Myxoma is a benign tumour with a propensity for local infiltration and recurrence. Laryngeal myxoma presents as a submucosal polyp. Being an uncommon tumour and mimicking vocal cord polyp, only anecdotal evidence is available in the literature. The literature was reviewed from 1986 onwards using the keywords “myxoma” and “larynx.” The databases used were PubMed, Google Scholar, Scopus, and Web of Science. Along with this, we also report our case of vocal fold myxoma. We found a total of 19 studies reporting laryngeal myxoma. Laryngeal myxoma typically affects males in the 6th decade with a history of smoking. Unlike myxomas originating outside the larynx, recurrence is not widely described, and microlaryngeal surgery will usually suffice. Laryngeal myxomas should definitely be kept in the list of differential diagnosis when dealing with a benign-looking vocal fold lesion.
Myxomas are a rare benign myxoid neoplasm of mesenchymal origin. They are a heterogeneous group of soft tissue neoplasms with a variable degree of invasiveness, ranging from benign to highly aggressive forms [
A 53-year-old male presented with hoarseness of 12-year duration. He gave no history of breathing or swallowing difficulty. On enquiring further, he had complaints related to gastric acid reflux. He was a smoker but had quit smoking 6 months back. He is a politician with a history of voice abuse. On flexible fibreoptic evaluation, there was a 0.5 cm polypoidal, cystic mass pedicled on the medial free edge of the middle 1/3 of the right true vocal fold. There was no abnormality of vocal fold mobility. Rest of the ENT examination was normal.
Based on a history of long-standing hoarseness, voice abuse, and presence of a solitary polypoidal lesion over the true vocal fold, a preoperative diagnosis of a laryngeal polyp was made. No preoperative radiology was taken due to the unambiguous nature of the clinical findings. The patient was taken up for microlaryngeal surgery (MLS), and the lesion was excised with cold instruments. Postoperative period was uneventful with patient reporting near-normal voice during first follow-up after one week. Surprisingly, the postoperative histology showed features consistent with laryngeal myxoma.
On histological examination, our case showed a polypoidal tumour lined by hyperplastic stratified squamous epithelium (Figure
Histopathology. (a–c) A polypoidal lesion lined by hyperplastic stratified squamous epithelium. Subepithelium shows a paucicellular mesenchymal tumour comprising small, bland, spindle, or stellate-shaped cells with small and hyperchromatic nuclei and inconspicuous cytoplasm dispersed in an abundant myxoid stroma. Cellular pleomorphism, mitotic figures, and necrosis are absent. (e–f) Results of immunohistochemistry with CD34, SMA, and S-100. The tumour cells are immunonegative for CD34, smooth muscle actin (SMA), and S100 ((a) HE 40x, (b) HE 100x, (c) HE 200x, (d) CD34 200x, (e) SMA 200x, and (f) S100 200x).
We did a comprehensive review of all the anecdotal cases of laryngeal myxoma reported in the literature so far which is summarized in Table
Systematic review of all cases of laryngeal myxoma.
Author (year) | Number of cases | Age/sex | Presentation | Addiction | Association | Site | Size | Approach | Follow-up | Histology | Need for tracheostomy |
---|---|---|---|---|---|---|---|---|---|---|---|
Tang et al. (2015) [ |
1 | ND | Dysphonia | ND | — | Glottis | ND | Two approaches | ?Recurrence | — | |
Ritchie et al. (2015) [ |
1 | 77/M | Hoarseness 6 months | Smoker | Reinke’s edema | Glottis | <1 cm | MLS | 3 months, NED | Stellate cell, spindle cell, and mucinous matrix. CD 34−, S100+, and SMA+ | — |
Singh et al. (2014) [ |
1 | 65/M | Hoarseness 4 months and dyspnoea 1 week | ND | — | Glottis | 17 × 12 mm | MLS | 8 months, NED | -do- | Tracheostomy |
No IHC | |||||||||||
Shah et al. (2014) [ |
1 | 50/M | Hoarseness 2 months | ND | — | Glottis | — | MLS | No f/u | -do- | — |
