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CASE REPORT Table of Contents   
Year : 2022  |  Volume : 19  |  Issue : 2  |  Page : 105-108
Advance oropharyngeal mucoepidermoid carcinoma in a 9-year-old boy: A case report and review of literature


1 Department of Otorhinolaryngology, Faculty of Clinical Sciences, College of Health Sciences, Bayero University, Kano, Nigeria
2 Department of Surgery, Division of Otorhinolaryngology, Faculty of Clinical Sciences, College of Medical Sciences, Ahmadu Bello University, Zaria, Nigeria
3 Department of Radiology, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria

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Date of Submission12-Sep-2020
Date of Decision17-Apr-2021
Date of Acceptance01-May-2021
Date of Web Publication01-Jan-2022
 

   Abstract 


Primary malignant salivary gland tumours are uncommon among the paediatric population, accounting for <10% of all head-and-neck tumours in childhood. Less than 5% of all salivary gland cancers occur during childhood, most of them are diagnosed over the age of 10 years and are histologically low or intermediate grade. Mucoepidermoid carcinoma (MEC) occurring in the oropharynx of children and arising from the posterior pharyngeal wall is rare and probably never reported. We hereby report a case of advanced MEC arising from the posterior wall of the oropharynx in a 9-year-old boy, managed via a transcervical excision in a resource-poor setting.

Keywords: Childhood, mucoepidermoid carcinoma, oropharyngeal

How to cite this article:
Ajiya A, Shuaibu IY, Yahuza MA. Advance oropharyngeal mucoepidermoid carcinoma in a 9-year-old boy: A case report and review of literature. Afr J Paediatr Surg 2022;19:105-8

How to cite this URL:
Ajiya A, Shuaibu IY, Yahuza MA. Advance oropharyngeal mucoepidermoid carcinoma in a 9-year-old boy: A case report and review of literature. Afr J Paediatr Surg [serial online] 2022 [cited 2022 Aug 10];19:105-8. Available from: https://www.afrjpaedsurg.org/text.asp?2022/19/2/105/333586



   Introduction Top


Primary malignant salivary gland tumours are uncommon among the paediatric population, accounting for <10% of all head-and-neck tumours in childhood.[1] Paediatric major salivary tumours are well known to the otolaryngologist. However, minor salivary gland malignancies are quite rare and are usually found on the palate.[2] Less than 5% of all salivary gland cancers occur during childhood, most of them are diagnosed over the age of 10 years and are histologically low or intermediate-grade.[3]

Mucoepidermoid carcinoma (MEC) is a common malignant salivary gland neoplasm presumed to arise from the reserve cells of the salivary gland ducts.[3] Girls are mostly affected.[4] It is the most common malignant lesion and represents around 35%–50% of all malignant salivary gland tumours in childhood.[5]

MEC primarily involves the salivary gland but can also be found in the upper aerodigestive tract, tracheobronchial tree, lacrimal sac, thyroid and liver.[6] Only a few paediatric and adolescent cases have been well-documented in the minor salivary glands. A review of English literature in 2012 revealed only 15 well-documented cases of MEC of minor salivary gland origin in this age group.[7]

MEC occurring in the oropharynx of children and arising from the posterior pharyngeal wall is rare and probably never reported. We hereby report a case of advanced MEC arising from the posterior wall of the oropharynx in a 9-year-old boy, managed via a transcervical excision in a resource-poor setting.


   Case Report Top


A 9-year-old boy with progressively enlarging left-sided neck mass referred from a secondary facility to our clinic with progressive dysphagia to solids, choking and difficulty breathing over a 3-month duration. The patient volunteered a history of left earache early in the onset of disease. There was no significant past medical history. Physical examination revealed an acutely ill-looking child, stridulous, in respiratory distress, dehydrated and drooling saliva. A huge fleshy mass was seen occupying the oropharynx making it difficult to assess the anatomical subsites of the region. A diagnosis of upper airway obstruction secondary to the advanced oropharyngeal tumour was made and the patient had emergency tracheostomy with examination under anaesthesia and biopsy [Figure 1]. Histology revealed MEC. Computed tomography scan showed a huge ill-defined irregularly shaped exophytic moderately enhancing mass centred to the left oropharyngeal wall [Figure 2]a. It extends from the posterior pharyngeal wall to the nasopharynx superiorly obliterating the fossa of Rosenmuller and inferiorly into the hypopharynx. Anteriorly, the mass fills the vallecula and distorts the epiglottis and base of the tongue [Figure 2]b. No vascular encasement or bony destruction is seen. Extensive lymph node enlargement is seen in the deep and posterior cervical groups. Definitively, the patient had a transcervical excision of the tumour with selective neck dissection and excision of the left submandibular gland [Figure 3]. The intraoperative finding was that of a huge fleshy mass from the posterior pharyngeal wall of the oropharynx and palpable matted lymph nodes at levels Ib, II and III of the neck.
Figure 1: Huge neck mass and tracheostomy tube in situ

