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| CASE REPORT |
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| Year : 2021 | Volume
: 5
| Issue : 1 | Page : 36-38 |
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Sarcomatoid carcinoma of the larynx: An aggressive tumor
Ipsita Dhal1, Krishna Gopal Birmiwal2, Bhagat Singh Lali1, Aseem Mishra2
1 Department of Pathology, Tata Memorial Centre, Homi Bhabha Cancer Hospital, Varanasi, Uttar Pradesh, India
2 Department of Head and Neck Oncology, Tata Memorial Centre, Homi Bhabha Cancer Hospital, Varanasi, Uttar Pradesh, India
| Date of Submission | 18-Jun-2020 |
| Date of Decision | 02-Oct-2020 |
| Date of Acceptance | 12-Oct-2020 |
| Date of Web Publication | 14-Apr-2021 |
Correspondence Address:
Bhagat Singh Lali
Department of Pathology, Tata Memorial Centre, Homi Bhabha Cancer Hospital, Varanasi - 221 010, Uttar Pradesh
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/oji.oji_29_20
Sarcomatoid carcinoma of the larynx is a rare and aggressive tumour with a unique biologic behavior and histogenesis. They were earlier called as collision tumors. They are classified under “epithelial tumors” in the World Health Organization classification. Due to the aggressive clinical behavior, they obstruct the airways soon and the patients present early. We present a case of a 55-year-old female who presented in acute state to the emergency department with dyspnea. On evaluation, a locally advanced laryngeal malignancy was detected radiologically. The biopsy was performed wherein a spindle cell pleomorphic tumor was seen which was positive for AE1/AE3 and vimentin, thus rendering a final diagnosis of sarcomatoid carcinoma. We discuss the clinical features, biological behavior, and management of these rare carcinomas.
Keywords: Aggressive behavior, larynx, sarcomatoid carcinoma
How to cite this article:
Dhal I, Birmiwal KG, Lali BS, Mishra A. Sarcomatoid carcinoma of the larynx: An aggressive tumor. Oncol J India 2021;5:36-8 |
| Introduction | |  |
Laryngeal sarcomatoid carcinomas are rare tumors accounting for <1% of all the laryngeal malignancies.[1] The first case was reported by Figi in 1933 wherein it was termed “larynx sarcoma.”[2] Currently, it is a distinct laryngeal malignancy classified under the malignant epithelial tumors, termed as spindle cell squamous carcinoma in the World Health Organization classification 2017.[3] These cases present early in the disease course with hoarseness of voice or obstructive symptoms.[3],[4] Diagnosis is difficult for the clinicians and pathologists alike and differentiation from primary sarcoma of the larynx is critical since the tumor biology, prognosis, and management differ significantly.[3],[4],[5] We present a case report of this rare subtype of tumor which presented in advanced stage.
| Case Report | | |
A 55-year-old female presented to the head and neck oncology department with a history of anterior lower neck swelling for the past 3 months. At present, she complained of progressive dyspnea for the past 20 days. She had a history of tobacco chewing over the past 15 years. On examination, her general condition was poor (PS-4). She was tachypneic with a heart rate of 140/min and oxygen saturation of 97%. An anterior midline lower neck swelling of hard consistency was palpated. To alleviate the symptoms, emergency tracheostomy was performed. During the tracheostomy procedure, a suspicious necrotic tissue was found around the thyroid gland and a biopsy was taken from the necrotic tissue for histopathological examination. Direct laryngoscopy revealed an ulceroproliferative growth in the supraglottic region completely obstructing the airway and a punch biopsy was taken from the growth as well.
Radiological finding with contrast-enhanced computed tomography scan of the neck revealed a 6.3 cm × 4.7 cm mass in the post cricoid region, extending from the oropharynx to cervical esophagus till the D4 vertebral body [Figure 1]. The mass was infiltrating the left lobe of the thyroid and trachea leading to luminal compromise. Posteriorly, the planes with prevertebral fascia were lost due to tumor infiltration with cricoid erosion. The vocal cords were normal. Multiple cervical lymph nodes were detected at left Level III and IV with left supraclavicular nodes. In the thorax, a well-defined subcentimeter-sized solid nodule was detected in the right upper lobe of the lung, which was suspicious for metastasis. | Figure 1: Computed tomography scan showing heterogeneous tumor of the larynx (a) infiltrating the left lobe of the thyroid and (b) trachea with significant luminal compromise (arrow)
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The histopathological examination of both the samples (thyroid and laryngeal biopsy) revealed a similar morphology. Fragmented bits of tissue were very focally lined by severely dysplastic stratified squamous epithelium but predominantly comprised a solid hypercellular tumor composed of sheets of haphazardly arranged spindle-shaped to epithelioid cells with large areas of necrosis [Figure 2]a. The individual cells exhibited markedly pleomorphic nuclei with coarse chromatin, prominent nucleoli, and moderate-to-scant eosinophilic cytoplasm [Figure 2]b. Few scattered bizarre cells were present along with atypical mitoses. It was a poorly differentiated spindle cell tumor on histomorphology which was diffusely immunoreactive for AE1/AE3 cytokeratin and vimentin whereas negative for TTF-1 [Figure 2]c and [Figure 2]d. Thus, confirming the diagnosis of sarcomatoid carcinoma. | Figure 2: Histology showing (a) tumor composed of sheets of pleomorphic cells with large areas of necrosis (H and E, ×10) and (b) markedly pleomorphic cells with few bizarre forms (H and E, ×20), and immunohistochemistry showing the tumor cells were positive for AE1/AE3 (c) and vimentin (d)
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Patient's general condition was stabilized and palliative chemotherapy was planned. She was found unfit for intravenous chemotherapy and oral metronomic chemotherapy was administered. Unfortunately, she expired at her residence 10 days later.
