Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 5  |  Issue : 2  |  Page : 135-137

Congenital sialoblastoma: A case report and review of literature


1 Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Pediatric Surgery, Sir Ganga Ram Hospital, New Delhi, India

Date of Web Publication3-Jan-2014

Correspondence Address:
Riti Aggarwal
Department of Pathology, Sir Ganga Ram Hospital, New Delhi - 110 060
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-8844.124261

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  Abstract 

Sialoblastomas are very rare epithelial tumors of salivary gland that usually occur in parotid or submandibular gland. Most of the sialoblastomas present in the neonatal period or early childhood. The biological behavior of these tumors is variable because of the potential for local and systemic recurrences. Only ten cases have been reported in submandibular gland so far. We report a case of newborn female child who presented with a left submandibular swelling.

Keywords: Childhood, salivary gland, sialoblastomas, submandibular


How to cite this article:
Aggarwal R, Bhalla S, Chopra P, Kulshreshtha R. Congenital sialoblastoma: A case report and review of literature. J Orofac Sci 2013;5:135-7

How to cite this URL:
Aggarwal R, Bhalla S, Chopra P, Kulshreshtha R. Congenital sialoblastoma: A case report and review of literature. J Orofac Sci [serial online] 2013 [cited 2020 Feb 23];5:135-7. Available from: http://www.jofs.in/text.asp?2013/5/2/135/124261


  Introduction Top


Sialoblastomas are very rare epithelial tumors of salivary gland origin of childhood. We reviewed the English literature and found that only 42 cases have been reported so far. Most common site of involvement was parotid gland with only ten cases in submandibular gland. Ectopic salivary gland tissue as a site of origin has also been reported. [1] We report a case of sialoblastoma in a newborn female child arising in left submandibular region.


  Case Report Top


A 1-day-old female infant presented with a firm, large lump in the left submandibular region. She was born as a full-term female neonate by normal vaginal delivery. Obstetric history was unremarkable. Soon after birth, she was noted to have a mass in left submandibular region measuring about 8 × 7 cm [Figure 1]. The mass was nodular, firm, and fixed to the deeper structures; but not with the overlying skin. Fine needle aspiration performed was reported as a biphasic soft tissue tumor composed of epithelial and spindle cells in a myxoid background. Magnetic resonance imaging (MRI) of head and neck showed a well-defined soft tissue mass measuring 5 × 5 cm in left submandibular region, abutting the left omohyoid and hyoglossus muscles in the parapharyngeal space and displacing the sternocleidomastoid muscle and parotid gland posteriorly. Bilateral carotid vessels were well preserved [Figure 2]. Chest X-ray was normal. Complete excision of the mass was then performed. The mass was easily enucleated.
Figure 1: Neonate with mass in the left submandibular region

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Figure 2: Magnetic resonance imaging (MRI) neck. Well-defined soft tissue mass in left submandibular region

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Grossly, the tumor was well circumscribed with a grey-yellow cut surface [Figure 3]. Microscopically, it was composed of islands of primitive basaloid cells with peripheral palisading separated by fibromyxoid stroma. Focal ductal differentiation in the basaloid cell nests and a few well-defined ducts between the lobules were present. There was no necrosis or neurovascular invasion [Figure 4]. On immunohistochemistry, the tumor cells in the lobules were positive for cytokeratin (CK) and smooth muscle actin (SMA) highlighted the peripheral myoepithelial cells. Ki67/MIB1 was seen in about 4-6% cells mainly at the periphery of the lobules [Figure 5]. Based on these findings, the mass was diagnosed as sialoblastoma. Postoperative course of the patient was uneventful and no recurrence was noted after 8 months of follow-up.
Figure 3: Gross specimen. Well-circumscribed mass with homogenous grey-yellow cut surface

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Figure 4: Microphotograph of the tumor. It is composed of islands of primitive basaloid cells in a fibromyxoid stroma arranged in lobules with ducts in the middle of lobules (hematoxylin and eosin (H and E), ×200 original magnification and inset ×400 original magnification)

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Figure 5: Microphotograph showing peripheral myoepithelial cells positive for SMA

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  Discussion Top


Salivary gland neoplasms are uncommon in children and account for only 3-5% of all salivary gland tumors in childhood. [2] Sialoblastoma is rare and was first described by Vawter and Tefft in 1966 under the name 'embryoma'. [3] Later, Taylor named it sialoblastoma. [4] He described these tumors to be in a primitive state of morphogenesis suggesting the maturation arrest of the epithelial salivary gland anlage. Most of the sialoblastomas present in the neonatal period or early childhood as a neck mass over the angle of mandible. There is no typical presentation. It is difficult to distinguish these from other more common neck masses like hemangioma, lymphangioma, vascular malformation, lymph node, and nerve sheath tumors or teratoma. Association of these tumors with hepatoblastoma or cutaneous hamartoma has been reported. [5],[6]

