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Year : 2021  |  Volume : 10  |  Issue : 4  |  Page : 286-288

Juvenile onset pleomorphic adenoma presenting as giant tumor of parotid gland in a young female

1 Department of General Surgery, PGIMS, Rohtak, Haryana, India
2 Department of Pediatrics, PGIMS, Rohtak, Haryana, India

Date of Submission05-Jul-2021
Date of Acceptance16-Aug-2021
Date of Web Publication22-Mar-2022

Correspondence Address:
Dr. Surender Verma
117-A, Sector 14, Rohtak, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jdrntruhs.jdrntruhs_91_21

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Salivary gland tumors are rare and carry diverse histologies. There is a paucity of clinical and biological details about pediatric salivary gland tumors and their clinical behavior in the literature. We present a case of 25-year-old female with giant pleomorphic adenoma of the parotid which started in her childhood at the age of 13 years. Presentation at such an early age is a rare entity. The patient underwent a superficial parotidectomy. Periodic follow-up was done for 1 year and there was no recurrence. For benign salivary gland tumor, surgical excision with preservation of neurovascular structure is the main treatment option.

Keywords: Giant, juvenile, management, salivary gland tumor

How to cite this article:
Verma S, Aggarwal S, Garg P, Verma A, Gera M, Yadav SS. Juvenile onset pleomorphic adenoma presenting as giant tumor of parotid gland in a young female. J NTR Univ Health Sci 2021;10:286-8

How to cite this URL:
Verma S, Aggarwal S, Garg P, Verma A, Gera M, Yadav SS. Juvenile onset pleomorphic adenoma presenting as giant tumor of parotid gland in a young female. J NTR Univ Health Sci [serial online] 2021 [cited 2023 Feb 7];10:286-8. Available from: https://www.jdrntruhs.org/text.asp?2021/10/4/286/339827

  Introduction Top

Pleomorphic adenoma (PA) is also known as benign mixed tumors (BMTs) because of its dual origin from epithelial and myoepithelial elements. It is the commonest constituting up to two-thirds of all salivary gland tumors.[1],[2],[3] About 84% of all adenomas is found in parotid gland, 8% in submandibular gland, and 6.5% in minor salivary gland. In most of the cases, it originates in the superficial lobe. Occasionally, it may involve the deep lobe of the parotid gland and the parapharyngeal space. PA usually manifests as a slow-growing, asymptomatic parotid gland swelling without facial nerve involvement. It is best treated by a wide local excision with good safety margins and follow-up is required for at least 3–4 years.[4]

  Case Report Top

A 25-year-old female patient reported to the Outpatient Department with a chief complaint of slow-growing, painless swelling on the left side of face since 12 years. She stated that the mass had begun to develop 12 years ago in the region below her swelling. It was painless initially, small in size, and had progressively increased with time to attain the present size. Past medical and surgical history was noncontributory. Extra-oral clinical examination showed a well-defined, ovoid, multilobular swelling, 10 × 8 cm in diameter on the left side of the face. The swelling had a superoinferior extent 6 cm below from left zygomatic arch to about 4 cm below the lower border of the mandible, and anteroposterior extent from mid body region to 3 cm behind the posterior border of mandible. The left ear lobule was everted and loss of wrinkling of the skin, and engorged veins were also seen [Figure 1].
Figure 1: showing Posterolateral aspect of huge Parotid tumor

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The swelling was nontender, firm in consistency, and not fixed to underlying structures and overlying skin. There was no facial asymmetry and movements of eye were normal. Intraoral clinical examination was unremarkable. The patient had no cranial nerve deficits and the remainder of her physical examination was remarkable.

A provisional diagnosis of benign parotid tumor was made and patient underwent investigations in the form of Magnetic resonance imaging (MRI) and Fine needle aspiration cytology (FNAC) of the swelling. MRI revealed a large (9.9 × 7.8 × 10.7 cm), well-defined, lobulated, heterogeneous lesion involving the superficial lobe of Left parotid gland extending into its deep lobe. Anteriorly, the fat planes were maintained with the masseter, medial pterygoid muscles, and ramus of mandible. Postero-medially, the lesion shows maintained fat planes with sternocleidomastoid muscle. Lesion shows T2 hyperintense areas s/o cystic component and T1/T2 hyperintense areas were suggestive of hemorrhagic component. FNAC showed features suggestive of PA with no evidence of malignancy.

