Malignant Mesenchymal Tumors Flashcards
1
Q
Malignant mesenchymal tumor of fibrous origin
A
Fibrosarcoma
2
Q
Malignant mesenchymal tumor of nerve origin
A
Malignant peripheral nerve sheath tumor (MPNST)
3
Q
Malignant mesenchymal tumor of endothelial cell origin
A
Kaposi cell
4
Q
Malignant mesenchymal tumor of muscle origin
A
Rhabdomyosarcoma
5
Q
Lymphoreticular tumors (4)
A
Langerhans Cell Histiocytosis
Leukemia
Lymphoma
Multiple Myeloma/Plasmocytoma
6
Q
Malignancy of fibroblastic differentiation
A
Fibrosarcoma
7
Q
Fibrosarcoma can be seen as a ___ ___ mass or as an ____ lesion
A
Soft tissue
Intrabony
8
Q
When do patients report pain with a fibrosarcoma?
A
Late
9
Q
Describe the histopathology of fibrosarcoma
A
Fascicles of spindle-shaped cells often forming a “herringbone” pattern. Variable number of mitoses
10
Q
Treatment for fibrosarcoma
A
Wide to radical surgical excision
11
Q
Does fibroscarcoma respond to radiation and chemotherapy?
A
No
12
Q
Describe the prognosis of fibrosarcoma
A
Only about 1/2 live past 5 years
13
Q
Routes of metastasis for fibrosarcoma via the blood
A
Lung, liver, bone
14
Q
Also known as neurogenic sarcoma or neurofibrosarcoma
A
Malignant Peripheral Nerve Sheath Tumor (MPNST)
15
Q
What are half of MPNSTs associated with?
A
NF1
16
Q
Is the mean age of an MPNST patient higher or lower if the patient has NF1
A
Younger if the patient has NF1 (29 vs 46)
17
Q
What are radiographic features associated with MPNST?
A
Widening of the canal and mental foramen, scooping of cortex. Obliteration of mandibular canal
18
Q
What shape are the cells and nuclei of MPNST? Is there mitosis?
A
Spindle-shaped cells with wavy nuclei
Yes
19
Q
What is the treatment for MPNST?
A
Surgical resection, radical excision, amputation +/- radiation
20
Q
Is the prognosis better or worse for an MPNST patient with NF1
A
Prognosis is worse with NF1
21
Q
First described as a disease affecting elderly white males of Mediterranean descent but was also seen in sub-Saharan Africans, transplant recipients, and HIV+ males
A
Kaposi Sarcoma
22
Q
Kaposi Sarcoma is caused by an infection with ___-__
A
HHV-8
23
Q
4 clinical presentations of Kaposi sarcoma
A
- Classic
- Endemic (African)
- Iatrogenic (Transplant-associated)
- Epidemic (AIDS-related)
24
Q
Where does the classic form of Kaposi sarcoma usually appear? In what types of patients?
A
On the lower extremities of elderly patients, most often male
25
Kaposi Sarcoma is _____ (painful/painless), with a ____ color
Painless
| Purple-red
26
Describe the transformative stages for Kaposi Sarcoma
Macule --> Plaque --> Tumor
27
Clinical feature suggesting Kaposi Sarcoma
Does not blanch on pressure
28
What age range is mainly affected by Endemic Kaposi Sarcoma?
Wide age range - esp. young adults and children
29
How can Endemic Kaposi Sarcoma present?
Anywhere from indolent skin lesions to aggressive tumors
30
Young children with Endemic Kaposi Sarcoma will often have tumors within their ___ ___
Lymph nodes (lymphadenopathic)
31
What type of transplants are mainly associated with Iatrogenic Kaposi Sarcoma?
Solid organ transplants (0.5% renal)
32
When will a patient develop Iatrogenic Kaposi Sarcoma?
Months to years after their transplant
33
What areas do Iatrogenic Kaposi Sarcomas affect?
Skin and oral mucosa
34
What may cause regression of Iatrogenic Kaposi Sarcoma?
Reducing degree of immune-suppression
35
Epidemic Kaposi Sarcoma mainly affects:
HIV-infected adult male homosexuals
36
Epidemic Kaposi Sarcoma skin lesions have a predilection for what areas?
