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Indian Journal of Medical and Paediatric Oncology
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LETTER TO EDITOR
Year : 2019  |  Volume : 40  |  Issue : 2  |  Page : 314-315  

Progressive metastatic sialoblastoma in a young child: Challenges in treatment


1 Department of Pediatric Hematology/Oncology, Health Care Global, Bengaluru, Karnataka, India
2 Department of Head and Neck Oncology, Health Care Global, Bengaluru, Karnataka, India

Date of Web Publication17-Oct-2019

Correspondence Address:
Intezar Mehdi
Department of Pediatric Hematology/Oncology, Health Care Global, No. 8, HCG Towers, P Kalinga Rao Road, Sampangiram Nagar, Bengaluru - 560 020, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmpo.ijmpo_183_19

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How to cite this article:
Ranjani S, Mehdi I, Rao V. Progressive metastatic sialoblastoma in a young child: Challenges in treatment. Indian J Med Paediatr Oncol 2019;40:314-5

How to cite this URL:
Ranjani S, Mehdi I, Rao V. Progressive metastatic sialoblastoma in a young child: Challenges in treatment. Indian J Med Paediatr Oncol [serial online] 2019 [cited 2019 Nov 13];40:314-5. Available from: http://www.ijmpo.org/text.asp?2019/40/2/314/269452

Sialoblastoma is one of the rare tumors of the salivary gland. It is a congenital tumor arising most often from the parotid gland. This tumor is also known as basaloid adenocarcinoma, basal cell adenoma, and congenital basal cell adenoma. It has significant variability in biologic behavior. It was originally considered a benign tumor, but locoregional and distant metastasis have been described. Here, we describe a child with metastatic progressive sialoblastoma.

A 1-year 7-month-old Indian girl presented to us with complaints of swelling of the left cheek for a year. She was treated with multiple courses of antibiotics, despite which the swelling persisted. She was noted to have a 5 cm × 6 cm swelling over the left parotid area. Ultrasound of the parotid region revealed lobulated hypoechoic lesion in the superior lobe of the left parotid gland. Magnetic resonance imaging neck showed enlargement of the superficial lobe suggestive of parotid neoplasm. Fine-needle aspiration cytology was suggestive of rhabdomyosarcoma elsewhere, and she underwent superficial left parotidectomy. Histopathological evaluation of the specimen was suggestive of Grade III sialoblastoma with immunohistochemistry positive for S100, vimentin and patchy staining for EMA. She developed a 6 cm × 8 cm firm swelling in the parotid area extending from the inferior orbital margin to the mandible and from the angle of the mouth to the retroauricular region 1 month after surgery [Figure 1]. Fluorodeoxyglucose positron emission tomography–computed tomography (PET CT) scan revealed multiple lung nodules. She underwent left radical parotidectomy [Figure 2]. Chemotherapy and radiotherapy were offered as treatment options, but parents refused treatment. PET CT was repeated one-month post surgery. It showed new lesion in the infratemporal fossa and increase in the size and number of lung lesions. The family was counseled regarding repeat surgery followed by radiation and chemotherapy; however, they did not opt for any treatment. She was followed up telephonically for 10 months postsurgery during which she was doing well. She was lost to follow-up after 10 months.
Figure 1: Preoperative picture showing massive swelling on the left cheek

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Figure 2: Postoperative picture showing considerable decrease in the size of the swelling

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Sialoblastomas are rare salivary gland tumors usually present at birth or recognized shortly after birth. Age of presentation is reported from the prenatal period to 4 years.[1] Sitthichaiyakul et al. observed that the median age of diagnosis was 9.8 years.[2] There have also been isolated case reports of sialoblastoma in adults.[1]

The incidence is extremely rare with only single case reports being reported. Choudhary et al. did a literature search and reported 46 cases from 1966 to 2011.[1] Irace et al. reviewed 62 cases of pediatric sialoblastoma in 2016.[3]

There is no clear consensus regarding the pathogenesis of sialoblastoma. It is thought to arise from the retained blastemal cells in the salivary gland.[2]

Sialoblastomas usually tend to progress locally, with local recurrences and rarely metastasize.[2] Irace et al. did a literature review of 62 cases, of which only nine patients had metastatic disease.[3] In a review by Choudhary et al., 10 cases out of 46 recurred. Most cases recurred within 24 months.[1]

Due to the rare incidence of the tumor, there are no standard treatment protocols.[4] Wang et al. reported three cases of congenital sialoblastomas, which did not recur after surgery and after a mean follow-up of 34 months. They concluded that surgical resection of the tumor is the safest modality of treatment.[5] Chemotherapy has also been tried as a treatment modality as this is a very chemosensitive tumor. Saribeyoglu et al. published a case of recurrent sialoblastoma who was treated with vincristine-, actinomycin D-, and cyclophosphamide-based chemotherapy. They suggested chemotherapy for unresectable or recurrent sialoblastoma.[6] Neoadjuvant chemotherapy has also been used with good outcome by Prigent et al.[7] Radiation has also proved beneficial in treatment of sialoblastoma with incomplete removal.[2]


  Conclusion Top


Sialoblastoma is a rare tumor in the pediatric age group that arises from the salivary gland. Our case highlights the aggressive nature of the disease with local and distant metastasis. Surgery remains the cornerstone of treatment with radiation and chemotherapy considered useful in metastatic disease. However, as there are no standard protocols, treatment has to be individualized.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Choudhary K, Panda S, Beena VT, Rajeev R, Sivakumar R, Krishanan S, et al. Sialoblastoma: A literature review from 1966-2011. Natl J Maxillofac Surg 2013;4:13-8.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Sitthichaiyakul P, Somran J, Oilmungmool N, Worasakwuttipong S, Larbcharoensub N. Sialoblastoma of the cheek: A case report and review of the literature. Mol Clin Oncol 2016;4:925-8.  Back to cited text no. 2
    
3.
Irace AL, Adil EA, Archer NM, Silvera VM, Perez-Atayde A, Rahbar R, et al. Pediatric sialoblastoma: Evaluation and management. Int J Pediatr Otorhinolaryngol 2016;87:44-9.  Back to cited text no. 3
    
4.
Saravakos P, Hartwein J, Fayyazi A. Sialoblastoma of the parotid gland in a 13-year-old girl with multiple recurrences and long-term follow-up. Head Neck 2016;38:E13-5.  Back to cited text no. 4
    
5.
Wang L, Chen WL, Chen JF, Pan CB, Zhao XP. Surgical excision of sialoblastoma in the parotid gland in newborn. Int J Pediatr Otorhinolaryngol 2013;77:1268-71.  Back to cited text no. 5
    
6.
Saribeyoglu ET, Devecioglu O, Karakas Z, Anak S, Unuvar A, Agaoglu L, et al. How to manage an unresectable or recurrent sialoblastoma. Pediatr Blood Cancer 2010;55:374-6.  Back to cited text no. 6
    
7.
Prigent M, Teissier N, Peuchmaur M, El Maleh-Berges M, Philippe-Chomette P, Cardin P, et al. Sialoblastoma of salivary glands in children: Chemotherapy should be discussed as an alternative to mutilating surgery. Int J Pediatr Otorhinolaryngol 2010;74:942-5.  Back to cited text no. 7
    


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  [Figure 1], [Figure 2]



 

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