Chapter 12 - Part 2 Flashcards

1
Q

What is Multiple endocrine neoplasia Type 2B characterized by?

A

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

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2
Q

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

A

Pheochromocytoma – 50% develop

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3
Q

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

A

Medullary carcinoma – 90% develop

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4
Q

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

A

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

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5
Q

What is typically the first sign of multiple endocrine neoplasia?

A

Oral mucosal neuromas – bilateral neuromas at the commissural mucosa

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6
Q

When is multiple endocrine neoplasia usually diagnosed?

A

Between the ages of 18-25

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7
Q

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

A

Multiple endocrine neoplasia has a marked propensity for metastasis

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8
Q

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

A

Melanotic neuroectodermal tumor of infancy

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9
Q

Where do melanotic neuroectodermal tumors of infancy usually occur?

A

Stirking predilection for the anterior maxilla (61%)

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10
Q

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

A

Rapidly expanding mass that is clinically black and/or blue

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11
Q

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

A

High urinary levels of vanillylmandelic acid

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12
Q

Are melanotic neuroectodermal tumor of infancy usually benign or malignant?

A

Most are benign

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13
Q

What is a granular cell tumor?

A

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

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14
Q

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

A

The dorsal tongue

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15
Q

At what age do granular cell tumors usually occur?

A

Around 40, rare in kids

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16
Q

What is the clinical presentation for a granular cell tumor?

A

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

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17
Q

What population might experience multiple granular cell tumors?

A

African Americans

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18
Q

What is a significant microscopic feature of granular cell tumors?

A

Pseudoepitheliomatous hyperplasia

What other thing causes PEH? Blastomycosis infection

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19
Q

What test is used to diagnose a granular cell tumor?

A

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

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20
Q

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

A

Congenital epulis

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21
Q

Where does a congenital epulis commonly occur?

A

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

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22
Q

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

A

90% occur in females

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23
Q

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

A

No pseudoepitheliomatous hyperplasia, and S-100 test is negative

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24
Q

How is a congential epulis treated?

A

Surgical excision

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25
Q

How is a granular cell tumor treated?

A

Surgical excision

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26
Q

What term describes a benign tumor of blood vessels?

A

Hemangioma

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27
Q

What is the most common tumor of infancy?

A

Hemangioma

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28
Q

In what population are hemangiomas most common?

A

White females

29
Q

What is the most common location of hemangiomas?

A

The head and neck

30
Q

What are the 2 types of hemangiomas?

A

Capillary and cavernous

31
Q

What is the difference between capillary and cavernous hemangioma?

A

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
Q

What term describes a rare, nonhereditary developmental condition?

A

Sturge-Weber angiomatosis

33
Q

What is the cause of sturge-weber angiomatosis?

A

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

34
Q

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

A

Port wine stain or nevus flammeus

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

35
Q

What nerve is usually involved with Sturge-weber angiomatosis?

A

The trigeminal nerve = unilateral distribution

36
Q

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

A

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

37
Q

What is sturge-weber angiomatosis associated with?

A

A convulsive disorder (anticonvulsants = drug induced gingival hyperplasia)

38
Q

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

A

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

39
Q

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

A

Hemorrhage

40
Q

What term describes a benign tumor of lymphatic vessels?

A

Lymphangioma

41
Q

What are the 3 types of lymphangiomas?

A

Capillar, cavernous, and cystic

42
Q

Where are cavernous lymphangiomas commonly found?

A

In the mouth

43
Q

Where are cystic lymphangiomas commonly found?

A

In the neck

44
Q

When are lymphangioma lesions commonly noted?

A

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

45
Q

Where do oral lymphangiomas occur most frequently occur?

A

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

46
Q

What do lymphangiomas clinically look like?

A

Frog eggs, or tapioca pudding

47
Q

What term describes a benign tumor of smooth muscle?

A

Leiomyoma

48
Q

Where do leiomyomas most commonly occur?

A

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

49
Q

What term describes a benign tumor or skeletal muscle?

A

Rhabdomyoma

50
Q

Where do rhabdomyomas usually occur?

A

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

51
Q

What are the 2 types of rhabdomyomas?

A

Adult and fetal

52
Q

What is the difference in adult and fetal type rhabdomyoma?

A

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
Q

What is the treatment for leiomyomas and rhabdomyomas?

A

Surgical excision

54
Q

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

A

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

55
Q

What virus is Kaposi’s sarcoma caused by?

A

HHV-8

56
Q

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

A

Classic, Endemic, Iatrogenic immunosuppression associated, AIDS related

57
Q

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

A

Classic type

58
Q

Describe the classic type of Kaposi’s sarcoma.

A

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
Q

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

A

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

60
Q

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

A

Transplant patients

61
Q

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

A

Type, stage, location

62
Q

How is Kaposi’s sarcoma treated?

A

Radiation, surgery, and chemotherapy

63
Q

What term describes a malignant tumor of skeletal muscles?

A

Rhabdomyosarcoma

64
Q

Rhabdomyosarcoma accounts for how many soft tissue sarcomas of childhood?

A

60% of soft tissue sarcomas of childhood

65
Q

How many of rhabdomyosarcomas occur in the head and neck?

A

35%, face and orbit are common locations

66
Q

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

A

Gingiva, then tongue

67
Q

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

A

Carcinomas rather than sarcomas

68
Q

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

A

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