Pleomorphic Adenoma (PA) is the most common benign salivary gland tumor, accounting for approximately 60–70% of benign salivary neoplasms and about 40% of all salivary gland tumors [1,2]. It primarily affects the parotid gland, followed by the minor salivary glands— most frequently located in the hard palate, upper lip, and buccal mucosa [3]. Ectopic or unusual presentations, particularly in the nasal vestibule or nasogenian region, are extremely rare and often misdiagnosed due to their atypical location and indolent progression.

The pathogenesis of PA in ectopic sites is not fully understood. Two main hypotheses have been proposed: (1) heterotopic salivary tissue remnants during embryologic development, particularly in areas where salivary glands are normally sparse (e.g., nasal cavity, nasopharynx, upper neck), and (2) neoplastic transformation of minor salivary gland cells present in ectopic mucosa or subcutaneous tissue [4,5].

Clinically, PA typically presents as a slow-growing, painless, and well-circumscribed mass. Its indolent evolution often leads to diagnostic delays, particularly in elderly patients who may ignore or adapt to the mass. In our case, the patient had a five-year history of a growing lesion without alarming symptoms. The simultaneous presence of a second lesion (inner cheek) added diagnostic complexity and initially suggested distinct etiologies.

Radiologically, PA appears as a well-defined, homogenous soft-tissue mass. On CT scans, it typically shows a non-invasive, rounded lesion without bone erosion or calcifications. MRI, which was not used in this case but is considered superior for soft tissue characterization, usually shows a lesion with low-to-intermediate signal intensity on T1-weighted images and high intensity on T2-weighted images, often with a well-defined capsule [6,7]. In our patient, the CT showed regular, ovoid lesions without bony destruction or signs of deep invasion, which supported a benign etiology.

Histologically, pleomorphic adenoma is composed of a mixture of epithelial and myoepithelial cells embedded in a chondromyxoid stroma. The lesion is usually well- encapsulated but may show microscopic pseudopodial extensions beyond its capsule, which can account for recurrence if incompletely excised [8]. The surprise in our case was the definitive histopathological diagnosis of PA in a highly atypical location—near the nasal vestibule and nasogenian sulcus—where minor salivary glands are not commonly found.

While pleomorphic adenoma is benign, there is a known risk of malignant transformation, particularly in long-standing lesions. The risk of transformation into carcinoma ex- pleomorphic adenoma is estimated at 5–10%, especially in tumors present for more than 10 years or those recurring multiple times [9,10]. Thus, complete surgical excision with adequate margins is essential, regardless of the location.

Surgical management of PA depends on the tumor's location, size, and proximity to critical structures. For intraoral or superficial facial lesions, a transoral or minimal access approach may suffice. In contrast, for lesions adherent to surrounding tissue or located in anatomically confined areas, more extensive approaches may be required. In our case, the para-lateral nasal approach was selected due to the lesion's subcutaneous infiltration and its firm adhesion to superficial planes, allowing for safe and complete removal. The cheek lesion was resected via an intraoral approach, which is standard for such locations [11].

The rarity of pleomorphic adenoma in the nasal vestibule or adjacent areas has been documented only in isolated case reports or small series. Compagno and Wong (1977) first described intranasal PA in a series of 20 cases, emphasizing their histologic similarity with their oral counterparts but clinical variability [12]. Since then, sporadic reports have described PA in locations such as the nasal septum, columella, and even the lateral nasal wall [13–15].

Our case is notable for presenting two synchronous pleomorphic adenomas, one in a typical site (inner cheek) and another in a highly atypical one (left nasogenian sulcus / nasal vestibule), both histologically benign. To our knowledge, such a synchronous dual presentation in an elderly patient is extremely rare.