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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
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
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
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
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
Genetic syndromes associated with the development of soft tissue tumors?
- Neurofibromatosis 1
- Neurofibromatosis 2
- Gardner syndrome
- Li-Fraumeni syndrome
Fibrous tumor-like lesions?
= Pseudosarcomas;
- Nodular fasciitis
- Proliferative fasciitis
- Myositis ossificans
What is nodular fasciitis?
Bening, self-limited proliferation of fibroblasts
- typically occurs in adults on their palmar side of their forarm
What is myositis ossificans?
Pseudosarcoma characterized by an ossifying mass
- usually develops in muscles, in the extremities of adults after repeated mechanical trauma
Myositis ossificans should be distinguished from?
Extraskeletal osteosarcoma
What are fibromas? examples?
Benign fibrous tumors
- dermatofibroma
- tendon sheath fibroma
- nuchal keloid
What is fibromatoses?
Group of intermediate (between benign and malignant) fibroblastic tumors
- locally aggressive, but never metastasize
What types of fibromatoses can we distinguish?
Superficial types
- Palmar/plantar fibromatosis
- Penile fibromatosis
Deep type
- Desmoid tumors
What is palmar/plantar fibromatosis?
Fibrous growth in the palmar or plantar aponeurosis
What is penile fibromatosis?
Fibrous growth in the penis
- cause abnormal curving and pain
What are desmoid tumors?
Fibrous growths in the extremities, abdominal wall or abdominal cavity
What are fibrosarcomas? where do they most commonly occur?
Malignant tumors composed of fibroblasts
- most commonly occur in the extremities and retroperitoneal area
Subtypes of fibrosarcoma?
- Adult type = fibrosarcoma not otherwise specified
- Infantile type
- Inflammatory myofibroblastic tumors
- Dermatofibrosarcoma protuberans
Histological characteristic pattern of fibrosarcomas?
Herringbone pattern
What are fibrohistiocytic tumors?
Tumors comprised of fibroblasts and histiocytes
- range from self-limiting benign lesions to aggressive high-grade sarcomas
What are histiocytes?
Lipid filled tissue macrophages
Example of a fibohistiocytic tumor?
Benign fibrous histiocytoma
Benign fibrous histiocytoma?
= Dermatofibroma
Common benign lesion that manifests as a small (<1cm), circumscribed brown spot in the dermis or subcutaneous fat
- usually harmless
Undifferentiated pleomorphic sarcoma?
Aggressive large sarcoma that most commonly occurs in the extremities or retroperitoneum
- frequently recurs and metastasizes
How do we diagnose a undifferentiated pleomorphic sarcoma?
We have to rule out the other pleomorphic sarcoma types