Sialoblastoma of the cheek: A case report and review of the literature

Abstract

Sialoblastoma is a rare salivary gland tumor that recapitulates the primitive salivary gland anlage. The authors herein report a case of sialoblastoma of a minor salivary gland, clinically presenting with progressive enlargement of a mass in the cheek of a 1-year-old female infant. Histopathologically, the mass consisted of tight clusters of basaloid cells and partially formed ductal and pseudo-ductal spaces separated by thin fibrous bands. Immunohistchemical studies demonstrated the presence of cytokeratin AE1/AE3, p63, CD99, α-fetoprotein (AFP) and Hep Par-1 expression in a considerable number of tumor cells. The clinical and pathological characteristics are presented and relevant literature is reviewed. Early complete surgical excision is recommended for the treatment of sialoblastoma. Radiation may be considered in cases with incomplete resection of the tumor. Chemotherapy may play a vital role in extensive, metastatic, or relapsed cases, or in cases with inadequate excision. The follow-up treatment should be frequent and prolonged. To the best of our knowledge, this is the first reported case of sialoblastoma of the cheek with immunoreactivity for AFP and Hep Par-1, which may be associated with the embryonic origin of the tumor. AFP may be a useful marker of tumor response in patient with sialoblastoma.

Sialoblastoma and Hepatoblastoma in an Infant: A Case Report and Review of the Literature

“…The imaging features of sialoblastoma have been documented in a few cases. On CT scan, they are detected as lobulated lesions that are isodense to muscle (11,12). On MRI, these tumors show low or intermediate signal intensity on T1-weighted images and intermediate or mildly high signal intensity on T2-weighted images, suggesting a high nucleus-to-cytoplasmic ratio (13)(14)(15).…”

Progressive Metastatic Sialoblastoma in a Young Child: Challenges in Treatment

“…[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.…”

“…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.…”

“…[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.…”

“…[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]…”