25. Pathogenesis and frequency of soft tissue tumors. Fibrous tumors and tumor-like lesions. Firbosarcoma. Fibromatoses. Fibrohistiocytic tumors

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25 Terms

1

What is "soft tissue"?

Any non-epithelial tissue other than bone, cartilage, CNS, haematopoietic and lymphoid tissue

In practice, refers to;

- fibrous tissue

- adipose tissue

- muscle tissue

- vessels

- peripheral nervous system

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2

How can we classify the soft tissue tumors?

According to their dignity;

- Benign soft tissue tumors

- Intermediate, locally aggressive soft tissue tumors

- Intermediate, rarely metastasizing soft tissue tumors

- Malignant soft tissue tumors

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3

Malignant sarcomas vs. benign sarcomas?

Benign tumors are 100 times more frequent than malignant ones

- malignant soft tissue tumors account for less than 1% of all malignancies

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4

Grading of soft tissue tumors?

We use a grading system called; FNCLCC

- based on the degree of pleomorphism, necrosis and mitotic activity

- High grade

- Intermediate grade

- Low grade

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5

Pathogenesis of soft tissue tumors?

Most soft tisse tumors develop without any apparent cause, but some may be caused by;

- irradiation

- oncogenic viruses - HHV8 - Kaposi Sarcoma

- genetic syndromes

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6

Genetic syndromes associated with the development of soft tissue tumors?

- Neurofibromatosis 1

- Neurofibromatosis 2

- Gardner syndrome

- Li-Fraumeni syndrome

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7

Fibrous tumor-like lesions?

= Pseudosarcomas;

- Nodular fasciitis

- Proliferative fasciitis

- Myositis ossificans

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8

What is nodular fasciitis?

Bening, self-limited proliferation of fibroblasts

- typically occurs in adults on their palmar side of their forarm

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9

What is myositis ossificans?

Pseudosarcoma characterized by an ossifying mass

- usually develops in muscles, in the extremities of adults after repeated mechanical trauma

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10

Myositis ossificans should be distinguished from?

Extraskeletal osteosarcoma

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11

What are fibromas? examples?

Benign fibrous tumors

- dermatofibroma

- tendon sheath fibroma

- nuchal keloid

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12

What is fibromatoses?

Group of intermediate (between benign and malignant) fibroblastic tumors

- locally aggressive, but never metastasize

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13

What types of fibromatoses can we distinguish?

Superficial types

- Palmar/plantar fibromatosis

- Penile fibromatosis

Deep type

- Desmoid tumors

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14

What is palmar/plantar fibromatosis?

Fibrous growth in the palmar or plantar aponeurosis

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15

What is penile fibromatosis?

Fibrous growth in the penis

- cause abnormal curving and pain

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16

What are desmoid tumors?

Fibrous growths in the extremities, abdominal wall or abdominal cavity

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17

What are fibrosarcomas? where do they most commonly occur?

Malignant tumors composed of fibroblasts

- most commonly occur in the extremities and retroperitoneal area

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18

Subtypes of fibrosarcoma?

- Adult type = fibrosarcoma not otherwise specified

- Infantile type

- Inflammatory myofibroblastic tumors

- Dermatofibrosarcoma protuberans

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19

Histological characteristic pattern of fibrosarcomas?

Herringbone pattern

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20

What are fibrohistiocytic tumors?

Tumors comprised of fibroblasts and histiocytes

- range from self-limiting benign lesions to aggressive high-grade sarcomas

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21

What are histiocytes?

Lipid filled tissue macrophages

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22

Example of a fibohistiocytic tumor?

Benign fibrous histiocytoma

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23

Benign fibrous histiocytoma?

= Dermatofibroma

Common benign lesion that manifests as a small (<1cm), circumscribed brown spot in the dermis or subcutaneous fat

- usually harmless

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24

Undifferentiated pleomorphic sarcoma?

Aggressive large sarcoma that most commonly occurs in the extremities or retroperitoneum

- frequently recurs and metastasizes

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25

How do we diagnose a undifferentiated pleomorphic sarcoma?

We have to rule out the other pleomorphic sarcoma types

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