Table 2. Management of syndromic CGCG.
| NS | NF1/JC | OES | SS | CFC | OGD | Total | |
|---|---|---|---|---|---|---|---|
| Number of CGCGs with management/ follow-up information | 43 | 22 | 11 | 6 | 5 | 1 | 88 |
| Conservative excision Recurrence |
12 1 |
11 4 |
11 3 |
4 0 |
0 0 |
0 0 |
38 8 (21.1%) |
| Resection Recurrence |
8 1 |
5 3 |
0 0 |
2 1 |
5 0 |
0 0 |
20 5 (25%) |
| Observation only Continued growth |
23 9a |
6 4 |
0 0 |
0 0 |
0 0 |
1 1b |
30 14 (46.7%) |
NS denotes Noonan syndrome; NF1/JC denotes neurofibromatosis type 1/Jaffe-Campanacci syndrome; OES denotes oculoectodermal syndrome; SS denotes Schimmelpenning syndrome; CFC denotes cardiofaciocutaneous syndrome; OGD denotes osteoglophonic dysplasia
a Makis, et al reported continued CGCG growth in setting of radiation therapy, antiangiogenic therapy, and steroid therapy
b White, et al reported continued CGCG growth in setting of vinblastine and methotrexate therapy but with response to IV bisphosphonate