This case report discusses a 76-year-old male diagnosed with sarcomatoid carcinoma, a rare variant of squamous cell carcinoma of the larynx, characterized by a polypoid mass on the left true vocal cord. The patient underwent direct laryngoscopy for excision of the tumor and received radiotherapy, resulting in a complete resolution of the tumor. Sarcomatoid carcinoma accounts for 2-3% of laryngeal cancers and is often detected early, leading to a favorable prognosis.
This case report discusses a 76-year-old male diagnosed with sarcomatoid carcinoma, a rare variant of squamous cell carcinoma of the larynx, characterized by a polypoid mass on the left true vocal cord. The patient underwent direct laryngoscopy for excision of the tumor and received radiotherapy, resulting in a complete resolution of the tumor. Sarcomatoid carcinoma accounts for 2-3% of laryngeal cancers and is often detected early, leading to a favorable prognosis.
This case report discusses a 76-year-old male diagnosed with sarcomatoid carcinoma, a rare variant of squamous cell carcinoma of the larynx, characterized by a polypoid mass on the left true vocal cord. The patient underwent direct laryngoscopy for excision of the tumor and received radiotherapy, resulting in a complete resolution of the tumor. Sarcomatoid carcinoma accounts for 2-3% of laryngeal cancers and is often detected early, leading to a favorable prognosis.
This case report discusses a 76-year-old male diagnosed with sarcomatoid carcinoma, a rare variant of squamous cell carcinoma of the larynx, characterized by a polypoid mass on the left true vocal cord. The patient underwent direct laryngoscopy for excision of the tumor and received radiotherapy, resulting in a complete resolution of the tumor. Sarcomatoid carcinoma accounts for 2-3% of laryngeal cancers and is often detected early, leading to a favorable prognosis.
DOI: http://dx.doi.org/10.18203/issn.2454-5929.ijohns20173067 Case Report
Sarcomatoid carcinoma a rare variant of squamous cell carcinoma
of larynx: a case report Trilok C. Guleria1*, Shobha Mohindroo2, Narender K. Mohindroo1, Ramesh K. Azad1 1 Department of Otolaryngology Head and Neck Surgery, 2Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
Received: 10 May 2017
Accepted: 31 May 2017
*Correspondence: Dr. Trilok C. Guleria, E-mail: tcguleria@gmail.com
Sarcomatoid (spindle cell) carcinoma is a rare variant of squamous cell carcinoma (SCC). It compromises of 2–3% of laryngeal cancers. Tumor arises from the oral cavity, tonsil, larynx and pharynx. Majority of these tumors are polypoid or pedunculated and tend to cause obstructive symptoms. These tumors are often detected at an early stage, removed by polypectomy during diagnosis and tend to have a very good prognosis. In this case report, 76 years male who presented with progressive hoarseness of his voice and pain in throat. The patient underwent direct laryngoscopy with excision of the malignant mass and received radiotherapy.
INTRODUCTION chronic debilitating disease. Physical examination of the
patient showed no palpable cervical lymphadenopathy. Squamous cell carcinoma is the most malignant carcinoma of the larynx.1 Sarcomatoid tumor is The patient underwent a flexible laryngoscopy that considered a highly malignant variant of squamous cell showed the presence of whitish polypoidal mass carcinoma and rare tumor of larynx. It is a unique and involving left true vocal cord (Figure 1). The patient then rare neoplasm, composed of both malignant epithelial and underwent direct laryngoscopy, which showed a mass mesenchymal components. Till date, only few cases are with filiform projections located in the midregion of left reported of sarcomatoid carcinoma of larynx.2 Since a true vocal cord. The lesion was excised and sent for majority of these tumors are polypoid or pedunculated histopathological examination. and tend to cause obstructive symptoms, these tumors are often detected at an early stage and tend to have a very The pathology report showed a sarcomatoid carcinoma good prognosis. The following is a case report of one (Figure 2) that on immunohistochemistry strongly such patient who presented to our institution with positive for Ki -67, SMA and focal positive for EMA but sarcomatoid carcinoma of the larynx. negative for desmin, HMB – 45, CK 5/6, CK HMW, Anti p63 and S-100. CASE REPORT A CT scan of his neck with contrast after the A 76 years old male presented to the ENT OPD with 3 histopathological confirmation revealed a minimal months history of progressive hoarseness and pain in thickening of size 1×0.5 cm involving the left true vocal throat. The patient history was significant for 40 pack cord (Figure 3); however the nasopharynx, pharynx, years of smoking. There was no history of hemoptysis, parapharyngeal space, epiglottis, major neck vessels weight loss, neck masses or any signs suggestive of any appeared normal. The parotid glands and submandibular
International Journal of Otorhinolaryngology and Head and Neck Surgery | July-September 2017 | Vol 3 | Issue 3 Page 768 Guleria TC et al. Int J Otorhinolaryngol Head Neck Surg. 2017 Jul;3(3):768-770
salivary glands were normal. There was no significant complete resolution of tumor after radiotherapy (Figure cervical lymphadenopathy. 4).
