Path Soft Tissue Ex 2

Front 1

Benign Conditions of Fibrous Tissue

Back 1

Nodular Fasciitis
Palmar fibromatosis
Abdominal fibromatosis (desmoid tumor)

Front 2

Conditions of Fibrous Tissue Malignant

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Fibrosarcoma

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Nodular Fasciitis characteristics

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Self-limiting pseudosarcomatous process composed of fibroblasts and myofibroblasts

Rapidly growing, richly cellular with mitoses

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Nodular Fasciitis Signs/Symptoms:

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Rapidly growing solitary mass lesion over 1-2 weeks often at site of previous trauma
50% of lesions are painful
Numbness or paresthesia if compression of peripheral nerve

Age:
Young adults (20-40 years)
Sex:
M = F

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Nodular Fasciitis Anatomic Distribution:

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May occur anywhere in the body, though predilection for upper extremities (volar surface of forearm), chest wall, back, head & neck and lower extremities

Head & neck most common in children and infants

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Nodular Fasciitis Gross Findings:

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Well-circumscribed, non-encapsulated
Most lesions small (< 2.0 cm); lesions up to 10.5 cm have been described
Divided into subcutaneous, intramuscular and fascial subtypes

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Nodular Fasciitis Microscopic Findings:

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Proliferating fibroblasts and myofibroblasts in short irregular bundles
Reticulin stromal fibrosis
Mitotic activity
Cellular and myxoid areas
Lipid-filled macrophages and giant cells

Front 8

Nodular Fasciitis
ancillary test
prognosis/tx

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1)IHC
Actin – positive
Cytokeratin and S100 – negative
2)Benign process
Surgical excision curative

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Palmar Fibromatosis (Dupuytren’s Contracture)

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Benign fibrous tissue proliferation
More common in Northern Europeans; rare in Blacks

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Palmar Fibromatosis (Dupuytren’s Contracture) Association with unrelated diseases and social behaviors

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Types I and II DM
Epilepsy
Alcoholism
Hypercholesterolemia
Cigarette smoking
Manual labor

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Palmar Fibromatosis Signs/Symptoms

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Slow growing nodule of hand progression to cord-like band with contracture of 4th or 5th digit
Age:
Older adults (20% over 65 years); rare in children
Sex:
M >>> F

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Palmar Fibromatosis Anatomic Distribution

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Palmar surface of hand (R > L)
50% of cases bilateral

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Palmar Fibromatosis tx

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Surgical excision
Recurrence is high unless dermis and fascia are removed

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Abdominal Fibromatosis (Abdominal Desmoid Tumor)

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Locally more aggressive than superficial fibromatoses
High recurrence rate
Previous trauma
Common in patients with Gardner’s syndrome

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Abdominal Fibromatosis Signs/Symptoms:

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Deep-seated, poorly-circumscribed firm mass in abdominal wall
Little to no pain
Age:
Young adults (20-30 years)
Sex:
F >>>>>>> M
Gravid or parous females

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Abdominal Fibromatosis Anatomic Distribution:

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Muscles and fascia of rectus abdominus and internal oblique

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Abdominal Fibromatosis Gross Findings:

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Poorly-circumscribed tumors
Off-white and firm
3-10 cm in diameter

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Abdominal Fibromatosis Microscopic Findings

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Proliferating spindle cells in bundles surrounded by collagen
Limited mitoses

Front 19

Abdominal Fibromatosis

Prognosis/Treatment:

Back 19

Surgical excision
Recurrence rate 15-30%
Local radiation

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Fibrosarcoma

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Malignant mesenchymal tumor
Variety of microscopic appearances
Tumors arise from intramuscular and intermuscular fibrous tissue, fascia, aponeuroses or tendons

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Fibrosarcoma Signs/Symptoms:

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Solitary palpable mass (3.0 to 8.0 cm)
Slow-growing
1/3 of cases present with pain
Age:
Adult-type
30-55 years
Infantile type
First 2 years of life
Congenital
Sex:
M > F

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Fibrosarcoma Anatomic Distribution:
Adult-type

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Deep soft tissues of lower extremities
Upper extremities; head & neck
Rare organ involvement (heart, lung, liver, CNS)

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Fibrosarcoma Anatomic Infantile-type

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Distal extremities

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Fibrosarcoma Gross Findings:

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Solitary, lobulated, circumscribed and frequently encapsulated
May invade adjacent structures

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Fibrosarcoma Ancillary Testing

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IHC
Vimentin – positive
Cytokeratin and S100 – negative
Actin – variable

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Fibrosarcoma Prognosis/Treatment:

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Local recurrence rate 50%
Metastasis via bloodstream to lung and bone
5-year survival
Adult-type: 80% for grade 1; 21% for grade 4
Infantile-type: > 80%
Wide surgical excision, RT, chemo

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Tumors and Tumor-Like Conditions of Fibrohistiocytic Origin

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Benign:
Benign Fibrous Histiocytoma (Dermatofibroma)
Intermediate:
Dermatofibrosarcoma Protuberans (DFSP)
Malignant:
Malignant Fibrous Histiocytoma (MFH)

