Malignant Mesenchymal Tumors and Lymphoreticular Malignancies - part I Flashcards

1
Q

malignant mesenchymal tumors

A
  1. fibrous - fibrosarcoma
  2. nerve - malignant peropheral nerve sheath tumor (MPNST)
  3. endothelial cell - Kaposi sarcoma
  4. muscle - rhabdomyosarcoma
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2
Q

lymphoreticular malignancies

A
  1. Langerhans cell histiocytosis
  2. leukemia
  3. lymphoma
  4. multiple myeloma/plasmacytoma
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3
Q

fibrosarcoma

A

malignancy of fibroblastic differentiation

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4
Q

who is affected by fibrosarcoma?

A

adults but any age

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5
Q

clinical features of fibrosarcoma

A
  1. soft tissue mass or centrally as an intrabony lesion

2. slow growing

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6
Q

T/F: pain is an early clinical feature of fibrosarcoma

A

false, late feature

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7
Q

histopathologic features of fibrosarcoma

A
  1. fascicles of spindle-shaped cells often forming a “herringbone” pattern
  2. variable number of mitoses
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8
Q

treatment of fibrosarcoma

A

wide to radical surgical excision

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9
Q

T/F: fibrosarcoma responds well to radiation or chemotherapy

A

false, little to no response to radiation or chemotherapy

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10
Q

how does fibrosarcoma metastasize?

A

blood

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11
Q

which organs does fibrosarcoma metastasize to?

A
  1. lung
  2. liver
  3. bone
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12
Q

what is malignant peripheral nerve sheath tumor (MPNST) also known as?

A
  1. neurogenic sarcoma

2. neurofibrosarcoma

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13
Q

what percent of MPNST is associated with NF1?

A

~50%

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14
Q

T/F: MPNST arise spontaneously

A

true

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15
Q

clinical features of MPNST

A
  1. may have pain
  2. may have nerve deficit
  3. may be in soft tissue or centrally as an intrabony lesion
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16
Q

histopathologic features of MPNST

A
  1. spindle-shaped cells with wavy nuclei

2. mitoses

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17
Q

treatment for MPNST

A
  1. surgical resection
  2. radical excision
  3. amputation
  4. ± radiation
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18
Q

how was Kaposi sarcoma (KS) first described by Moritz Kaposi in the late 19th century?

A

described as a disease affecting elderly white males of Mediterranean descent

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19
Q

Kaposi sarcoma was identified in what type of people?

A
  1. sub-Saharan Africans
  2. transplant recipients
  3. HIV+ males
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20
Q

what is KS caused by?

A

infection with HHV-8

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21
Q

what are the 4 clinical presentations of KS?

A
  1. classic
  2. endemic (African)
  3. iatrogenic (transplant-associated)
  4. epidemic (AIDS-related)
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22
Q

who is affected by classic KS?

A

elderly patients, most often male

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23
Q

where does classic KS typically appears?

A

on lower extremities

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24
Q

clinical features of classic KS

A
  1. slow growing
  2. painless
  3. purple-red
  4. macules –> plaques –> tumors
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25
Q

T/F: classic KS blanches on pressure

A

false, does NOT

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26
Q

who is affected by endemic (African) KS?

A

wide age range especially young adults and children

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27
Q

T/F: course of endemic (African) KS can vary widely from indolent skin lesions to aggressive tumors

A

true

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28
Q

who is most often affected by lymphadenopathic endemic (African) KS?

A

young children –> causes visceral tumors

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29
Q

cause of iatrogenic (transplant-associated) KS

A

solid organ transplants

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30
Q

T/F: iatrogenic KS can occur several months to years after transplant

A

true

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31
Q

where does iatrogenic KS affect?

A

skin and oral mucosa

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32
Q

what can cause regression of iatrogenic KS?

A

reducing degree of immune-suppression

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33
Q

who is affected by AIDS-related KS?

