Melanotic Neuroectodermal Tumor of Infancy
Clinical
- Rapidly growing painless lesion
- Over 90% involve the bones of the head
- Up to 80% arise in the maxilla and/or palate
Maxilla |
80% |
Skull |
10% |
Mandible |
5% |
Brain |
4% |
Other (epididymis, ovary, mediastinum, etc…) |
< 1% |
- Over 90% occur within the first year of life
- Classically regarded as benign but may recur and infrequently metastasize
- Local recurrence rate 10-36%
- Often associated with incomplete surgical removal
- Metastasis rate 5- 7%
- Usually liver, bone, lymph nodes, spinal cord or brain