Miscellaneous deck 1

Question 1

Provide an alternate name for Gorlin syndrome

Answer 1

Nevoid basal cell carcinoma syndrome

(NBCCS)

Question 2

How is Gorlin syndrome inherited?

Answer 2

Autosomal dominant

Question 3

What is the underlying genetic defect in Gorlin syndrome?

Answer 3

Pathogenic variants in the PTCH1 region of SUFU tumor suppressor genes.

Question 4

Apart from basal cell carcinoma, what other tumors are associated with Gorlin syndrome? Name 3.

Answer 4

Odontogenic keratocysts
Cardiac and ovarian fibromas
Medulloblastomas

Question 5

Name 4 non-neoplastic features of Gorlin syndrome.

Answer 5

1. Palmar and plantar pits
2. Craniofacial and skeletal abnormalities
3. Ocular abnormalities
4. Ectopic intracranial calcification

Question 6

Name an important risk factor for basal cell carcinoma development in Gorlin syndrome.

Answer 6

Ionizing radiation

Question 7

Recommended nutritional supplements and Gorlin syndrome

Answer 7

Vitamin D
Oral nicotinamide (has been shown to reduce the rate of BCC development by 20%)

Question 8

Imiquimod 3.75% cream for actinic keratosis: Dosage instructions

Answer 8

Apply thin film or 2 full pump actuations once daily before bedtime for 2 weeks to the skin of the affected area (either the entire face or balding scalp but not both concurrently), leave on for approximately 8 hours then remove with mild soap and water.

Following 2 weeks of no treatment, repeat with a second 2-week treatment.

Question 9

Imiquimod 3.75% cream for actinic keratosis: Maximum to be prescribed:

Answer 9

56 packets or 2 x 7.5 g pumps per 2 cycles of treatment.

Question 10

Imiquimod 5% cream for actinic keratosis: Dosage instructions

Answer 10

Apply twice a week using up to 1 packet per application prior to normal sleeping hours to a defined treatment area on the face or scalp but not both concurrently. Leave on for approximately 8 hours then remove with mild soap and water.

Treatment should continue for 16 weeks.

Question 11

Imiquimod 5% cream for actinic keratosis: Maximum to be prescribed:

Answer 11

36 packets per 16-week treatment..

Question 12

Imiquimod 5% cream for basal cell carcinoma, superficial. Directions for application.

Answer 12

Apply once daily 5 days/week, prior to normal sleeping hours, for 6 weeks. Leave on skin for approximately 8 hours and remove with mild soap and water. Apply enough cream to cover the treatment area including 1 cm skin surrounding the tumor.
Treatment area should not exceed 3 cm.
The diameter of cream droplet applied should range from 4 mm to 7 mm for tumor areas of 0.5 cm to 2 cm respectively.

Question 13

Imiquimod 5% cream for basal cell carcinoma, superficial. Directions for application. Off label dosing for better tolerability:

Answer 13

Apply once every other day. May include a treatment holiday during weekends or in the middle of the treatment course.

Question 14

Imiquimod 5% cream for basal cell cancer, maximum to be supplied.

Answer 14

36 packets during the 6-week treatment..

Question 15

Imiquimod 5% cream for cutaneous squamous cell carcinoma in situ, low risk, off label use. Directions for application.

Answer 15

Apply to the target lesion once daily for 16 weeks.

OR

Apply to target lesion once daily every other day if genital lesion for at least 6 weeks and up to 20 weeks.

Question 16

Imiquimod 5% cream for melanoma in situ, lentigo maligna type, off label use.

Answer 16

Apply to lesion including a margin of 0.5 to 1 cm around the lesion, 5 nights a week for 12 weeks.

OR

Apply to lesion including a margin of 2 cm around the lesion 3-5 times a week for 12 weeks.