"Never doubt that a small group of thoughtful, committed citizens can change the world. Indeed, it is the only thing that ever has."

Margaret Mead
Case report
peer-reviewed

Age Limit and Radiotherapy Option for Sarcomatoid Carcinoma of the Larynx: A Case Report with Literature Review



Abstract

Sarcomatoid carcinomas, also known as spindle cell carcinomas (SPCCs), are rare carcinomas, predominantly developing in the lung. They have lots of features of sarcoma in their histological features. The standard laryngeal carcinoma classification is based on tumor size, lymph node affection, and metastasis (TNM), it is the classification scheme of the American Joint Committee on Cancer Staging (AJCC), and it is used in the same way for stage spindle cell carcinoma (SPCC). We present a case report of a young female along with a literature review of sarcomatoid carcinoma of the larynx.

Introduction

Sarcomatoid carcinomas, also known as spindle cell carcinomas (SPCC), are rare carcinomas that predominantly develop in the lung. They have lots of features of sarcomas in their histological features [1-3]. In the larynx, sarcomatoid carcinomas are considered high-grade variants of squamous cell carcinomas, with no randomized clinical trials, conducted to specify a treatment modality [2]. The standard laryngeal carcinoma classification scheme of the American Joint Committee on Cancer Staging (AJCC) is used in the same way as to stage SPCC [3]. The classification is based on tumor size, lymph node affection, and metastasis (TNM) [3]. Most laryngeal SPCCs give symptoms early, predominantly hoarseness of voice. The reason for hoarseness of voice is that they most commonly appear as glottic pedunculated polypoidal masses (T1 or T2 lesions) with minimal invasion of the underlying stroma. The presenting gross appearance allows early wide complete transoral local excisions [3]. Scholars suggested good disease-controlled outcomes for early-stage glottic sarcomatoid carcinomas when treated with irradiation, comparing favorably with early glottic squamous cell carcinomas [4].

Case Presentation

A 24-year-old female patient presented to the otolaryngology clinic with a six-month history of progressive hoarseness of voice. In addition, she has a recent history of mild dyspnea on exertion and dry cough. The patient did not have any weight loss nor dysphagia. The patient was a cigarette smoker of around one pack per day for five years. There was no past family history of cancer, and she did not have any medical illness of significance. The patient also did not have any prior history of radiotherapy. In the clinic, fiberoptic nasoendoscopy showed a right vocal fold mass reaching the anterior commissure. The vocal fold mobility was normal. The neck examination was unremarkable. Our clinical impression, at this stage, was that the patient had early glottic laryngeal cancer. Consequently, the patient had a computed tomography (CT) scan showing the mass with no cervical lymphadenopathy (Figure 1).

The vocal fold mobility was normal. The neck examination was unremarkable. The patient underwent a laryngotracheoscopy under general anesthetic, with a biopsy taken from the lesion, which appeared to be arising from the right vocal fold without a subglottic extension. The initial histopathology report confirmed sarcomatoid carcinoma and subsequent immunohistochemistry was positive for epithelial membrane antigens (EMA), cytokeratin CK 5/6, and cytokeratin AE1/AE3AE 1/3 (Figures 2-4).

The patient’s spindle cell (sarcomatoid) carcinoma stage was T2N0M0 according to the AJCC cancer staging system for laryngeal carcinomas. The options for management were evaluated by the head and neck multidisciplinary team, who preferred a transoral surgical excision as a modality of treatment. We discussed the treatment options with the patient, who refused surgical intervention. Consequently, she received intensity-modulated radiotherapy (IMRT). The patient had followed up at a six-month interval, and she remains free of the disease (Figure 5).

Discussion

Methods

We also conducted a review of the literature based on a search performed on June 9, 2018, in PubMed. We discovered 167 articles, and on further screening, we excluded 103 articles, as they were not related to our topic or we could not download it. Then, 49 studies were excluded in the full-text analysis due to irrelevant disease. Fifteen articles were included in our review of the literature. We reviewed 59 patients with laryngeal carcinosarcoma having different ages, sexes, and modalities of carcinosarcoma treatment. The following table illustrates the review of the included patients (Table 1).