| Discussion | | |
Laryngeal sarcomatoid carcinomas are rare aggressive biphasic tumors comprising the epithelial and mesenchymal elements.[3] The definite diagnosis is challenging from both the clinician and pathologist's perspective.[4] The major predisposing factors include tobacco and alcohol consumption and poor oral hygiene.[5] Thompson et al. reported tobacco consumption in 87% of their spindle cell carcinoma patients and alcohol consumption in 48%.[1] Our patient also had a history of tobacco smoking over the past 15 years. Previous irradiation to the larynx is another risk factor. However, it has been reported in only 7.7%–9.1% cases in the literature. The mortality is significantly higher in patients exposed to previous radiation when compared to the nonexposed individuals.[1],[3]
These carcinomas are common in males and present at the mean age of 58–65 years.[1],[3],[6] Our patient was female presenting at nearly same age, i.e., at a 55-year age. These patients usually present at an early stage of the disease due to hoarseness of voice and/or obstructive symptoms.[6],[7] Our patient also presented in an acute state to the emergency department, but the disease was locally advanced at presentation along with radiologically suspected lung metastasis. In the larynx, glottic malignancies are more common when compared to the supraglottic and infraglottic sites. The site of involvement impacts the prognosis since glottic sarcomatoid carcinomas have more favorable prognosis (mean 7.8 years) when compared to the supraglottic (mean 5.6 years) and infraglottic carcinomas (mean 3.5 years).[1]
These tumors were historically considered as collision tumors or combination tumors. The absence of mingling of the stromal and epithelial components was believed to be against calling them transformed carcinomas. The spindle cell proliferation was thought to be a reactive phenomenon to the tumor.[8],[9] Over the years, we have understood that this is a biphasic tumor comprising the malignant surface epithelial changes in the form of in situ or invasive carcinoma and an underlying spindle cell proliferation.[1] In tumors where the surface epithelium is ulcerated or denuded, rendering a correct diagnosis is a major challenge. At the same time, the squamous component may be inconspicuous and a diligent search for it becomes mandatory.[7]
On immunohistochemistry, Thompson et al., in a large series of cases, found that 64% of these tumors express one of the four epithelial markers, namely, AE1/AE3, epithelial membrane antigen, CK1, and CK18. However, they also found that the poorly differentiated tumors even lack the expression of epithelial markers.[1] Among mesenchymal markers, vimentin is expressed in all the cases and the less commonly expressed markers include smooth muscle actin (35%), S-100 (5%), and desmin (2%).[1],[3] In our case, AE1/AE3 and vimentin were positive.
The surgery is the mainstay of treatment and outcomes are better after surgery when compared with radiotherapy alone. Since these tumors are mostly pedunculated or polypoidal, a wide local excision is considered to be the surgery of choice.[1],[4] The overall metastatic rate is 19.3% in these tumors. It is lower in glottic carcinomas (12.1%) in comparison with transglottic tumors (26.1%), supraglottic tumors (35.7%), and infraglottic tumors (100%).[1] The 5 years disease-specific survival is 84.2% with surgery and radiotherapy, 84% with surgery alone, and 60.5% with radiotherapy alone. Compared to conventional squamous cell carcinomas, the survival rates over the short term (90.9% v. 88%, respectively) and long term (57.9% vs. 50.6%, respectively) are similar in these tumors.[4] Tumor size, location, and stage have been significantly implicated as prognostic factors in sarcomatoid carcinomas of the larynx in large case series.[1],[7]
To conclude, sarcomatoid carcinoma of the larynx is a rare and unique entity with distinct biologic behavior and histogenesis. It holds its distinct position in the World Health Organization classification. These tumors present early in the disease course and are overly aggressive. They pose diagnostic challenges to the clinicians and the pathologists. Surgery is the mainstay of treatment and survival rates are comparable to conventional squamous cell carcinomas of the larynx.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Thompson LD, Wieneke JA, Miettinen M, Heffner DK. Spindle cell (sarcomatoid) carcinomas of the larynx: A clinicopathologic study of 187 cases. Am J Surg Pathol 2002;26:153-70.  |
| 2. | Figi FA. Sarcoma of the larynx. Arch Otolaryngol Head Neck Surg 1933;18:21. |
| 3. | Bishop J, Cardesa A, Helliwell T, Hille J, Nadal A. Spindle cell squamous cell carcinoma. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ, editors. WHO Classification of Head and Neck Tumours. 4 th ed.. Lyon: International Agency for Research on Cancer; 2017. p. 87-8. |
| 4. | Bostanci A, Ozbilim G, Turhan M. Spindle cell carcinoma of the larynx: A confusing diagnosis for the pathologist and clinician. Case Rep Otolaryngol 2015;2015:1-4. |
| 5. | Zheng Y, Xiao M, Tang J. Clinicopathological and immunohistochemical analysis of spindle cell carcinoma of the larynx or hypopharynx: A report of three cases. Oncol Lett 2014;8:748-52. |
| 6. | Boamah H, Ballard B. A case report of spindle cell (sarcomatoid) carcinoma of the larynx. Case Rep Med 2012;2012:1-5. |
| 7. | Viswanathan S, Rahman K, Pallavi S, Sachin J, Patil A, Chaturvedi P, et al. Sarcomatoid (spindle cell) carcinoma of the head and neck mucosal region: a clinicopathologic review of 103 cases from a tertiary referral cancer centre. Head Neck Pathol 2010;4:265-75. |
| 8. | Baker DC. Pseudosarcoma of the pharynx and larynx. Ann Otol Rhinol Laryngol 1959;68:471-7. |
| 9. | Goellner JR, Devine KD, Weiland LH. Pseudosarcoma of the larynx. Am J Clin Pathol 1973;59:312-26. |
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