Inflammatory Myofibroblastic Tumor of the Supraglottis: A Rare Entity

Abstract

Inflammatory myofibroblastictumour of larynx is a rare and an unexplored area. Though benign, it has capabilities to transform into malignancy and also has a very high recurrence rate. The purpose of this paper is to shed light on treatment modality for IMT of the supraglottis. As per our experience, wide excision of the tumor with coblation is not an ideal treatment. Hence more research needs to be done to explore better and effective treatment for this tumor.

Introduction

Inflammatory myofibroblastictumour (IMT) are extremely rare in the larynx with approximately 41 published cases. Most commonly it affectslung, liver, gastrointestinal tract (omentum and mesentery) and the central nervous system. It is known by many other terms, including plasma cell granuloma, plasma cell pseudotumor, xanthomatous pseudotumor, pseudosarcomatousmyofibroblastic proliferation, inflammatory myofibrohistiocytic proliferation, inflammatory fibrosarcoma, and, most commonly, inflammatory pseudotumor [1–3].Spindle cell lesions of the larynx are an unusual occurrence (1.3%) [4].Rarely when involving the larynx, laryngeal IMT most commonly involves the true vocal cord [5].

Here we present a case of inflammatory myofibroblastic tumor of the aretynoids and aryepiglottic fold in an adult in 3rd decade of life. To the best of our knowledge, no such case has been documented in the literature.

Case Report

A 34 years old female came to our OPD with complaints of change in voice and dysphagia (solids >  > Liquids) since 4 month.

On Examination, throat, nose and bilateral ear were normal. In neck, no palpable lymph nodewere present. And on auscultation, rhonchi was present bilaterally. Patient was advised fibre-optic laryngoscopy to evaluate the larynx.

On Fibre-optic laryngoscopy, a smooth mass arising from right Aryepiglottic fold was seen. It was completely occupying the laryngeal inlet. The mass was extending from the Right aryepiglotticfold, right ventricular fold, epiglottis, till the left vocal cord.Left vocal cord was partially visible and was found to be mobile. The right true vocal cord, right side cuneiform, corniculate, arytenoid and inter-arytenoid fold were not visible. (Fig. 1).

Fig. 1.

Supraglotttic growth on right side

Due to the distinct look of the mass, the patient was advised both CECT and MRI Neck for proper evaluation.On CECT NECK—Nodular mass lesion was seen on right aryepiglottic fold measuring 16 mm × 21 mm. The mass was endophytic and protruding in the lumen. Partial luminal narrowing was seen. Mass was hyper enhancing and was suggestive of Schwannoma/Haemangioma/ Mitotic pathology. MRI NECK (PLAIN + CONTRAST) stated that a well defined lobulated endophytic homogenously enhancing soft tissue signal intensity mass lesion seen in right side supraglottis predominantly involving right aryepiglottic fold and false vocal cord. This mass lesion was causing compression of right pyriform fossa and superior laryngeal narrowing. It was predominately arising from posteromedial surface of right aryepiglottic fold and bulging into the right vallecula superiorly and compressing the right paraglottic space. It was abutting the inferior surface of right side epiglottis. It measures approximately 1.7 × 2.3 × 2.1 cm. (Fig. 2 and 3).

Fig. 2.

MRI NECK(PLAIN) in coronal section showing a large supraglottic growth

Fig. 3.

MRI NECK(PLAIN) in axial section showing a large supraglottic growth

The patient was planned for Microlaryngeal surgery under GA. Patient was intubated under C-MAC video laryngoscope by cardiac anesthesiologist (Fig. 4). Multiple biopsies were taken. Mass was firm in consistency and did not bleed on touch. It was completely adherent to the right A-E fold and right arytenoid and was removed with coblationprocizeMLW laryngeal wand at the setting ofcoblate—7 and coag- 3. Post—operative period was uneventful.

Fig. 4.

Showing endoscopic picture

Histopathology report was suggestive of benign spindle cell neoplasm in favour of nerve sheath origin.On Immunohistochemistry, cells expressed ALK (cytoplasmic) and SMA (weak) and were immune negative for CD34, S100, Desmin and SOX-10. Immunophenotype was suggestive of inflammatory myofibroblastic tumor.

Patient was under regular follow up. After 1 month of surgery, recurrence of the mass was seen which almost attained its pre-op size on gradual follow-up. Patient refused any further surgical or medical management and did not follow up after that.

Discussion

The most common location of the IMT is the lung, followed by soft tissue and viscera. IMT of the head and neck, especially of the larynx, are rare [6].

In the larynx Wenig et al. identify the true vocal fold as the most common site of presentation[5]. In another study by Guilemany et. al, laryngoscopy showed an exophytic tumor located in the right true vocal cord [7].

In a study by Alaani et. al, he stated that direct rigid laryngoscopy showed a right subglottic mass with a normal glottis and supraglottis which was on HPE diagnosed as IMT post-operatively [8].

Inflammatory myofibroblastic tumor (IMT) attached to the right aryepiglottic fold was seen in a study by Rodrigues et. al in a 2 year old boy [9].

This predilection of glottis involvement can be credited to the voice trauma. There has been studies demonstating subglottic IMT as well. Supraglottic IMT is a rare entity and only seen in pediatric population till date.

Surgical excision is the most common management approach reported in the literature [10]. Other treatment options, however, have been described, including radiotherapy and laser ablation [11, 12].

Martinez et. al in a study stated that Conservative excision is the appropriate treatment because the general behaviour of IMT is benign[13].

On the contrary, In a study by Volker et. al, laryngoscopy showed a polyp (0.8 cm) of the right vocal fold which was diagnosed as inflammatory myofibroblastic tumor. Wide local excision was done. Recurrence occurred within 10 days of resection [14].

Similarly, a 57-year-old Italian man reported a 5-month history of progressive hoarseness, shortness of breath, and intermittent dysphonia. The lesion was completely excised, and histological examination was performed. The patient was referred months later for the same symptoms [15].

Also in a study by Rodrigues et. al, recurrence was seen in 7 months after microlaryngeal resection of the lesion [9].

Recurrence or persistence of disease and the need for further procedures are potential problems and has been already been acknowledged in the literature. In a study by Wenig et. al, Follow-up surgery included total laryngectomy[5].

The mischievous nature of this tumor was seen when recurrence occurred post radical surgery as well. In a study by Marioni et. al, Right hemilaryngectomy extending to the anterior third of the left vocal fold, was performed using an endoscopic CO2 laser. Three months after surgery, recurrence involving the larynx at the level of the posterior third of the right vocal fold was seen[4].

A transformation of an IMT to higher malignancy is possible, especially in cases with repeated recurrences[14]. This also does not favour the debate that conservative approach is appropriate.

Conclusion

Recurrence was seen 1 month later in our patient post excision and hence we advocate against the use of coblation or conservative surgical excision as the effective treatment of choice.Here we shed some light on an ineffective treatment modality so when in future such cases are encountered, it is well established what not to do. No set guidelines are present in the literature on management of laryngeal IMTs owing to their such rare occurence.Newer and better treatment modalities are yet to be explored to tackle the notorious nature of laryngeal IMTs.

Funding

Not applicable.

Data Availability

Can be made available at request.

Declarations

Conflict of interest

Not applicable.

Consent for Publication

Informed consent has been taken from the patients and can be made available at request