Keywords
sarcomatoid - spindle cell - vocal cords - microlaryngoscopy - radiotherapy
Introduction
Carcinoma of the larynx is one of the common malignancies in India. The most common
histological type of laryngeal carcinoma is squamous cell carcinoma (SCC). Sarcomatoid
or spindle cell carcinoma is the rarest variant of SCC, which comprises 1.3% of it.[1] Sarcomatoid carcinoma is considered the most malignant among laryngeal carcinoma.[2] Because it is an uncommon variety, it is often misdiagnosed as reactive lesions
or mesenchymal malignancy.[3] Sarcomatoid squamous cell carcinoma is the most commonly noted at the level of the
glottis.[4] It is a biphasic tumor due to its epithelial and mesenchymal components.[5] Here, we present a patient diagnosed with sarcomatoid squamous cell carcinoma of
the right vocal cord in the early stage and was treated successfully.
Case Report
A 78-year-old male patient presented to the department of ENT with a history of change
in voice for the last 2 months, which progressed over the previous month. He is a
chronic smoker with 110 pack-years of smoking. There was no history of chronic cough,
weight loss, fever, voice trauma, hemoptysis, or difficulty breathing. He did not
have any other comorbidities. The external laryngeal framework was normal on physical
examination, and there was no cervical lymphadenopathy. On videolaryngoscopy, an exophytic
lesion was noted in the anterior one-third of the right vocal cord ([Fig. 1]). Bilateral vocal cords were normal and mobile.
Fig. 1 Videolaryngoscopic image showing an exophytic lesion in the right vocal cord.
As a part of further investigation, a contrast-enhanced CT scan of the neck was done,
which showed mild asymmetrical thickening of the right true vocal cord measuring 6 mm
when compared with the left side of size 3.5 m ([Fig. 2]). The rest of the larynx and paraglottic space seems to be normal. The postcricoid
region, esophagus, thyroid gland, parotid, submandibular salivary glands, and parapharyngeal
spaces were normal. There was no evidence of cervical lymphadenopathy.
Fig. 2 CECTNeck (A-axial; B-coronal) showing asymmetrical thickening of the right vocal cord.
We performed micro laryngeal surgery, which showed a firm exophytic lesion with a
narrow stalk in the anterior one-third of the right true vocal cord without extending
to the anterior commissure. The lesion was excised and sent for histopathological
examination. Microscopy showed squamous cells in nests and sheets ([Fig. 3]) with spindle cells in sheets and marked pleomorphism ([Figs. 4] and [5]).
Fig. 3 Microscopic image shows squamous cells in sheets and keratin pearl formation (H&E,
10X).
Fig. 4 Microscopic image shows spindle-shaped cells in sheets with pleomorphism (H&E, 10X).
Fig. 5 Microscopic image shows pleomorphic tumor cells (H&E, 40X).
Histological features were suggestive of the spindle cell variant of squamous cell
carcinoma.
The patient was staged as T1N0Mx (stage I) and radiotherapy was advised. The patient
received a total dose of 66 Gy in 30 fractions of external beam radiotherapy. He tolerated
the radical radiotherapy and responded well to the treatment. Video laryngoscopic
examination post-radiotherapy showed no residual disease. This patient was called
for follow-up after 1month, which showed no residual tumor.
Discussion
Most laryngeal malignancies are squamous cell carcinoma which comprises >95% of all
laryngeal malignancies. Sarcomatoid or spindle cell carcinoma is a rare variant that
comprises 1.3%.[1] Due to its rare, aggressive, unique nature, and histology, it is called a collision
tumor.[6] It is predominantly noted in men in the older age group of 60 to 70 years.[7] The patient reported here is a male aged 78 years. Spindle cell carcinoma has a
strong association with a history of cigarette smoking and alcohol intake.[8] The commonest site of involvement is the true vocal cord and anterior commissure.[9] The majority of patients present with dysphonia, dysphagia, and airway obstruction.[10]
Thompson et al described the tumor macroscopically as polypoidal with a mean size
of 2.1 cm. Of the 187 cases they studied, only two were noted as sessile or ulcerated.[3]
Microscopically, sarcomatoid SCC shows a combination of a malignant mesenchymal spindle
cell component in a homologous or heterologous pattern along with a surface squamous
cell component.[11] Epithelial and mesenchymal components are presented in the nested form, with spindle
components constituting the majority of the tumor.[9]
As it is a rare entity, there is no clear consensus regarding the management of sarcomatoid
squamous cell carcinoma. Radiotherapy can be used as a single treatment technique
for sarcomatoid carcinoma.[8] However, mesenchymal cells are usually not sensitive to radiotherapy, so it is combined
with surgical techniques to reduce the local recurrence rate.[12] The treatment protocol we followed is radical radiotherapy, as it was a T1 glottic
carcinoma. The patient had an excellent response to radiotherapy.
The prognosis of the disease depends on the T stage, lymph node metastasis, and tumor
location. The overall survival rate is better for the T1 stage than for T2 and T3.[3] The survival rate with stage T 1 is higher compared with other stages. The 5-year
survival rate is 80% for sarcomatoid squamous cell carcinoma, whereas 84% for squamous
cell carcinoma.[13] For other stages (II-IV), the 5-year overall survival rates were 43% for sarcomatoid
tumors, and 51% for squamous cell carcinomas. Even though the sarcomatoid SCC is highly
malignant, the prognosis is good because of the location on the vocal cord, resulting
in early presentation.
Conclusion
Sarcomatoid or spindle cell carcinoma is a rare variant of squamous cell carcinoma,
which is more commonly seen on the vocal folds. Histologically, it demonstrates both
spindle and squamous cell components. The best treatment modality is surgery followed
by radical Radiotherapy. However, in early-stage, radiotherapy gives good results.
Even though the sarcomatoid SCC is highly malignant, the prognosis is good because
of the location on the vocal cord resulting in early presentation.
