pleomorphic adenoma

Tumors arising from the minor salivary glands are uncommon clinical entities. Among them the palate is the most commonly affected site followed by the upper lip and buccal mucosa respectively [5]. Pleomorphic adenoma is considered to be the most common benign salivary gland tumor. Our literature search indicated that eighty-four percent of pleomorphic adenomas occur in the parotid gland, eight percent in the submandibular gland, and four to six percent in the minor salivary glands. Spiro et al. conducted a study o patients with salivary gland neoplasia in which he reported that twenty to forty percent of all salivary gland tumors arise from minor salivary glands. [7], mostly seen in fourth to sixth decades of life with a slight predilection for female gender. This case report is consistent with the finding of gender. Rahnama et reported that etiology of pleomorphic adenomais, in 70%, result of chromosome abnormalities involving pleomor adenoma gene 1 (Plag 1) located on 8q12 and 12q15 [8].

4,5 Previous studies have shown that HER2 is overexpressed in salivary malignant pleomorphic adenoma and is involved in multiple cellular pathways, including cell proliferation, adhesi[r]

35. Koral K, Sayre J, Bhuta S, Abemayor E, Lufkin R. Recurrent pleomorphic adenoma of the parotid gland in pediatric and adult patients: value of multiple lesions as a diagnostic indicator. AJR Am J Roentgenol. 2003 Apr;180(4):1171-4. 36. Glas AS, Hollema H, Nap RE, Plukker JT. Expression of estrogen receptor, progesterone receptor, and insulin-like growth factor receptor-1 and of MIB-1 in patients with recurrent pleomorphic adenoma of the parotid gland. Cancer. 2002 Apr 15;94(8):2211-6.

Of the 12 patients included in the study, 8 were males and 4 were females. The age at the time of presentation ranged from 14 years to 52 years. A painless slowly enlarging swelling in the palate was the most common complaint at the time of presentation. Postero-lateral region of the hard and soft palate was the most common site. Soft palate involvement was seen in all cases. The right side was involved in 8 cases. The size of the tumor ranged from 3cm X 2cm to 7cm X 8cm in long standing cases. Mucosal ulceration was seen in 2 cases. In one case it was due to a previous incisional biopsy done elsewhere and in one case it was due to occlusal trauma. 3 patients had altered speech while one patient reported difficulty in swallowing. None of the patients had pain at the time of presentation. FNAC was done pre-operatively in all patients and was sensitive and reliable towards the diagnosis of pleomorphic adenoma. Coronal CT scans were done for all patients. Wide local excision was done in all cases and sufficient to completely excise the tumor. Greater palatine artery was clamped, cauterised and the foramen packed with bone wax in all our cases. No residual palatal defects were seen in our cases and all wounds healed by secondary healing. No reconstruction of the palate was needed in any of our cases. Acrylic palatal splint was given in all patients and were well tolerated. Palatal splints were retained till 3 weeks post operatively.

Generally, parapharyngeal space (PPS) tumours are less than 1% of all head and neck tumours [1]. Most PPS tumours are benign (70-80%). The malignant ones are fewer. The post - styloid compartment lesions tend to be of neurogenic origin whereas the prestyloid ones arise from the minor salivary glands in the lateral pharyngeal wall or extensions of tumours of the deep lobe of the parotid [2]. Among the benign tumours of the PPS, the pleomorphic adenoma is the commonest [1]. We present a case of a left parapharyngeal mass which caused a cerebrovascular accident (CVA) and was excised transorally with resolution of the CVA. We believe that the left parapharyngeal tumour in our case was compressing on the ipsilateral carotid sheath and therefore by inference on the walls of the internal carotid artery thus limiting the blood supply to the left half of the brain. This led to the left brainstem infarct with the resultant right hemiparesis. The occluded vessel recanalized after the tumour was excised with resolution of

Currently, a defined time period prior to the des- ignation as a metachronous lesion has not been established. Ethunandan [5] used an interval of 6 months in their study to define it. In this case, it took a long time (approximately 58 months) to discover the tumor in the posterior part of the parotid gland, after the tumor found in the anterior part. As a result, it was consid- ered a synchronous lesions on both parts (ante- rior and posterior of the left parotid gland), and also, metachronous lesions on both sides (left and right parotid glands). According to another Yu’s [6] statistics, multiple tumors makes up 3.4% of all the tumors of parotid gland with males outnumber females by 0.3-4.9 on aver- age, where metachronous ones could account for 30%. It could also be read in Ethunandan’s [8] statistics that metachronous tumors acco- unted for 20% of multiple tumors and were all bilateral in distribution. He concluded that 83% metachronous tumors were of the same histo- logical type and Warthin’s tumor is the most common metachronous tumor (50%). We can come to realize that bilateral Warthin’s tumors with a pleomorphic adenoma is extremely rare.