ID Article Age (Years) /Gender Specific Habits/ Symptoms/Duration Site/Classification Treatment Pathology Report of Surgical Specimen Subsequent course/Follow-Up
1 Randall et al. [5] 58/M Smoker/hoarseness for two Mo Rt anterior commissure/T1bN0M0 Local excision Pseudo-Sarcocarcinoma in situ Neck metastasis four Mo, and FOD eight Y later
56/M Smoker/hoarseness for two Wks Lt anterior commissure/ T2N1M0 SSL Carcinoma in situ and invasive epidermoid carcinoma pseudo sarcoma Neck metastasis nine Mo, lt RND, one node has cancer/FOD four M0 before death from MI
43/F Smoker/hoarseness for four Mo Lt TVC/T1aN0M0 Lt HL Invasive epidermoid carcinoma with underlying pseudosarcoma FOD nine and a half Y
74/M Smoker/hoarseness for one Y Anterior commissure/T2N0M0 Frontal HL Pseudosarcoma FOD four Y
60/M Smoker/hoarseness for three Mo Rt FVC/T2N0M0 SSL with foldover Rt RND Osteosarcoma FOD three and a half Y
67/M Smoker/hoarseness for one Mo FVC/TV bilateral/T3N1M0 TL Pseudosarcoma FOD 12 Mo
55/M Dysphagia for three Mo Epiglottis rt T3N1M0 SSL, Bilateral RND Pseudosarcoma Died with cancer three Y
80/F Dysphagia for eight Mo Lt AE/ T3N0M0 SSL, LT RND Pseudosarcoma, carcinoma in situ Died Free of Ds two Y
80/M Dysphagia for three Y Lt TVC/ T3N0M0 TL Pseudosarcoma Died with cancer 13 M0
2 Appelman et al. [2] 62/M Smoker/hoarseness for one Y/dyspnea for one wk Rt ventricle, Rt VC Radiotherapy Fusion of SCCt and SPCC Local recurrence, 10 Mo/died one and a half Y
51/M Smoker/hoarseness for three M/otalgia Anterior half of Lt VC HL SCC with sarcoma-like areas FOD 15 Y
72/M Hoarseness for three mo Base of the epiglottis Rd Fusion of SCC and spindle cells Local recurrence, Five Mo, radium implant/died 14 months later
49/M None Right arytenoid Snare excision +radiotherapy SCC intermixed with spindle cells; osteoid and chondroid areas Cervical node metastasis, one Mo/died two and a half Y
56/M Hoarseness for three mo Left true cord None Predominant bizarre spindle cell neoplasm Died five mo
59/M Difficulty swallowing for six mo Hypopharynx, false cord None SCC intermixed with spindle cells; osteoid and chondroid areas Died eight Mo
48/M Dysphagia for seven Mo Right, true cord Rd then local recurrence, Four yr.; total laryngectomy SCC intermixed with spindle cells; osteoid metaplasia Died, five and a half Y, cervical node metastasis
42/M Hoarseness (nine mo) neck pain (one Mo) Right, true cord HL Fusion of SCC and spindle cells Alive and ell after 13.5 Y
59/M Hoarseness for two yr, dysphagia for three Mo. Both true cords and commissure TL/Metastasis in cervical lymph nodes, three Mo then RD Fusion of SCC and spindle cells Died, 10 Mo
73/F Hoarseness, dysphagia, weight losssix Wks Right ventricular fold TL/regional recurrence, cervical lymph node metastasis, 10 Mo followed by radiotherapy Fusion of SCC and spindle cells Died two and a half Y later
77/M Hoarseness for 10 mo Right, true cord TL SCC with demarcated sarcoma-like areas FOD one and a half year later
3 Katholm et al. [6] 50/M Smoker/hoarseness LT VC/T1AN0M0 Radiotherapy Interlacing bundles of large spindle-shaped cells with pleomorphic nuclei and nucleoli. FOD 22 Mo
4 Alguacil-Garcia et al. [7] 59/M Smoker/hoarseness for one year Rt VC TL Sarcomatoid carcinoma FOD two Y
5 Hellquist et al. [8] 64/M Hoarseness of voice for seven Mo VC TL SPCC  
66/F Hoarseness of voice for eight Mo Epiglottis Rad SPCC FOD three and a half Y
54/M Hoarseness of voice for three Mo VC TL SPCC FOD three Y
69/M Hoarseness of voice for four Mo VC Excision, rad SPCC FOD one and a half Y
71/M Hoarseness of voice for two Mo VC Rad/then recurrence after five Y, then laryngofissure SPCC FOD eight and a half Y
62/F Hoarseness of voice for three Mo VC Excision SPCC FOD one and a half Y
57/M Hoarseness of voice for four Mo VC TL+Radio SPCC FOD one and a half Y
36/M Hoarseness of voice for four Mo Subglottis Excision + Radio SPCC FOD three Y
63/M Hoarseness of voice for five Mo VC Radio SPCC FOD two Y
75/F Hoarseness of voice for six Mo VC Radio SPCC LNs metastasis After one Y died with the ds after three Y
59/M Hoarseness of voice for three and a half Mo VC Radio SPCC FOD 11.5 Y
67/M Hoarseness of voice for two and a half Mo VC Excision+radio SPCC FOD six Y
57/M Hoarseness of voice for two Mo VC Excision SPCC FOD 10.5 Y
69/M Hoarseness of voice for two Mo VC Excision SPCC FOD five Y
6 Lassaletta et al. [9] 48/F Smoke 40 Cig/day/ hoarseness and intermitted stridor Lt VC Functional neck dissection and total laryngectomy GCT Lt VC/SPCC in subglottic/positive for EMA, cytokeratin CK 5/6 and cytokeratin AE1/AE3AE 1/3, keratin 8, anion exchange keratin 1,3. Negative for S100 FOD six Mo
7 Miyahara et al. [10] 88/M Smoker/hoarseness RT VC/T2N0 TL Carcinosarcoma Died seven Y later of old age not the Ds
86/M Smoker/hoarseness RT VC/ T1aN0 Total laryngectomy Spindle cell carcinoma FOD six Y
68/M Smoker/hoarseness Anterior commissure/T1bN0 Rad. Spindle cell carcinoma FOD six Y
76/M Smoker/hoarseness VC/T1aN0 Extirpation Spindle cell carcinoma FOD six Y
8 Onishi et al. [11] 73/M Smoker/hoarseness two Mo Lt VC/T1aN0M0 Partially resection/radio invasive poorly differentiated SCC with massive sarcomatoid changes (spindle cells) Died eight M0 after diagnosis with the tumor
9 Franzen et al. [12] 61/M Hoarseness four Mo, dysphonia two Wks
Right VC and anterior commissure/T2 N0 M0 R0 G3
Partial laryngectomy and modified radical neck dissection
 