Figure 1: Fiberoptic laryngoscopy showing whitish Figure 4: After radiotherapy fiberoptic laryngoscopy polypoidal mass involving the left true vocal cords. showing complete resolution of tumour.
DISCUSSION
SCC is considered to be the most common type of
malignant laryngeal tumor.1 Sarcomatoid carcinoma is a highly malignant variant of SCC. It is a rare tumor with a reported incidence of 2–3% of all laryngeal cancers.2 Sarcomatoid is an aggressive malignant tumor typically observed in upper aerodigestive tract, in which nearly half of the cases are of larynx origin.3 It was first reported in the literature in 1933 by Figi, under the name of “larynx sarcoma”.4
Although the exact cause of spindle cell carcinoma
(SpCC) is not known, it is strongly associated with a Figure 2: Computed tomography neck showing history of cigarette smoking and alcohol abuse. It has also minimal thickening of size 5×1 cm involving the left been suggested that SpCC is associated with radiation true vocal cord. exposure although the determination of radiation risk may be complicated by the dose and duration of radiation exposure.5 SpCC is more predominant in men compared to females (12:1 ratio) although it is becoming more common in females and it is usually seen in the 6th and 7th decades of life.6
SpCC most commonly affects the glottis in the majority
of cases (70%), and the majority of patients present with symptoms of hoarseness, dyspnea, cough, and dysphagia often of <1 year duration.1 The majority of these tumors are characterized as being polypoid or pedunculated Figure 3: Histological findings. (98.9%) tumors that are often <2 cm in size. 6 Lining epithelium shows acanthosis and parakeratosis. Stroma shows pleomorphic spindle cell in isolation and in fascicles. Spindle cells have hyperchromatic, pleomorphic nuclei with The diagnosis of SpCC requires histological eosinophilic cytoplasm. Occasional cells show intranuclear demonstration of both the squamous cell component and inclusions and occasional mitotic figures. Occasional bi, the spindle shape cells with sarcomatous appearances.2 trinucleate and pleomorphic giant cells are seen. Focal areas The histological examination can often show the presence show dense collagen bundles. Histopathology revealed of SCC at the surface or deeper within the tumor although sarcomatoid carcinoma variant of squamous cell carcinoma. this is rare especially with tumors where the surface is ulcerated or denuded. It is often seen is a blending of The patient sarcomatoid carcinoma was staged as squamous cells and spindle cells which can be T1N0M0 after investigations and received radiotherapy. differentiated by their different arrangement which The patient’s symptoms gradually improved and includes storiform, solid, and fascicular appearance. 6
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In addition to histological studies, immunohistochemical Because most spindle cell tumors are polypoid and studies of epithelial and mesenchymal markers are used pedunculated and tend to cause obstructive symptoms to diagnose the tumor. Epithelial markers include keratin such as hoarseness, dyspnea, and dysphagia most tumors (AE1/AE3, CK1, 8, 9), epithelial membrane antigens, KI, without metastasis are detected early and tend to have a and K18. Mesenchymal markers include vimentin, very good 5 year prognosis. desmin, S-100, Osteopontin, and BMP (2, 4).7 For spindle cell carcinomas with poorly differentiated Funding: No funding sources epithelial tumor components Lewis et al. have shown that Conflict of interest: None declared p53, a transcription factor that is important for epithelial Ethical approval: Not required proliferation and differentiation, is particularly useful for diagnosing SpCC of the head and neck region.8 REFERENCES
