Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 

 Table of Contents  
Year : 2013  |  Volume : 9  |  Issue : 2  |  Page : 284-286

Low-grade myofibroblastic sarcoma of the larynx: A rare entity with review of literature

1 Department of Radiotherapy and Oncology, Regional Cancer Centre, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Otolaryngology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication13-Jun-2013

Correspondence Address:
Budhi Singh Yadav
Department of Radiotherapy and Oncology, Regional Cancer Centre, Post Graduate Institute of Medical Education & Research (PGIMER), Sector 12, Chandigarh - 160012
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.113387

Rights and Permissions
 > Abstract 

Mesenchymal neoplasms of the larynx are rare and make up approximately 0.3% to 1.0% of all malignancies at this location. Low grade myofibroblastic sarcoma (LGMS) of larynx is a rare entity. We describe a rare case of LGMS of larynx who presented with complaint of hoarseness of voice. The patient was treated with total laryngectomy plus partial pharyngectomy followed by post-operative radiotherapy. Histopathologically, the lesion was composed of spindle cells that manifested variable cellular anaplasia and expressed smooth muscle actin (SMA) and focally S-100.One of the resection limits was involved so patient was given post-operative radiotherapy. The patient is alive and disease free 14 months after surgery. The characteristic clinical, histopathological features and treatment of this case are described with a literature review.

Keywords: Larynx, low grade, myofibroblast, myofibroblastic sarcoma, radiotherapy, surgery

How to cite this article:
Khosla D, Yadav BS, Kumar R, Ghoshal S, Vaiphei K, Verma R, Sharma SC. Low-grade myofibroblastic sarcoma of the larynx: A rare entity with review of literature. J Can Res Ther 2013;9:284-6

How to cite this URL:
Khosla D, Yadav BS, Kumar R, Ghoshal S, Vaiphei K, Verma R, Sharma SC. Low-grade myofibroblastic sarcoma of the larynx: A rare entity with review of literature. J Can Res Ther [serial online] 2013 [cited 2022 Oct 1];9:284-6. Available from: https://www.cancerjournal.net/text.asp?2013/9/2/284/113387

 > Introduction Top

Primary nonsquamous tumors of the larynx are typically submucosal, derived mainly from mesenchymal cells, and compose approximately 0.3-1.0% of all laryngeal tumors. [1] Tumors and tumor-like lesions of myofibroblasts constitute one of the more recent areas of mesenchymal/soft-tissue pathology to have become recognized.

Myofibroblasts are spindle cells having ultrastructural features in common with smooth muscle cells and fibroblasts. They constitute nosologic entities, which are termed "myofibroblastic tumors". Tumors with benign and rarely, malignant behavior are reported to belong to this group of lesions. Inflammatory myofibroblastomas, benign tumors composed of myofibroblastic proliferation and chronic inflammatory infiltrate, have been described at many locations. [2],[3],[4],[5] Myofibroblastomas are rare in the head and neck region and low-grade myofibroblastic sarcoma (LGMS) is even rarer. To the best of our knowledge, there are only three cases of myofibroblastic sarcoma of larynx reported in English literature. [6],[7],[8] Herein, we describe a rare case of LGMS that originated in the larynx with its clinicopathologic features and review of literature.

 > Case Report Top

A 55-year-old man presented at the otolaryngology clinic with 6 months history of hoarseness of voice. General examination revealed no abnormality. Indirect laryngoscopy revealed an ulcerative growth on laryngeal surface of epiglottis, anterior commissure and anterior two-thirds of both vocal cords. The biopsy was suggestive of laryngeal sarcoma of low- grade. Contrast enhanced computed tomography (CECT) showed a laryngeal mass with involvement of supraglottic larynx and hypopharynx with extension to the thyroid cartilage and thyroid gland. The patient was tracheostomized for the compromised respiratory function and then underwent total laryngectomy plus total thyroidectomy plus partial pharyngectomy and voice prosthesis. Histopathological examination revealed an infiltrative tumor composed of interlacing fascicles of spindle shaped tumor cells with focal herringbone pattern. The tumor cells consisted of moderately pleomorphic fusiform vesicular nucleus, conspicuous nucleoli, blunted ends and mitotic activity of 6-7 per high power field. In between these spindle cells, there were collections of polygonal cells [Figure 1]a. Collagen production, scattered multinucleate giant cells and focal chondroid differentiation were noted [Figure 1]b. Large areas of haemorrhage and necrosis were seen. The lateral resection limit was involved by tumor. The thyroid cartilage and gland were free of tumor. The tumor cells showed strong positivity for smooth muscle actin (SMA) [Figure 1]c, focally positive for S-100 [Figure 1]d but were negative for desmin, h Caldesmon, c-kit and pancytokeratin.
Figure 1: (a) Medium power photomicrograph of the tumor showing interlacing bundles of spindle shaped cells with moderate degree of anisonucleiosis. Scant amount of collagen in between the individual tumor cells is seen. There are collections of polygonal cells to stellate shaped cells in between the spindle shaped cells (H and E, × 300) (b) Medium power photomicrograph of the tumor to show chondroid differentiation and multinucleated cells amongst the spindle shaped tumor cells (H and E, ×300) (c) Medium power hotomicrograph to show cytoplasmic positivity for smooth muscle actin of the tumor cells (PAP, ×300) (d) Medium power photomicrograph to show occasional cytoplasmic positivity for S-100 by the spindle shaped cells, chondroid cells and the multinucleated giant cells (PAP, × 300)

Click here to view

The patient underwent post-operative radiotherapy in view of margin involvementfor a total dose of 45 Gy in 20 fractions. CECT at last follow-up did not reveal any tumor recurrence. The patient is doing well 14-month post-operatively.

