Soft Tissue Neoplasms Flashcards
Osteoblastoma > 2 cm Posterior spine Pain unresponsive to NSAIDs No thick rim of reactive bone Tx: en bloc excision or curettage
characteristics of both osteoma and osteoblastoma
Round to oval
Hemorrhagic, gritty
Well circumscribed
Randomly interconnecting trabeculae of woven bone
Rimmed by osteoblasts
Loose connective tissue with congested capillaries
osteochondroma Most common benign bone tumor Adolescence & early adulthood M:F; 3:1 Metaphysis of long tubular bones (knee) Slow growing Benign, cartilage capped tumor with bony stalk
osteochondroma Sessile or pedunculated Cap – hyaline cartilage Endochondral ossification New bone forms inner portion of head & stalk Tx: simple excision
Endochondroma Benign tumor of hyaline cartilage Most common intraosseous cartilage tumor 20s-50s Solitary metaphyseal lesion Hands & feet Circumscribed, central calcifications, sclerotic rim, intact cortex Detected incidentally
Endochondroma Benign hyaline cartilage tumors within medullary cavities < 3 cm Grey-blue Translucent Nodules of hyaline cartilage Benign chondrocytes Tx: observation or curettage Non-hereditary disorders characterized by multiple enchondromas (Maffucci Syndrome & Ollier disease)
Osteosarcoma epidemiology and definition
Most common primary malignant bone tumor
20% of primary bone cancers
75% occur in children younger than 20
Second peak in older adults with underlying conditions (Paget disease, bone infarcts, prior radiation)
M>F
Metaphysis of long bones of extremities
Approx. 50% around knee (i.e. distal femur or proximal tibia)
Treatment and prognosis for osteosarcoma
Tx: chemo & surgery
Neoadjuvant chemo (assumed all patients have occult metastases)
Followed by surgery
Five year survival: 60%-70% for those without overt metastases
< 20% for those with metastases, recurrent disease, or secondary osteosarcoma
osteosarcoma
Painful, progressively enlarging mass
Large destructive lytic & blastic lesion
Infiltrative margins
Codman triangle – tumor breaks through cortex & lifts periosteum
75% have acquired genetic abnormalities
Mutations in tumor suppressor genes & oncogenes
RB, TP53, INK4a, MDM2, CDK4a
Subtypes related to
Site of origin: intramedullary, intracortical, surface
Histologic grade: low vs. high
Primary vs. secondary to pre-existing disorders
Histologic features: osteoblastic, chondroblastic, fibroblastic, telangiectatic, small cell, and giant cell
Most common subtypes: metaphysis of long bones, primary, intramedullary, osteoblastic, high grade
osteosarcoma Bulky, gritty Grey-white Hemorrhage & cystic degeneration Destroy surrounding bone Replace bone marrow May penetrate epiphysis or enter joint
Ostseosarcoma Production of osteoid/bony matrix Large hyperchromatic nuclei Bizarre giant cells Abundant mitoses Vascular invasion Extensive necrosis
chondrosarcoma epidemiology and definition
Adults – 40s and older; M:F=2:1
Axial skeleton
Low grade & slow growing or high grade & aggressive
Conventional type – produce hyaline cartilage
Central (intramedullary)
Most common (approx. 90%)
Peripheral (juxtacortical)
Clear cell, dedifferentiated, mesenchymal variants
Painful, progressively enlarging masses
Direct correlation between grade & biologic behavior
Majority are grade 1
5 year survival 80%-90%
Grade 3: 5 year survival of 43%
70% of grade 3 tumors metastasize, especially to the lungs
Tx: wide surgical excision
Chemo for dedifferentiated & mesenchymal types
chondrosarcoma Large, bulky Glistening, grey-white, translucent Gelatinous/myxoid matrix Spotty calcifications Central necrosis
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Chondrosarcoma
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Chondrosarcoma
Dedifferentiated variant
Low grade chondrosarcoma with high grade component that does not produce cartilage
chondrosarcoma variant
Clear cell variant
Abundant clear cytoplasm
Osteoclast type giant cells
Reactive bone formation
Chondrosarcoma
Mesenchymal variant
Islands of cartilage
Sheets of small round cells
Mimicks Ewing sarcoma
Lipoma
Benign Soft, mobile, painless Mature adipocytes Proximal extremities & trunk Tx: simple excision
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Liposarcoma
50s-60s
Extremities & retroperitoneum
Lipoblast
Cytoplasmic vacuoles with fat
liposarcoma
Well differentiated variant Atypical lipomatous tumor in extremities Resemble lipomas grossly Scattered lipoblasts Atypical spindled cells
Liposarcoma Dedifferentiated liposarcoma Retroperitoneum Heterologous elements MDM2 amplification
Liposarcoma
Myxoid liposarcoma Lower extremities Prominent capillaries Mucoid matrix t(12;16) FUS-DDIT3
Pleomorphic liposarcoma Highly cellular Poorly differentiated Tumor giant cells Abundant mitoses Necrosis
Variants of liposarcoma
Recurrence common
Complete excision!!
