Maller Vijetha,  Arora Anurag,  Boulden Thomas,  Choudhri Asim

Final Pr. ID: Poster #: CR-013

A newborn male child with antenatal diagnosis of left facial mass presumably hemangioma, presented for postnatal ultrasound. Ultrasound of the left face and neck, showed a large homogenous mass measuring 5x7x4 cm arising from the inferior aspect of the superficial lobe and entire deep lobe of the parotid gland extending over the angle of mandible to the left parapharyngeal space. On MRI the mass was heterogenously enhancing with low signal intensity on T1 and intermediate signal on T2 weighted imaging. The mass was also extending into the left masticator, sublingual, and submandibular spaces. The neonate also had a right upper abdominal swelling, and an ultrasound abdomen detected heterogeneously hypo to hyperechoic mass in the segment 4 of liver. On triple phase computed tomography, the mass showed early peripheral arterial enhancement and heterogeneous enhancement in portal and venous phase. On magnetic resonance imaging, the mass was hyperintense to liver on T2-weigthed imaging with restricted diffusion and heterogenous enhancement less than the background liver on all phases. Biopsy result of the left facial mass was sialoblastoma and that of liver was hepatoblastoma. Alpha fetoprotein was also elevated. Wide margin surgical resection of sialoblastoma and left lobe hepatectomy was performed followed by chemotherapy.

Sialoblastoma is a rare congenital tumor of the salivary glands, mainly arising from the parotid gland, which is locally aggressive and potentially malignant. A total of approximately 40 cases of congenital sialoblastoma has been reported. Neonatal hepatoblastomas comprises 10% of pediatric hepatoblastomas. An association of sialoblastoma and centromere division has been reported, which in turn is associated with embryonal tumors. There have been only two reported cases of concomitant sialoblastoma and hepatoblastoma. Further research with genetic analysis is required to document association of these two embryonal tumors. The current management of these tumors is complete surgical resection followed by chemotherapy. Read More

Authors:  Maller Vijetha , Arora Anurag , Boulden Thomas , Choudhri Asim

Keywords:  Sialoblastoma, Hepatoblastoma