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Soft Tissue Tumors (ALL IN ONE) Flashcards

(106 cards)

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1
Q

What is the most common soft tissue tumor in adults?

A

Lipoma

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2
Q

Where are most lipomas located?

A

In superficial subcutaneous tissue

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3
Q

Can lipomas be single or multiple?

A

Yes, they can be single or multiple and may be familial

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4
Q

Do lipomas have a risk of malignant transformation?

A

No

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5
Q

What is the gross appearance of a lipoma?

A

Circumscribed yellow mass

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6
Q

What is the histological composition of a lipoma?

A

Mature fatty (adipose) tissue

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7
Q

Are there many histological variants of lipomas?

A

Yes

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8
Q

At what age do liposarcomas commonly occur?

A

50-60 years

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9
Q

Where are liposarcomas typically located?

A

Deep soft tissue and retroperitoneum

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10
Q

What is the gross appearance of a liposarcoma?

A

Large yellow glistening mass

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11
Q

What characterizes low-grade liposarcoma histologically?

A

Well-differentiated with 12q amplification and MDM2 gene involvement

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12
Q

What is the role of MDM2 in liposarcoma development?

A

MDM2 inhibits TP53, leading to sarcoma formation

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13
Q

What genetic abnormality is seen in myxoid type liposarcoma?

A

t(12;16)

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14
Q

What is the histological feature of myxoid liposarcoma?

A

Myxoid stroma with a vascular network showing a “chicken-wire” appearance

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15
Q

What defines a round cell liposarcoma?

A

Same t(12;16) genetics as myxoid type but more aggressive

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16
Q

What is the feature of pleomorphic liposarcoma?

A

Hard to distinguish from other pleomorphic sarcomas

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17
Q

What is essential for the diagnosis of liposarcoma?

A

Identification of lipoblasts

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18
Q

What factors determine the prognosis of liposarcoma?

A

Type and site (deep vs superficial)

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19
Q

What is Nodular Fasciitis?

A

Reactive or neoplastic fibroblastic and myofibroblastic proliferation

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20
Q

What tumor is Nodular Fasciitis often misdiagnosed as?

A

Sarcoma

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21
Q

Which age group is mostly affected by Nodular Fasciitis?

A

Young adults

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22
Q

How does Nodular Fasciitis usually present?

A

Rapidly enlarging painful mass

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23
Q

Where is Nodular Fasciitis commonly located?

A

Upper extremity and trunk

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24
Q

What percentage of Nodular Fasciitis cases have a history of local trauma?