Face and lower extremities
37
The oral cavity is the initial site of presentation for this lesion in ~22% of patients
Epidemic (AIDS-related) Kaposi Sarcoma
38
True/False: Oral involvement is rare in Epidemic (AIDs-related) Kaposi Sarcoma?
False - 70% will develop oral lesions
39
What areas in the oral cavity are affected by Epidemic Kaposi Sarcoma?
Hard palate, gingiva, tongue
40
How can Epidemic Kaposi Sarcoma cause tooth movement?
If it invades the bone
41
Histology of Kaposi Sarcoma: Cellular ____ cell tumor within _____ tissue and _____ blood
Spindle
Connective
Extravasated
42
Kaposi Sarcoma has many poorly defined ___ ___
Vascular slits
43
Kaposi Sarcoma has ____ positivity in endothelial cells
HHV-8
44
Treatment for Kaposi Sarcoma for small lesions? For larger lesions?
Excision for small cosmetically problematic lesions
Radiation
Injections of chemotherapeutic agents
45
Prognosis for Classic Kaposi Sarcoma
Fair - patients usually die of something else (MI, CVA, etc.)
46
Prognosis for Endemic Lymphadenopathic Kaposi Sarcoma
Poor
47
Prognosis for Iatrogenic Kaposi Sarcoma
Fair to poor
48
Prognosis for AIDs related Kaposi Sarcoma
Fair
49
3 types of Rhabdomyosarcoma
1. Embryonal
2. Alveolar
3. Pleomorphic
50
How common is Rhabdomyosarcoma? Who does is affect?
Rare
| Children or adolescents
51
Most common soft tissue sarcoma in children under 15 yo
Rhabdomyosarcoma
52
Most common intraoral site for Rhabdomyosarcoma
Palate
53
Radiographic feature of Rhabdomyosarcoma
Expansion
54
Term to describe Rhabdomyosarcoma that grows in a body cavity
Sarcoma Botryoides
55
What does Sarcoma Botryoides look like?
A bunch of grapes
56
What kind of cells does Rhabdomyosarcoma have? What type of nuclei?
Small cells with hyperchromatic nuclei
57
What might you see in the histology of Rhabdomyosarcoma
Strap-shaped rhabdomyoblasts with cross striations
58
Treatment for Rhabdomyosarcoma
Wide excision, multiagent chemotherapy, post-op radiation
59
What does prognosis for Rhabdomyosarcoma depend on?
Type, location, stage, age
60
Spectrum of disorders characterized by proliferation of histiocyte-like cells accompanied by varying numbers of eosinophils, lymphocytes, plasma cells and multinucleated giant cells
Langerhans Cell Histiocytosis
61
Langerhans Cell Histiocytosis was previously known as:
Histiocytosis X
62
What are Langerhans Cells?
Tissue-resident Macrophages (related to monocytes) that serve as antigen-presenting cells
63
Clonal proliferation confirms that Langerhans Cell Histiocytosis is a ____ process
Neoplastic
64
What is significant about the age range for Langerhans Cell Histiocytosis?
More than half of cases are in patients younger than 15
65
3 Clinical presentations of Langerhans Cell Histiocytosis
1. Acute Disseminated
2. Chronic Disseminated
3. Eosinophilic Granuloma (monostotic or polyostotic)
66
Acute Disseminated Histiocytosis is also called
Letterer-Siwe Disease
67
Who is affected by Acute Disseminated Histiocytosis?
Infants
68
What can occur due to Acute Disseminated Histiocytosis?
Skin rash, splenic, hepatic, and marrow involvement.
69
Chronic Disseminated Histiocytosis is also called
Hand-Schuller-Christian Disease
70
Who is affected by Chronic Disseminated Histiocytosis?
Older children
71
Which is more aggressive: Acute or Chronic Disseminated Histiocytosis?
Acute
72
What is the classic triad for Chronic Disseminated Histiocytosis?
1. Exopthalmos
2. Diabetes insipidus (kidneys unable to prevent water excretion)
3. Bone lesions
73
What is significant about Eosinophilic granulomas in Langerhans Cell Histiocytosis
They are intrabony only - no visceral involvement
74
What patients are affected by eosinophilic granulomas?
Teenagers and young adults
75
Which patients have polyostotic eosinophilic granulomas? Which have monostotic?