 > Discussion Top

The myofibroblast has been well known as a cellular participant in wound-healing (granulation tissue), a variety of inflammatory or fibrosing non-neoplastic conditions and reactive tumor stroma. [9] The histologic appearance of myofibroblasts includes the presence of large cells, which are spindle-shaped to stellate in appearance, have round to oval nuclei with deep indentations and conspicuous nucleoli, and are characterized by the presence of abundant acidophilic and fibrillary appearing cytoplasm often demonstrating long cytoplasmic extensions. [5] This is supported by their reactivity to SMA and vimentin and the negative staining for desmin, S-100 protein, keratin, and h Caldesmon. [10] Further evidence for the myofibroblastic derivation is highlighted by the presence of fibronexus, and an epithelial subepithelial anchoring structure by electron microscopy. [11]

The most relevant differential diagnoses for our case are inflammatory myofibroblastic tumors, fibrosarcoma, leiomyosarcoma, spindle cell carcinoma and other benign mesenchymal spindle cell lesions. Inflammatory myofibroblastic tumors can readily be distinguished by the presence of the myofibroblastic spindle cells admixed with mature lymphocytes, histiocytes, and plasma cells. [5] Characteristic histological patterns include the fasciitis-like, compact spindle cell and hypocellular fibrous patterns, which are often seen in combination within the same tumor. Chromosomal translocations leading to activation of the ALK tyrosine kinase can be detected in approximately 50% of these tumors, particularly those arising in young patients. [12] Inflammatory myofibroblastic tumors of larynx have been reported by Wenig et al. [5] The laryngeal inflammatory myofibroblastic tumors primarily affect adults, are localized lesions producing regional but not systemic symptoms, predilect to the glottis, are amenable to conservative surgical resection, and behave indolently.

In a clinicopathologic study of 15 cases of myofibrosarcoma by Montgomery et al., [13] lesions mainly involved the head and neck, extremities, and trunk and ranged in size from 1.5 to 12 cm. All sarcomas displayed fascicular or storiform patterns. They concluded that overall behavior of LGMS is indolent but intermediate grade tumors may have higher incidence of recurrence and metastasis. Covello et al. [6] reported a case of LGMS in larynx of 69-year-old woman with a history of metastatic skin melanoma. The patient underwent a supracricoid partial laryngectomy with cricohyoidoepiglottopexy. All of the resection margins were negative and no vascular invasion was identified. No further treatment was given and the patient was disease free 1 year after resection of the laryngeal tumor. Ni et al. [7] described a case of LGMS of epiglottic-glossal surface in a 41-year-old woman who underwent an extended incision. Post-operatively, patient was not given any adjuvant treatment in view of negative surgical margins. Friedman et al. [8] retrospectively reviewed 38 patients of submucosal neoplasms of laryngeal introitus and reported one case of glottic myofibroblastic sarcoma who was treated with endoscopic surgery. MacGreggor et al. [14] reported two cases of low-grade malignant myofibroblastic tumors of the larynx. One patient underwent laryngectomy due to chronic obstructive pulmonary airway disease and compromised respiratory function and the second patient had local excision of the lesion. In our patient, tumor was infiltrative with findings of increased cellularity, nuclear pleomorphism and elevated mitotic activity reflecting its low-grade malignant features. Immunohistochemical studies showed strong reactivity to SMA, focally positive for S-100 and negative staining for desmin, h Caldesmon supporting their myofibroblastic derivation. Our patient underwent total laryngectomy, partial pharyngectomy and total thyroidectomy due to involvement of supraglottic larynx, glottis larynx and hypopharynx with extension to thyroid cartilage and gland on CECT. Post-operative radiotherapy was given in view of margin involvement. The patient is alive and disease free at last follow-up.

 > Conclusion Top

LGMS of the larynx is extremely rare. Excision with clear margins is considered to be adequate; however, tumors with positive margins may be treated with post-operative radiotherapy to prevent recurrence. More case reports and series are needed for determining the nature, prognosis and treatment options for these rare tumors.