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Benign Fibrous Histiocytoma Signs/Symptoms:

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Solitary slow-growing nodule of skin
Often elevated or pedunculated
1/3 of cases multiple
Age:
Young adults to mid-adulthood (20-45 years)
Sex:
M = F

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Benign Fibrous Histiocytoma Anatomic Distribution:

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Most common on distal extremities

Front 30

Benign Fibrous Histiocytoma microscopic findings

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Poorly-circumscribed, non-encapsulated, cellular proliferation in dermis; fibroblastic cells arranged in “storiform” pattern; numerous foreign body (Touton) giant cells; Mitoses rare to absent

Front 31

Benign Fibrous Histiocytoma prognosis tx

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Simple surgical excision
< 5% recurrence

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Dermatofibrosarcoma Protuberans Signs/Symptoms

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Slow-growing, non-painful tumor
Antecedent trauma in 10-20% of cases
Age:
Younger than dermatofibroma
Sex:
M > F

Front 33

Dermatofibrosarcoma Protuberans Anatomic Distribution:

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Trunk, groin and proximal extremities (as opposed to dermatofibroma)

Front 34

Dermatofibrosarcoma Protuberans microscopical findings

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Cellular tumors of dermis and subcutis; slender fibroblasts in monotonous storiform pattern

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Dermatofibrosarcoma Protuberans Ancillary test

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IHC
CD34 – positive
Cytogenetics
Supernumerary ring structure formed by amplified sequences from chromosomes 17 and 22
Fusion of COL1A1 and PDGF genes
Overexpression of PDGF may lead to DFSP

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Dermatofibrosarcoma Protuberans Prognosis/Treatment:

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Locally aggressive; recurs in 50% of cases
Rarely metastasizes (<4% of cases)
Wide surgical excision; RT for large tumors or positive margins

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Most common sarcoma of late adult life

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Malignant Fibrous Histiocytoma

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Malignant Fibrous Histiocytoma Signs/Symptoms:

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Painless, enlarging mass if on extremity
Retroperitoneal tumors may present with obstruction and anorexia
Fever and leukocytosis
Tumor production of IL-6, IL-8 and TNF
Age:
Late adulthood (50-70 years)
Sex:
M >> F (70% of MFH in men)

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Malignant Fibrous Histiocytoma Anatomic Distribution:

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Lower extremities (thigh), followed by upper extremities and retroperitoneum

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Malignant Fibrous Histiocytoma Gross Findings:

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Solitary, multilobulated fleshy masses
5-10 cm
Two-thirds within skeletal muscle
Prominent areas of hemorrhage and necrosis

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Malignant Fibrous Histiocytoma Ancillary Testing:

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> 80% show some cytogenetic abnormality

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Malignant Fibrous Histiocytoma
Prognosis/Treatment:

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Local recurrence rate between 19-31%
Metastatic rate between 31-35%
Most common site is lung
Usually within 12-24 months of diagnosis
Treatment by wide radical excision
RT and chemo

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Most common mesenchymal neoplasm

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Lipoma

Tumors may be single, multiple, superficial or deep

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Lipoma Signs/Symptoms:

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Painless (except angiolipoma),soft, solitary, mobile mass
Age:
Adults (40-60 years)
Rare in 1st 2 decades
Sex:
M > F

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Lipoma Anatomic Distribution:

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Upper back, neck, shoulder and abdomen, proximal extremities, buttocks and upper thigh
Seldom in face,hands or feet

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Lipoma Gross Findings:

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Soft, well-circumscribed, thinly-encapsulated rounded masses
Few millimeters to > 10 cm

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Lipoma Microscopic Findings:

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Mature adipose tissue
Many histologic subtypes

Front 48

Lipoma Ancillary Testing:

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50-80% have clonal cytogenetic abnormalities with most common alteration involving translocations of 12q

Front 49

Lipoma Prognosis/Treatment

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Simple surgical excision
Recurrence rate < 5%

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Liposarcoma Signs/Symptoms

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Extremity tumors develop as slow-growing masses
Retroperitoneal tumors are associated with abdominal symptoms
Age:
Late adult life (60-70 years)
Myxoid and round cell subtypes occur in 5th decade
Sex:
M = F

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Liposarcoma Anatomic Distribution:

Back 51

Deep muscles of extremities or retroperitoneum

Front 52

Liposarcoma Ancillary Testing:

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Well-differentiated liposarcomas often possess a giant ring chromosome (RCG) involving chromosome 12
Myxoid liposarcomas are characterized by t(12;16)(q13;p11) resulting in fusion of CHOP gene with TLS gene. CHOP encodes a DNA transcription factor and TLS encodes an RNA binding protein

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Liposarcoma Prognosis/Treatment

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Well-differentiated tumors of extremities recur around 50% of time though rarely cause death
Poorly differentiated tumors of retroperitoneum often recur, metastasize to lung and bone and have a 5-year survival of 20%
Surgery with chemo and RT