Chapter 12 - Part 2 Flashcards Preview

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Flashcards in Chapter 12 - Part 2 Deck (68):
1

What is Multiple endocrine neoplasia Type 2B characterized by?

Parathyroid tumors, pituitary tumors, pancreatic tumors, adrenal gland tumors, thyroid tumors, mucosal neuromas

2

What are adrenal gland tumors called, and how many MEN patients get it?

Pheochromocytoma -- 50% develop

3

What are thyroid tumors called, and how many MEN patients get it?

Medullary carcinoma -- 90% develop

4

In addition to systemic disease, what is characteristic about the patient's features?

A marfanoid build, with thin elongated limbs, and a narrow face with thick protuberant lips

5

What is typically the first sign of multiple endocrine neoplasia?

Oral mucosal neuromas -- bilateral neuromas at the commissural mucosa

6

When is multiple endocrine neoplasia usually diagnosed?

Between the ages of 18-25

7

Why is it important to catch multiple endocrine neoplasia early on?

Multiple endocrine neoplasia has a marked propensity for metastasis

8

What term describes a rare pigmented tumor that usually occurs during the 1st year of life?

Melanotic neuroectodermal tumor of infancy

9

Where do melanotic neuroectodermal tumors of infancy usually occur?

Stirking predilection for the anterior maxilla (61%)

10

What is the clinical presentation of melanotic neuroectodermal tumors of infancy?

Rapidly expanding mass that is clinically black and/or blue

11

What urine test is associated with melanotic neuroectodermal tumor of infancy?

High urinary levels of vanillylmandelic acid

12

Are melanotic neuroectodermal tumor of infancy usually benign or malignant?

Most are benign

13

What is a granular cell tumor?

A benign soft tissue neoplasm that shows a predilection for the oral cavity

14

What is the most common site for a granular cell tumor?

The dorsal tongue

15

At what age do granular cell tumors usually occur?

Around 40, rare in kids

16

What is the clinical presentation for a granular cell tumor?

Asymptomatic, sessile nodule less than 2 cm, that may be yellow or mucosal colored

17

What population might experience multiple granular cell tumors?

African Americans

18

What is a significant microscopic feature of granular cell tumors?

Pseudoepitheliomatous hyperplasia

What other thing causes PEH? Blastomycosis infection

19

What test is used to diagnose a granular cell tumor?

The S-100 test, and it will be positive!!!

20

What term describes a mucosal colored, smooth surfaced mass that is present at birth?

Congenital epulis

21

Where does a congenital epulis commonly occur?

3x more common in the maxillary ridge (almost exclusively on the alveolar ridges),

22

In what population do congenital epulis (epuli/epulises??) commonly occur?

90% occur in females

23

What tests are done and test results are found to diagnose congenital epulis?

No pseudoepitheliomatous hyperplasia, and S-100 test is negative

24

How is a congential epulis treated?

Surgical excision

25

How is a granular cell tumor treated?

Surgical excision

26

What term describes a benign tumor of blood vessels?

Hemangioma

27

What is the most common tumor of infancy?

Hemangioma

28

In what population are hemangiomas most common?

White females

29

What is the most common location of hemangiomas?

The head and neck

30

What are the 2 types of hemangiomas?

Capillary and cavernous

31

What is the difference between capillary and cavernous hemangioma?

Capillary hemangiomas may not blanch and are usually red in color, where cavernous hemangiomas typically blanches and may be darker red to purple
--> diascopy: poke it

32

What term describes a rare, nonhereditary developmental condition?

Sturge-Weber angiomatosis

33

What is the cause of sturge-weber angiomatosis?

Caused by the persistence of a vascular plexus around the cephalic portion of the neural tube

34

What are the congenital dermal capillary vascular malformations referred to as?

Port wine stain or nevus flammeus

*not all patients with port wine stain have sturge-weber angiomatosis

35

What nerve is usually involved with Sturge-weber angiomatosis?