Patient was explained about the possible risk of facial nerve injury and accordingly, consent was obtained. An incision starting from front of ear, then elliptical belly and down into the cervical crease was made. Platysma muscle and superficial aponeurotic layer were dissected. Facial nerve was identified and preserved [Figure 2]. Tumor was resected from underlying facial nerve branches and masseter muscle. Superficial parotidectomy was completed and the wound was closed with mini-VAC drain in situ.
Figure 2: Showing main facial trunk and its branches intraoperatively

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The excised tumor mass was 10 cms × 10 cms × 6.5 cms in dimension. The weight of the excised mass was 1.4 kg. Histopathology revealed a well-encapsulated, well-demarcated, solid cystic gray white mass with myxoid areas [Figure 3]. Microsections examined showed features suggestive of PA. The patient was periodically followed up for 1 year and no recurrences or complications were observed during this period.
Figure 3: Showing cut specimen showing well demarcated solid cystic grey white mass with myxoid areas suggestive of Pleomorphic adenoma

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  Discussion Top

According to WHO, PA is a well-defined tumor characterized by its pleomorphic or mixed appearance. Although the lesion presents features due to the different compounds with a myxoid or chondroid matrix, it is generally considered to be a benign neoplasm.[5]

PAs generally present as asymptomatic masses and their size varies from 2 to 6 cm.[6] However, in the index case size was 12 × 10 × 8 cm and was weighing 1.4 kg. Large tumor and heavy tumors have been rarely reported in literature.[7] Alves et al.[8] reported that the tumor occurred commonly between the third and fifth decades of life, and women were most commonly affected.

Our patient was a 25 = year-old female who presented with a slowly enlarging, multilobular, asymptomatic swelling on the left side of the face for 12 years. As per literature, only 5% of the affected population is pediatric.[3] Our index case presented in childhood as an asymptomatic swelling.

MRI is a favored imaging modality over CT scan as it provides better soft-tissue delineation, margin description, and its relationship with the surrounding structures. MRI findings in the present case were suggestive of a large well-defined, heterogeneous, lobulated lesion involving the superficial lobe of Left parotid gland sparing its deep lobe.

USG helps to differentiate solid from cystic masses as well as vascular masses. USG-guided FNAC can guide surgeon to clinch the correct diagnosis and thereby perform the right surgical procedure.

Tumor size and facial nerve relation are important parameters, which guide the choice of surgical procedure. Enucleation and superficial or total parotidectomy with preservation of the facial nerve formed the mainstay of surgical treatment. However, enucleation procedures are not performed nowadays because of high associated rates of recurrence.

In the present case, the superior lobe of the Left parotid gland with the tumor mass was excised with utmost care to preserve all the facial nerve branches. PAs need to be managed cautiously because of their tendency for recurrence and malignant transformation (1.5% in the first 5 years, 9.5% after 15 years). Capsule rupture and tumor spillage during surgery are important risk factors for recurrence. Around 10% cases undergo malignant changes and predisposing factors are old age, large tumor, long standing mass, submandibular gland tumor, and so on.[9]

To summarize, an exhaustive pre-operative diagnostic algorithm is required before approaching PAs. MRI provides important information about the location and margins and can guide the surgeon in planning the right approach. FNAC is mandatory for histological confirmation of diagnosis. Hence, surgical dissection should be meticulous to secure neurovascular structures and to prevent recurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her names and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Mc Loughlin L, Gillanders SL, Smith S, Young O. The role of adjuvant radiotherapy in management of recurrent pleomorphic adenoma of the parotid gland: A systematic review. Eur Arch Otorhinolaryngol 2019;276:283-95.  Back to cited text no. 1
Lee JH, Kang HJ, Yoo CW, Park WS, Ryu JS, Jung YS, et al. PLAG1, SOX10, and Myb expression in benign and malignant salivary gland neoplasms. J Pathol Transl Med 2019;53:23-30.  Back to cited text no. 2
Meshram GG, Kaur N, Hura KS. Pediatric pleomorphic adenoma of the parotid: Case report, review of literature and novel therapeutic targets. Children (Basel) 2018;5:127.  Back to cited text no. 3
Sergi B, Limongelli A, Scarano E, Fetoni AR, Paludetti G. Giant deep lobe parotid gland pleomorphic adenoma involving the parapharyngeal space. Report of three cases and review of the diagnostic and therapeutic approaches. Acta Otorhinolaryngol Ital 2008;28:261-5.  Back to cited text no. 4
Carr RJ, Bowerman JE. A review of tumors of the deep lobe of the parotid salivary gland. Br J Oral Maxillofac Surg 1986;24:155-68.  Back to cited text no. 5
Beunting JE, Smith TL, Holmes DK. Giant pleomorphic adenoma of the parotid gland: Case report and review of the literature. Ear Nose Throat J 1998;77:643, 637-8, 640.  Back to cited text no. 6
Guerriere CN, Goff JJ, Cummings GH, Auber AE. An unusually large, solid tumor of the parotid gland. Ann Plast Surg 1999;43:529-32.  Back to cited text no. 7
Alves FA, Perez DE, Almeida OP, Lopes MA, Kowalski LP. Pleomorphic adenoma of the submandibular gland: Clinicopathological and immunohistochemical features of 60 cases in Brazil. Arch Otolaryngol Head Neck Surg 2002;128:1400-3.  Back to cited text no. 8
Zarbo RJ. Salivary gland neoplasia: A review for the practicing pathologist. Mod Pathol 2002;15:298-323.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]


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