Poly - Teenagers
| Mono - Adults
76
What are the most frequent bones affected by Langerhans Cell Histiocytosis
Skull, Mandible, Ribs, Vertebrae
77
Severe bone loss from Langerhans Cell Histiocytosis can resembly:
Periodontal disease
78
Superficial bone has a ____ ____ radiographic appearance with Langerhans Cell Histiocytosis. Teeth appear to be _____ due to extensive _____ involvement.
Scooped out
Floating
Alveolar
79
____ _____ in the cytoplasm of Langerhans cells can be seen by an electron microscope
Birbeck bodies
80
Treatment for Acute Langerhans Cell Histiocytosis
Chemotherapy
81
Treatment for Chronic Langerhans Cell Histiocytosis
Radiation and/or chemotherapy
82
Treatment for Eosinophilic Granuloma
Curettage or radiation
83
Prognosis for Acute Langerhans Cell Histiocytosis
Poor
84
Prognosis for Chronic Langerhans Cell Histiocytosis
Guarded
85
Prognosis for Eosinophilic Granuloma
Good
86
Group of hematologic malignancies characterized by tumor cells circulating in the blood
Leukemia
87
Where does leukemia begin?
In the bone marrow
88
In leukemia, what type of cells go through malignant transformation and proliferation - eventually overflowing into the peripheral blood
Hematopoietic stem cells
89
4 broadly divided types of leukemia
1. Lymphocytic
2. Myelomonocytic
3. Acute
4. Chronic
90
2 groups of lymphocytic leukemia
1. Acute Lymphocytic Leukemia (ALL)
| 2. Chronic Lymphocytic Leukemia (CLL)
91
2 groups of myelomonocytic leukemia
1. Acute Myeloid Leukemia (AML)
| 2. Chronic Myeloid Leukemia (CML)
92
Patients with leukemia often present with ____ _____
Myelophthisic anemia
93
Myelophthisic anemia
Normal bone marrow cells replaced by leukemic cells
94
Symptoms of myelophthisic anemia and their causes
1. Fatigue, shortness of breath, pallor (decreased RBCs)
2. Easy bruising (decreased platelets)
3. Infection (decreased WBCs)
95
Oral involvement is most often in ______ forms of leukemia
Myelomonocytic
96
Focal proliferation of leukemic cells at one soft tissue site
Granulocytic sarcoma
97
Oral clinical feature of leukemia
Diffuse gingival enlargement
98
How do you diagnose leukemia?
1. Finding increased atypical WBCs
| 2. Type determined by immunohistochemical/cytogenetic studies
99
General histology of leukemia
Diffuse infiltration and destruction of normal tissue by sheets of poorly differentiated cells with either myelomonocytic or lymphoid characteristics
100
3 treatments for leukemia
Chemotherapy, bone marrow/stem cell transplant, targeted gene therapy
101
Is the prognosis for ALL better for adults or children?
Children
102
Is the prognosis for AML better or worse if the patient is under 60 yo
Better if the patient is under 60
103
What is the prognosis for CLL
Incurable
104
What is the prognosis for CML
Indolent period has a better survival. Quick death after blast transformation
105
Where does Hodgkin Lymphoma develop?
Lymph nodes
106
Where does Non-Hodgkin Lymphoma arise from/
Mostly lymph nodes, but also soft tissue or bone
107
Hodgkin Lymphoma has a _____ predilection
Male
108
_______ is a common site of initial involvement for Hodgkin Lymphoma; however, _____ involvement is rare
H&N
| Oral
109
Hodgkin Lymphoma has a ______ age distribution
Bimodal
110
Clinical feature of Hodgkin Lymphoma
One or more non-tender, palpable, rubbery-firm, enlarging lymph nodes
111
Most involved lymph nodes in Hodgkin Lymphoma
Supraclavicular and Cervical
112
Describe Category A and B for Hodgkin Lymphoma
A - no systemic signs
| B - "B signs" (fever, weight loss, night sweats, generalized itching (pruritus))
113
What type of cells are seen in Hodgkin Lymphoma
Reed-Sternberg cells
114
What do Reed-Sternberg cells look like?
