Discussion
Pleomorphic Adenoma is a benign tumor, the histology is derived from leap tube epithelium of salivary glands. it is also called a mixed adenomatosis due to its tendency towards multidirectional differentiation (myoepithelial differentiation and glandular epithelium differentiation). They are mainly distributed in the major salivary gland, of which about 85% occur in the parotid gland, 8% in the submandibular gland, and about 7% in the minor salivary gland [
5]. Which includes the hard and soft palate, the nasal cavity, pharynx, larynx, trachea, and lacrimal glands [
1]. The clinical incidence of septum is rare.
Pleomorphic adenoma which originates from the mucosa of nasal septum have the different hypotheses [
6]. Matthew et al. suggested that the abnormal origin of these tumors may come from the mucosa of the nasal septum, possibly due to the misplacement of embryonic epithelial cells, which come from the epidermis and enter the nasal septum through the septal region. According to Stevenson pointed out, the tumors in this area originates from a remnant of the vomeronasal organ, an epithelium-lined duct in the septum that degenerated in early fetal life. Conversely, Evans and Cruickshank state that, at present, mixed neoplasms are almost universally believed to originate entirely from epithelial cells and occur in fully developed salivary gland tissues rather than embryonic remains [
7].
Pleomorphic Adenoma is usually unilateral nasal diseases, growth slow, mostly expansive growth. There are different clinical features according to the tumor progression and size [
2,
3,
7‐
9]. In the early stages, the tumor is small, may not have obvious clinical manifestations, and is often diagnosed by physical examination or incidental discovery. As the tumor continues to grow, the nasal passages are blocked, it can be present as a unilateral nasal congestion, progressive aggravation, and accompanied by nose bleeding or headache, smell disorders and so on. If the tumor expands further and grows along the nasal space, it often leads to poor sinus drainage, sinusitis or mucosal edema, and even polyps. At the same time, the surface of the enlarged tumor envelope may become necrotic and form granulation. Swelling, pain and deformities may occur in the nose or face if the surrounding tissues are involved. If the dacryocyst and orbit are violated, tears overflow, eyeball displacement, and diplopia may occur. Physical examination showed that the tumors were mostly round, oval, nodular or lobular, with wide base, smooth or uneven surface, medium hardness, complete envelope, clear boundary, poor mobility, and easy palpation bleeding.
The radiographic findings of pleomorphic adenomas in the nasal cavity are nonspecific, much like the clinical presentation. CT usually shows a clear soft tissue mass; Calcification.
occurs only in rare cases.The clinician can evaluate bone involvement or destruction based on CT, which may occur when a neoplasm has been untreated for a long time [
10].
Magnetic Resonance Imaging (MRI) is primarily used to evaluate the epithelial and stromal components of tumors, as well as the surrounding soft tissues. MRI of interstitial components showed T1-weighted low signal intensity and T2-weighted medium-high signal intensity. Epithelial components have low signal intensity on T2-weighted imaging [
6]. Kajiyama et al. [
11] found that MRI of nasal polymorform adenoma mainly showed submucosa lesion, and suggesting that preoperative MRI examination should be performed to ensure the complete resection of the tumor.
Diagnosis of Pleomorphic Adenoma mainly relies on pathologic examination of the mass [
2‐
4,
6].
At low magnification, the outer cells of the lumen were arranged in palisades, nests, sheets or strips, with obvious proliferation and abundant lumen structure. At high magnification, tumor myoepithelial cells were mixed with epithelial cells, or are arranged like spindle, plasma cells, clear cells, and epithelial cells. Unlike the pleomorphic adenoma of the large salivary gland, the PANS contain more cellularity; predominant epithelial components; Low stromal component and absent capsule.
Immunohistochemistry can helpful in the diagnosis of pleomorphic adenoma: [
2,
4] Ki-67 is used to judge the proliferation activity of cells, and previous studies have shown that the positive expression rate of Ki-67 in pleomorphic adenoma of the nose is 1% ~ 5%. S-100, P63, CK (AE1/AE3) and SMA are all sensitive markers of myoepithelial cells, and studies have shown that they are all positive in pleomorphic adenoma of nose.
Differential diagnosis of intranasal pleomorphic adenoma includes both benign and malignant tumours such as polyps, capillary tumor, papillomas, angiofibromas, myoepithelioma, retention cyst, osteomas, squamous cell carcinoma, mucoepidermoid carcinoma, adenocarcinoma, adenoid cystic carcinoma, melanoma and olfactory esthesioneuroblastoma [
12].
Surgical treatment is the preferred treatment for nasal pleomorphic adenomas [
6,
8]. The goal is to preserve a safe margin, remove the tumor completely, and prevent metastasis of tumor cells through blood or lymph node implantation [
13]. Different surgical methods can be used according to tumor growth site and size. It include endonasal endoscopic resection, midfacial degloving approach, lateral rhinotomy, and partial maxillectomy. [
2,
14] Endoscopic resection of this type of tumor has become the preferred surgical method for this tumor due to its advantages of less damage, less damage to the tumor envelope, better protection of the normal structure of the nasal cavity, and better recovery of the physiological function of the nasal cavity after surgery [
15].
However, surgical indications should be considered in endoscopic resection of septum pleomorphic adenoma.
If the nasal tumor is large, poorly defined, or suspected of malignant metastasis, rhinotomy is recommended to avoid recurrence or tumor tumor rupture due to incomplete or insufficient resection [
8,
16].
There are reports that malignant transformation of PNAS is still very rare, with fewer than 10 cases [
17]. The incidence has been reported in 2.4 to 10% of cases [
14]. The reasons for recurrence are may be as follows:
(1) Partial tumor tissue capsule adheres to normal nasal mucosa, which is not completely removed during the operation, resulting in residual tumor tissue.
(2) Tumor rupture, tumor tissue overflow and lead to implant recurrence.
Therefore, Freeman et al. [
18] suggested extending local resection to ensure the integrity of tumor capsule so as to prevent local and distant implantation of tumor cells. At the same time, if the tumor is suspected to be preoperatively, routine biopsy should not be performed to prevent capsule rupture, and rapid frozen sections can be performed intraoperatively. In order to completely remove the tumor, an incision should be made in the normal tissue outside the tumor boundary. If the tumor is found to be broken intraoperatively, the tumor tissue should be carefully removed, and the normal saline should be repeatedly rinsed and the surgical instruments replaced [
16].
Pleomorphic adenomas do not respond well to chemotherapy and radiotherapy. In addition, radiation may induce malignant transformation of tumors [
19]. Therefore, it is generally not recommended. However, it has also been reported that postoperative adjuvant radiotherapy can reduce the recurrence rate of positive surgical margin and polynodular pleomorphic adenoma [
16,
20].
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