Pleomorphic Adenoma: An Unusual Presentation on the Nasal Septum

Ameen Sumais AP¹ https://orcid.org/0000-0002-2584-7617, Santhosh Kumar N², Smrithi S Thomas³, Sudheesh Manoharan⁴, Bharathi K⁵, Padma E⁶

^1,2,4-6Department of Head and Neck Surgical Oncology, MVR Cancer Centre & Research Institute, Calicut, Kerala, India

³Department of Pathology, MVR Cancer Centre & Research Institute, Calicut, Kerala, India

Corresponding Author: Ameen Sumais AP, Department of Head and Neck Surgical Oncology, MVR Cancer Centre & Research Institute, Calicut, Kerala, India, Phone: +91 8921085244, e-mail: ameensumaiz@gmail.com

Received on: 13 April 2023; Accepted on: 05 June 2024; Published on: 17 July 2024

ABSTRACT

Pleomorphic adenoma rarely arises from the minor salivary glands. It has been reported at various sites where minor salivary glands are found, including the nasal cavity, oral cavity, nasopharynx, oropharynx, larynx, hypopharynx, trachea, and even lacrimal glands. In the nasal cavity, it usually arises from the minor salivary glands of septal mucosa. Intranasal pleomorphic adenoma may be misdiagnosed because it has high myoepithelial cellularity and fewer myxoid stroma compared to those arising from other sites. We report a case of pleomorphic adenoma in the nasal septum of an elderly woman who was treated for olfactory neuroblastoma 25 years ago. She presented with epistaxis from the left nostril for the last 4 months. Diagnostic nasal endoscopy showed a fleshy growth with crusting attached to the anterior nasal septum. She was evaluated elsewhere, and the histopathology report was suggestive of olfactory neuroblastoma. The slide review at our center was suggestive of pleomorphic adenoma, and immunohistochemistry (IHC) was positive for cytokeratin and p63 and negative for synaptophysin. Her case was discussed by a multidisciplinary team (MDT), and she underwent excision of the lesion with wide surgical margins using a lateral rhinotomy approach. Though pleomorphic adenoma from the septum is rare, it should be kept in mind while evaluating unilateral nasal obstruction/epistaxis in middle-aged patients.

How to cite this article: AP AS, N SK, Thomas SS, et al. Pleomorphic Adenoma: An Unusual Presentation on the Nasal Septum. Int J Head Neck Surg 2024;15(1):14-16.

Source of support: Nil

Conflict of interest: None

Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.

Keywords: Case report, Pleomorphic adenoma, Pleomorphic adenoma of nasal septum, Pleomorphic adenoma of minor salivary glands, Sinonasal pleomorphic adenoma.

INTRODUCTION

Pleomorphic adenoma (PA) is the most common benign tumor of the major and minor salivary glands. Only 8–10% of PA arises from the minor salivary glands distributed in the upper aerodigestive tract and is most commonly found in the palate.¹ Histologically there are three main groups: Myxoid (80% stroma), cellular (myoepithelial predominant), and mixed (classic). The classic variant is the most common type from minor salivary glands. PA from septal mucosa is unusual and unique. It usually arises from the septum, even though the majority of minor salivary glands are located on the lateral nasal wall and inferior turbinate, and it is cellular (myoepithelial predominant) compared to its counterparts from major salivary glands.² Greater epithelial cellularity of these tumors may contribute to misdiagnosis but also distinguishes these lesions from those mixed tumors commonly found in major salivary glands. We report a case of PA of nasal septum in an elderly woman with a history of olfactory neuroblastoma in the past.