 > References Top

1.Batsakis JG, Fox JE. Supporting tissue neoplasms of the larynx. Surg Gynecol Obstet 1970;131:989-97.  Back to cited text no. 1
2.Wargotz ES, Weiss SW, Norris HJ. Myofibroblastoma of the breast. Sixteen cases of a distinctive benign mesenchymal tumor. Am J Surg Pathol 1987;11:493-502.  Back to cited text no. 2
3.Prayson RA, Estes ML, McMahon JT, Kalfas I, Sebek BA. Meningeal myofibroblastoma. Am J Surg Pathol 1993;17:931-6.  Back to cited text no. 3
4.Sahin AA, Ro JY, Ordoñez NG, Luna MA, el-Naggar AK, Goepfert H, et al. Myofibroblastoma of the tongue. An immunohistochemical, ultrastructural, and flow cytometric study. Am J Clin Pathol 1990;94:773-7.  Back to cited text no. 4
5.Wenig BM, Devaney K, Bisceglia M. Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. Cancer 1995;76:2217-29.  Back to cited text no. 5
6.Covello R, Licci S, Pichi B, Spriano G, Vidiri A, Morelli L, et al. Low-grade myofibroblastic sarcoma of the larynx. Int J Surg Pathol 2011;19:822-6.  Back to cited text no. 6
7.Ni C, Xu YY, Zhou SH, Wang SQ. Differential diagnosis of inflammatory myofibroblastictumour and low-grade myofibroblastic sarcoma: Two case reports with a literature review. J Int Med Res 2011;39:311-20.  Back to cited text no. 7
8.Friedman AD, Burns JA, Lutch MJ, Zeitels SM. Submucosal neoplasms of the laryngeal introitus. J Laryngol Otol 2012;126:706-13.  Back to cited text no. 8
9.Eyden B, Banerjee SS, Shenjere P, Fisher C. The myofibroblast and its tumours. J Clin Pathol 2009;62:236-49.  Back to cited text no. 9
10.Watanabe K. Leiomyosarcoma versus myofibrosarcoma. Am J Surg Pathol 2002;26:393-4.  Back to cited text no. 10
11.Eyden B. The fibronexus in reactive and tumoral myofibroblasts: Further characterisation by electron microscopy. Histol Histopathol 2001;16:57-70.  Back to cited text no. 11
12.Gleason BC, Hornick JL. Inflammatory myofibroblastic tumours: Where are we now? J Clin Pathol 2008;61:428-37.  Back to cited text no. 12
13.Montgomery E, Goldblum JR, Fisher C. Myofibrosarcoma: A clinicopathologic study. Am J Surg Pathol 2001;25:219-28.  Back to cited text no. 13
14.MacGregor AR, Batsakis JG, El-Naggar AK. Myofibroblastoma of the larynx: A study of two cases. Head Neck 2003;25:606-11.  Back to cited text no. 14


  [Figure 1]

This article has been cited by
1 Effective Treatment of Low-Grade Myofibroblastic Sarcoma with Apatinib: A Case Report and Literature Review
Yan Lin, Xing Gao, Ziyu Liu, Zhihui Liu, Yongqiang Li, Rong Liang, Zhiling Liao, Jiazhou Ye
Pharmacogenomics and Personalized Medicine. 2022; Volume 15: 573
[Pubmed] | [DOI]
2 Low-grade myofibroblastic sarcoma of the tongue with difficulty of diagnosis: A case report and review of the literature
Masami Kawaharada, Wataru Katagiri, Satoshi Maruyama, Hideyoshi Nishiyama, Takafumi Hayashi, Tadaharu Kobayashi, Jun-ichi Tanuma
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology. 2021; 33(1): 93
[Pubmed] | [DOI]
3 Primary low-grade myofibroblastic sarcoma: A rare case report of this tumor in the orbit and literature review
Lijuan Tang, Hua Xu, Huanhuan Gao, Huasheng Yang, Shuxia Chen, Ping Zhang
European Journal of Ophthalmology. 2020; : 1120672120
[Pubmed] | [DOI]
4 Low-grade myofibroblastic sarcoma of the levator scapulae muscle: a case report and literature review
Hirotaka Yonezawa, Norio Yamamoto, Katsuhiro Hayashi, Akihiko Takeuchi, Shinji Miwa, Kentaro Igarashi, Mickhael Bang Langit, Hiroaki Kimura, Shingo Shimozaki, Takashi Kato, Sei Morinaga, Yoshihiro Araki, Yohei Asano, Hiroko Ikeda, Takayuki Nojima, Hiroyuki Tsuchiya
BMC Musculoskeletal Disorders. 2020; 21(1)
[Pubmed] | [DOI]
5 Evaluation and Management of an Uncommon Tumor of the Larynx: A Case Report and Literature Review of Laryngeal Low-Grade Myofibroblastic Sarcoma
Nithin P Nair, Darwin Kaushal, Meenakshi Rao, Kapil Soni, Sameema Vaithankalath
Cureus. 2020;
[Pubmed] | [DOI]
6 Low-Grade Myofibroblastic Sarcoma of the Larynx
Diana Vlad, Silviu Albu
Journal of Craniofacial Surgery. 2016; 27(3): e270
[Pubmed] | [DOI]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  >Abstract>Introduction>Case Report>Discussion>Conclusion>Article Figures
  In this article

 Article Access Statistics
    PDF Downloaded260    
    Comments [Add]    
    Cited by others 6    

Recommend this journal