Benign mesenchymal neoplasm 3

Question 1

where are Granular cell tumors fount intraorally?

Answer 1

Develop on any cutaneous or mucosal surface, but 40% occur on the tongue

Question 2

CLINICAL characteristics of Granular Cell Tumors:

Answer 2

• Slow-growing, demarcated (though unencapsulated), non-tender submucosal nodule
• Most are less than one cm. in size
• Tongue is favorite site, but buccal mucosa and floor of mouth may be affected

Question 3

Prognosis/treatment for Granular cell tumors:

Answer 3

Conservative excision is usually curative

Prognosis is excellent

Question 4

___________ is a rare lesion of undetermined histogenesis, that appears on the maxillary ridge of newborns

Answer 4

Congenital Epulis

Question 5

general characteristics of Congenital Epulis:

Answer 5

• Found at birth on the maxillary ridge of girl babies
• Smooth-surfaced, often pedunculated
• Vary in size

Question 6

Histological characteristics of Congenital Epulis:

Answer 6

A) Microscopically shows a benign proliferation of cells having granular cytoplasms

B) No pseudoepitheliomatous hyperplasia

C) No S-100 positivity

Question 7

What is the treatment protocol for Congenital Epulis?

Answer 7

• Conservative excision
• No tendency to recur
• Some reports of spontaneous involution without surgery

Question 8

what is the most common tumor of childhood?

Answer 8

Hemangioma

Question 9

what are the most common sites for hemangiomas?

Answer 9

Head and neck region is frequently affected

Tongue is most common intraoral site

Question 10

clinical presentation of Hemangiomas:

Answer 10

A) Usually red in color, but can range through various shades of purple, depending on the caliber of vessels

B) Most are elevated, but macular lesions are also seen (“port wine stain”; nevus flammeus)

C) Variable in size

Question 11

what diagnostic tool can be used to determine whether a red lesion (hemangioma) is due to extravasated blood or to blood that is contained within vessels

Answer 11

diascopy

Question 12

T/F: Hemangiomas will “blanch” if pressed with a glass slide

Answer 12

True

Question 13

What are the treatment options for hemangiomas?

Answer 13

1) Surgical excision, cryotherapy, embolization or use of sclerosing agents if cosmetically unacceptable
2) If lesion impairs vision, treat as soon as possible
3) Otherwise, wait until child is at least 6 years of age

Question 14

_________________ is also known as Sturge-Weber syndrome (really should be “anomalad”)

Answer 14

Encephalotrigeminal Angiomatosis

Question 15

T/F: Encephalotrigeminal Angiomatosis is a congenital abnormality, not an inherited or genetic problem

Answer 15

True

Question 16

what is the definition of Encephalotrigeminal Angiomatosis?

Answer 16

Hemangiomatous process affecting superficial and deeper tissues in region of cranial nerve V

Question 17

Clinical symptoms of Encephalotrigeminal Angiomatosis:

Answer 17

1) Patients exhibit nevus flammeus in distribution of 1st, and sometimes 2nd or 3rd, division of C.N. V
2) Involvement of deeper soft tissues as well as meninges of brain
3) Seizure disorders, mental retardation, and hemiplegia may be present

Question 18

Radiographic signs of Encephalotrigeminal Angiomatosis:

Answer 18

A) Classic radiographic finding of “tram-line” calcifications seen on skull film

B) Parallel calcifications probably represent the calcified walls of abnormal blood vessels

Question 19

_________ is a benign neoplasm of lymphatic vessel differentiation

Answer 19

Lymphangioma

Question 20

Clinical characteristics of Lymphangiomas:

Answer 20

1) Often present at birth
2) Tongue is most common intraoral site
3) Surface has a vesicular appearance, similar to “frog eggs” or “tapioca pudding”

Question 21

Cystic Hygroma are simply a large form of what condition?

Answer 21

lymphangioma

Question 22

what areas of the body can be effected by Cystic Hygroma? why is this especially concerning?

Answer 22

1) May affect the neck, mediastinum, axilla and the oral cavity
2) Important because this lesion may cause airway obstruction, particularly if it becomes secondarily infected

Question 23

Treatment protocol for Cystic Hygromas:

Answer 23

• Management depends on size of lesion
• More difficult to treat than hemangioma because it is often difficult to discern the borders of the tumor
• Surgical excision is the treatment of choice
• Relatively high recurrence rate: up to 40% in some series

Question 24

____________ is a benign tumor of smooth muscle differentiation

Answer 24

Leiomyoma

Question 25

clinical characteristics of Leiomyomas:

Answer 25

1) Most in the oral region probably arise from vascular smooth muscle
2) Well-demarcated, rubbery firm, less than 1 cm in diameter
3) May have a reddish or purplish color due to their vascular nature

Question 26

where are Leiomyomas usually found?

Answer 26

Most common in the upper lip, buccal mucosa and palate

Question 27

Histological features of Leiomyomas:

Answer 27

1) Benign proliferation of cells that resemble smooth muscle
2) Spindle-shaped, with cigar-shaped nuclei and eosinophilic cytoplasms
3) No significant atypia, and no mitotic activity
4) Positive for markers of muscle differentiation

Question 28

Treatment options for Leiomyomas:

Answer 28

Treatment consists of conservative excision
Prognosis is excellent
Virtually no chance of recurrence

Question 29

____________ are very rare, benign tumor of skeletal muscle differentiation

Answer 29

Rhabdomyoma

Question 30

T/F: Most rhabdomyomas arise in the heart (“cardiac rhabdomyoma”)

Answer 30

True

Question 31

Cardiac Rhabdomyomas are associated with what other syndrome?

Answer 31

tuberous sclerosis

Question 32

Extra-cardiac rhabdomyomas usually develop in what region?

Answer 32

head and neck region

Question 33

what are the clinical features of a Rhabdomyoma?

Answer 33

Non-tender, well-circumscribed mass in the submandibular or base of tongue region

Question 34

what is the treatment for Rhabdomyoma?

Answer 34

• Conservative surgical excision is appropriate

- Recurrence has been reported, but is not common

Question 35

what are the histological characteristics of Granular cell Tumors?

Answer 35

Collection of mesenchymal cells with a granular-appearing cytoplasm

Question 36

Granular cell tumors can be incorrectly diagnosed as Squamous cell carcinoma due to the presence of ___________ in 30% of cases

Answer 36

PEH (pseudoepitheliomatous hyperplasia)