CASE DESCRIPTION

A 59-year-old woman presented with bleeding from the left nostril in the last 4 months. She had a history of olfactory neuroblastoma 25 years ago and underwent endoscopic endonasal surgery, but the treatment details were not available. She was evaluated at a local hospital for the nasal bleed, and biopsy from the left nasal cavity growth was suggestive of olfactory neuroblastoma. On diagnostic nasal endoscopy at our center, she had a fleshy growth with crusting attached to the anterosuperior part of the cartilaginous nasal septum. Contrast-enhanced magnetic resonance imaging (MRI) (Figs 1A and B) showed a circumscribed polypoidal lesion in the left anterior nasal cavity measuring 8.6 × 20 × 12 mm [craniocaudal (CC) × anteroposterior (AP) × transverse (TR)]; T2/STIR hyperintensity and homogeneous postcontrast enhancement. The histopathology slides from the local hospital were reviewed and reported as PA. On immunohistochemistry, tumor cells were positive for cytokeratin and p63 and negative for synaptophysin (Figs 2A to D). The case was discussed by a multidisciplinary team (MDT) and planned for surgical excision. After obtaining consent and fitness for general anesthesia, she underwent a wide excision of the lesion using a lateral rhinotomy approach.³ A segment of cartilage attached to the mass was removed, and mucoperichondrium on the right side was preserved. Her postoperative period was uneventful. She is now kept under follow-up in view of the chance of recurrence.⁴^,⁵

Figs 1A and B: (A) MRI shows a circumscribed polypoidal lesion in the left anterior nasal cavity with T2/STIR hyperintensity; (B) MRI shows circumscribed polypoidal lesion in the left anterior nasal cavity with homogeneous postcontrast enhancement

Figs 2A to D: (A) Histopathology shows myoepithelial cells, which are spindle cells and ductal cells (epithelial cells) in a myxoid stroma; (B) IHC cytokeratin-positive, highlighting the ductal cells; (C) IHC p63-positive, highlighting the myoepithelial cells; (D) IHC synaptophysin shows negative staining

DISCUSSION

Pleomorphic adenoma is the most common benign salivary gland tumor. It is composed of epithelial (ductal) cells, myoepithelial cells, and chondromyxoid stroma. This tumor develops from the intercalated duct and myoepithelial cells of the glandular component. It can arise from the parotid gland, submandibular gland, and other minor salivary glands. Minor salivary glands are distributed submucosally in the upper aerodigestive tract. PA from minor salivary glands has been reported in the sinonasal cavity, oral cavity, nasopharynx, oropharynx, hypopharynx, larynx, trachea, auditory canal, and even lacrimal glands.³^,⁴

Compagno and Wong² in 1977 published one of the largest case series on intranasal PA from 1949 to 1971 (n = 40). The majority of these tumors (25 of 40) originated from bony or cartilaginous septum. The majority of cases occurred through the third to sixth decades of life with no significant sex predilection. They also reported some previously unrecognized features that the histologic features of these mixed tumors of the nasal cavity differed in certain characteristics. Cellularity was usually greater in these tumors than in mixed tumors of the major salivary glands, with little or no stromal components.

Rha et al.⁴ in 2019 published a comprehensive literature review of sinonasal PA from 1966 to 2016 and identified 101 cases. The septum was the primary origin in 57 cases. Other subsites in decreasing order include lateral nasal wall (16) inferior turbinate (8) maxillary sinus (6), and nasal vestibule (5). Their study reported a female predominance (59.4%). Eight patients had locoregional recurrence on follow-up.

Diagnosis of PA usually relies on histopathological examination and is confirmed by immunohistochemical studies. In our case, the histopathology showed myoepithelial cell predominance, and the stroma showed a myxoid component. Immunohistochemistry (IHC) was positive for cytokeratin, which highlights the epithelial cells, and p63, a marker for myoepithelial cells in salivary gland neoplasms. IHC was negative for synaptophysin, NKX2.2, desmin and GATA3, which ruled out neuroendocrine tumors, sarcomas, and salivary gland malignancies.

The treatment for sinonasal PA is wide local excision with negative margins.⁵ The approaches include endoscopic endonasal, lateral rhinotomy, and midfacial degloving. The choice of approach is determined by the anatomical location of the tumor. Intranasal PAs have a relatively low rate of recurrence (10%) compared with recurrence rates as high as 50% for parotid gland and 25% for intraoral tumors.⁶ In few cases, the tumor can turn into carcinoma ex PA. So, regular follow-up with nasal endoscopy is recommended.

CONCLUSION

Pleomorphic adenoma from the nasal septum is unusual. It may bring diagnostic dilemmas because of its unique feature of greater myoepithelial cellularity. Though rare, the differential diagnosis of PA should be kept in mind while evaluating unilateral nasal obstruction/epistaxis in middle-aged patients.

ORCID

Ameen Sumais AP https://orcid.org/0000-0002-2584-7617

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