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Figure 2: (a) A huge exophytic oropharyngeal mass.(b) Mass in the oropharynx extending to the nasopharynx, hypopharynx and vallecula

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Figure 3: Transcervical tumour excision

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A 3 cm by 10 cm mass, weighing 240 gram was removed [Figure 4] and histology revealed sections showing infiltrating clusters of malignant epidermoid cells admixed with neoplastic mucous cells and intermediate type cells and disposed within a fibro collagenous stroma containing moderate inflammation.[Figure 5] The submandibular gland was unremarkable. The patient was transfused whole blood intraoperatively and was fed postoperatively via a nasogastric tube. Sutures were removed on the 8th post-operative day, the tracheostomy tube on the 13th day and the nasogastric tube on the 17th. The patient had a barium swallow on the 17th day post-operative, which showed the normal study. He was discharged on the 20th day post-operative. The patient had been on regular follow-up since then without a sign of recurrence.
Figure 4: Ahuge excised mass

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Figure 5: Intermediate gradeMucoepidermoid carcinoma showing islands of malignant epithelial cells as well as clear cells containing mucin.(HandE×10)

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   Discussion Top


MEC was first described by Volkmann in 1895.[6] Although rare, MEC is the most common malignant salivary gland tumour in childhood accounting for 50% of cases.[7] The minor salivary glands are roughly around 500–1000 glands distributed throughout the oral cavity and oropharynx, mostly found in the palate, other areas where they are less commonly found include buccal mucosa, retromolar trigone, tongue and floor of the mouth.[2] In the present case, the tumour arises from the posterior wall of the oropharynx. This is the first reported of such to the best of our knowledge.

Most patients present with an isolated, painless and slow-growing mass. Our patient had a history of otalgia probably from the affectation of nerves in the pharynx. The clinical presentation of MEC may vary according to the histologic grade. Low and intermediate grade may appear as slow-growing masses; high grade generally appears as a rapidly enlarging mass.[8] The disease in our patient evolved just over 2 months. Enlarged cervical lymph nodes related to metastasis occurred in 30% of patients, but nodal enlargement was seldom found in patients with minor salivary gland neoplasms at presentation.[9] Our patient presented with neck nodes at multiple levels in the neck. Up to 5% of major glands and 2.5% of minor gland low-grade tumours were reported to metastasise to regional lymph nodes. MEC among children is commonly reported among girls.[4],[10] On the contrary, our patient was a boy.

Diagnosis of MEC may be difficult based on imaging alone, and therefore, tissue diagnosis is usually needed. On computed tomography, low-grade tumours frequently appear as well-defined masses with moderate enhancement, but high-grade tumours exhibit ill-defined margins.[11],[12] The scan in our patients showed an ill-defined but moderately enhancing mass.

Low-grade tumours are well-circumscribed and mucoid material is abundant. On the contrary, high-grade tumours have a greater epithelial component. Also, epithelial pearls and intercellular bridging may be found. In some higher-grade specimens, special stains may be required to demonstrate mucin production, thereby differentiating these tumours from squamous cell carcinoma.[2] The microscopic feature in our specimen showed more of the malignant epidermoid cells commensurate to a intermediate-grade lesion.

Complete surgical extirpation of the mass with free surgical margins is the treatment modality of choice in both children and adults. It is also the one that offers the best chance of local control of the disease.[9] Radiation therapy with 5500–6500 cGy is recommended for high-grade carcinomas, stage III and IV tumours, incompletely excised tumours and/or nodal involvement. However, strong indications should exist to justify post-operative radiotherapy in children, as adverse sequelae are common as high as 60%.[13] The role of chemotherapy in the treatment of salivary MEC remains speculative. Prophylactic neck dissection is not warranted for clinically negative neck, as recurrence is dependent on tumour stage and extent of surgical resection.[8] Our patient had complete surgical excision with selective neck dissection and has been referred for post-operative radiation therapy.

