Tumor/Lesion Pathogenesis Histology/Other

FIBROUS TISSUE
 plump fibroblasts (myofibroblasts) randomly
 most common pseudosarcoma, usually adults arranged/short fascicles
 located on (1) forearm, (2) chest, (3) back  myxoid background, extravasated RBCs,
Nodular fasciitis
 solitary, rapidly growing, sometimes painful mass lymphocytes
 usually have preceding trauma  mitoses and collagenization progresses with
time
 reactive pseudosarcoma lesion seen in athletic adolescents and
young adults  Early phase(similar to nodular fasciitis →
Myositis ossificans  50% of cases report previous trauma Middle phase (within three weeks, get
zonation) → Late phase (complete bone
 Early phase(swelling/pain) → several weeks(less pain/firmer) → metaplasia)
Late phase(painless, hard, well circumscribed mass)
 Benign but BAD
 Non-metastasizing fibroblastic tumors which invade locally and recur
if not excised properly  Made of fibroblasts and myofibroblasts
Fibromatosis
 Ex. Palmar and plantar fibromatosis (Dupuytren contraction-fixed producing lots of collagen (desmoid)
flexion of hand)
 Abdominal fibromatosis (Desmoid tumor)
 affects adults(40-60 y/o); arise from deep connective tissue like
fascia, tendon, and grow slowly; affects extremities
 spindle-shaped fibroblasts arranged in
Fibrosarcoma intersecting fascicles producing Herringbone
 grayish, white, firm, well delineated
pattern
 Survival correlates with differentiation
 Min. pleomorphism
 Recurrence after excision = 60%
 most common malignant sarcoma of adults
 occurs in deep structures: deep fascia of skeletal muscles
Malignant (pleomorphic)
 metastasize widely  mono/multinucleated bizarre tumor giant cells
Fibrohistiocytoma
 cell origin is NOT HISTIOCYTIC

Atypical fibroxanthoma
 located superficially  lacks storiform pattern
(benign fibrohistiocytoma)
FIBROHISTIOCYTIC

 single/multiple small firm skin nodule; non-encapsulated
 Gross: reddish brown; poorly circumscribed
 Recurrent infantile digital fibroma: nodules on fingers/toes that
Dermatofibroma
spontaneously involute
 Fibroma Molle: skin tag, common, soft pedunculated growth made of
mature fibrous tissue + mature fat on body surface
Dermatofibrosarcoma  nodular polypoid lesion in dermis and invades subcutaneous
protuberans  slow growing
 low grade sarcoma seen in young adults;
 involve deep fascia
 fibroblastic prolif, collagen capillaries +
histiocytes, fibroblasts arranged in intertwining
bands, numerous capillaries(sclerosing
hemangioma), lipid containing histiocytes,
hyperplasia of epidermis
 bland spindle cells with delicate cytoplasm in a
storiform pattern
 Radial whorls of spindle cells with storiform
pattern and Honeycomb pattern of infiltration
into surrounding adipose tissue
 Tumor/Lesion Pathogenesis Histology/Other
TISSUEADIPOSE

 most common tumor in adults(benign)

