Pleomorphic adenoma is a benign, slow-growing neoplasm that most commonly affects the parotid gland. It has epithelial, myoepithelial, and stromal components. The prognosis is generally good after the surgical removal, however, there is an increased incidence of recurrence particularly if the parotid gland is affected.
Presentation
Pleomorphic adenoma (PA) is the most common type of benign tumor of the salivary gland [1] [2] [3].
Pleomorphic adenoma or benign mixed tumor, occur most frequently (85%) in the parotid glands [2], followed by the minor salivary glands in 10% of the cases, and then by the submandibular glands at 5% [2]. The adenoma presents as a small, mobile, slowly growing, painless mass of 2-6 cm; however, occasionally much larger tumors have been encountered [4]. Patients have varying symptoms, which may depend on the location of the tumor, and may include hoarseness of voice, dyspnea, and difficulty in chewing and eating.
The tumors occur most frequently in middle-aged women. They originate in most cases from the superficial lobe of the parotid gland and present as a swelling of the gland below the ear; however, they can develop in the deep lobe of the parotid gland, where they will not be easily noticed in early stages [5]. Tumors of the deep lobe of the parotid gland, and of the minor salivary glands sometimes appear in the parapharyngeal space [6]. More common locations for adenomas of the minor salivary glands are the hard and soft palate, and the mucosa of the lip [7]. Most tumors are solitary, but bilateral tumors have also been found [5]. The facial nerve is rarely involved.
The adenomas are often encapsulated. The histological pattern varies case by case [3[. All tumors are mixtures of epithelial cells, myoepithelial and basal cells, with varying contributions from stroma; using the relative amounts of different cell types and stroma for predicting future outcomes has not yet met with success [3].
Although PA is a benign tumor, it can become malignant. Surgical removal of the tumor by enucleation –or preferably by parotidectomy at the first incidence [8]- is the cornerstone of treatment. Surgery has to be done carefully to preserve the facial nerve and to avoid rupture, which could lead to recurrence of the tumor.
Pleomorphic adenomas of the lacrimal glands (PALG) constitute about a tenth of the lacrimal gland tumors. Patients with PALG present with slowly progressing proptosis. The treatment for PALG is total removal of the tumor [9].
Entire Body System
- Swelling
Initial imaging with CT showed that the swelling was grossly involving the extraocular muscles. [ncbi.nlm.nih.gov]
- Pathologist
Thus, "infiltrative growth" of LG CXPA is difficult to define and diagnosis is challenging to many practicing pathologists. In this study, we report three cases of LG CXPA. [ncbi.nlm.nih.gov]
The pathologist will use a syringe and needle to withdraw a sample of the tumor and then the tissue samples are processed in a lab and read by the pathologist who performed the test. [healthool.com]
A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. [dovemed.com]
Conclusion Pleomorphic adenoma, though a common entity, is still a challenging tumor for pathologist, radiologist, and the surgeon. Its diverse histological and topographical property makes the tumor special. [panafrican-med-journal.com]
- Fever
There was no history of fever, weight loss, bleeding, pus discharge or any other type of discharge from the swelling. The personal history of the patient did not reveal any history of smoking, or any other addiction. [panafrican-med-journal.com]
No history of associated pain and fever. Patient also gave a history of restoration done 6 months back. Family history was not contributory. His vital signs were within normal limits. [pubs.sciepub.com]
Tumors localized in the lung parenchyma are frequently asymptomatic, although symptoms such as fever, weight loss, pleural effusion and shortness of breath have been reported [1, 2]. [academic.oup.com]
There was no history of fever, bleeding, pain, sensory changes and disturbance of salivation or trauma. The past dental history and medical history was unremarkable and no other abnormalities were found. [ijdr.in]
