Malgnant Mesenchymal (3) Flashcards

1
Q

Which malignant mesenchymal tumor is of fibrous tissue?

A

Fibrosarcoma

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2
Q

Which malignant mesenchymal tumor is of nervous tissue?

A

Malignant peripheral nerve sheath tumor (MPNST)

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3
Q

Which malignant mesenchymal tumor is of endothelial cell tissue?

A

Kaposi sarcoma

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4
Q

Which malignant mesenchymal tumor is of muscle tissue?

A

Rhabdomyosarcoma

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5
Q

What are the clinical features of a fibrosarcoma?

A

Slow-growing, pain in late stage

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6
Q

What are the histo features of a fibrosarcoma?

A

Spindle shaped cells in a “herringbone” pattern

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7
Q

T/F: Treatment for fibrosarcoma includes radiation and chemotherapy.

A

False

Wide surgical excision

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8
Q

What is the 5 year survival rate of fibrosarcoma and how does it metastasize?

A

50%

Via blood

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9
Q

What are two other names for a MPNST?

A

Neurogenic sarcoma or neurofibrosarcoma

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10
Q

What are some clinical features of MPNST?

A

50% associated with NF1

May arise spontaneously

May have pain

Those with NF1 will develop lesion earlier in life (late 20s)

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11
Q

What are the histo features of MPNST?

A

Spindle-shaped cells with wavy nuclei

+ mitosis

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12
Q

What is the treatment and prognosis for MPNST?

A

Surgical resection and radical excision

50% 5-year survival without NF-1

Worse prognosis with NF-1

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13
Q

What causes Kaposi sarcoma?

A

Infection with HHV-8

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14
Q

What are the clinical features of classic kaposi syndrome?

A

Lower extremities of older patients

Painless purple. Goes from macule -> plaque -> tumor.

Does not blanch with pressure

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15
Q

What are the clinical presentations of endemic kaposi syndrome?

A

Seen in Africa

Varies from indolent skin lesions to aggressive tumors

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16
Q

T/F: Latrogenic kaposi syndrome will be seen weeks after organ transplant.

A

False

Months-years

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17
Q

What tissues are affected with latrogenic kaposi syndrome?

A

Skin and oral mucosa

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18
Q

T/F: Oral cavity is often the initial site of presentation in patients with classic kaposi syndrome.

A

False

AIDS-related kaposi syndrome

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19
Q

Where are there likely to be oral lesions with AIDS related kaposi syndrome?

A

Hard palate, gingiva, tongue

Will invade bone leading to mobility

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20
Q

What is the treatment and prognosis for kaposi syndrome?

A

Excision of small lesions, radiation and chemo injections

Classic and AIDS related -fair
Endemic - poor
latrogenic -fair to poor

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21
Q

What are the three types of Rhabdomyosarcoma?

A
  1. Embryonal
  2. Alveolar
  3. Pleomorphic
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22
Q

What is the most common soft tissue sarcoma in children under 15 y.o.?

A

Rhabdomyosarcoma

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23
Q

What is the most common intraoral site for Rhabdomyosarcoma?

A

Palate

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24
Q

What is the proper term and appearance when a Rhabdomyosarcoma grows within a body cavity?

A

Sarcoma botryoides

Looks like a bunch of grapes

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25
Q

What are the histo features of rhabdomyosarcoma?

A

Small round cells with hyperchromatic nuclei

Strap-shaped rhabdomyoblasts

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26
Q

What is treatment/prognosis for Rhabdomyosarcoma?

A

Wide excision, chemo, radiation

Good; 90% 5 year survival

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27
Q

Which lymphoreticular malignancy involves a spectrum of disorders characterized by proliferation of histiocyte-like cells with varying numbers of eosinophils, plasma cells, lymphocytes, and giant cells?

A

Langerhans cell histiocytosis

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28
Q

What is a langerhans cell?

A

Tissue-resistant macrophages that serve as antigen-presenting cells

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29
Q

T/F: Langerhans cell histiocytosis is a neoplastic process.

A

True

30
Q

Which of the three types of LCH is often seen as a skin rash in infants and has an aggressive course?

A

Acute disseminated histiocytosis

31
Q

____________ is not as aggressive as acute disseminated histiocytosis and is often seen in older children.

A

Chronic disseminated histiocytosis

32
Q

What is the classic triad of issues involved with chronic disseminated histiocytosis?

A

Bones, skin, viscera

33
Q

What characterizes eosinophilic granuloma type of LCH?