 
53/M Smoker/swallowing difficulty 2 Mo/ wt loss > 12 kg Right laryngeal wall of the sinus piriformis and the aryepiglottic fold/T3 N2B Mo Right-sided laryngeal hypopharyngeal resection and neck dissection
Light microscopic and immunohistochemically becomes a biphasic tumor(carcinosarcoma)
 
10 Boamah et al. [13] 67/M Smoker/hoarseness 2 Mo, dysphagia Anterior commissure/T1 Excision + radiotherapy Spindle cell carcinoma, positive for EMA, CK 5/6 and AE 1/3, and a high MIB-1 but negative for myoD1, SMA, desmin, and myf4 Improved
11 Rutt et al. [14] 69/M Smoker (15 Y) quit (30 Y prior)/ Dysphonia/six Mo Rt VC/ T1N0M0 Micro flap excision then wider excision SCC, spindle cell variant FOD one Y
61/M Rough, unstable voice with phonatory breaks Lt VC/T1N0M0 Surgical excision SCC, spindle-cell variant Recurrence six Mo, Co2 laser excision with margin control/ free, six Mo
12 Zhang et al. [15] 66 /M Smoker/hoarseness Bilat vocal folds/ T3N0M0 TL SCC, malignant fibrous histiocytoma 88 Mo/AWD
64 /M Smoker/ hoarseness Lt false VC, laryngeal Ventricle/ T2N0M0 Vertical partial laryngectomy SCC, malignant fibrous histiocytoma 62/AWD
59 /M Smoker/hoarseness Lt false VC, laryngeal ventricle/T2N1M0 SCPL-CHP + RND Poorly differentiated Ca, leiomyosarcoma 60/DOD
60 /M Smoker/hoarseness, dyspnea Lt false vocal fold, bilat vocal folds/T3N0M0 Total laryngectomy + RND Mucinous adenocarcinoma, fibrosarcoma 24/AWD
57 /M Smoker/hoarseness, dyspnea Bilat vocal folds/T3N0M0 Total laryngectomy + SND Poorly differentiated Ca, osteosarcoma 20 Mo/AWD
50 /M Abnormal throat sensation Epiglottis, R aryepiglottic fold/T3N1M0 Total laryngectomy + RND Poorly differentiated Ca, embryonal rhabdomyosarcoma 17 Mo/AWD
42 /M Smoker/hoarseness R VC/T2N0M0 Vertical partial laryngectomy Ca in situ, leiomyosarcoma 13 Mo/AWD
13 Zheng et al. [16] 55/M Smoker/neck mass Lt pyriform sinus Excision, reconstruction, and neck dissection SPCC Free eight M0
62/M Smoker/hoarseness Lt VC TL+ dissection SPCC Pulmonary metastasis six M0
57/M Smoker/foreign body Posterior wall of hypopharynx Total hypopharyngectomy SPCC Free five and a half M0
14 Bostanci et al. [17] 60/M Smoker/hoarseness five Y Rt VC/T1N0M0 Excision + Radiotherapy Atypical spindle cells/epithelial component positive cytokeratin and p63 FOD 12 M0
15 Rao et al. [18] 45/M Smoker 10Y / hoarseness, difficulty in swallowing and breathing six Mo Lt VC/ T1N0M0 Mass excision Pleomorphic spindle-shaped cells FOD three Mo

