Term
|
Definition
| mesenchymal proliferations, including extraskeletal and nonepithelial; excludes viscera, meninges, and lymphoreticular system |
|
|
Term
| What are the different types of soft tissue? |
|
Definition
| muslce, fat, fibrous tissue, vessels, nerve |
|
|
Term
| How many sarcomas occur in the US annually? How many cancers? |
|
Definition
| 8,000; 1.5 million cancers |
|
|
Term
| A syndrome of soft-tissue sarcomas is called... |
|
Definition
|
|
Term
| What neoplasms are associated with NF type 1? |
|
Definition
| neurofibroma, malignant peripheral nerve sheath tumor |
|
|
Term
| What soft tissue tumor is associate with osler-weber-rendu? |
|
Definition
| telangiectasia/arteriovenous malformations (AVM) |
|
|
Term
| what types of soft tissue tumors is gardner syndrome associated with |
|
Definition
| fibromatosis/desmoid tumors, colonic adenomas |
|
|
Term
| Where do soft tissues occur? At what percent do they occur in each place? |
|
Definition
40% lower extremity, thigh
30% trunk and retroperitoneum
20% upper extremity
10% head and neck |
|
|
Term
| Are soft tissue tumors more common in men or women? |
|
Definition
|
|
Term
| At what age do soft tissue tumors occur? |
|
Definition
| incidence increases with age but about 15% occur in children |
|
|
Term
| What sarcoma is typical in children? |
|
Definition
|
|
Term
| What sarcoma typically are found in young adults? |
|
Definition
|
|
Term
| What two types of sarcomas are typically found in middle to late adulthood? |
|
Definition
| liposarcoma and fibrosarcoma |
|
|
Term
| What are the different grades of soft tissue tumors? |
|
Definition
| I to III based on degree of differentiation, average number of mitoses per high-power feild, cellularity, pleomorphism and an estimate of the extent of necrosis |
|
|
Term
| What is staging of soft tissue tumors based off of? |
|
Definition
| size, location, depth, grade, and the presence or absence of metastases |
|
|
Term
| Which sarcomas have a better prognosis: superficial or deep-seated? |
|
Definition
|
|
Term
| What is the overall 10 year suvival rate for sarcomas? |
|
Definition
|
|
Term
| What is the most common soft tissue tumor of adulthood? |
|
Definition
|
|
Term
| Where are lipomas most often found? |
|
Definition
| subq on the proximal extremities and trunk |
|
|
Term
|
Definition
| well encapsulated mass of mature adipose tissue that is soft, mobile, painless (except for angiolipoma) |
|
|
Term
|
Definition
|
|
Term
| What is the most common malignant tumor of adulthood? |
|
Definition
|
|
Term
| Where do liposarcomas occur? |
|
Definition
| deep soft tissue, proximal extremities and retroperitoneum |
|
|
Term
| Describe the appearance of liposarcomas. |
|
Definition
|
|
Term
| What are the gross physical characteristics/presentation of fat necrosis? |
|
Definition
| painless palpable mass of skin thickening or retraction. Can show up on mamogram as density or calcification |
|
|
Term
| What are masses in the breast associated with pregnancy and breast-feeding called? |
|
Definition
|
|
Term
| Fat necrosis is caused by... |
|
Definition
|
|
Term
| Describe fibrous tumors and tumor-like conditions. |
|
Definition
| non-neoplastic, secondary to trauma (physical, ischemic), develop and grow rapidly, hypercellular, plump fibroblasts, mitosis |
|
|
Term
| Name two tumor-like conditions taht are pseudosarcomatous proliferations. |
|
Definition
| nodular fasciitis, myositis ossificans |
|
|
Term
| What's another name for nodular fasciitis? |
|
Definition
| pseudosarcomatous fasciitis |
|
|
Term
| What is the most common reactive pseudosarcoma? |
|
Definition
| pseudosarcomatous fasciitis or nodular fasciitis |
|
|
Term
| What age group gets pseudosarcomatous fasciitis? |