Low-grade tumours have recurrence rates of 15%–20% in contradistinction with 30%–40% recurrence rates seen among patients with high-grade tumours. The 5-year survival of 85%–95% for patients with low-grade tumours has been reported variously. On the contrary, patients with high-grade tumours have survival rates of 30%–50% from several reported series.[14] However, the prognosis appears to be better for children than adults. It is still early to be able to reliably comment on the prognosis in the index patient.


   Conclusion Top


In the evaluation of children with oropharyngeal tumours arising from the posterior and lateral walls of the oropharynx with associated palpably enlarged cervical lymph nodes, MEC should be entertained. A transcervical approach to tumour excision with neck dissection with or without post-operative radiotherapy is a useful treatment modality.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Bhagat S, Varshney S, Singh RK, Bist S. Mucoepidermoid carcinoma of the larynx with transglottic involvement in a child: A case report. Ear Nose Throat J 2012;91:533-5.  Back to cited text no. 1
    
2.
Winslow CP, Batuello S, Chan KC. Pediatric mucoepidermoid carcinoma of the minor salivary glands. Ear Nose Throat J 1998;77:390-1, 395.  Back to cited text no. 2
    
3.
Mesolella M, Iengo M, Testa D, DI Lullo AM, Salzano G, Salzano FA. Mucoepidermoid carcinoma of the base of tongue. Acta Otorhinolaryngol Ital 2015;35:58-61.  Back to cited text no. 3
    
4.
Martínez OM, Dorado ED, García MD, Ramírez MI, de la Fuente Muñoz I, Soler JL. Locally advanced stage high-grade mucoepidermoid carcinoma of salivary gland in a 9-year-old girl: The controversy of adjuvant therapy. Rare Tumors 2016;8:6429.  Back to cited text no. 4
    
5.
Thariat J, Vedrine PO, Temam S, Ali AM, Orbach D, Odin G, et al. The role of radiation therapy in pediatric mucoepidermoid carcinomas of the salivary glands. J Pediatr 2013;162:839-43.  Back to cited text no. 5
    
6.
Techavichit P, Hicks MJ, López-Terrada DH, Quintanilla NM, Guillerman RP, Sarabia SF, et al. Mucoepidermoid carcinoma in children: A single institutional experience. Pediatr Blood Cancer 2016;63:27-31.  Back to cited text no. 6
    
7.
Ritwik P, Cordell KG, Brannon RB. Minor salivary gland mucoepidermoid carcinoma in children and adolescents: A case series and review of the literature. J Med Case Rep 2012;6:182.  Back to cited text no. 7
    
8.
Re M, Pasquini E. Nasopharyngeal mucoepidermoid carcinoma in children. Int J Pediatr Otorhinolaryngol 2013;77:565-9.  Back to cited text no. 8
    
9.
Spiro RH, Huvos AG, Berk R, Strong EW. Mucoepidermoid carcinoma of salivary gland origin. A clinicopathologic study of 367 cases. Am J Surg 1978;136:461-8.  Back to cited text no. 9
    
10.
Sengul F, Simsek S, Cakur B. Mucoepidermoid carcinoma in a minor salivary gland in a child. Case Rep Dent 2013;2013:615948.  Back to cited text no. 10
    
11.
Som PM, Brandwein MS. “ Anatomy and Pathology of the Salivary Glands”, in Head and Neck Imaging. In: Som PM, Curtin HD, editors. St. Louis, Mo, USA: Mosby Elsevier; 2011. p. 2448-609.  Back to cited text no. 11
    
12.
Gamoh S, Akiyama H, Tsuji K, Nakazawa T, Morita S, Tanaka A, et al. Non-contrast computed tomography and magnetic resonance imaging features of mucoepidermoid carcinoma in the salivary glands. Oral Radiol 2018;34:24-30.  Back to cited text no. 12
    
13.
Mendenhall WM, Morris CG, Amdur RJ, Werning JW, Villaret DB. Radiotherapy alone or combined with surgery for salivary gland carcinoma. Cancer 2005;103:2544-2550.  Back to cited text no. 13
    
14.
Ellis MA, Graboyes EM, Day TA, Neskey DM. Prognostic factors and occult nodal disease in mucoepidermoid carcinoma of the oral cavity and oropharynx: An analysis of the National Cancer Database. Oral Oncol 2017;72:174-8.  Back to cited text no. 14
    

Top
Correspondence Address:
Dr. Iliyasu Yunusa Shuaibu
Department of Surgery, Division of Otorhinolaryngology, Faculty of Clinical Sciences, College of Medical Sciences, Ahmadu Bello University, Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajps.AJPS_133_20

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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