Nerve tumors and syndromes

Question 1

Diff dx of swelling occurring commonly on the gingiva

Answer 1

Fibroma (normal color)
Fibrous epulis (normal to red mucosal color)
Pyogenic granuloma (red and bleeds easily)
Peripheral giant cell granuloma (red or red-blue nodular mass)
Peripheral ossifying granuloma (Normal mucosal color or red and ulcerated)

Question 2

Diff dx of bilateral Schwannomas in vestibulococchlear nerve (CN viii)

Answer 2

To look for other signs of neufibromatosis type 2

Question 3

What are other signs of neurofibromatosis type 2

Answer 3

Bilateral Schwannomas of vestibulococclear nerve
- progressive sensorineural deafness 
- dizziness
- tinnitus
Cafe au lait spots

Question 4

Signs of Schwannomas?

Answer 4

Slow growing encapsulated nerve tumor arising from Schwann cells

• soft tissue mass
• pain/tenderness
• radiolucent unilocular/multilocular for intrabony lesions
• paraesthesia

Question 5

Histopath of Scwannomas

Answer 5

AntoninA
Antoni B
Verocay bodies

Question 6

How is Schwannomas treated?

Answer 6

Excision.
Recurrence is rare
Malignant transformation is rare

Question 7

Treatment of Vestibular Schwannomas

Answer 7

Surgical excision - deafness and risk facial nerve injury

Stereotactic radiosurgery

Question 8

Neurofibroma?

Answer 8

Most common nerve tumor occurring in young adults

• slow growing soft painless mass
• skin most common
• buccal mucosa/ tongue
• can occur centrally producing well-demarcated/poorly defined unilocular/multilocular radiolucency
• assoc with neurofibromatosis type 1 (von recklinghausen disease)

Question 9

Histology of neurofibroma

Answer 9

Spindle-shaped cells with wavy nuclei
Myxoid mass
Abundant mast cells

Question 10

Von recklinghausen disease is also known as?

Answer 10

Neurofibromatosis type 1

Question 11

DIAGNOSTIC FEATURES OF NF1

Answer 11

Multiple neurofibromas on skin (papules, nodules, huge masses)
Cafe au lait spots that are smooth (coast of California)
Iris harmartoma
Optic glioma
Crowe sign (feckles on axilla)

Question 12

Importance in diagnosing von Recklinghausen disease (NF1)

Answer 12

Malignant transformation

Association with CGCG of mandible and Noonan syndrome

Question 13

Tx of NF1

Answer 13

Prevention of cancer - most common is malignant transformation to malignant peripheral nerve sheath tumor
Other tumors- cns tumors, leukemia, rhabdomyosarcoma

Question 14

MEN 2b

Multiple endocrine neoplasia type 2b

Answer 14

Oral/intestinal neuromas (first signs)
Medullary thryoid ca
Pheochromocytoma

Assoc with RET gene mutation

Marfanoid facies and body built - narrow face, elongated limbs with muscle wasting

Question 15

Why is MEN2b important in omfs

Answer 15

First to detect oral neuromas which are the early signs

Prompt referral to gen surgery or oncology for tx of medullary throid carcinoma and pheochromocytoma

Risk for emergncy life threatening hypertensive crisis

Question 16

Carotid body

Answer 16

Paraganglia of neural crest origin assoc with autonomic nerves

• chemoreceptors detecting oxygen and pH level
• baroreceptors detecting pressure

Question 17

Carotid body tumor

Answer 17

Carotid body paraganglioma

Bifurcation of internal and external carotid arteries

Slow growing painless mass at the upper lateral neck below angle of the mandible