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Sialoblastoma of the submandibular gland: a distinct entity?

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Abstract

Sialoblastoma is a rare congenital malignant tumor of the salivary glands. A case of a submandibular sialoblastoma in a 1.5-year-old child is presented. A comparative analysis on 79 pediatric cases reported in the literature suggests a less aggressive behavior for submandibular sialoblastoma in comparison with other sites. Classically, diagnosis is confirmed by open biopsy, but fine-needle aspiration may offer an alternative with reduced morbidity. Expression of AFP and high levels of Ki-67 have been associated with poor prognosis. Whilst early surgical resection with negative margins is widely accepted as first-line treatment, there is no consensus on therapy of recurrence and follow-up. MRI and sonography represent valid tools for the follow-up, which is usually restricted to 3–5 years.

Conclusion: Submandibular sialoblastomas may have a different biological profile in comparison with parotid tumors with the absence of metastasis and much lower rate of recurrence. Comprehensive diagnostics should include additional options such as fine-needle aspiration and markers to assess cell proliferation and AFP. Literature suggests that surgery alone is sufficient for the treatment of tumors with low malignancy. Follow-up should be tailored according to the tumor site and might be limited to 3–5 years.

What is Known:

• Sialoblastoma is a rare congenital malignant tumor with an unpredictable clinical outcome.

What is New:

• Sialoblastoma of submandibular origin seems to have a less aggressive behavior in comparison with other sites.

• Fine-needle aspiration and markers to assess proliferation index (i.e., suggestive of potential more aggressive course/malignancy) should be strongly considered in the diagnostic work-up.

• Radical surgery as first-line therapy and a 3–5-year follow-up are acceptable for tumors with a low malignancy.

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Authors and Affiliations

  1. Department of Otolaryngology, University Hospital Basel, University of Basel, 4031, Basel, Switzerland

    Riccardo Di Micco & Nicolas Gürtler

  2. Department of Radiology, Children’s University Hospital Basel, 4056, Basel, Switzerland

    Friederike Prüfer

  3. Department of Pathology, University Hospital Basel, 4056, Basel, Switzerland

    Elisabeth Bruder

  4. Department of Oncology, Children’s University Hospital Basel, 4056, Basel, Switzerland

    Alexandra Schifferli

  5. Department of Otolaryngology, Children’s University Hospital Basel, 4056, Basel, Switzerland

    Nicolas Gürtler

Authors
  1. Riccardo Di Micco
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  2. Friederike Prüfer
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  3. Elisabeth Bruder
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  4. Alexandra Schifferli
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  5. Nicolas Gürtler
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Contributions

Authors' Contributions All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by all authors. The first draft of the manuscript was written by Riccardo Di Micco and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Nicolas Gürtler.

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The authors declare that they have no conflict of interest.

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This article does not contain any studies with human participants or animals performed by any of the authors.

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Informed consent was obtained from all individual participants included in the study.

Additional information

Communicated by Peter de Winter

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Revisions received: 7 June 2019 / 9 June 2019

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Di Micco, R., Prüfer, F., Bruder, E. et al. Sialoblastoma of the submandibular gland: a distinct entity?. Eur J Pediatr 178, 1301–1304 (2019). https://doi.org/10.1007/s00431-019-03411-x

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  • DOI: https://doi.org/10.1007/s00431-019-03411-x

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