- Severe Pain
The peak incidence of this malignant tumor is between ages 40 and 60, and symptoms include severe pain and, often, facial nerve paralysis. [msdmanuals.com]
- Pallor
There was no icterus or pallor. The examination of remaining body did not reveal any other swelling. The systemic examination of other systems was within normal limits. [panafrican-med-journal.com]
Jaw & Teeth
- Parotid Mass
METHODS: Forty patients with the clinical suspicion of a parotid mass underwent both CT and MRI of the parotid region. [doi.org]
We report a case of a 77-year-old man, who presented with a 6-year history of a parotid mass that had undergone rapid growth within weeks. [ncbi.nlm.nih.gov]
A large mass with a heterogeneous medial portion involves primarily the deep lobe of the parotid gland. [emedicine.com]
Psychiatrical
- Aggressive Behavior
Furthermore, the increased expression of this protein is associated with a more aggressive behavior (invasive growth) in salivary gland tumors. [ncbi.nlm.nih.gov]
The present investigation demonstrates the predominance of myxoid and hyalinized stroma which is similar to Ito et al results.3 Some studies show that prominent zones of hyalinization are related to the aggressive behavior or malignant transformation [journalgateway.com]
Face, Head & Neck
- Neck Mass
Ectopic Warthin’s tumour presenting as a neck mass. B-ENT 2008;4:111-5. [ PubMed ] Wang MC, Tsai TL, Chen PC, et al. Extraparotid Warthin’s tumor presented as a neck mass. [gs.amegroups.com]
Neck mass with marked squamous metaplasia: A diagnostic pitfall in aspiration cytology. J Oral Pathol Med 2008;37:56-8. 6. Siddaraju N, Murugan P, Basu D, Verma SK. [ccij-online.org]
Workup
The radiological tools for the diagnosis and differentiation of salivary gland disease are computerized tomography (CT) scans, magnetic resonance imaging (MRI), and ultrasonography [1] [10]. MRI may be the instrument of choice over CT scan, because of its superior definition of soft tissue and tumor margins [2]. Ultrasound may also be used to serve as the first diagnostic tool; it can differentiate malignant lesions from benign ones with very high accuracy (96%), and can successfully predict PA cases [10]. Apart from location, shape, dimensions and margin [10], ultrasound can distinguish between cystic and solid masses, and between intra- and extracapsular tumors [2]. It can also be used for guiding fine needle biopsy [10].
Fine needle aspiration biopsy is also an important tool for preoperative diagnosis; it is safe and reliable but its sensitivity is variable and is more accurate for the diagnosis of benign lesions than for malignant ones [11] [12] [13].
Because of the diverse histological appearance of PAs, it may not be easy to differentiate them histologically from other neoplasms, such as myoepithelioma or some carcinomas [2].
Treatment
Knowledge of the recurrence risk factors could allow selection of treatments that minimize the risk. [ncbi.nlm.nih.gov]
Carew, JF, Spiro, RH, Singh, B, Treatment of recurrent pleomorphic adenomas of the parotid gland. Otol Head Neck Surg 1999 ; 121: 539 – 42. Google Scholar | SAGE Journals | ISI 4. Phillips, PP, Olsen, KD. [doi.org]
Prognosis
Further accumulation of cases and long follow-up data are needed to elucidate the pathophysiology and prognosis of MEC ex PA. [ncbi.nlm.nih.gov]
Etiology
The markedly different Ga-DOTA-TOC avidity suggested a different etiology, and histological examination demonstrated a pleomorphic adenoma. [ncbi.nlm.nih.gov]
On the basis of history and clinical examination a diagnosis of solid non infectious mass of soft palate likely of benign etiology was made. [panafrican-med-journal.com]
Etiology Although the etiology of pleomorphic adenoma is unknown, the incidence of this tumor has been found to increase 15-20 years after exposure to radiation. [1] One study suggests that the simian virus (SV40) may play a causative role in the development [emedicine.medscape.com]
Relevant investigations consisting of hemogram, total and differential counts (done to exclude infective etiology) were within normal limits. [ijo.in]
