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Table 8 Details of patients with NF1 showing mass effect and/or enhancement of focal abnormal signal intensities (FASI)

From: Cerebellar radiological abnormalities in children with neurofibromatosis type 1: part 1 - clinical and neuroimaging findings

Patient study number

Age at finding in years (y)

Location

FASI diameter(cm)

Mass effect

Enhancement

FASI outcome

7

17.7

L cerebellum, R basal ganglia

1.2, 0.8

–

+

Remained stable in size with persisting enhancement on a repeat scan 1.3y later. Died at age 28.1y from a pelvic sarcoma

8

12.8

L cerebellum

3.7

+ initially only

+

Enlarged to 4 cm at age 13.1y then shrunk to 2.8 cm with resolution of mass effect and enhancement at age 13.5y. Disappeared at 21.3y

12.8

R cerebellum

2

+

+

Enlarged to 2.7 cm. Partial excision at age 13y (benign ganglioma). Remnant enhanced then resolved at age 21.8y. Died at age 23.4y from a malignant nerve sheath tumor

13

2.8

L cerebellum

1.2

+

–

Decreased to 0.8 cm with resolution of mass effect at age 5.5y. Disappeared at age 7.6y

2.8

R cerebellum

1

–

+

Decreased to 0.5 cm with resolution of enhancement at age 5.5y. Disappeared at age 7.6y

7

R cerebellum

0.8

+

+

Resolved at age 11.2y

20

1.9

L thalamus

1.6

+

–

Resolved at age 2.3y

33

3

R periventricular region

5.6 (few confluent FASI)

+ initially only

+ at 4.6y

Diameter was 4 cm and enhancement persisted on last scan at age 16y

3

Midbrain

1.6

–

+

Resolved at age 4y

41

11.7

Pons/ L superior cerebellar peduncle

1.6

+

+

Enlarged to 2.2 cm at age 17.2y then shrunk to 1.3 cm with resolution of mass effect at age 19.9y. Enhancement persisted on last scan at age 19.9y

14.2

L cerebellum

0.8

–

+ at 18.8y

Enlarged to 2 cm and developed into a cystic lesion with a solid component. Fully resected and proven to be a low grade pilocytic astrocytoma

44

1.8

Bilateral basal ganglia

4.9 each

+

+

Shrunk to 2.5, 2.7 cm at age 2.3y then enlarged to 3.9, 5 cm on last scan at age 3.8y. Both mass effect and enhancement persisted

1.8

L fornix

1.4

+ initially only

–

Enlarged to 2.5 cm at age 2.3y and to 2.7 cm on last scan at age 3.8y

1.8

Bilateral cerebral peduncles

1.7, 1.4

+ initially only

+

Size remained stable and enhancement persisted on last scan at age 3.8y

50

18.1

R periventricular region

2

+ at 29.3y

+ at 27.4y

Enlarged to 6.7 cm then showed necrosis at age 28.3y, followed by further enlargement to 10.5 cm with edema and mass effect at age 29.3y. Resected and proven to be an anaplastic astrocytoma

28

Corpus callosum

1.7

–

+

Enlarged to 2.4 cm at age 28.5y then shrunk to 1.9 cm on last scan at age 29.3y with a persisting enhancement

54

3.7

L subcortical white matter

0.5

–

+ at 5.3y

Enlarged to 1.4 cm at age 5.3y then shrunk to 1 cm at age 5.8y. Remained the same size with persistent enhancement on last scan at age 9.2y

  1. L left, R right, − absent, + present