A

Seen in teenagers and adults

No visceral involvement, only bone

34
Q

What are the most frequent bones affected with LCH?

A

Skull, mandible, ribs

35
Q

What is the radiographic appearance of LCH in the jaws?

A

Scooped out appearance can mimic periodontitis and make teeth look like they are floating

36
Q

How can LCH be identified histologically?

A

Birbeck bodies seen in cytoplasm of langerhans cells

37
Q

Which type of LCH can be treated with curettage?

A

Eosinophilic granuloma

38
Q

Which type of LCH has the worst prognosis?

A

Acute

39
Q

__________ describes a group of malignancies characterized by tumor cells circulating in the blood.

A

Leukemia

40
Q

Where does leukemia start?

A

In the bone marrow

Hematopoietic stem cells turns malignant

41
Q

What is the order of prevalence for the types of leukemia in the U.S.?

A
  1. Acute myeloid leukemia
  2. Chronic lymphocytic leukemia
  3. Chronic myeloid leukemia
  4. acute lymphocytic leukemia
42
Q

What are the symptoms of myelophthisic anemia?

A

Fatigue, SOB
Easy bruising
Infection

All associated with decreased normal blood cells

43
Q

Which types of leukemia are most likely to produce oral involvement?

A

AML and CML

44
Q

What is a granulocytic sarcoma?

A

Focal proliferation of leukemic cells at one soft tissue site

45
Q

T/F: Gingival enlargement can be a feature of CML and AML.

A

True

46
Q

What are the treatment options for leukemia?

A

Chemotherapy, bone marrow transplant, targeted gene therapy

47
Q

What is the prognosis for ALL?

A

Children 90% cured

Adults 80% remission

48
Q

What is the prognosis for AML?

A

<60y.o. 40% 5 year survival

> 60 y.o. Less than 10%

49
Q

Which type of leukemia does not have a cure?

A

CLL

2-10 year survival

50
Q

What is the prognosis for CML?

A

5 year survival 80%

If blast transformation death in 3-6 months

51
Q

Which type of lymphoma can be seen in soft tissue or bone?

A

Non-Hodgkin

52
Q

T/F: Hodgkin lymphoma has a male predilection.

A

True

53
Q

What is a bimodal age distribution?

A

Hodgkin lymphoma will affect teenagers and young adults and will also affect those over 50 y.o.

54
Q

Where is Hodgkin lymphoma often seen?

A

Cervical and supraclavicular nodes

55
Q

T/F: Category A Hodgkin lymphoma has systemic signs.

A

False

Category B Hodgkin

Fever, weight loss, night sweats, pruritus

56
Q

What cells are seen in a histo section of Hodgkin lymphoma?

A

Reed-Sternberg cells

57
Q

T/F: Hodgkin lymphoma has a good prognosis.

A

True

58
Q

T/F: Non-Hodgkin lymphoma is more common than Hodgkin.

A

True

Seen in older population

59
Q

What are some features of oral non-Hodgkin lymphoma?

A

Often primary site, soft palate or buccal mucosa

May be mistaken for periapical/perio disease

60
Q

Roughly how many patients diagnosed with non-Hodgkin lymphoma die each year?

A

1/3

61
Q

What is the origin for multiple myeloma?

A

Plasma cells

Start proliferating an antibody that is not normal or functional

62
Q

What results in Bence Jones proteins found in urine of 30-50% of multiple myeloma cases?

A

Manny unattached light chain antibodies filtered through the kidney

63
Q

T/F: Smoldering is an asymptomatic finding in blood that can be very early stages of multiple myeloma.

A

True

64
Q

Who is at the most risk for multiple myeloma?

A

Black males around 65 years old

65
Q

What is the most common hematologist malignancy in black persons?

A

Multiple myeloma

66
Q

What is the most characteristic symptom of multiple myeloma?

A

Bone pain, esp lumbar spine

67
Q

T/F: Patients with multiple myeloma will have pathological fractures and renal failure.

A

True

68
Q

T/F: Calcifications in soft tissue are common with multiple myeloma.

A

True

69
Q

Accumulation of light chains can lead to deposition of __________ in various soft tissues in patients with multiple myeloma.

A

Amyloid

70
Q

What are the sites most often effected with deposition of amyloid?

A

Periorbital skin - waxy, firm lesions

Oral mucosa (tongue) - enlargement, firmness

71
Q

What is the radiographic presentation of multiple myeloma?

A

Widespread “punched out” lesions in the bone

72
Q

What is the survival rate for a patient with multiple myeloma?

A

6-7 years