Owl-eyes or Pennies on a plate
115
What is a disadvantage of treatment for Hodgkin Lymphoma
Treatment can result in post-treatment complications
116
Is the prognosis better for Hodgkin Lymphoma or Non-Hodgkin Lymphoma
Hodgkin Lymphoma
117
Is the prognosis better or worse for patients who show "B signs"
Worse
118
Most post-treatment mortality with Hodgkin Lymphoma is due to:
Secondary malignancy or cardiovascular disease
119
Are patients with Non-Hodgkin Lymphoma typically older or younger than patients with Hodgkin Lymphoma?
Older
120
Which is more common: Hodgkin or Non-Hodgkin Lymphoma
Non-Hodgkin Lymphoma
121
Prevalence of Non-Hodgkin Lymphoma is increased in patients who have ____ ____
Immunologic problems (ex: HIV, organ transplant, congenital and autoimmune disease)
122
Where do most Non-Hodgkin Lymphomas arise?
Lymph nodes but some can be extranodal
123
What is often the primary site for extranodal Non-Hodgkin Lymphoma
Oral cavity
124
Where is Non-Hodgkin Lymphoma often found within the oral cavity?
Soft palate or buccal mucosa
125
Non-Hodgkin Lymphoma lesions of the PDL may be mistaken for:
Periapical or periodontal disease
126
What sign may a patient with Non-Hodgkin Lymphoma express if there is mandibular canal involvement?
Numb chin (paresthesia)
127
Non-Hodgkin Lymphoma has a ____ consistency
Boggy
128
What may an edentulous patient with Non-Hodgkin Lymphoma complain of?
"Denture is too tight"
129
What might a vague oral pain in a patient with Non-Hodgkin Lymphoma be mistaken for?
Toothache
130
Radiographic feature of Non-Hodgkin Lymphoma
"Moth-eaten" or ill-defined radiolucency and expansion
131
What do you need to ID Non-Hodgkin Lymphoma
Immunohistochemistry
132
What is the treatment if a patient has localized Non-Hodgkin Lymphoma
Radiation and chemo
133
What is the treatment if a patient has generalized Non-Hodgkin Lymphoma
Chemo
134
What do plasma cells produce?
Protein immunoglobulins (Ig) (also known as antibodies)
135
What does clonal proliferation of one specific immunoglobulin type indicate?
Disease
136
Multiple Myeloma/Plasmacytoma has ___ ___ origin
Plasma cell
137
What are immunoglobulins made of?
2 heavy chains and 2 light chains (2 light = kappa and lambda)
138
What is wrong with the heavy and light chains in multiple myeloma?
The light chain (usually kappa) is not attached to the heavy chain
139
What happens to circulating unattached light chains?
They are filtered in the kidney and secreted in the urine (Bence Jones proteins)
140
What is smoldering multiple myeloma? How is it diagnosed?
Asymptomatic - often by a chance finding on a blood test
141
What type of patients often get MM?
Older patients (rare < 40)
142
MM has a ____ predilection and affects ___ ___ 2x more than ___ ___
Male
Black men
White men
143
Most common hematologic malignancy in black persons in the US
MM
144
Most characteristic symptom of MM
Bone pain, especially lumbar spine
145
Why would patients with MM experience pathologic fractures and renal failure?
Fractures due to tumor destruction of bone. Renal failure due to circulating light chain proteins
146
What are metastatic calcifications (soft tissue) caused by in MM
Hypercalcemia secondary to tumor-related osteolysis
147
Why would a patient with MM have petechial hemorrhages of skin and oral mucosa?
If platelet production is affected
148
What would cause fever in MM?
Neutropenia and increased susceptibility of infection
149
What may be the initial manifestation in patients with MM?
Deposition of amyloid (accumulation of light chains) in soft tissue
150
Sites classically affected by amyloid:
Periorbital skin and oral mucosa (especially tongue)
151
Periorbital skin lesion in MM appears:
Waxy, firm, plaque-like
152
Tongue lesion in MM appears:
Diffuse enlargement, firmness, nodular, sometimes ulcerated
153
Radiographic feature of MM
Punched out non-corticated radiolucencies, especially skull
154
What might MM appear as in a radiograph?
Osteomyelitis
155
What is the purpose of treatment for MM?
To control disease and keep patient comfortable
156
Is the prognosis better or worse for younger patients? What about patients with comorbidities?
Better for younger patients. Worse for comorbidities
