Soft Tissue Neoplasms Flashcards

Question

Variants of liposarcoma

Answer 1

Recurrence common Complete excision!! Well differentiated variant – indolent behavior Myxoid variant – intermediate behavior Pleomorphic variant – aggressive behavior

Answer 2

Self-limited Fibroblastic & myofibroblastic proliferation Young adults Rapidly growing, history of trauma common t(17;22) MYH9-USP6 Spontaneous regression

Answer 3

Nodular Fasciitis ``` < 5 cm Circumscribed or slightly infiltrative Cellular “Tissue culture fibroblasts” Maturation gradient: loose and cellular to organized and fibrous Abundant mitoses ```

Answer 4

Superficial fibrzomatosis ``` Infiltrative fibroblastic proliferation Causes deformity Clinically innocuous M>F Nodular proliferation of fibroblasts & dense collagen ```

Answer 5

``` Superficial firbromatosis Palmar – Dupuytren contracture Puckering & dimpling Flexion contracture Plantar Younger patients No contractures ```

Answer 6

Superficial fibrzomatosis Penile – Peyronie disease Dorsolateral penis Abnormal curvature of shaft &/or Urethral constriction

Answer 7

``` Large & infiltrative Recurrence common Young patients; F>M APC or β-catenin mutations ↑ Wnt signaling FAP patients are predisposed ```

Answer 8

Deep fibromatosis ``` Grey-white, firm Poorly demarcated Bland fibroblasts & dense collagen Broad fascicles Anterior abdominal wall Complete excision difficult ```

Answer 9

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

Answer 10

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

Answer 11

Rhabdomyosarcoma: Ebmryonal ``` Botryoid variant Polypoid growth Mucosa-lined, hollow organs Cambium layer Subepithelial condensation of tumor cells Loose stromal connective tissue ```

Answer 12

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 ```

Answer 13

Rhabdomyosarcoma: Pleomorphic ``` Rare; 6th decade; M>F Extremities Haphazardly oriented pleomorphic cells Hyperchromatic nuclei Eosinophilic cytoplasm Bizarre tumor giant cells ```

Answer 14

``` Aggressive Tx: surgery, chemo, +/- radiation Botryoid embryonal – best prognosis Embryonal – intermediate prognosis Alveolar – poor prognosis Pleomorphic – often fatal ```

Answer 15

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

Answer 16

Leiomyoma ``` 1-2 cm Fascicles of spindle cells Blunt ended elongated nuclei Minimal atypia Tx: resection ```

Answer 17

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 ```

Answer 18

Leiomyosarcoma ``` Spindle cells Hyperchromatic nuclei Interweaving fascicles Variable nuclear atypia High mitotic activity  malignant ```

Answer 19

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)

Answer 20

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)

Answer 21

``` t(11;22) EWS-FLI1 Hemorrhage & necrosis Uniform small round cells Hyperchromatic Scant cytoplasm Tx: neoadjuvant chemo Surgery +/- radiation ```

Answer 22

10% of all sarcomas 20s-40s Deep seated mass present for several years t(X;18)  SS18-SSX1, -SSX2, or –SSX4

Answer 23

Monophasic – uniform spindle cells in fascicles Biphasic – spindle cells & gland-like structures Staghorn vessels Tx: surgical +/- chemo & radiation

Answer 24

``` High grade pleomorphic tumors Diagnosis of exclusion Largest category of adult sarcomas Deep soft tissues of extremities (thigh) Middle aged to older adults ```

Answer 25

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 ```