Pleomorphic adenoma (PA) is a common tumor occurred in salivary glands. The occurrence of PA located in breast is extremely rare and PAB was mostly found in the mammary subareola [1] of postmenopausal female [2]. PA has a characteristic mixture of epithelial components and myoepithelial components embedded in myxochondroid matrix [3]. To our knowledge, only 77 cases of PAB [4-7] have been reported in the literature since the first reported case of PAB by French scholar Lecène in 1906 [8]. Indeed, the breast is a rare location of the PA and the rarity causes the confusion and diffi- culty in pathological diagnosis among other breast neoplasms, such as mucinous carcino- ma [9] and metaplastic carcinoma [10]. Herein, we reported two cases of PAB and reviewed rel- evant literature briefly in order to avoid misdiag- nosis in this rare location.

In summary, this case demonstrates a case of pleomorphic adenoma occurring in a young female. Neoplasms in the minor salivary glands are relatively rare, but pleomor- phic adenoma is the most common type of tumor. The treatment goal is the surgical excision of the tumor, considering the relative high rate of malignant transformation. Follow-up examinations are recommended in order to detect recurrences early.

9-year-old Japanese girl with pleomorphic adenoma of the cheek mucosa. Cohen and Kronenberg reported two more cases of juvenile pleomorphic adenomas of the cheek (girls, age <18 years) [13-15]. No recurrence was reported in these cases [13-15]. Dhanuthai et al reported the first case of palatal pleomorphic adenoma in a 13 year-old child [17]. In children, Jorge et al reported five cases of intraoral pleomorphic adenoma in patients under 18 years of age in two Brazilian institutions. Four patients were females and one was male; two cases affected the pal- ate, two the upper lip and one the tongue. The cases were treated by local excision and long-follow up showed no The radiological and gross features of juvenile pleomorphic adenoma of the cheek

Yu et al. [2] reported that synchronous lesions affect men almost 6 times more frequently than women. Tumors with bilateral involvement present with high frequence an histopathology exam that show a Warthin’s tumour (79.2% to 92.5% of cases) [5], the second neoplasm more represented is pleomorphic adenoma, repre- senting approximately 6% of the tumors [1].

Pleomorphic adenoma of the parotid gland typically appears as slow growing, painless rounded mass with bosselated borders, usually located in the superficial lobe above the plane of facial nerve near the angle of mandible. 8 Occasionally it may involve deep lobe involving pre styloid area and may extend into the parapharyngeal space widening the stylomandibular tunnel. Tumors located in parapharyngeal space may present as swelling in the retromandibular area of upper neck, as a smooth bulge in the soft palate or causing medial displacement of the tonsil in lateral pharyngeal space extending from skull base to hyoid bone. Otalgia, trismus or neuralgias may be seen. 9,10 In the cheek, a firm, bimanually palpable mobile swelling without involvement of facial tissues may be present. 11,12 Pleomorphic adenoma arising from minor salivary gland of the lip tends to appear as sessile masses which may ulcerate and cause difficulty in speaking and those in palate may cause mechanical obstruction of the airway. 13 Pain, facial weakness or palsy is uncommon and usually heralds malignant change. Cervical heterotopias are uncommon, but usually presents as painless, discharging masses or sinus openings in neck.

Fig 1. A, 55-year-old woman who underwent superficial parotidectomy 22 years before recurrence. Axial fat-sup- pressed T2-weighted MR image demonstrates marked T2 hyperintensity of multiple recurrent pleomorphic adenoma lesions on the right. Subcutaneous location of lesions is also noted on this image (arrows). B, Also noted are multiple T2 hyperintense recurrent lesions at the right stylomastoid foramen (white arrow), the right parapharyngeal space (as- terisk), and the superficial parotidectomy bed on this axial fat-suppressed T2-weighted MR image.

Background. Potential epigenetic biomarkers for malignant transformation to Carcinoma ex- Pleomorphic Adenoma (Ca ex PSA) have been sought previously with and without specific comparison to the benign variant, Pleomorphic Salivary Adenoma (PSA). Previous analysis has been limited by a non-quantitative approach. We sought to demonstrate quantitative promoter methylation across a panel of tumour suppressor genes in both Ca ex PSA and PSA.