The etiology of PA's is unknown, however some authors mention the myoepithelial cell as responsible for the development of this pathology (Ledesma-Montes et al.). [scielo.conicyt.cl]
Epidemiology
INTRODUCTION: Whereas salivary gland pleomorphic adenoma (SGPA) is the most common type of salivary gland tumor, little is known about its epidemiology because national cancer registries do not register this disease. [ncbi.nlm.nih.gov]
Epidemiology Mean age of presentation is mid-forties but the tumour can occur at any age after 10. Women are more commonly affected. Most tumours arise in the parotid gland (80%). Natural History The tumour is usually slowly growing. [ozradonc.wikidot.com]
The epidemiology of pleomorphic adenoma can cover all ages and both genders but is more prevalent among women from thirty to sixty years of age. From its name, pleomorphic depicts variability in its morphology. [cancerwall.com]
The epidemiology of this tumor shows that adults are the most affected, with rare occurrence in children or adolescents. We present the case report of pleomorphic adenoma located on the palate of a 10 year old. [scielo.conicyt.cl]
Pathophysiology
Further accumulation of cases and long follow-up data are needed to elucidate the pathophysiology and prognosis of MEC ex PA. [ncbi.nlm.nih.gov]
Genetic rearrangements, such as chromosomal translocations involving 8q12 target the PLAG1 gene, may contribute to the pathophysiology of PALG. [eyewiki.aao.org]
Prevention
Total parotidectomy or removal of the entire parotid gland, is considered by the healthcare provider to prevent exacerbation in cases with high probability of recurrence. [cancerwall.com]
An acquaintance with its varied cytomorphologic features and a knowledge of its unusual sites of occurrence is essential to prevent its misdiagnosis as other myxoid benign and malignant spindle cell lesions. [ncbi.nlm.nih.gov]
Treatment of the benign tumor is essential is preventing a malignant tumor from occurring. [healthyliving.azcentral.com]
References
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- Jain S, Hasan S, Vyas N, Shah N, Dalal S. Pleomorphic Adenoma of the Parotid Gland: Report of a Case With Review of Literature. Ethiop J Health Sci. 2015 Apr; 25(2): 189–194.
- Di Palma S, Simpson RHW, Skalova A, Leivo I. Major and Minor Salivary Glands. In: Cardesa A, Slootweg PJ, eds. Pathology of the head and neck. Berlin:Springer; 2006:131-170.
- Guerriere CN, Goff BJ, Cummings GH, Auber AE. An unusually large, solid tumor of the parotid gland. Ann Plast Surg. 1999 Nov;43(5):529-532.
- Silva SJ, Costa Junior GT, Brant Filho AC, Faria PR, Loyola AM. Metachronous bilateral pleomorphic adenoma of the parotid gland. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006 Mar;101(3):333-338.
- Hakeem AH, Hazarika B, Pradhan SA, Kannan R. Primary pleomorphic adenoma of minor salivary gland in the parapharyngeal space. World J Surg Oncol. 2009 Nov 12;7:85.
- Pons Vicente O, Almendros Marqués N, Berini Aytés L, Gay Escoda C. Minor salivary gland tumors: A clinicopathological study of 18 cases. Med Oral Patol Oral Cir Bucal. 2008 Sep 1;13(9):E582-588.
- Wittekindt C, Streubel K, Arnold G, Stennert E, Guntinas-Lichius O. Recurrent pleomorphic adenoma of the parotid gland: analysis of 108 consecutive patients. Head Neck. 2007; 29(9):822-828.
- Rose GE, Wright JE. Pleomorphic adenoma of the lacrimal gland. Br J Ophthalmol. 1992 Jul;76(7):395-400.
- Bialek EJ, Jakubowski W, Karpinska G. Role of ultrasonography in diagnosis and differentiation of pleomorphic adenomas. Arch Otolaryngol. Head Neck Surg. 2003;129:929–933.
- Contucci AM, Corina L, Sergi B, Fadda G, Paludetti G. Correlation between fine needle aspiration biopsy and histologic findings in parotid masses. Acta Otorhinolaryngol Ital. 2003 Aug;23(4):314-318.
- Sergi B, Contucci AM, Corina L, Paludetti G. Value of fine-needle aspiration cytology of parotid gland masses. Laryngoscope. 2004 Apr;114(4):789.
- Akhavan-Moghadam J, Afaaghi M, Maleki AR, Saburi A. Fine Needle Aspiration: An Atraumatic Method to Diagnose Head and Neck Masses. Trauma Mon. 2013 Dec; 18(3): 117–121.