Unmasking Laryngeal Spindle Cell Carcinoma Presenting as a Benign Laryngeal Lesion: Challenges in Diagnosis and Therapeutic Decision-Making
Piriya Darishini Renganathen 1, Nik Fariza Husna Nik Hassan *1, Dinie Qistina Rajalie 3,
Nurul Anis Mohd Fauzi 2, Irise Hoi Khin Chen 3, R Komathi S Ramachandran 2, Zakinah Yahaya 3
*Correspondence to: Nik Fariza Husna Nik Hassan, Department of Otorhinolaryngology Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia, Kelantan, ORCID: 0000-0001-6335-1631.
Copyright.
© 2026 Nik Fariza Husna Nik Hassan, This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received: 18 August 2026
Published: 01 September 2026
DOI: https://doi.org/10.5281/zenodo.22225403
Abstract
Spindle cell carcinoma (SpCC) is a rare and aggressive variant of squamous cell carcinoma, constituting approximately 0.5% of all squamous malignancies and up to 3% of head and neck cancers, with laryngeal involvement being particularly uncommon (1). It predominantly affects males in their mid-fifties and is strongly associated with tobacco and alcohol exposure, poor oral hygiene, and prior irradiation (2). Our case demonstrated an a single lobulated and pedunculated smooth-surfaced huge mass producing a ball-valve effect with near total obstruction of the laryngeal airway. The gross mass appearance was more of a benign lesion, the urgency of the tumour excision was for airway urgency rather than suspicious of malignancy. A meticulous histopathological and immunohistochemical evaluation revealed SpCC as it exhibits a biphasic architecture with a minor squamous component and a predominant malignant spindle cell element showing fasciculate growth (4). Immunoprofile demonstrating cytokeratin and epithelial membrane antigen (EMA) positivity, along with cytokeratin–vimentin co-expression in spindle cells, support a monoclonal epithelial origin with sarcomatoid differentiation (5). Prognosis for this pathology is generally poorer than that of laryngeal squamous cell carcinoma furthermore it has a higher rate of local recurrence. The prognosis is also influenced by tumour subsite, stage, and size (6). Surgical excision remains the primary treatment due to limited responsiveness to radiotherapy and chemotherapy, although combined surgery and radiotherapy offers the most favourable outcomes, with radiotherapy reserved mostly for recurrent disease following salvage procedures (7).
Keywords: Spindle cell carcinoma; Sarcamatoid carcinoma; Laryngeal carcinoma; Squamous cell carcinoma; Immunohistochemistry.
Abbreviations
SpCC - Spindle cell carcinoma
EMA - Epithelial membrane antigen
GRBAS - Grade, Roughness, Breathiness, Asthenia, and Strain scale
CECT - Contrast-enhanced computed tomography
Cm – Centimetre
SATB2 - Special AT-rich sequence -binding protein 2
Case Report
A 52-year- old gentleman with underlying hyperlipidemia presented to emergency department with history of shortness of breath for two weeks, which was worsening for two days. The patient also complained of dysphagia, worsening hoarseness and multiple episodes of hemoptysis for the past two months. The patient did not have any ear or nasal complaints, and was neither a smoker nor alcohol consumer. Patient working as chef in restaurant using fire wok. Upon review in the emergency, patient appeared in significant respiratory distress with biphasic stridor and hoarseness GRBAS grade 3 with the main component of breathiness. Flexible nasopharyngolaryngoscope done showed a single lobulated smooth-surfaced, pedunculated mass arising from anterior commissure, with near total occlusion of the glottic airway, the pedunculated mass moved up and down with respiration, giving a ball valve effect. No prominent vessels were seen over its surface. Other examination findings were unremarkable. Tracheostomy was performed under local anesthesia to secure the airway and proceeded with excision biopsy of the laryngeal mass.
This was followed by contrast-enhanced computed tomography (CECT) 1mm per slice of the neck, thorax , abdomen and pelvis describing ill- defined heterogeneously enhancing soft tissue lesion at the anterior commissure region measuring approximately 0.7centimere (cm) x 0.5cm x 0cm causing narrowing of glottis lumen with no sign of invasion, and no neck nodes involvement no distant metastasis reported which brings to TNM staging of T2N0M0 in stage 2. The histopathological examination reported spindle cell carcinoma with osteosarcomatous differentiation. The tumor cells were positive for Vimentin and Special AT-rich sequence -binding protein 2 (SATB2) stains. Focal staining noted for Smooth muscle actin (SMA) and weak staining for P63 (<10%). Weak staining for MNF 116, EMA, Cytokeratin 5/6, p40.