No IHC | |||||||||||
Garca et al. (2013) [ |
1 | 61/M | Hoarseness 3 months | Smoker | — | Glottis | 0.5 × 0.5 cm | MLS | No f/u | -do- | — |
CD 34−, S100−, desmin−, and SMA− | |||||||||||
Kanlıada et al. (2012) [ |
1 | 42/f | Hoarseness 3 months | — | — | Glottis | 4 mm | MLS | 12 months, NED | -do- | — |
No IHC | |||||||||||
Nakamura et al. (2008) [ |
1 | 74/f | Hoarseness | Alcohol | — | Glottis | 4 mm | MLS | NED | -do- | — |
No IHC | |||||||||||
Song et al. (2008) [ |
1 | 36/M | Hoarseness 2 months | — | — | Glottis | 7 mm × 5 mm | MLS | 4 months, NED | -do- | — |
No IHC | |||||||||||
Ali et al. (2008) [ |
1 | 48/F | Hoarseness childhood | Smoker | Reinke’s edema | Glottis | — | MLS | — | -do- | — |
CD34+, S100−, and SMA−,Ki-67+ | |||||||||||
Leu et al. (2007) [ |
1 | 53/m | Hoarseness 2 years | — | — | Glottis | 5 mm | MLS | — | -do- | — |
No IHC | |||||||||||
Kim et al. (2007) [ |
2 | 62/m | Hoarseness 5 years | Smoker | — | Glottis | 1.5 × 0.8 × 0.4 cm | MLS | 8 years, NED | -do- | — |
No IHC | |||||||||||
52/M | Hoarseness and dyspnoea 3 weeks | Smoker | — | Glottis | 2 × 0.4 × 0.4 cm | MLS | 3 years, 6 months, NED | -do- | — | ||
NED | |||||||||||
Idrees et al. (2005) [ |
1 | 46/M | — | Smoker | — | Glottis | 8 mm | MLS | — | -do- | — |
No IHC | |||||||||||
Baruah et al. (2001) [ |
1 | 57/M | Hoarseness | — | — | AEF/epiglottis | 6.5 × 5.0 × 1 cm | MLS | ND | -do- | — |
No IHC | |||||||||||
Kim et al. (1997) [ |
1 | 62/M | Dyspnoea 3 days | Smoker, alcoholic | Reinke’s edema | Glottis, obstructing airway | 2.5 × 2.5 × 1.5 cm | MLS | — | -do- | Tracheostomy |
No IHC | |||||||||||
Tsunoda et al. (1997) [ |
1 | 57/m | Hoarseness | Alcoholic | — | Glottis | 7 × 7 × 7 mm | MLS | NED | No IHC | — |
Hadley et al. (1994) [ |
1 | 64/M | Hoarseness 4 years | Alcoholic, smoker | — | Glottis | 1 × 0.6 × 0.2 cm | MLS | 18 months, NED | No IHC | — |
Sena et al. (1991) [ |
1 | 70/M | Hoarseness | Alcoholic, smoker | — | AEF | 0.5 × 0.5 × 2.5 cm | Transcervical | 12 months, NED | No IHC | — |
Chen and Ballecer (1986) [ |
1 | 37/M | Dysphonia and dysphagia | — | — | Epiglottis | 5.6 × 4.3 × 2.4 cm | MLS | 12 months, NED | No IHC | — |
Present study (2016) | 1 | 53/M | Hoarseness 12 years | Smoker | — | Glottis | 0.5 cm | MLS | 4 months, NED | Immunonegative for CD34, SMA, and S100 | — |
Larynx as a site for head and neck myxoma is extremely rare. Our comprehensive literature search revealed 19 cases of laryngeal myxoma till date (Table
Although we did not find any systemic association, at least three studies have revealed an association with Reinke’s edema [
Demographically, laryngeal myxomas differ from other head and neck myxomas. A comprehensive review on head and neck myxoma by Andrews et al. [
Various theories for its etiopathogenesis have been put forth. Due to the abundance of mucinous matrix and glycosaminoglycans, fibroblast immaturity has been cited as a factor [
Histopathology forms the mainstay for diagnosing this entity. The usual clinical appearance is that of a benign vocal fold lesion like an intracordal cyst, polyp, or a nodule. It is the presence of myxoid stroma and abundance of stellate cells and spindle cells that help in clinching the diagnosis. There is no general consensus regarding myxoma-specific immunohistochemistry markers. All studies (Table
Head and neck myxomas are known for locally infiltrative nature and tendency to recur if not excised radically [
Laryngeal myxomas should definitely be kept in the list of differential diagnosis when dealing with a benign looking vocal fold lesion. Patients need to be kept on close follow-up if excision has been suboptimal due to the absence of capsule and locally infiltrative nature.
The datasets generated or analysed during this study are available from the corresponding author on reasonable request.
The authors declare that they have no conflicts of interest.