Results

The included articles described a total of 59 patients, 52 of which were males and seven were females. The age range was between 36 and 88 years. The reported cases were from 1960 till 2016. Most of the patients were smokers, and most of them underwent total laryngectomy followed by radiotherapy. On the other hand, eight patients received radiotherapy only, while the rest had different types of surgery. The general survival outcome was better with a combination of both surgery and radiation, compared to radiotherapy alone.

Discussion

Carcinosarcoma is a rare lesion; it can be present in many body parts, including the larynx. The larynx appears to be an unusual site, as only a few cases can be found in the literature. Carcinosarcoma is considered a malignant tumor composed of both epithelial and mesenchymal components. It accounts for less than 1% of all malignant tumors of the larynx and hypopharynx [19]. In our review of the literature, we noticed that there is a broad age range for laryngeal carcinosarcoma (36-88 years), while our case was 24 years old. Despite being a rare condition, spindle cell carcinoma should be considered a valid diagnosis in any age group, especially if the patient presents with hoarseness of voice or dyspnea and a history of smoking. Surgical intervention was the first line of management in the treatment of most of the laryngeal carcinosarcomas mentioned in our literature with a favored outcome. However, scholars suggested good disease-controlled results for early-stage glottic sarcomatoid carcinomas when treated with irradiation, comparing favorably with early glottic squamous cell carcinomas [4]. We had eight patients in our review that received radiotherapy only, without any surgical intervention [2,6,8,10}. These patients had different ages, cancer stages, and laryngeal cancer locations. Five out of the eight patients who were treated only with radiation have no evidence of the disease at a range of two to 11.5 years while the other three developed a recurrence and died of the disease later on. So radiotherapy might be a valid alternative option instead of surgical therapy in selected patients. Our patient has stage T2N0M0 glottic carcinoma. Due to the early detection of the tumor and its site, the surgical excision was a valid and reasonable option for management. However, the patient preferred the radiotherapy option. The follow-up plan will be the same as for other glottic carcinomas.

Conclusions

Spindle cell carcinoma (SPCC) or sarcomatoid carcinoma of the larynx is a rare, highly malignant variant of squamous cell carcinoma. It can present at an early age with symptoms and diagnosis is possible in the early stages. Although in some patients, surgery is the best modality of therapy, radiation only can be a reasonable alternative.