The decision was then made for the patient to undergo a total laryngectomy with bilateral neck dissection followed by post operative radiotherapy of 60Gy in 30 fractions. The postoperative course was uneventful an the patient recovered well. At the latest follow up following 6 months after completion of therapy , patient remains clinically free of disease with no evidence of local or regional recurrence.
Discussion
Spindle cell carcinoma (SpCC) also referred as sarcamatoid carcinoma represents a rare and distinctive variant of squamous cell carcinoma, representing 0.5% of all squamous cell carcinoma (1). Spindle cell squamous cell carcinoma accounts for approximately 2%–3% of all head and neck malignancies and less than 1% of laryngeal cancers in the adult population (2). SpCC predominantly affects males, with a mean age of presentation around 57 years triggered by risk factors including chronic tobacco and alcohol use, poor oral hygiene, and a history of prior irradiation at the affected site (3).
The clinical presentation of laryngeal SpCC is variable and is largely dependant on anatomical subsite and extent of airway involvement. Hoarseness is the most frequently reported presenting symptoms attributable to the predilection of the tumour involvement in the glottic region (4). Other reported manifestation include dysphagia, dyspnoea, haemoptysis and symptoms related to progressive airway compromise. In the present case, patient experienced progressive hoarseness and dysphagia followed by rapidly worsening dyspnoea over 2 weeks. The clinical deterioration culminated in acute respiratory distress with biphasic stridor, necessitating urgent airway intervention.
A particularly distinctive feature of the present case was the gross appearance of the tumour. The initial endoscopic evaluation revealed a solitary, smooth-surfaced, pedunculated polypoidal mass arising from anterior commissure, obstructing the glottic airway.
The mass moved synchronously with respiratory, producing a pronounced ball valve effect (5). Such a freely mobile pedunculated lesion may clinically suggest benign laryngeal polyp, particularly in the abscense of ulceration or irregular surface. Such an atypical presentation elevated clinical suspicion for a benign aetiology, presenting significant diagnostic and therapeutic challenges. In this setting, the immediate clinical priority was airway stabilisation rather than definitive oncological assessment.
SpCC is characterized histologically by the coexistence of conventional squamous epithelial differentiation and a malignant spindle-cell proliferation. In most cases, the spindle-cell component represents the dominant morphological feature, while the conventional squamous component is relatively limited. The spindle cells may demonstrate a loose, wispy or fascicular arrangement, consistent with the histological findings in the present case (6). This morphological spectrum is clinically important because a limited biopsy may sample exclusively spindle cell component and may lead to misdiagnosis. The epithelial nature and proposed monoclonal origin of the spindle-cell component are further supported by immunohistochemical findings, with expression of epithelial markers including cytokeratin and EMA. In addition, dual-immunolabelling studies have demonstrated co-expression of cytokeratin and vimentin within the same spindle cells, supporting their epithelial derivation despite their sarcomatoid morphology (7).
The prognostic behaviours of laryngeal SpCC is generally poorer than that of conventional laryngeal squamous cell carcinoma (SCC), owing to its highly aggressive biological behaviour. Spector et al. reported that recurrence is influenced by several clinicopathological variables, including tumour subsite, stage and size (8). The aggressive clinical behaviour has historically been attributed to the sarcomatoid phenotype and the potential for early local and distant dissemination. In our patient, the absence of radiologically evident cervical nodal disease and distant metastasis at presentation was favourable, although the large tumour burden and unusual histological differentiation warranted definitive oncological management.
Surgery stays as the mainstay treatment for SpCC, particularly for localised disease and lesions causing significant airway compromise. According to Dubal Et Al, best prognosis was seen in cases of which underwent both surgery and radiotherapy. Radiotherapy alone did not provide any significant survival rate and poses least favourable outcomes (4). However radiotherapy is suggested as adjuvant option for patients who experience disease recurrence after salvage surgery (9).
Conclusion
Laryngeal SpCC remains a diagnostically challenging and clinically aggressive malignancy that may mimic benign airway lesions, underscoring the importance of maintaining a high index of suspicion in atypical presentations. Precise diagnosis hinges on comprehensive histopathological and immunohistochemical assessment to distinguish its characteristic biphasic architecture and confirm its epithelial origin. Given its propensity for local recurrence and poorer prognosis compared to laryngeal squamous cell carcinoma, early surgical intervention remains the cornerstone of management, with adjuvant radiotherapy offering benefit primarily in recurrent or high-risk cases. Continued reporting of atypical presentations and outcomes is essential to refine diagnostic accuracy and optimize therapeutic strategies for this rare but clinically significant entity.
Acknowledgements
The authors wants to express their heartfelt gratitude all institutions and individuals whom played a major role in producing this case report.
Conflict of Interest
The authors have declared that there are no conflict of interests.
References