Deck 22: Neurofibromatosis

Question 1

A young adult client diagnosed with neurofibromatosis asks if the disorder is cancer. How should you respond to the client?

A) "No, it is a benign encapsulated tumor caused by a specific type of cell."
B) "Unfortunately yes. Neurofibromatosis always produces cancerous tumors."
C) "It depends on what the tissue sample reveals after the biopsy of the mass."
D) "It may or may not be cancerous and depends upon how the tumor responds to treatment."

Answer 1

A
Explanation: A neurofibroma is defined as a benign, encapsulated tumor resulting from proliferation of Schwann cells that are of ectodermal (neural crest) origin and that form a continuous envelope around each nerve ¬fiber of peripheral nerves. The tumors are not cancerous. Determining if the tumor is cancerous is not dependent on the results of a biopsy or the response of the tumor to treatment.

Question 2

The parent of a preschool-age child is upset because the child is developing brown spots over the lower legs. Which should you explain to the parent about this finding?

A) "These spots are harmless and should not cause any concern."
B) "These are called café-au-lait spots. They are an indication of type 1 neurofibromatosis (NF-1)."
C) "These spots indicate an underlying bone cancer and must be biopsied immediately."
D) "These spots indicate type 2 NF and indicate that a change in hearing will occur in a few years."

Answer 2

B
Explanation: Type 1 NF manifests as tumors of the subcutaneous tissues and hyperpigmented skin lesions known as café-au-lait spots. The spots are not harmless and need to be identified. The spots do not indicate an underlying bone cancer. NF type 2 is characterized by the development of noncancerous tumors called schwannomas on the auditory and vestibular nerves that control hearing and balance. Although the tumors usually develop in late adolescence, some people do not develop problems until they are in their 40s and 50s.

Question 3

You are concerned that a young adult client has underdiagnosed neurofibromatosis type 2. What did you assess to make this clinical decision?

A) Severe itching
B) Muscle wasting
C) High blood pressure
D) Freckles under the armpits

Answer 3

B
Explanation: Muscle wasting is a manifestation of NF type 2. Severe itching, high blood pressure, and freckles where the skin meet skin as under the arms are all manifestations of NF type 1.

Question 4

Genetic testing shows the parents of a child with neurofibromatosis type 1 have no genetic evidence of the disorder. What should you say when the parents ask if any other future offspring will have the same disorder?

A) "Unfortunately, all offspring will have the disorder."
B) "There is a 50/50 chance that future offspring will have the disorder."
C) "The disorder can be stopped if specific lifestyle actions are taken now."
D) "Since neither of you are effected, the mutation occurred in utero and cannot be passed on to other offspring."

Question 5

A young adult diagnosed with neurofibromatosis type 2 asks what causes the disorder. What should you say in response?

A) "The gene that suppresses tumors malfunctions after puberty."
B) "The gene on chromosome 22 causes the formation of tumors."
C) "There is no known reason for the development of the disorder."
D) "The gene that suppresses the formation of tumors malfunctioned."

Question 6

A school-age child has 2 café-au-lait spots that measure 0.6 cm in size located on the inner thigh. Which action should be taken in order to diagnose this client with neurofibromatosis (NF) type 2?

A) Biopsy the areas
B) Reexamine the child in a year
C) Schedule for an immediate eye examination
D) Collect a skin scraping to have a chromosome analysis completed

Question 7

You are completing a physical assessment of a prepubescent client. For which reason should you mention the need for testing for neurofibromatosis type 1 (NF-1) with the healthcare provider?

A) Bilateral hearing loss
B) Cataract development in the left eye
C) Hypertrichosis noted on the forearm above the elbow
D) Freckles along the bridge and nose, extending onto both cheeks

Question 8

The parents of a child with neurofibromatosis type 1 (NF-1) learn about the disorder. Which statement indicates that you need to provide more teaching to the parents?

A) "There is no cure for the disorder."
B) "The spots can be removed which cures the disorder."
C) "The disorder is managed by detecting problems as they occur."
D) "Our child should be examined every year to check for problems."

Question 9

A young adult client is demonstrating signs of neurofibromatosis type 2 (NF-2). Which diagnostic test should you anticipate being scheduled for this client?

A) Spinal tap
B) Myelogram
C) MRI of the head
D) Cerebral angiogram

Question 10

A client with neurofibromatosis type 1 (NF-1) is upset about having Lisch nodules. What should you explain about this finding?

A) "They do not interfere with vision."
B) "You will need surgery to remove these nodules."
C) "They have no impact on the bones around your eyes."
D) "They have no effect on the development of an optic glioma on the future."