A

25-50%

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25
What is the behavior of reactive Nodular Fasciitis?
Self-limiting
26
What genetic abnormality is associated with neoplastic Nodular Fasciitis?
t(17;22) MYH9-USP6 fusion gene
27
What lesion is similar to Nodular Fasciitis but occurs in muscle? (DDx)
Myositis Ossificans
28
What is the gross appearance of Nodular Fasciitis?
Unencapsulated, circumscribed, slightly infiltrative mass less than 3 cm
29
What are the microscopic features of Nodular Fasciitis?
Immature fibroblasts, numerous mitoses without atypia, myxoid background, zonation
30
What is Fibromatosis?
A group of fibroblastic proliferations
31
How does Fibromatosis grow?
Infiltratively
32
What is the behavior of Fibromatosis after surgical excision?
Tends to recur but does not metastasize
33
Does Fibromatosis affect specific age groups?
It affects various age groups
34
What are the two types of Fibromatosis?
Superficial and Deep (desmoid tumors)
35
What are examples of Superficial Fibromatosis?
Palmar (Dupuytren’s contracture), Plantar (Ledderhose’s disease), Penile (Peyronie’s disease)
36
Where are Deep (desmoid) fibromatoses typically located?
Abdominal wall, trunk, extremities, and within the abdomen
37
How aggressive are Deep Fibromatoses?
Locally aggressive with low risk of metastasis
38
Are Deep Fibromatoses usually single or multiple?
They can be single or multiple
39
What genetic mutation is associated with Desmoid / Deep Fibromatosis?
Gardner syndrome: APC/B-Catenin (CTNNB1) gene mutation
40
Who does Fibrosarcoma mostly affect?
Adults
41
Where does Fibrosarcoma commonly occur?
Deep tissues of thighs, knees, and retroperitoneum
42
How do Fibrosarcomas tend to grow?
Slowly
43
What is the gross appearance of Fibrosarcoma?
Solitary, infiltrative, or well-circumscribed mass
44
What is the microscopic pattern of Fibrosarcoma?
Fascicles of fibroblasts arranged in a herringbone pattern
45
What determines recurrence and metastatic rates in Fibrosarcoma?
The tumor grade (Grade I-III)
46
What types of Fibrohistiocytic tumors exist?
Benign, borderline, and malignant types
47
Where are benign Fibrohistiocytic tumors typically located?
Superficial tissues
48
Where are malignant Fibrohistiocytic tumors typically located?
Deep tissues, larger, highly pleomorphic
49
In which age group do most Fibrohistiocytic tumors occur?
Adults
50
What is the treatment for Fibrohistiocytic tumors?
Excision
51
What is another name for Benign Fibrous Histiocytoma if dermal?
Dermatofibroma
52
What is the size and location of a Benign Fibrous Histiocytoma?
Circumscribed tumor in dermis or subcutaneous tissue less than 1 cm
53
What is the histology of Benign Fibrous Histiocytoma?
Spindle cells and histiocytes
54
What are the immunohistochemical markers for Benign Fibrous Histiocytoma?
Positive Factor 13A, Negative CD34
55
How does Dermatofibrosarcoma Protuberans differ from Benign Fibrous Histiocytoma?
Larger, deeper, mitoses present, CD34 positive
56
What is the characteristic appearance of Dermatofibrosarcoma Protuberans?
Honeycomb appearance
57
What is the recurrence behavior of Dermatofibrosarcoma Protuberans?
Recurs after excision
58
What is Undifferentiated Pleomorphic Sarcoma (UPS)?
A high-grade tumor with pleomorphic cells not classifiable by H/E, IHC, EM, or genetics
59
What was the old name for Undifferentiated Pleomorphic Sarcoma?
Malignant Fibrous Histiocytoma (MFH)
60
Where does UPS usually occur?
Deep soft tissues of extremities, especially the thigh
61
Which age group is commonly affected by UPS?
Middle-aged to older adults
62
What is the ploidy status of most UPS tumors?
Aneuploid
63
What is the gross appearance of UPS?
Large, fleshy mass with necrosis and hemorrhage
64
What are the microscopic features of UPS?
Bizarre spindle or polygonal cells with mitoses
65
What is the treatment for UPS?
Surgery plus chemotherapy with or without radiotherapy
66
What is the benign tumor of smooth muscle called?
Leiomyoma
67
Where are Leiomyomas mainly found?
Uterus
68
What are other sites where Leiomyomas can occur?
Vascular smooth muscle and arrector pili muscle in skin
69
What is the malignant tumor of smooth muscle called?
Leiomyosarcoma
70
What percentage of soft tissue sarcomas are Leiomyosarcomas?
10-20%
71
Where do Leiomyosarcomas commonly occur?
Uterus or soft tissue
72
What prognosis is associated with superficial skin Leiomyosarcomas?
Better prognosis
73
Where are large, deep Leiomyosarcomas commonly found?
Extremities or retroperitoneum
74
What type of tumors are most skeletal muscle tumors?
Malignant
75
What is the most common soft tissue sarcoma of children, adolescents, and young adults?
Rhabdomyosarcoma
76
What genetic mutation is associated with alveolar Rhabdomyosarcoma?
t(2;13) PAX3-FKHR (FOXO1) fusion gene
77
What does the PAX3-FKHR fusion gene interfere with?
Muscle differentiation
78
Where are Rhabdomyosarcomas commonly located?
Soft tissue, head and neck, genitourinary tract
79
How aggressive is Rhabdomyosarcoma?
Highly aggressive
80
What is the most common type of Rhabdomyosarcoma?
Embryonal type (60%)
81
Where is Embryonal Rhabdomyosarcoma commonly found?
Head and neck, genitourinary tract, and retroperitoneum
82
Where is Alveolar Rhabdomyosarcoma commonly found?
Extremities of adolescents
83
Where is Pleomorphic Rhabdomyosarcoma commonly found?
Deep soft tissues of adults
84
What immunohistochemical stains are positive in Rhabdomyosarcoma?
Desmin, Myogenin, and MYOD-1
85
What type of staining does Desmin show?
Cytoplasmic staining
86
What type of staining do Myogenin and MYOD-1 show?
Nuclear staining indicating skeletal muscle differentiation
87
What is the diagnostic cell of Rhabdomyosarcoma?
Rhabdomyoblast
88
What is the appearance of a Rhabdomyoblast?
Tadpole-shaped or Strap-shaped cell
89
In which order does prognosis worsen in Rhabdomyosarcoma types?
Embryonal → Pleomorphic → Alveolar
90
What is the typical appearance of cells in Embryonal Rhabdomyosarcoma?
Round to spindle-shaped
91
What is the typical appearance of cells in Alveolar Rhabdomyosarcoma?
Round cells
92
What is the cell of origin for Synovial Sarcoma?
Unknown
93
What age group is most affected by Synovial Sarcoma?
20-50 years
94
What percentage of soft tissue sarcomas are Synovial Sarcomas?
0.1
95
Where are most Synovial Sarcomas located?
Deep soft tissue adjacent to joints
96
What is the most common joint area involved in Synovial Sarcoma?
Around the knee
97
How often is Synovial Sarcoma intra-articular?
Less than 10%
98
What specific genetic translocation is associated with Synovial Sarcoma?
t(X;18)
99
What fusion gene is formed by t(X;18) in Synovial Sarcoma?
SS18-SSX fusion gene
100
What is the histologic appearance of Synovial Sarcoma?
Biphasic or monophasic tumor of epithelial and stromal elements
101
What immunohistochemical markers are positive in Synovial Sarcoma?
Cytokeratin (CK) and Epithelial Membrane Antigen (EMA)
102
How is Synovial Sarcoma typically treated?
Limb-sparing surgery and chemotherapy
103
Where does Synovial Sarcoma commonly metastasize?
Lung, bone, and regional lymph nodes
104
How do most sarcomas metastasize?
Through the blood
105
What is the 5-year survival rate of Synovial Sarcoma?
25-62% depending on stage and age
106
What percentage of Synovial Sarcoma patients live more than 10 years?
10-30%

MSS Pathology (13 decks)

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