PDF 
Views 
Share
Progressive Metastatic Sialoblastoma in a Young Child: Challenges in Treatment
CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2019; 40(02): 314-315
DOI: DOI: 10.4103/ijmpo.ijmpo_183_19
| Figure 1 Preoperative picture showing massive swelling on the left cheek
| Figure 2 Postoperative picture showing considerable decrease in the size of the swelling
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]
Publication History
Article published online:
03 June 2021
© 2019. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
| Figure 1 Preoperative picture showing massive swelling on the left cheek
| Figure 2 Postoperative picture showing considerable decrease in the size of the swelling
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
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.
- 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
- 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
- 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
- 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
- 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
- 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
- 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
Address for correspondence
Publication History
Article published online:
03 June 2021
© 2019. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
| Figure 1 Preoperative picture showing massive swelling on the left cheek
| Figure 2 Postoperative picture showing considerable decrease in the size of the swelling
- 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
- 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
- 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
- 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
- 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
- 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
- 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