References

  1. Travis WD, Brambilla E, Müller-Hermelink HK, Harris CC: Tumors of the Lung, Pleura, Thymus, and Heart. World Health Organization Classification of Tumours. IARC Press, 2004.
  2. Appleman HD, Oberman HA: Squamous cell carcinoma of the larynx, with sarcoma-like stroma. A Clinical-Pathological assessment of spindle cell carcinoma and pseudosarcoma. AJCP. 1965, 44(2):135-145. 10.1093/ajcp/44.2.135
  3. El-Naggar AK, Chan JKC, Takata T, Grandis JR, Slootweg PJ: The fourth edition of the head and neck World Health Organization blue book: editors' perspectives. Hum Pathol. 2017, 66:10-12. 10.1016/j.humpath.2017.05.014
  4. Ballo MT, Garden AS, El-Naggar AK, Gillenwater AM, Morrison WH, Goepfert H, Ang KK: Radiation therapy for early stage (T1-T2) sarcomatoid carcinoma of true vocal cords: outcomes and patterns of failure. Laryngoscope. 2009, 108:760-763. 10.1097/00005537-199805000-00024
  5. Randall G, Alonso WA, Ogura JH: Spindle cell carcinoma (pseudosarcoma) of the larynx. JAMA Otolaryngol Head Neck Surg. 1975, 101:63-66. 10.1001/archotol.1975.00780300067018
  6. Katholm M, Krogdahl A, Hainau B, Bretlau P: Spindle cell carcinoma of the larynx. Acta Otolaryngol Case Rep. 1984, 98:163-166. 10.3109/00016488409107550
  7. Alguacil-Garcia A, Alonso A, Pettigrew NM: Sarcomatoid carcinoma (so-called pseudosarcoma) of the larynx simulating malignant giant cell tumor of soft parts. Am J Clin Pathol. 1983, 82:340-343. 10.1093/ajcp/82.3.340
  8. Hellquist H, Olofsson J: Spindle cell carcinoma of the larynx. APMIS. 1989, 97:1103-1113. 10.1111/j.1699-0463.1989.tb00524.x
  9. Lassaletta L, Alonso S, Granell J, Ballestín C, Serrano A, Álvarez-Vicent JJ: Synchronous glottic granular cell tumor and subglottic spindle cell carcinoma. JAMA Otolaryngol Head Neck Surg. 1998, 124:1031-1034. 10.1001/archotol.124.9.1031
  10. Miyahara H, Tsuruta Y, Yane K, Ogawa Y: Spindle cell carcinoma of the larynx. Auris Nasus Larynx. 2004, 31:177-182. 10.1016/j.anl.2004.01.008
  11. Onishi H, Kuriyama K, Komiyama T, et al.: T1N0 laryngeal sarcomatoid carcinoma that showed rapid systemic metastases after radical radiotherapy: a case report and review of literature. Otolaryngol Head Neck Surg. 2005, 26:400-402. 10.1016/j.amjoto.2005.02.017
  12. Franzen A, Theegarten D: Karzinosarkome des larynx und hypopharynx [Article in German]. Laryngorhinootologie. 2007, 86:209-212. 10.1055/s-2006-925440
  13. Boamah H, Ballard B: A case report of spindle cell (sarcomatoid) carcinoma of the larynx. Case Rep Med. 2012, 2012:1-5. 10.1155/2012/370204
  14. Rutt AL, Mintz I, Jackson-Menaldi C, Johns M, McHugh JB, Rubin AD: Spindle cell carcinoma of the larynx presenting as a vocal fold cyst. J Voice. 2014, 28:524. 10.1016/j.jvoice.2013.10.013
  15. Zhang M, Zhao LM, Li XM, Zhou L, Lin L, Wang SY: True carcinosarcoma of the larynx. J Laryngol Otol. 2013, 127:100-103. 10.1017/S002221511200285X
  16. 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, 8:748-752. 10.3892/ol.2014.2172
  17. 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. 10.1155/2015/831835
  18. Rao BSS, Grandhi B, Shanthi V, Rao NM, Murthy BK: Carcinosarcoma of the larynx - a rare site entity. J Clin Diagn Res. 2016, 10:1-2. 10.7860/JCDR/2016/17174.7758
  19. Luna-Ortiz K, Mosqueda-Taylor A: Supracricoid partial laryngectomy as a primary treatment for carcinosarcoma of the larynx. Ear Nose Throat J. 2006, 85:337-341.
Case report
peer-reviewed

Age Limit and Radiotherapy Option for Sarcomatoid Carcinoma of the Larynx: A Case Report with Literature Review


Author Information

Mina Fransawy Alkomos Corresponding Author

Research, California Institute of Behavioral Neurosciences & Psychology, Sacramento, USA

Michael Rizk

Otolaryngology, Ain Shams University, Cairo, EGY

Goubran Eskander

Otolaryngology, Ain Shams University, Cairo, EGY

Ahmed Elkheshen

Internal Medicine, The Icahn School of Medicine at Mount Sinai, New York, USA

Rupak Mahendhar

Internal Medicine, Icahn School of Medicine at Mount Sinai/Queens Hospital Center, New York, USA

Amir Shahbaz

Internal Medicine, Icahn School of Medicine at Mount Sinai/Queens Hospital Center, New York, USA

Paria Zarghamravanbakhsh

Endocrinology, Icahn School of Medicine at Mount Sinai Queens Hospital Center, New York, USA

Phoebe Younan

Faculty of Medicine, Ain Shams University, Cairo, EGY

Nasim Golchin

Endocrinology, Icahn School of Medicine at Mount Sinai/Queens Hospital Center, New York, USA

Issac Sachmechi

Internal Medicine, Icahn School of Medicine at Mount Sinai/Queens Hospital Center, New York, USA


Ethics Statement and Conflict of Interest Disclosures

Human subjects: Consent was obtained by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.


Case report
peer-reviewed

Age Limit and Radiotherapy Option for Sarcomatoid Carcinoma of the Larynx: A Case Report with Literature Review


Figures etc.

SIQ
7.1
RATED BY 4 READERS
CONTRIBUTE RATING

Scholary Impact Quotient™ (SIQ™) is our unique post-publication peer review rating process. Learn more here.