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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 2  |  Issue : 2  |  Page : 179-181

Myofibroblastic sarcoma of the nasal sill presenting as extranasal polyp: An extreme rarity


Department of Burns and Plastic Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Date of Submission27-May-2020
Date of Decision07-Jun-2020
Date of Acceptance18-Nov-2020
Date of Web Publication08-Jul-2021

Correspondence Address:
Dr. A J Praveen
Department of Burns and Plastic Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JME.JME_58_20

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How to cite this article:
Praveen A J, Jagtap MP, Chattopadhyay D. Myofibroblastic sarcoma of the nasal sill presenting as extranasal polyp: An extreme rarity. J Med Evid 2021;2:179-81

How to cite this URL:
Praveen A J, Jagtap MP, Chattopadhyay D. Myofibroblastic sarcoma of the nasal sill presenting as extranasal polyp: An extreme rarity. J Med Evid [serial online] 2021 [cited 2021 Dec 8];2:179-81. Available from: http://www.journaljme.org/text.asp?2021/2/2/179/324977




  Introduction Top


Myofibroblastic sarcoma is an extremely rare condition in the nasal cavity and paranasal sinuses, with only seven cases reported in literature till date.[1] Clinically, they present as intranasal mass with episodes of epistaxis and copious rhinorrhoea. Here, we present an unusual presentation of this extremely rare tumour as an extranasal mass.


  Case Report Top


A 21-year-old female presented with a painless mass protruding from her left nostril for 5 months' duration. There was no associated epistaxis, rhinorrhoea, nasal obstruction or proptosis. There was no history of antecedent trauma. The patient did not have any constitutional symptoms such as fever, anaemia and weight loss. Examination revealed a polypoid mass of approximately 3 cm × 3 cm in size [Figure 1]; pinkish brown in colour, with lobulated surface and firm in consistency, which was non-tender, non-compressible and non-pulsatile. The tumour appeared to be attached to the nasal sill at the left nostril with a short stalk of 4 mm in length and 6 mm in width, separate from the columella and ala, and was freely mobile and clinically, it was not showing any fixity to the underlying structures. Nasal endoscopy showed the mass to be arising from the nasal sill area and was not involving the nasal cavity or the paranasal sinuses. Because both these observations were clearly pointing towards the fact that the swelling is a benign entity limited to the nasal sill area, imaging was not done and the tumour was taken up for excision biopsy. Because nasal polyps are generally vascular and also because we had to do a detailed nasal endoscopy to visualise all the paranasal sinuses, we decided to excise the tumour under general anaesthesia. The tumour was excised including an adequate margin of skin and soft tissue of 0.5 cm all around the base of the tumour stalk. No destruction of local cartilage or bone was seen preoperatively. On histological examination [Figure 2]a and [Figure 2]b, the tumour sections were cellular, well vascularised and composed of spindle-shaped and polygonal cells, with vesicular nuclei showing nuclear pleomorphism and mitotic figures. The histopathology was consistent with spindle cell sarcoma of intermediate grade. Immunohistochemistry revealed that the tumour cells expressed smooth muscle actin, desmin, CD34 (focal) and cytokeratin and were immunonegative for S-100 protein, Ets-related gene protein, epithelial membrane antigen, HMB-45, Melan A, CD21, CD23 and CD35. The final impression was that of an intermediate-grade spindle cell sarcoma showing a myofibroblastic phenotype. Once the diagnosis of myofibroblastic sarcoma was made, chest X-ray and ultrasonography (USG) abdomen were performed, which did not show any features of metastasis. Neck examination did not reveal any features of lymphatic metastasis to the neck nodes. The case was discussed in the tumour board and the patient was advised to be on regular follow-up. Nasal endoscopy was done on the follow-up visits at 1 month, 3 months and 6 months after the surgery. The patient is doing well on follow-up, with no sign of local recurrence or distant metastasis at 7 months after tumour excision.
Figure 1: Polypoid mass protruding from the nasal sill

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Figure 2: (a) Tumour cells seen as a lobulated mass present in the subcutis below the keratinised stratified squamous epithelium (H and E, ×4). (b) The oval tumour cells exhibit abundant, pale cytoplasm; ovoid nuclei with vesicular chromatin and moderate atypia; and occasional eosinophilic nucleoli. Mitotic figures are seen (H and E, ×40)

