TABLE 1.
Pt | Age at NF1 Dx |
Clinical criteria at NF1 Dx |
Other NF1 stigmata |
Decade of age at tumor Dx |
Tumor histology |
Tumor location |
Indication for evaluation |
Timeline of symptomatic progression (months) |
Treatment | Survival from symptom onset (days) |
---|---|---|---|---|---|---|---|---|---|---|
1 | Infancy | CALM, dermal NF |
DD | 40’s | PA | Medulla | Clinical progression |
4 | STR, RT | 330 |
2 | Childhood | CALM, freckling, LN |
DD | 20’s | PAa | Brainstem | Radiographic progression |
5 | STR, Carbo/Vinc, RT+Bev |
611 |
3 | 5 yrs | CALM, dermal NF |
OPG | 30’s | PA | Cerebellum | Clinical progression |
7 | STR, Carbo/Vinc, everolimus+Bev |
844 |
Dx, diagnosis; NF1, neurofibromatosis type 1; CALM, café au lait macules; NF, neurofbroma; LN, Lisch nodule; DD, developmental delay; OPG, optic pathway glioma; PA, pilocytic astrocytoma; STR, subtotal resection; Carbo, carboplatinum; Vinc, vincristine; RT, radiotherapy; Bev, bevacizumab.
Denotes that histopathologic diagnosis was pilocytic astrocytoma with anaplastic features as detailed in Table II.