Congenital granular cell myoblastoma
A congenital granular cell myoblastoma (also known as congenital epulis) is a very rare benign tumour which classically presents in the fetal-neonatal population.
It occurs almost exclusively in females when it presents in the fetal-neonatal population although no such predilection occur when it rarely presents in older children.
It is thought to occur as a result of excessive oestrogen production from the fetal ovaries under HCG stimulation.
The histogenesis is uncertain, and proposed cells of origin include odontogenic epithelium, undifferentiated mesenchymal cells, pericytes, fibroblasts, smooth muscle cells, nerve-related cells, and histiocytes.
It most commonly occurs along the alveolar ridge of the maxilla although less commonly, it can also occur in relation to the mandible.
Rarely a congenital epulis may be detected on antenatal ultrasound as an intra-oral mass. Colour and power Doppler ultrasound examination may show marked blood flow within the tumour 5.
General considerations include
- 1. Kusukawa J, Kuhara S, Koga C et-al. Congenital granular cell tumor (congenital epulis) in the fetus: a case report. J. Oral Maxillofac. Surg. 1997;55 (11): 1356-9. J. Oral Maxillofac. Surg. (link) - Pubmed citation
- 2. Cussen LJ, Macmahon RA. Congenital granular-cell myoblastoma. J. Pediatr. Surg. 1975;10 (2): 249-53. J. Pediatr. Surg. (link) - Pubmed citation
- 3. Koch BL, Myer C, Egelhoff JC. Congenital epulis. AJNR Am J Neuroradiol. 1997;18 (4): 739-41. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 4. Kim SK, Won HS, Lee SW et-al. Prenatal diagnosis of congenital epulis by three-dimensional ultrasound and magnetic resonance imaging. Prenat. Diagn. 2006;26 (2): 171-4. doi:10.1002/pd.1368 - Pubmed citation
- 5. Nakata M, Anno K, Matsumori LT et-al. Prenatal diagnosis of congenital epulis: a case report. Ultrasound Obstet Gynecol. 2002;20 (6): 627-9. doi:10.1046/j.1469-0705.2002.00867 - Pubmed citation
- 6. Jiang L, Hu B, Guo Q. Prenatal sonographic diagnosis of congenital epulis. J Clin Ultrasound. 2011;39 (4): 217-20. doi:10.1002/jcu.20765 - Pubmed citation
- 7. Roy S, Sinsky A, Williams B et-al. Congenital epulis: prenatal imaging with MRI and ultrasound. Pediatr Radiol. 2003;33 (11): 800-3. doi:10.1007/s00247-003-1024-4 - Pubmed citation