Epithelial-Myoepithelial Carcinoma of Nasal Cavity at an Unusual Age: A Case Report and Review of Literature

Abstract

Introduction: Epithelial-myoepithelial carcinoma (EMC) was recognised as a distinct pathologic entity in World Health Organisation classification. It is an extremely rare low grade carcinoma of salivary gland, with characteristic biphasic tubular structures. It predominantly occurs in Parotid gland but can also be seen in nasopharynx, lacrimal gland, paranasal sinuses, larynx, lung. Nasal EMC (excluding the paranasal sinuses as primary tumour site) are very rare with only 13 cases reported till date. In this case report, we described a case of nasal EMC extending into nasopharynx, its clinical features and management. We have also done a literature review of all the relevant cases of nasal EMC.

Material and methods: We searched the PubMed database for articles between January 1950 and December 2022 for nasal EMC for this review.

Results: We found 13 relevant case reports of nasal EMC and median age was 58 years with female preponderance. We found that our patient was the youngest to be reported till date. Two cases, including the current study, showed epicentre of the tumour in posterior nasal cavity, extending to choana and nasopharynx. Most common presentation was epistaxis, followed by nasal obstruction. Only 4 out of 14 cases had information on surgical margin status, out of which only one has positive surgical margin. Five patients (including the patient in the current study) received adjuvant radiotherapy; however 6 patients (42.8%) did not receive any adjuvant radiotherapy.

Keywords: EMC; Epithelial–myoepithelial carcinoma; Sinonasal.

Fig. 1

Post operative CT scan

Fig. 2

Histopathology (H&E stain, 2 × view)

Fig. 3

IHC showing Calponin positive cells

Fig. 4

PET scan showing FDG avid soft tissue thickening at post-operative site

Fig. 5

Planning target volume for radiotherapy