 encapsulated, golden yellow, soft, solitary, freely movable, painless,
 Lobules of mature adipose tissue separated by
well-defined mass
thin fibrous septa
Lipoma  grows slowly; seen in 4th-6th decade in subQ of neck, shoulder, arm,  Myxoid areas, fat necrosis from trauma
trunk
 found in pts w/neurofibromatosis and multiple endocrine adenomas
syndrome
Spindle Cell  middle aged (45-60) y/o male; located on back, neck, shoulder  Bipolar spindle cells, mature fat cells, myxoid
 well demarcated stroma, collagen fibers
 painful, subcutaneous mass in forearm of young adults
 non-infiltrative: rare in children
Angiolipoma  mature fat w/thin walled blood vessels;
 infiltrative: infiltrates muscle, same histo as above; occurs in children;
not painful
 Multivacuolated lipoblasts and multinucleated
Pleomorphic (atypioid  well circumscribed and encapsulated unless intramuscular in location cells with atypical nuclei (floret cells)
lipoma)  differential w/liposarcoma difficult  bw atypical cells are mature fat cells, fibrous
collagen, focal spindle cells and myxoid areas
 Lobular architecture w/ fibrous connective tissue
septa
 benign tumor of children(< 6y/o boys) located in upper or lower limb
Lipoblastoma  Lobules consist of mono/multivacuolated
 encapsulated
lipoblasts w/immature spindle and stellate
 20% recurrence mesenchymal cells, mature fat cells
 Myxoid stroma w/immature cells
 Small lobules created by thin fibrous septa
Hibernoma  Tumor from brown fat located in neck, axilla, mediastinum  Brown intracytoplasmic lipofuscin pigment seen
 Infants and adults; encapsulated, tan brown subQ mass in many cells
 malignant; lower extremities of adults
 one of the most common soft tissue sarcomas  Overall histo pattern w/ lipoblast is diagnostic
Liposarcoma  4th-6th decade and arise from deep soft tissue bw large muscles  Lipoblasts may be monovacuolated or
 Ex. Thigh, retroperitoneum, shoulder multivacuolated
 Appear well-circumscribed but are infiltrative
 Lipoma-like (hyperchromatic lipobalsts)
 usually poorly circumscribed
Well-differentiated  Sclerosing-fibrous tissue bands w/ atypical lipoblasts
 atypical lipoblasts
 Good prognosis
 monomorphic fusiform or stellate cells in myxoid
 most common histologic type stroma (gelatinous)
Myxoid
 good prognosis  rich in hyaluronic acid w/plexiform capillary
network (chicken wire vessels)
 poorly differentiated  large w/bizarre lipoblasts w/frequent mitoses
Pleomorphic
 poor prognosis and spindle cells
SKELETAL MUSCLE Rhabdomyoma(benign)
 cardiac type: associated w/tuberous sclerosis, hamartoma
 well differentiated skeletal muscle cells
 extremely rare, most found in buccal cavity
Rhabdomyosarcoma  malignant tumor of skeletal muscle and most common soft 
tissue sarcoma of childhood
 small spindle shaped cells w/eosinophilic
 arises from undifferentiated mesoderm; common sites: head and
cytoplasm condensed around blood vessels or
neck, retroperitoneum, bile duct, urogenital tract (penis and
underneath mucosa
vagina); affects children under 5;
Embryonal  In between cells are loose arrangement of cells
 Gross: poorly circumscribed, soft, white; when growing under in mucoid material
mucosal membrane forms sarcoma botyroides (grape-like)  Skeletal muscle differentiation is detected via
 aggressive tumors light microscopy and immunohistochemistry
 small round cells grouped in alveolar pattern
w/peripheral cells in contact with fibrous tissue
 seen in adolescence (10-25 y/o) in the deep muscles of extremities
Alveolar and central cells are loose and non-cohesive
 Poor prognosis
 scant cytoplasm is eosinophilic
 EM: primitive cells
 Similar to malignant pleomorphic liposarcoma
and malignant fibrous histiocytoma but cross-
Pleomorphic  Thigh is most common site; occurs exclusively in adults striation must be demonstrated
 Grows rapidly  EM: great value in diagnosis of this tumor; five
year survival is 30%
Tumor/Lesion Pathogenesis Histology/Other
SMOOTH MUSCLE

 benign tumor in soft tissue, spread thru blood vessels, female

genital tract (fibroids)
 Interlacing bundles of spindle cells with blunt
 Skin/subQ tissue locations: (1) Erectores pilorum, (2) Erector muscle
nuclear ends
of nipple, (3) media of blood vessel
Leiomyoma  EM: similar to normal smooth muscle cells
 Deep soft tissue locations: retroperitoneum; uterus, stomach  No cell pleomorphism, no mitotic activity, no
 Most common benign tumor in GI necrosis
 Well-delineated, easily shelled out, firm and yellowish tan
 Vascular leiomyoma, epithelioid leiomyoma
 malignant tumor
 less frequent than leiomyomas
Leiomyosarcoma
 common sites similar to benign
 most common sarcoma of GI and uterus
 SYNOVIUM  Benign synovioma: giant cell tumor of tendon sheath
 malignant tumor: Majority around knee, ankle jts in young adults
 Tumor grows close to joints, tendon sheaths, bursa but doesn’t
involve synovial membrane
Synovial Sarcoma  Gross: well circumscribed, firm, grayish pink
 Biphasic pattern: epithelial cells forming glands and intervening
spindle cells
BLOOD VESSELS
 Sarcomatous stroma and gland-like structures
mimicking synovial membrane
 Hyaluronidase resistant mucin in glandular
spaces and in cytoplasm of epithelial-like cells
 Some tumors are predominantly sarcomatous
and some epithelial-like
 Origin: mesencymal cells with capacity to
differentiate into synovial elements

 Benign
 Present at birth and involve head and neck region
 Many are hamartomas or developmental defects
Hemangiomas
 Some composed of closely arrange capillaries: capillary 
hemangiomas
 Some consist of large dilated spaces: cavernous hemangiomas
 Can spontaneously regress

 Malignant tumors composed of anastomosing vascular channels

Angiosarcomas lined by malignant endothelial cells 
 Often seen in head and neck of elderly adults

 Arise in extremities subjected to longstanding lymphedema

Lymphangiosarcomas 
 Assoc. w/ postmastectomy lymphedema: Stewart-Treves syndrome

 Bland plump spindle cells assoc. w/slit-like

vascular spaces containing erythrocytes and
Kaposi’s sarcoma  Assoc. with AIDS patients hemosiderin deposits
 Cell of origin is unknown
Important Points to Remember
1. The most common soft tissue tumor is a lipoma
2. The most common malignant soft tissue tumor is metastatic cancer(s)
3. The most common malignant soft tissue tumor of childhood is a rhabdomyosarcoma
4. Sarcomas metastasize through the bloodstream
5. Liposarcomas in the retroperitoneum eventually are fatal b/c of local aggressiveness to (1) blood vessels, (2)ureter, (3)
other vital organs
6. Soft tissue tumors are 5th leading cause of deaths from cancer in children
7. Sarcomas-undifferentiated mesenchymal cells