Within the striking Perchance about the recurring predisposition, tumor cells are highly expected to possess low biological requirements: an exegesis which may account for the neoplastic cellular survival on splitting into an operative site. Many recurrent pleomorphic adenomas are multifocal and some are so widely distributed that surgical control becomes impossible. Another possible justification for recurrences or persistence in pleomorphic adenoma is the diffluent nature of predominantly mucoid tumors [8].

Pleomorphic adenoma (PA) is the most common tumor of the salivary glands (it includes about 65% of all salivary gland tumors). PA originates more commonly in parotid glands (65% to 75%), 8% in submandibular glands, and only 6% to 7% in minor salivary glands. Generally, it is considered a benign and slow-growing tumor, but about 1.6% to 7.5% of PA shows malignant changes during its natural history, including three different types: carcinoma ex pleomorphic adenoma (CXPA), also called malignant mixed tumor, carcinosarcoma, and metastasizing PA (MPA); the latter two are exceedingly rare [1,2].

Salivary gland tumors represent 1% to 4% of all human neoplasias [1]. Pleomorphic adenoma is the most common tumor that a ff ects the aforementioned glands [1–3]. Rarely PA undergoes malignant transformation into Carcinoma Ex-Pleomorphic Adenoma (CEPA) or carcinosarcoma. It is even more uncommon that PA metastasizes. Among sali- vary gland neoplasms, metastasizing pleomorphic adenoma (MPA) constitutes an extremely rare group of tumors [2, 3]. Although apparently benign, the MPA-associated mortality could be as high as 22% [2]. According to Nouraei and colleagues’ review of case reports, the 5-year disease-specific and disease-free survivals of patients suffering from MPA are 58% and 50%, respectively [3].

Pleomorphic adenoma (PA) is the most com- mon type of salivary gland tumors, and more than half arise in the parotid gland. This type of tumor is marked by slow-growing, painless mass, and tumor can achieve different sizes and weights if untreated. Diagnosis of PA is based on clinical and cytological features. It is a benign disease characterized with some malignant biological behaviors, like prolifera- tion, recurrence, infiltration, diffusion and me- tastasis. Entire surgical excision and facial nerve preservation of the tumor are the chief principles of operation. Fear of surgery or so- ciocultural factors may lead to the delayed diagnosis and intervention, resulting in enor- mous size of the tumor. Giant pleomorphic ad- enomas (GPA) are not seen frequently and usu- ally present in patients with a long history of the tumor, even several decades.

feature of being composed of both epithelial and mesenchymal-like tissues. The proportion of each of these elements varies widely and one or the other is often predominant. The “cellular” type of pleomorphic adenoma is one in which the epithelial element predominates, whereas the “myxoid” type is composed mostly of a myxomatous or myxochondromatous mesenchymal-like element. The “mixed” type is a classic form. Distinctive epithelial cell types include spindle, clear, squamous, basaloid, cuboidal, plasmacytoid, oncocytic, mucous and sebaceous. 11, 13, 14

Pleomorphic adenoma (PA) is the most common tumor type in the salivary gland. PA is uncommon in the breast tissue. Only 73 cases of PA of the breast have been reported in the world literature. We are reporting the 74th case of PA of the breast. A 61- year-old woman was referred to Shahid Beheshti Hospital Obstetric Clinic with bloody painless discharge from the right nipple. A bean size mass was detected immediately below the right nipple. After an excisional biopsy, the pathologist found proliferation in epithelial and myoepithelial cells that had small and multiple nuclei, myxoid and chondroid stroma. Immunohistochemistry stain was positive for S-100 and patchy for GFAP in tumor cells and for SMA around the tubule-glandular and tumor cell aggregates and suggested PA of the breast. It is essential for the pathologists to consider PA of the breast as a differential diagnosis of a rounded circumscribed mass in the juxta-areolar areas. Careful paraffin sections should be performed to avoid an unnecessary mastectomy.

Incision biopsy of the submandibular discrete lobule con- firmed the diagnosis pleomorphic adenoma. Computed tomographic (CT) scan could not be done, as the patient could not afford the cost, while magnetic resonance imag- ing (MRI) facilities are not available at our centre. The patient was subsequently admitted for surgical exci- sion of the submandibular and parapharyngeal tumor. The submandibular lesion was approached through a left submandibular incision. The mass was found to be com- pletely distinct from the parapharyngeal tumor, which was enucleated by blunt dissection. Recovery and imme- diate post-operative period were uneventful.