucial tonsils, ears, gums, pterygopalatine fossa, and tissue around the teeth. Laryngeal and tracheal involvement is very rare. Only a few cases of inflammatory myoblastic tumor in the laryngeal and tracheal area have been reported previously.2-5
We report a case of inflammatory myoblastic tumor of the upper airway involving the larynx and trachea, and describe the clinical presentation, radiological findings, and successful decannulation of the patient after surgical excision of the tumor followed by expansion laryngoplasty.
CASE DESCRIPTION
A 21-year-old female patient presented with complaints of dyspnea 5 months postdelivery. She had to undergo a tracheostomy for the same. She took antituberculosis treatment (ATT) for 6 months as it was a suspicious case of tuberculosis (TB). An endoscopic biopsy was taken, which gave the histopathological examination (HPE) result as leiomyoma. The patient underwent video laryngoscopy (VLS) followed by examination under anesthesia (EUA), which showed a large vascular mass attached to the subglottis (Fig. 1). Contrast-enhanced computed tomography (CECT) of the neck revealed a well-demarcated soft tissue density lesion in the subglottis, extending toward the anterior wall up to the 4th tracheal ring, causing total luminal occlusion of the trachea (Figs 2A to C).
Fig. 1: Videolaryngsocopy image showing large vascular mass attached to subglottis
Figs 2A to C: (A) Coronal section; (B, C) Sagittal section of Contrast-enhanced computed tomography showing heterogenous lesion involving subglottis and trachea
The patient underwent complete excision of the mass, followed by expansion laryngoplasty with the help of a costochondral graft (Fig. 3). A horizontal skin crease incision was made after inducing general anesthesia (GA) through the flexometallic tube introduced through the tracheostomy site, with the patient positioned supine. A horizontal skin crease incision was made between the cricoid and the first tracheal ring. Thyroid isthmusotomy was performed. The mass was seen attached to the left inner aspect of the lateral wall of the cricoid and extending to the first three tracheal rings. A midline incision was made on the cricoid. The involved rim of the cricoid cartilage was removed along with the mass and the attached part of the first, second, and third tracheal rings. The mass was seen infiltrating outside through the tracheal rings. A costochondral graft was harvested from the 8th rib on the right side and used to reconstruct the defect with the help of 3-0 vicryl and tissue glue. A corrugated rubber drain was placed and removed on postoperative day 3 (POD3). Tracheostomy care was provided. The HPE report came back as an inflammatory myoblastic tumor of the larynx (Fig. 4). The patient was decannulated after 1 month, as the videolaryngoscopic examination showed an adequate airway and no remnants of the disease. On follow-up after 6 months, there was no evidence of any recurrence seen on videolaryngoscopic examination.
Fig. 3: Mass seen attached to left inner aspect of lateral wall of cricoid and extending to first three tracheal rings
Fig. 4: Histopathological examination showed myofibroblastic cells on a background of inflammatory cells suggestive of inflammatory myoblastic tumor
DISCUSSION
Inflammatory myofibroblastic tumor is a characteristic borderline tumor containing myofibroblastic cells with a varying mixture of other inflammatory cells such as mature lymphocytes, histiocytes, plasma cells, eosinophils, and collagen. It is seen more frequently in pulmonary tissue and other internal organs, but it can also occur in the head and neck.6 In the head and neck region, IMTs are predominantly seen in the larynx, most commonly in the area of the true vocal cord. Nonlaryngeal sites of IMT include the mouth, tonsil, parapharyngeal space, periodontal tissue, ears, orbit, gums, salivary gland, paranasal sinuses, and trachea. Tracheal IMTs account for <1% of all respiratory tract tumors.7 There are a few reports of tracheal IMT in the pediatric age group, but in adults, it is extremely rare. IMTs have been reported in various locations throughout the body, with pulmonary tissue being the most commonly involved site.8,9
Different etiologies have been postulated for IMT, which include chronic infections, autoimmune diseases, and trauma.10 Autoimmune diseases, like immunoglobulin G4 (IgG4) disease, have also been recently associated.11,12 In certain patients, a rapidly progressing disease with metastases has been described.10,13,14
There are reports proposing a link between IMT occurrence and multiple translocations involving 2p23, specifically the anaplastic lymphoma kinase (ALK) gene, suggestive of its neoplastic potential.8 It is more frequently seen in IMTs of the pediatric population and young adults but is unheard of in individuals over 40 years old. This gene rearrangement causing enzyme stimulation is confined to the myofibroblasts, while the inflammatory cells remain normal.6
Immunohistochemistry (IHC) of T and B cell subpopulations, CD34, CD117, S-100, and c-Kit helps differentiate IMTs from other neoplasms.15,16 At the molecular level, about half of IMTs contain a clonal cytogenetic anomaly that activates the ALK. Positive IHC staining for ALK occurs in almost 40–100% of these tumors.15-17 Muscle-specific actin, vimentin, and smooth muscle actin are seen in IMTs, along with desmin and cytokeratins.18
Laryngeal IMTs present with symptoms such as voice change and noisy breathing. Sometimes, a feeling of a lump in the throat may be the only symptom. Other symptoms and signs including pyrexia (fever), cachexia (weight loss), body ache, malaise, anemia, increase in platelets, polyclonal hyperglobulinemia, and elevated erythrocyte sedimentation rate (ESR), though seen in cases with soft tissue and visceral IMTs, are not usually associated with aerodigestive tract IMTs.6
Inflammatory myofibroblastic tumors have a distinct pathological pattern characterized by myofibroblastic cells surrounded by inflammatory cells. These myofibroblastic cells appear as submucosal, loose, cellular proliferation of spindled to stellate cells arranged in a storiform to fascicular pattern. The inflammatory infiltrate consists of mature lymphocytes, histiocytes, plasma cells, eosinophils, and scattered neutrophils. The stroma is highly vascular and varies from edematous to fibromyxoid to collagenous. The vascular component ranges from widely dilated medium-sized blood vessels to narrow, slit-like vascular channels that can be compressed by the myofibroblasts and inflammatory cells.6
The classification by Coffin divided it into three distinct types: (1) hypocellular; (2) hypercellular; and (3) collagen sheets pattern. In the hypocellular type, myofibroblasts are arranged loosely amidst a background of inflammatory cells such as plasma cells, lymphocytes, and eosinophils. The second type shows densely aggregated, elongated, short spindle cells or stellate cells arranged on a collagenized background. In the third type, the cellular component is loosely arranged, resembling scar tissue.5,19,20
Conservative surgical resection can often achieve complete clearance of laryngeal IMTs. Symptom relief may be achieved in some patients with the use of corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs). Approximately, one-fourth of extrapulmonary IMTs are reported to recur.6 Laryngeal IMTs have a recurrence rate of 8–18%. Recurrence is typically observed within 2–12 months after initial treatment. In cases of multiple recurrences, laryngectomy may be considered a treatment option.21
CONCLUSION
Inflammatory myofibroblastic tumor is a rare entity in the trachea, especially in adults. Management depends on preoperative diagnosis aided by endoscopy and radiological evaluation. IHC is essential to arrive at an accurate diagnosis. For limited cases, endoscopic resection can be performed. However, if the skeleton is involved, treatment includes resection of the affected part of the skeleton with expansion laryngoplasty using a costochondral graft. Serial follow-up for two years is necessary as recurrence is known to occur in 8–18% of cases.
ORCID
Vishnu Vinayakumar https://orcid.org/0009-0004-0212-2367
Manju E Issac https://orcid.org/0009-0002-6990-8589
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