A

primarily HIV-infected adult male homosexuals (other types of KS seen in both genders; children in endemic)

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34
Q

AIDS-related KS skin lesions have a predilection for which locations?

A

face and lower extremities

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35
Q

where is the initial site of AIDS-related KS presentation?

A

oral cavity in ~22% of patients

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36
Q

where does AIDS-related KS affect intraorally?

A
  1. hard palate
  2. gingiva
  3. tongue
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37
Q

T/F: AIDS-related KS can invade bone

A

true

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38
Q

what happens when AIDS-related KS invades bone?

A

tooth mobility

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39
Q

histopathologic features of KS

A
  1. cellular spindle cell tumor within the CT, extravasated blood
  2. many poorly defined vascular slits
  3. HHV-8 positivity in endothelial cells and some spindle cells
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40
Q

treatment for KS

A
  1. excision for small cosmetically problematic lesions
  2. radiation
  3. injections of chemotherapeutic agents
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41
Q

prognosis of classic KS

A

fair

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42
Q

T/F: patients with classic KS usually die of something else and not the disease

A

true, MI, CVA, etc.

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43
Q

prognosis of endemic lymphadenopathic KS

A

poor

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44
Q

prognosis of iatrogenic KS

A

fair to poor

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45
Q

prognosis of AIDS-related KS

A

fair

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46
Q

what are the different types of rhabdomyosarcoma?

A
  1. embryonal
  2. alveolar
  3. pleomorphic
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47
Q

T/F: rhabdomyosarcoma is common

A

false, rare

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48
Q

who does rhabdomyosarcoma affect?

A

children or adolescents

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49
Q

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

A

rhabdomyosarcoma

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50
Q

clinical features of rhabdomyosarcoma

A
  1. usually painless
  2. rapidly growing
  3. sarcoma botryoides
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51
Q

what is the most common location for rhabdomyosarcoma?

A

head and neck

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52
Q

what is the most common intraoral site for rhabdomyosarcoma?

A

palate

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53
Q

sarcoma botryoides

A

term used when rhabdomyosarcoma grows in a body cavity e.g. oral cavity, vagina

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54
Q

how does sarcoma botryoides appear clinically?

A

“bunch of grapes”

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55
Q

histopathologic features of rhabdomyosarcoma

A
  1. small round cells, hyperchromatic nuclei

2. may see strap-shaped rhabdomyoblasts with cross striations

56
Q

treatment of rhabdomyosarcoma

A
  1. wide excision
  2. multiagent chemotherapy
  3. post-op radiation
57
Q

prognosis for rhabdomyosarcoma depends on what?

A
  1. type
  2. location
  3. stage
  4. age
58
Q

Langerhans cell histiocytosis (LCH)

A

SPECTRUM of disorders characterized by proliferation of histiocyte-like cells accompanied by varying number of eosinophils, lymphocytes, plasma cells and multinucleated giant cells

59
Q

what was Langerhans cell histiocytosis previously known as?

A

histiocytosis X

60
Q

what are distinctive histiocytic cells?

A

Langerhans cells

61
Q

Langerhans cells

A

tissue-resident macrophages (ralted to monocytes) serve as antigen-presenting cells

62
Q

there was previous controversy on whether LCH was neoplastic or non-neoplastic but what confirms the neoplastic process?

A

clonal proliferation

63
Q

T/F: LCH is rare

A

true

64
Q

who is affected by LCH?

A

wide age range

65
Q

T/F: LCH has a slight female predilection

A

false, NO gender predilection

66
Q

T/F: LCH is painless

A

false, dull pain or tenderness

67
Q

what are the 3 clinical presentations of LCH?

A
  1. acute disseminated histiocytosis
  2. chronic disseminated histiocytosis
  3. eosiniohilic granuloma (monostotic or polyostotic)
68
Q

polyostotic

A

localized to multiple skeletal sites

69
Q

who is affected by acute disseminated histiocytosis (Letterer-Siwe disease)?