Well differentiated variant – indolent behavior
Myxoid variant – intermediate behavior
Pleomorphic variant – aggressive behavior
Nodular fasciitis
Self-limited
Fibroblastic & myofibroblastic proliferation
Young adults
Rapidly growing, history of trauma common
t(17;22) MYH9-USP6
Spontaneous regression
Nodular Fasciitis
< 5 cm Circumscribed or slightly infiltrative Cellular “Tissue culture fibroblasts” Maturation gradient: loose and cellular to organized and fibrous Abundant mitoses
Superficial fibrzomatosis
Infiltrative fibroblastic proliferation Causes deformity Clinically innocuous M>F Nodular proliferation of fibroblasts & dense collagen
Superficial firbromatosis Palmar – Dupuytren contracture Puckering & dimpling Flexion contracture Plantar Younger patients No contractures
Superficial fibrzomatosis
Penile – Peyronie disease
Dorsolateral penis
Abnormal curvature of shaft &/or
Urethral constriction
Deep fibrzomatosis
Large & infiltrative Recurrence common Young patients; F>M APC or β-catenin mutations ↑ Wnt signaling FAP patients are predisposed
Deep fibromatosis
Grey-white, firm Poorly demarcated Bland fibroblasts & dense collagen Broad fascicles Anterior abdominal wall Complete excision difficult
Rhabadomyosarcoma
4.5% of all childhood cancers
Bimodal ages distribution
2-6 years old; 10-18 years old
3 major subtypes: embryonal*, alveolar, pleomorphic
Head & neck, GU tract & retroperitoneum, extremities
Glistening, gelatinous, grey-white or firm, rubbery, grey-white to pink-tan
Hemorrhage & necrosis
Rhabdomyosarcoma: Embryonal
60%; children < 10 yo
Head & neck most common
Alternating hypercellular & loose myxoid areas
Small hyperchromatic round to spindled cells
Rhabdomyoblasts with cross striations
Rhabdomyosarcoma: Ebmryonal
Botryoid variant Polypoid growth Mucosa-lined, hollow organs Cambium layer Subepithelial condensation of tumor cells Loose stromal connective tissue
Rhambomyosarcroma: Alveolar
30%; 10-25 yo Extremities Irregular alveolar spaces Central loss of cohesion Fibrous septae & dilated vascular channels t(1;13) PAX3-FOXO1A t(1;13) PAX7-FOXO1A
Rhabdomyosarcoma: Pleomorphic
Rare; 6th decade; M>F Extremities Haphazardly oriented pleomorphic cells Hyperchromatic nuclei Eosinophilic cytoplasm Bizarre tumor giant cells
Rhabdomyosarcoma tx and prognosis
Aggressive Tx: surgery, chemo, +/- radiation Botryoid embryonal – best prognosis Embryonal – intermediate prognosis Alveolar – poor prognosis Pleomorphic – often fatal
Leiomyoma
Benign smooth muscle tumor
Common in the uterus
Most common neoplasm in women
Erector pili muscles of skin, nipples, scrotum, labia
May be multiple & painful
Deep soft tissues & muscular layers of GI tract
HLRCC – uterine & cutaneous leiomyomata with RCC
Autosomal dominant mutation in fumarate hydratase gene
Leiomyoma
1-2 cm Fascicles of spindle cells Blunt ended elongated nuclei Minimal atypia Tx: resection
Leiomyosarcoma
10%-20% of sarcomas Older adults; F>M Extremities & retroperitoneum Great vessels – IVC (more lethal) Painless firm masses Large & bulky in retroperitoneum Necrosis, hemorrhage, cystic degeneration
Leiomyosarcoma
Spindle cells Hyperchromatic nuclei Interweaving fascicles Variable nuclear atypia High mitotic activity malignant
Leiomyosarcoma tx
Treatment depends on size, location, & grade
Superficial/cutaneous good prognosis
Retroperitoneum poor prognosis
Difficult to remove
Causes death by both local extension and metastatic spread (lungs)
Ewing Sarcoma/Primitive Neuroectodermal Tumor (PNET)
Bone & soft tissue tumor without obvious differentiation
6%-10% of primary bone malignancies
2nd most common bone sarcoma in children
80% < 20 yo
Present as painful, enlarging mass
Diaphysis of long tubular bones (i.e. femur)
Ewing Sarcoma/Primitive Neuroectodermal Tumor (PNET) tx and mechanism
t(11;22) EWS-FLI1 Hemorrhage & necrosis Uniform small round cells Hyperchromatic Scant cytoplasm Tx: neoadjuvant chemo Surgery +/- radiation
Synovial Sarcoma epidemiology and gene
10% of all sarcomas
20s-40s
Deep seated mass present for several years
t(X;18) SS18-SSX1, -SSX2, or –SSX4
Types and tx for synovial sarcoma
Monophasic – uniform spindle cells in fascicles
Biphasic – spindle cells & gland-like structures
Staghorn vessels
Tx: surgical
+/- chemo & radiation
Biphasic or monophonic synovial sarcoma
Undifferentiated Pleomorphic Sarcoma (UPS)
High grade pleomorphic tumors Diagnosis of exclusion Largest category of adult sarcomas Deep soft tissues of extremities (thigh) Middle aged to older adults
Undifferentiated Pleomorphic sarcoma
Grey-white & fleshy Grow up to 20 cm Anaplastic spindle to polygonal cells Hyperchromatic bizarre nuclei Abundant mitoses & necrosis Tx: surgery +/- chemo & radiation Poor prognosis