The trigeminal nerve = unilateral distribution

36

In addition to nevus flammeus, what do sturge-weber angiomatosis patients also have?

Leptomeningeal angiomas that overlie the ipsilateral cerebral cortex, and may result in mental retardation and contralateral hemiplagia (paralysis)

37

What is sturge-weber angiomatosis associated with?

A convulsive disorder (anticonvulsants = drug induced gingival hyperplasia)

38

Why are dentist's and oral pathologists concerned about sturge-weber angiomatosis?

Intraoral involvement is common and results in hypervascular changes to the ipsilateral mucosa

39

What may occur if sturge-weber angiomatosis lesions are manipulated?

Hemorrhage

40

What term describes a benign tumor of lymphatic vessels?

Lymphangioma

41

What are the 3 types of lymphangiomas?

Capillar, cavernous, and cystic

42

Where are cavernous lymphangiomas commonly found?

In the mouth

43

Where are cystic lymphangiomas commonly found?

In the neck

44

When are lymphangioma lesions commonly noted?

Half of all lesions are noted at birth, and 90% develop by 2 years of age

45

Where do oral lymphangiomas occur most frequently occur?

On the anterior 2/3 of the tongue and often result in MACROGLOSSIA

46

What do lymphangiomas clinically look like?

Frog eggs, or tapioca pudding

47

What term describes a benign tumor of smooth muscle?

Leiomyoma

48

Where do leiomyomas most commonly occur?

Uterus, GI tract, skin (can be a neoplasia of smooth muscle wall around blood vessels)

49

What term describes a benign tumor or skeletal muscle?

Rhabdomyoma

50

Where do rhabdomyomas usually occur?

Extracardiac, but very rarely they will occur in the head and neck

51

What are the 2 types of rhabdomyomas?

Adult and fetal

52

What is the difference in adult and fetal type rhabdomyoma?

Adult rhabdomyomas occurs in adult men in the pharynx and FOM, where fetal rhabdomyomas occur in young males and have a predilection for the face

53

What is the treatment for leiomyomas and rhabdomyomas?

Surgical excision

54

What are the common soft tissue sarcomas that can (but rarely) occur in the oral and maxillofacial region?

Fibrosarcomas, malignant fibrous histiocytoma, liposarcoma, leiomysocarcoma, angiosarcoma, malignant peripheral nerve sheath tumor

55

What virus is Kaposi's sarcoma caused by?

HHV-8

56

What are the 4 clinical presentations of Kaposi's sarcoma?

Classic, Endemic, Iatrogenic immunosuppression associated, AIDS related

57

What is the most common clinical presentation of Kaposi's sarcoma?

Classic type

58

Describe the classic type of Kaposi's sarcoma.

It is hereditary, occurs in older men or Italian, Jewish or Slavic descent (Mediterranean). Multiple blue-purple lesions occur on the lower extremities. BUT it has 90% survival

59

What are the 4 subtypes of the Endemic (African) type of Kaposi's sarcoma?

Benign nodular, aggressive, florid and lymphadenopathic... endemic has the poor prognosis

60

In what population do iatrogenic type kaposi's sarcoma commonly occur?

Transplant patients

61

What does the treatment of Kaposi's sarcoma depend on?

Type, stage, location

62

How is Kaposi's sarcoma treated?

Radiation, surgery, and chemotherapy

63

What term describes a malignant tumor of skeletal muscles?

Rhabdomyosarcoma

64

Rhabdomyosarcoma accounts for how many soft tissue sarcomas of childhood?

60% of soft tissue sarcomas of childhood

65

How many of rhabdomyosarcomas occur in the head and neck?

35%, face and orbit are common locations

66

What is the most common oral soft tissue site of metastases?

Gingiva, then tongue

67

What do metastases to the oral soft tissues most commonly represent?

Carcinomas rather than sarcomas

68

What is one possible explanation of Head and neck metastases in the absence of lung metastases?

Batson's plexus, which is a valveless vertebral venous plexus that might allow retrograde spread of tumor cells and bypass the lungs