A

infants

70
Q

what does acute disseminated histiocytosis involve?

A
  1. skin rash
  2. splenic
  3. hepatic
  4. marrow involvement
71
Q

T/F: acute disseminated histiocytosis are on an aggressive, malignant course

A

true

72
Q

who is affected by chronic disseminated histiocytosis (Hand-Schuller-Christian disease)?

A

older children

73
Q

T/F: chronic disseminated histiocytosis is more ggressive than acute disseminated histiocytosis

A

false, not as aggressive as acute disseminated histiocytosis

74
Q

classic triad (bone, skin, viscera) of chronic disseminated histiocytosis

A
  1. exophthalmos
  2. diabetes insipidus (kidneys unable to prevent excretion of water)
  3. bone lesions
75
Q

T/F: eosinophilic granuloma has both intrabony and visceral involvement

A

false, intrabony ONLY, no visceral involvement

76
Q

who is affected by eosinophilic granuloma?

A

teenagers, young adults

77
Q

who is most affected by polyostotic eosinophilic granuloma?

A

teenagers

78
Q

who is most affected by monostotic eosinophilic granuloma?

A

adults

79
Q

where can LCH be seen radiographically?

A

any bone can be affected but skull, mandibule, ribs, vertebrae most frequent

80
Q

radiographic features of LCH

A
  1. solitary or multiple
  2. may break out of bone
  3. radiolucent
  4. well-defined (usually) but not corticated
  5. occasionally ill-defined
  6. “scooped-out” appearance of superficial bone esp. posterior mandible
81
Q

what can severe bone loss due to LCH resemble?

A

perio disease

82
Q

extensive alveolar involvement can cause what to LCH on radiographs?

A

causes teeth to appear as if they’re “floating in air”

83
Q

histopathologic features of LCH

A
  1. sheets of large, pale histiocytic-appearing cells (neoplastic Langerhans cells)
  2. variable numbers of eosinophils
84
Q

what could be seen in the cytoplasm of Langerhans cells under an electron microscope?

A

Birbeck bodies

85
Q

treatment of LCH depends on what?

A

on form of LCH

86
Q

treatment for acute disseminated histiocytosis

A

chemotherapy

87
Q

treatment for chronic disseminated histiocytosis

A

radiation and/or chemotherapy

88
Q

treatment for eosinophilic granuloma

A

curettage or radiation

89
Q

prognosis of LCH are better for what type of patients?

A

patients who are older at the time of onset

90
Q

prognosis of acute disseminated histiocytosis

A

poor

91
Q

prognosis of chronic disseminated histiocytosis

A

guarded

92
Q

prognosis of eosinophilic granuloma

A

good

93
Q

leukemia

A

groups of hematologic malignancies characterized by tumor cells circulating in the blood

94
Q

where does leukemia begin?

A

in the bone marrow

95
Q

how does leukemia start?

A

with malignant transformation of one of the hematopoietic stem cells, which proliferates and eventually overflows into the peripheral blood

96
Q

types of leukemia

A
  1. lymphocytic
  2. myelomonocytic
  3. acute
  4. chronic
97
Q

lymphocytic leukemia

A

acute lymphocytic leukemia (ALL) < chronic lymphocytic leukemia (CLL)

98
Q

myelomonocytic leukemia

A

acute myeloid leukemia (AML) > chronic myeloid leukemia (CML)

99
Q

T/F: patients with leukemia often present with myelophthisic anemia

A

true

100
Q

myelophthisic anemia

A

normal bone marrow cells replaced by leukemic cells

101
Q

decreased RBC’s in leukemic patients with myelophthisic anemia suffer from what?

A
  1. fatigue, shortness of breath (SOB)

2. pallor

102
Q

decreased platelets in leukemic patients with myelophthisic anemia are more prone to what?

