Muscu - Connective Tissue - Neoplastic

Question 1

Benign Fibrous Histiocytoma

Answer 1

• Common lesion occuring in the dermis and subcutis

AKA - Dermatofibroma

Question 2

Benign Fibrous Histiocytoma - History

Answer 2

• Common lesion

* Typically seen in mid-adult life

Question 3

Benign Fibrous Histiocytoma - Presentation

Answer 3

• Presents as a small firm nodule in the dermis or subcutis

Question 4

Fibromatosis

Answer 4

• Fibromatosis involves the benign proliferation of fibroblasts and subsequent collagen deposition thickening the surrounding connective tissue.
• These disease have been termed “fibroproliferative diseases”
• Superficial and deep forms exist
• Several forms of superficial fibromatosis are recognized, including: palmar fibromatosis (Dupuytren’s contracture), plantar fibromatosis, and penile fibromatosis (peyronie’s disease).
• Note that a deep seated fibromatosis is termed a “desmoid tumor” and is separate entity from the superficial fibromatoses.

Question 5

Fibromatosis - History

Answer 5

• The incidence of Dupuytren’s contracture ranges from 1-20 cases per 1000 annually
• Male to female ratio if 5:1, and mainly in caucasions
• Irregular nodular thickening of the fascia
• Rate of progression is quite variable
• Initially the 5th digit is involved, gradually spreading to the other digits

Question 6

Fibromatosis - Presentation

Answer 6

• Slowly progressing contracture of the palmar fascia in the hand
• Thickening cord-like bands developing in the palmar fascia
• Dimpling or puckering of the overlying skin can help in diagnosis

Question 7

Fibrosarcoma

Answer 7

• A rare but aggressive neoplasm of the soft tissue

Question 8

Fibrosarcoma - History

Answer 8

• Very rare; approximately 500 reported each year

* Usually in the retroperitoneum, thigh, knee, or distal extremity

Question 9

Fibrosarcoma

Answer 9

• A rare but aggressive neoplasm of the soft tissue

Question 10

Fibrosarcoma - History

Answer 10

• Very rare, approx. 500 reported each year
• Usualy in the retroperitoneum, thigh, knee, or distal extremity
• Approx. 50% ocur in the extremities
• Most commonly in the 30-55 age range
• Characterized by immature proliferating fibroblasts with bundles of collagenous fibers
• Aggressive tumors that can metastasize

Question 11

Fibrosarcoma - Presentation

Answer 11

• No characteristic clinical findings

Question 12

Fibrosarcoma - DIagnostic Testing

Answer 12

• Biopsy

Question 13

Lipoma

Answer 13

• Benign tumors of fat

Question 14

Lipoma - History

Answer 14

• Most common soft tissue tumor of adulthood
• Can present anywhere in the body
• When deep-seated in the mediastinum or retroperitoneum, they can reach massive sizes

Question 15

Lipoma - Presentation

Answer 15

• Soft, mobile, painless mass

* Mass effect when deep seated

Question 16

Lipoma - Diagnostic Testing

Answer 16

• Biopsy

Question 17

Liposarcoma

Answer 17

• Sarcoma of adipose tissue

Question 18

Liposarcoma - History

Answer 18

• One of the most common sarcomas of adulthood
• Usually present in the age range from 40 to 60
• Arise in deep soft tissues of retroperitoneum or proximal extremities, but can occur anywhere
• Behavior ranges from benign to aggressive based on type

Question 19

Liposarcoma - Presentation

Answer 19

• No characteristic clinical findings
• Painless mass
• Mass effect when deep seated

Question 20

Liposarcoma - Diagnostic Testing

Answer 20

• Biopsy

Question 21

Malignant Fibrous Histiocytoma

Answer 21

• This is an aggressive malignant form of fibrous histiocytoma

Question 22

Malignant Fibrous Histiocytoma - History

Answer 22

• Peak incidence is in the 7th decade
• Several types recognized:
• storiform -pleomorphic
• myxoid
• inflammatory
• giant cell
• angiomatoid of which the former is the most common
• usually arise in the musculature of the proximal extremities, or in the mediastinum
• Recurrence rate is high

Question 23

Malignant Fibrous Histiocytoma - Presentation

Answer 23

• Painless mass

Question 24

Malignant Fibrous Histiocytoma - diagnostic testing

Answer 24

• Biopsy

Question 25

Myositis Ossificans

Answer 25

* The fibrotic proliferation and the formation of metaplastic bone in skeletal muscle usually following a trauma

Question 26

Myositis Ossificans - History

Answer 26

* Fairly rare event * Typically in an athletic young person following a traumatic event * Typically proximal extremity muscle

Question 27

Myositis Ossificans - Presentation

Answer 27

* Initially an area of pain and swelling in the muscle * Evolves into a painless mass * Can calcify to become a painful mass in muscle

Question 28

Myositis Ossificans - Diagnostic Testing

Answer 28

Radiographic Studies: - Plain films - CT scans - MRI when appropriate