Dubal et al reported that the majority of laryngeal SpCC 1. Volker HU, Scheich M, Holler S, Strobel P, Hagen cases have T1 (62%) and stage I (61%) tumors at R, Hermelink HKM, Eck M. Differential diagnosis presentation.9 Lymph node metastasis may occur (12.6%) of laryngeal spindle cell carcinoma and but distant metastasis is uncommon (3.7%). he surgery inflammatory myofibroblastic tumor – Report of yields a favorable prognosis when compared to two cases with similar morphology. Diagn Pathol. radiotherapy. The 5-year disease speciic survival (DSS) is 2007;2:1. 84% with surgery alone, 84.2% with surgery combined 2. Katase N, Tamamura R, Gunduz M, Murakami J, with radiotherapy, and 60.5% with radiotherapy alone. In Asaumi J, Tsukamoto G, et al. A spindle cell addition, a glottis location of the tumor, as opposed to a carcinoma presenting with osseous metaplasia in the non-glottic location, is significantly associated with gingiva: A case report with immunohistochemical improved prognosis (5 year DSS, 84.0% versus 51.9%). analysis. Head Face Med. 2008;4:28. Compared to other laryngeal malignancies, SpCCs have 3. Gerry D, Fritsch VA, Lentsch EJ. Spindle cell intermediate (5 years) survival benefit (74.1% versus carcinoma of the upper aerodigestive tract: An 64.6%), but short-term (1 year, 90.9% versus 88%) and analysis of 341 cases with comparison to long-term (10 years, 57.9% versus 50.6%) survival rates conventional squamous cell carcinoma. Annals are similar. Otol, Rhinol Laryngol. 2014;123(8):576-83. 4. Figi FA. Sarcoma of the larynx. Arch Otolaryngol On the other hand, in a retrospective study assessing the Head Neck Surg. 1933;18:21. outcomes of early-stage (T1-T2) glottic SpCC cases (n 5. National Cancer Institute. Cancer facts. Head and =28) treated with primary radiotherapy. Ballo et al neck cancer: Questions and answers. National reported that these patients had similar local-control rates Cancer Institute; 2012. to irradiated patients with similar volume disease with the 6. Thompson LD, Wieneke JA, Miettinen M, Heffner more typical squamous cell carcinoma.10 Therefore, the DK. Spindle cell (sarcomatoid) carcinomas of the authors suggested that the histologic diagnosis of SpCC larynx: A clinicopathologic study of 187 cases. Am instead of squamous cell carcinoma should not alter J Surg Pathol. 2002;26:153-70. treatment recommendations in patients with early-stage 7. Lewis JE, Olsen KD, Sebo TJ. Spindle cell disease. carcinoma of the larynx: Review of 26 cases including DNA content and immunohistochemistry. Tumors that are stage T2 or less can be managed Hum Pathol. 1997;28:664-73. conservatively with limited field irradiation and 8. Lewis JS, Ritter JH, El-Mofty S. Alternative conservative treatment to preserve the patient's voice. epithelial markers in sarcomatoid carcinomas of the Tumors that are stage 3–4 can be treated with local head and neck, lung, and bladder-p63, MOC-31, and resection, partial laryngectomy, total laryngectomy with TTF-1. Mod Pathol. 2005;18:1471-81. or without lymph node dissection followed by a 9. Dubal PM, Marchiano E, Kam D, Dutta R, combination of radiation therapy and chemotherapy. 6 Kalyoussef E, Baredes S, et al. Laryngeal spindle SpCC of the larynx has a very good 5-year prognosis of cell carcinoma: a population-based analysis of 65–95%.1 Poor prognostic factors include tumors incidence and survival. Laryngoscope. diagnosed at higher stages, large tumors (>3 cm) with a 2015;125(12):2709-14. predominance of epithelial component, nonglottic 10. Ballo MT, Garden AS, El-Naggar AK, Gillenwater tumors, fixed vocal cords, history of radiotherapy and AM, Morrison WH, Goepfert H, et al. Radiation metastasis to regional lymph nodes, and distant therapy for early stage (T1-T2) sarcomatoid metastasis.6 carcinoma of true vocal cords: outcomes and patterns of failure. Laryngoscope. 1998;108(5):760- 3. CONCLUSION Cite this article as: Guleria TC, Mohindroo S, SpCC or sarcomatoid carcinoma of the larynx is a highly Mohindroo NK, Azad RK. Sarcomatoid carcinoma a rare malignant variant of SCC that is very uncommon. variant of squamous cell carcinoma of larynx: a case report. Int J Otorhinolaryngol Head Neck Surg 2017;3:768-70.
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