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  Discussion Top


As the patient was a resident of West Bengal, the first differential diagnosis that was considered was of rhinosporidiosis.[2] Ethmoidal polyp also has a similar presentation but was ruled out as nasal endoscopy showed that the mass was arising from the nasal sill. Other rare tumours such as sebaceous gland carcinoma, ganglioneuroma and schwannoma also have a similar presentation in this anatomical region. Because there are no existing reports of myofibroblastic sarcoma presenting as an extranasal mass, it was not considered a possible diagnosis. However, to our surprise, histopathology of the excised specimen revealed that the tumour was a rare case of myofibroblastic sarcoma. However, the report showed that the deep structures such as nasal cartilage were not involved by the tumour. Nasal endoscopies done in the follow-up visits did not show any recurrence, and there was no other mass in the nasal cavity or paranasal sinuses. In addition, on examination of the neck, there were no palpable nodes. The chest X-ray and USG abdomen performed did not show any features of metastasis. Myofibroblastic sarcoma is a very rare tumour. Mentzel reported it for the first time as a distinct entity in 1998.[3] It is a solid tumour of mesenchymal origin.[4] There are only seven reported cases of these tumours presenting as an extranasal mass.[1] Our literature search revealed that there is no consensus regarding the treatment protocols or excision margins regarding this tumour presenting in the head-and-neck region.[5] In a similar case of myofibroblastic sarcoma of the orbit reported by Zhang et al., they have not taken any margins at all and in that case, there was no recurrence in the 1-year follow-up period. In their case too, they had initially considered the tumour as a benign entity based on clinical and radiological findings, but post-operative histopathology revealed the diagnosis of myofibroblastic sarcoma.[6] Studies show that although these tumours are sarcomas, they have relatively benign/low-grade malignant characteristics.[7] Surgery is the primary modality of treatment for myofibroblastic sarcoma.[8] Other modalities such as chemotherapy or radiotherapy are indicated only if there are features of local tissue invasion or distant metastasis.[1] All these points are in agreement with the fact that the surgical treatment given in the form of excision biopsy was adequate. The point to carry home from this case in our experience is that all nasal polyps have to be sent for histopathology irrespective of their clinical presentation.


  Conclusion Top


Myofibroblastic sarcomas of the nasal cavity and paranasal sinuses are extremely rare, with only seven cases described in the literature. All the reported patients had extensive involvement of the nasal cavity and paranasal sinuses, and presented with nasal obstruction, copious rhinorrhoea and epistaxis, with orbital involvement causing proptosis in 50% of cases. In our case, the tumour presented as an extranasal polypoid mass without any involvement of the nasal cavity or paranasal sinuses, and the typical clinical features were absent. To the knowledge of the authors, there is no previous report of myofibroblastic sarcoma arising from the nasal sill and also having such a unique presentation. Our case also reemphasises the importance of histopathology (and immunohistochemistry if indicated) for all nasal polyps irrespective of their clinical appearance.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Meng GZ, Zhang HY, Bu H, Yang GH, Zhang XL, Yang G. Myofibroblastic sarcoma of the nasal cavity and paranasal sinus: A clinicopathologic study of 6 cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:530-9.  Back to cited text no. 1
    
2.
Shetty V, Kulkarni A, Banerjee S. Long-term use of Le Fort I osteotomy for the management of nasopharyngeal rhinosporidiosis: A case series. Ear Nose Throat J 2018;97:E36-E43.  Back to cited text no. 2
    
3.
Agaimy A, Wünsch PH, Schroeder J, Gaumann A, Dietmaier W, Hartmann A, et al. Low-grade abdominopelvic sarcoma with myofibroblastic features (low-grade myofibroblastic sarcoma): Clinicopathological, immunohistochemical, molecular genetic and ultrastructural study of two cases with literature review. J Clin Pathol 2008;61:301-6.  Back to cited text no. 3
    
4.
Arora R, Gupta R, Sharma A, Dinda AK. A rare case of low-grade myofibroblastic sarcoma of the femur in a 38-year-old woman: A case report. J Med Case Rep 2010;4:121.  Back to cited text no. 4
    
5.
Maruyama T, Nakasone T, Nimura F, Matayoshi A, Kawano T, Nishihara K, et al. Indolent growth of low-grade myofibroblastic sarcoma of the cheek mimics benign lesions: A case report and literature review. Oncol Lett 2017;13:4307-14.  Back to cited text no. 5
    
6.
Zhang S, Ma Y, Ma T, Wang Z. Low-grade myofibroblastic sarcoma of the orbit: A case report and literature review. Medicine (Baltimore) 2017;96:e9172.  Back to cited text no. 6
    
7.
Montgomery E, Goldblum JR, Fisher C. Myofibrosarcoma: A clinicopathologic study. Am J Surg Pathol 2001;25:219-28.  Back to cited text no. 7
    
8.
Keller C, Gibbs CN, Kelly SM, Haller JR, White KS, Coffin CM, et al. Low-grade myofibrosarcoma of the head and neck: Importance of surgical therapy. J Pediatr Hematol Oncol 2004;26:119-20.  Back to cited text no. 8
    


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