Malgnant Mesenchymal (3) Flashcards

(72 cards)

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
What are the histo features of rhabdomyosarcoma?
Small round cells with hyperchromatic nuclei Strap-shaped rhabdomyoblasts
26
What is treatment/prognosis for Rhabdomyosarcoma?
Wide excision, chemo, radiation Good; 90% 5 year survival
27
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?
Langerhans cell histiocytosis
28
What is a langerhans cell?
Tissue-resistant macrophages that serve as antigen-presenting cells
29
T/F: Langerhans cell histiocytosis is a neoplastic process.
True
30
Which of the three types of LCH is often seen as a skin rash in infants and has an aggressive course?
Acute disseminated histiocytosis
31
____________ is not as aggressive as acute disseminated histiocytosis and is often seen in older children.
Chronic disseminated histiocytosis
32
What is the classic triad of issues involved with chronic disseminated histiocytosis?
Bones, skin, viscera
33
What characterizes eosinophilic granuloma type of LCH?
Seen in teenagers and adults No visceral involvement, only bone
34
What are the most frequent bones affected with LCH?
Skull, mandible, ribs
35
What is the radiographic appearance of LCH in the jaws?
Scooped out appearance can mimic periodontitis and make teeth look like they are floating
36
How can LCH be identified histologically?
Birbeck bodies seen in cytoplasm of langerhans cells
37
Which type of LCH can be treated with curettage?
Eosinophilic granuloma
38
Which type of LCH has the worst prognosis?
Acute
39
__________ describes a group of malignancies characterized by tumor cells circulating in the blood.
Leukemia
40
Where does leukemia start?
In the bone marrow Hematopoietic stem cells turns malignant
41
What is the order of prevalence for the types of leukemia in the U.S.?
1. Acute myeloid leukemia 2. Chronic lymphocytic leukemia 3. Chronic myeloid leukemia 4. acute lymphocytic leukemia
42
What are the symptoms of myelophthisic anemia?
Fatigue, SOB Easy bruising Infection All associated with decreased normal blood cells
43
Which types of leukemia are most likely to produce oral involvement?
AML and CML
44
What is a granulocytic sarcoma?
Focal proliferation of leukemic cells at one soft tissue site
45
T/F: Gingival enlargement can be a feature of CML and AML.
True
46
What are the treatment options for leukemia?
Chemotherapy, bone marrow transplant, targeted gene therapy
47
What is the prognosis for ALL?
Children 90% cured Adults 80% remission
48
What is the prognosis for AML?
<60y.o. 40% 5 year survival >60 y.o. Less than 10%
49
Which type of leukemia does not have a cure?
CLL 2-10 year survival
50
What is the prognosis for CML?
5 year survival 80% If blast transformation death in 3-6 months
51
Which type of lymphoma can be seen in soft tissue or bone?
Non-Hodgkin
52
T/F: Hodgkin lymphoma has a male predilection.
True
53
What is a bimodal age distribution?
Hodgkin lymphoma will affect teenagers and young adults and will also affect those over 50 y.o.
54
Where is Hodgkin lymphoma often seen?
Cervical and supraclavicular nodes
55
T/F: Category A Hodgkin lymphoma has systemic signs.
False Category B Hodgkin Fever, weight loss, night sweats, pruritus
56
What cells are seen in a histo section of Hodgkin lymphoma?
Reed-Sternberg cells
57
T/F: Hodgkin lymphoma has a good prognosis.
True
58
T/F: Non-Hodgkin lymphoma is more common than Hodgkin.
True Seen in older population
59
What are some features of oral non-Hodgkin lymphoma?
Often primary site, soft palate or buccal mucosa May be mistaken for periapical/perio disease
60
Roughly how many patients diagnosed with non-Hodgkin lymphoma die each year?
1/3
61
What is the origin for multiple myeloma?
Plasma cells Start proliferating an antibody that is not normal or functional
62
What results in Bence Jones proteins found in urine of 30-50% of multiple myeloma cases?
Manny unattached light chain antibodies filtered through the kidney
63
T/F: Smoldering is an asymptomatic finding in blood that can be very early stages of multiple myeloma.
True
64
Who is at the most risk for multiple myeloma?
Black males around 65 years old
65
What is the most common hematologist malignancy in black persons?
Multiple myeloma
66
What is the most characteristic symptom of multiple myeloma?
Bone pain, esp lumbar spine
67
T/F: Patients with multiple myeloma will have pathological fractures and renal failure.
True
68
T/F: Calcifications in soft tissue are common with multiple myeloma.
True
69
Accumulation of light chains can lead to deposition of __________ in various soft tissues in patients with multiple myeloma.
Amyloid
70
What are the sites most often effected with deposition of amyloid?
Periorbital skin - waxy, firm lesions Oral mucosa (tongue) - enlargement, firmness
71
What is the radiographic presentation of multiple myeloma?
Widespread “punched out” lesions in the bone
72
What is the survival rate for a patient with multiple myeloma?
6-7 years