A

easy bruising

103
Q

decreased WBC’s in leukemic patients with myelopthisic anemia are more prone to what?

A

infection

104
Q

oral involvement is most often seen in what form of leukemia?

A

myelomonocytic forms

105
Q

clinical features of leukemia

A
  1. diffuse gingival enlargement

2. “granulocytic sarcoma”

106
Q

granulocytic sarcoma

A

focal proliferation of leukemic cells at one soft tissue site

107
Q

diagnosis of leukemia is based on what?

A

finding of increased numbers of atypical white blood cells in the circulation and marrow

108
Q

how is the type of leukemia determined?

A

immunohistochemical/cytogenetic studies

109
Q

histopathologic features of leukemia

A

diffuse infiltration and destruction of normal host tissue by sheets of poorly differentiated cells with either myelomonocytic characeristics or lymphoid features

110
Q

treatment for leukemia

A
  1. chemotherapy
  2. bone marrow or stem cell transplantation
  3. targeted gene therapy
111
Q

prognosis of leukemia depends on what?

A
  1. type of leukemia
  2. age of patient
  3. cytogenetic alterations
112
Q

T/F: CLL is curable

A

false, incurable but highly variable course of disease, 2-10 years

113
Q

T/F: CML has an indolent period

A

true

114
Q

T/F: CML has a blast transformation that may lead to death in 3-6 months

A

true, less differentiated, proliferate wildly

115
Q

T/F: lymphoma tends to grow as solid masses

A

true

116
Q

what are the two forms of lymphoma?

A
  1. Hodgkin

2. non-Hodgkin

117
Q

where does Hodgkin lymphoma develop?

A

in the lymph nodes

118
Q

where does non-Hodgkin lymphoma arise in?

A

mostly arises in lymph nodes, but can also arise in soft tissue or bone

119
Q

T/F: lymphoma has a female predilection

A

false, MALE

120
Q

what is the most common site of initial involvement for Hodgkin lymphoma?

A

head and neck

121
Q

T/F: oral involvement for Hodgkin lymphoma is rare

A

true

122
Q

what is the age distribution for Hodgkin lymphoma?

A

BIMODAL

  1. teenagers and young adults, 2nd-3rd decades
  2. > 50 years old
123
Q

T/F: any lymph node can be involved in Hodgkin lymphoma

A

true

124
Q

clinical features of Hodgkin lymphoma

A
  1. one or more non-tender, palpable, rubbery-firm, enlarging lymph nodes
125
Q

70-75% of patients with Hodgkin lymphoma will experience what?

A

painless cervical, supraclavicular lymphadenopathy

126
Q

category A of Hodgkin lymphoma

A

no systemic signs

127
Q

category B of Hodgkin lymphoma

A

“B signs”

128
Q

B signs of Hodgkin lymphoma

A
  1. fever
  2. weight loss
  3. drenching night sweats
  4. generalized pruritus (itching)
129
Q

histopathologic features of Hodgkin lymphoma

A
  1. effacement of normal nodal architecture by infiltrate of inflammatory cells
  2. Reed-Sternberg cell
130
Q

Reed-Sternberg cells

A

atypical neoplastic lymphoid cell

131
Q

what are the two forms of Reed-Sternberg cells?

A
  1. “owl-eye”

2. pennies on a plate”

132
Q

treatment of Hodgkin lymphoma depends on what?

A

on stage

133
Q

treatment for Hodgkin lymphoma

A
  1. multiagent chemotherapy

2. radiation for later stages

134
Q

T/F: treatment of Hodgkin lymphoma can result in post-treatment complications

A

true

135
Q

prognosis of Hodgkin lymphoma

A

good

136
Q

prognosis for Hodgkin lymphoma is worse for what types of patients?

A

worse for patients who show “B” signs

137
Q

after 15 years post-tx mortality of Hodgkin lymphoma is due to what?

A

more to secondary malignancy or cardiovascular disease