Characteristically, sialoblastomas are composed of numerous solid islands of primitive basaloid epithelial cells and small ducts separated by fibrous to fibromyxoid stroma mimicing the fetal salivary gland. [7] Within some islands, distinct lumina containing basophilic secretory material are seen. There is peripheral palisading of nuclei in these islands of cells. The other salivary gland basaloid tumors are basal cell adenoma, basal cell adenocarcinoma, and adenoid cystic carcinoma. However, these tumors are rare in children under 5 years of age. The immunohistochemical markers used to identify myoepithelial and epithelial cells are SMA, S-100, P63, and CK. P-53 and Ki-67 highlights the proliferative activity of the tumor.

Given its rarity, there is no consensus regarding their biological behavior. Recurrence rate of 34% is reported in sialoblastomas involving parotid gland, while no recurrence has been noted in the submandibular gland. [2] These tumors are locally aggressive with recurrences as well as occasional metastasis to lungs and lymph nodes. [8] According to Batsakis et al., histological features that suggest malignant potential are necrosis, atypia, and neurovascular invasion. [9] Williams et al., [2] classified these into favorable and unfavorable tumors based on histological features such as mitotic figures/high Ki-67 index, anaplasia, necrosis, and circumscription. The favorable features in our case was well circumscription of the tumor, no atypia, necrosis, or neurovascular invasion. The tumor cells showed intermediate Ki-67 expression. However, only long-term follow-up will predict its biological behavior. Complete surgical excision with negative margins is said to be curative even for recurrent cases. The other treatment modalities if needed are chemotherapy and radiotherapy. [10]


  Conclusion Top


Sialoblastoma is a rare, aggressive, and potentially malignant salivary gland tumor of childhood. Although it is an extremely unusual pediatric neoplasm, it should be recognized by otolarygologists and pathologists and considered in the differential diagnosis of submandibular swellings. Early surgery is prudent and the patient must be followed-up to detect local recurrences.

 
  References Top

1.Saffari Y, Blei F, Warren SM, Milla S, Greco MA. Congenital minor salivary gland sialoblastoma: A case report and review of the literature. Fetal Pediatr Pathol 2011;30:32-9.  Back to cited text no. 1
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2.Williams SB, Ellis GL, Warnock GR. Sialoblastoma: A clinicopathologic and immunohistochemical study of 7 cases. Ann Diagn Pathol 2006;10:320-6.  Back to cited text no. 2
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3.Vawter GF, Teftt M. Congenital tumors of the parotid gland. Arch Pathol 1966;82:242-5.  Back to cited text no. 3
    
4.Taylor GP. Congenital epithelial tumor of the parotid sialoblastoma. Pediatr Pathol 1988;8:447-52.  Back to cited text no. 4
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5.Cheng YK, Chu WC, Law LW, Ting YH, Leung TY. A fetus with a huge neck mass and a large abdominal circumference - a rare case of sialoblastoma and hepatoblastoma. Prenat Diagn 2012;32:915-7.  Back to cited text no. 5
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6.Green RS, Tunkel DE, Small D, Westra WH, Argani P. Sialoblastoma: Association with cutaneous hamartoma (organoid nevus)? Pediatr Dev Pathol 2000;3:504-5.  Back to cited text no. 6
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7.Ellis GL. What's new in the AFIP fascicle on salivary gland tumors: A few highlights from the 4 th Series Atlas. Head Neck Pathol 2009;3:225-30.  Back to cited text no. 7
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8.Huang R, Jaffer S. Imprint cytology of metastatic sialoblastoma: A case report. Acta Cytol 2003;47:1123-6.  Back to cited text no. 8
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9.Batsakis JG, Mackay B, Ryka AF, Seifert RW. Perinatal salivary gland tumours (embryomas). J Laryngol Otol 1988;102:1007-11.  Back to cited text no. 9
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10.Farooqi KM, Kessel R, Brandwein-Gensler M, Granowetter L, Manwani D. Sialoblastoma-long-term follow-up and remission for a rare salivary malignancy. Rare Tumors 2011;3:e13.  Back to cited text no. 10
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    Figures

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


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