Neurofibromatosis type 1 (NF1) with plexiform neurofibromas (PN)

Active Ingredient: Selumetinib

Indication for Selumetinib

Population group: only children (1 year - 12 years old) , adolescents (12 years - 18 years old) , adults (18 - 65 years old)
Therapeutic intent: Curative procedure

Selumetinib as monotherapy is indicated for the treatment of symptomatic, inoperable plexiform neurofibromas (PN) in adult and paediatric patients with neurofibromatosis type 1 (NF1) aged 3 years and older.

For this indication, competent medicine agencies globally authorize below treatments:

25 mg/m² of body surface area (BSA) every 12 hours

For:

Dosage regimens

In case that patient age in years is ≥ 3

Oral, 25 milligrams selumetinib per square meter of body surface, once every 12 hours.

Detailed description

The recommended dose of selumetinib is 25 mg/m² of body surface area (BSA), taken orally twice daily (approximately every 12 hours).

Dosing is individualised based on BSA (mg/m²) and rounded to the nearest achievable 5 mg or 10 mg dose (up to a maximum single dose of 50 mg). Different strengths of selumetinib capsules can be combined to attain the desired dose (Table 1).

Table 1. Recommended dose based on body surface area:

Body surface area (BSA)aRecommended dose
0.55–0.69 m²20 mg in the morning and 10 mg in the evening
0.70–0.89 m²20 mg twice daily
0.90–1.09 m²25 mg twice daily
1.10–1.29 m²30 mg twice daily
1.30–1.49 m²35 mg twice daily
1.50–1.69 m²40 mg twice daily
1.70–1.89 m²45 mg twice daily
≥1.90 m²50 mg twice daily

a The recommended dose for patients with a BSA less than 0.55 m² has not been established.

Treatment with selumetinib should continue as long as clinical benefit is observed, or until PN progression or the development of unacceptable toxicity.

Missed dose

If a dose of selumetinib is missed, it should only be taken if it is more than 6 hours until the next scheduled dose.

Vomiting

If vomiting occurs after selumetinib is administered, an additional dose is not to be taken. The patient should continue with the next scheduled dose.

Dose adjustments

Interruption and/or dose reduction or permanent discontinuation of selumetinib may be required based on individual safety and tolerability.

Recommended dose reductions are given in Table 2 and may require the daily dose to be divided into two administrations of different strength or for treatment to be given as a once daily dose.

Table 2. Recommended dose reductions for adverse reactions:

Body surface area
(BSA)
Initial selumetinib
dosea
(mg/twice daily)
First dose reduction
(mg/dose)
Second dose reduction
(mg/dose)b
MorningEveningMorningEvening
0.55–0.69 m²20 mg in the
morning and 10 mg
in the evening
101010 once daily
0.70–0.89 m²2020101010
0.90–1.09 m²2525101010
1.10–1.29 m²3025202010
1.30–1.49 m²3525252510
1.50–1.69 m²4030302520
1.70–1.89 m²4535302520
≥1.90 m²5035352525

a Based on BSA as shown in Table 1.
b Permanently discontinue treatment in patients unable to tolerate selumetinib after two dose reductions.

Dose modifications for the management of adverse reactions associated with this medicinal product are presented in Table 3.

Table 3. Recommended dose modifications for adverse reactions:

CTCAE Grade*Recommended dose modification
Grade 1 or 2 (tolerable –
can be managed with
supportive care)
Continue treatment and monitor as clinically indicated
Grade 2 (intolerable –
cannot be managed with
supportive care) or
Grade 3
Interrupt treatment until toxicity is grade 0 or 1 and reduce by one dose
level when resuming therapy (see Table 2)
Grade 4Interrupt treatment until toxicity is grade 0 or 1, reduce by one dose level
when resuming therapy (see Table 2). Consider discontinuation

* Common Terminology Criteria for Adverse Events (CTCAE)

Active ingredient

Selumetinib

Selumetinib is a selective inhibitor of mitogen activated protein kinase kinases 1 and 2 (MEK ½). Selumetinib blocks MEK activity and the RAF-MEK-ERK pathway. Therefore, MEK inhibition can block the proliferation and survival of tumour cells in which the RAF-MEK-ERK pathway is activated.

Read more about Selumetinib

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