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. Author manuscript; available in PMC: 2016 Jul 9.
Published in final edited form as: Am J Med Genet A. 2016 Mar 14;170(6):1455–1461. doi: 10.1002/ajmg.a.37622

TABLE 1.

Characteristics of NF1 Adults With Progressive Extra-Optic Pilocytic Astrocytomas

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.

a

Denotes that histopathologic diagnosis was pilocytic astrocytoma with anaplastic features as detailed in Table II.