|
Definition
|
|
Term
| Where do pseudosarcomatous fasciitis occur? |
|
Definition
|
|
Term
| What is a typical history of psuedosarcomatous fasciitis? |
|
Definition
| several week history of a solitary, rapidly growing, painful mass with possible history of preceding trauma |
|
|
Term
| Where in the layers tissue between epidermis and bone/viscera pseudosarcomatous fasciitis occur? |
|
Definition
| deep dermis, subcutis and muscle |
|
|
Term
| What do pseudosarcomatous fasciitis look like? How big are pseudosarcomatous fasciitis? |
|
Definition
| several cm nodule with poorly defined borders |
|
|
Term
| What do nodular fasciitis nodules look like histologically? |
|
Definition
| plump, immature fibroblasts/myofibroblasts with pleomorphism, conspicuous nucleoli and abundant mitotic figures. As well as extravasated blood and lymphocytes |
|
|
Term
| If you suspect nodular fasciitis what should you be sure to rule out? |
|
Definition
|
|
Term
| Does nodular fasciitis recur after excision? |
|
Definition
|
|
Term
| What is myositis ossificans? |
|
Definition
| reactive fibroplastic proliferation with metaplasia that resembles bone. |
|
|
Term
| What is the size/shape of myositis ossificans? |
|
Definition
| 3-6 cm well demarcated lesion |
|
|
Term
| Where does myositis ossificans occur? |
|
Definition
| muscles of proximal extremities |
|
|
Term
| In what age group does myositis ossificans occur? |
|
Definition
|
|
Term
| What percent of myositis osssificans cases are associated with previous trauma? |
|
Definition
|
|
Term
| If you suspect myositis ossificans you should be sure to rule out... |
|
Definition
| extraskeletal osteosarcoma |
|
|
Term
| What is the clinical course of myositis ossificans? |
|
Definition
| initially swollen and painful, then circumscribed and firm, then painless, hard, well demarcated "zonation" |
|
|
Term
| How do you differentiate between myositis ossificans and osteosarcoma? |
|
Definition
| osteosarcoma lackks zonation of myositis ossificans |
|
|
Term
| How do you cure myositis ossificans? |
|
Definition
|
|
Term
| What are superficial fibromatoses? |
|
Definition
| superficial, nodular or poorly circumscribed fasiclesof fibroblasts surrounded by dense collagen |
|
|
Term
| What are the different types of superficial fibromatoses? |
|
Definition
| palmar= dupuytren contracture, plantar, penile= peyronie disease |
|
|
Term
| What is Dupuytren contracture? |
|
Definition
| palmar superficial fibromatoses; attachment to overlying skin can cause puckering and dimpling over years, and flexion contracture |
|
|
Term
| Is Dupuytren contracture unilateral or bilateral? |
|
Definition
| 50% of cases are bilateral |
|
|
Term
| What is the treatment for Dupuytren contracture? |
|
Definition
| steroid, radiation, needle aponeurotomy, surgery |
|
|
Term
| HOw do people end up getting duputren's contracture? |
|
Definition
| it is a genetic condition that is passed AD with a variable penetrance |
|
|
Term
| Besides being in different locations, what are the other differences between palmar and plantar fibromatosis? |
|
Definition
| plantar fibromatoses is NOT often bilateral and the contractures are rarer |
|
|
Term
| What is the apperance of peyronie disease? |
|
Definition
| causes a palpable mass or induration, usually on dorsolateral penis, may cause abnormal curvature of the shaft or constriction of urethra |
|
|
Term
| How is peyronie disease treated? |
|
Definition
| verapamil, interferon, collagenase, surgery (shortening unaffected side, lengthening affected side, implants) |
|
|
Term
| Which gender is more predisposed to superficial fibromatoses? |
|
Definition
|
|
Term
| What does a superficial fibromatosis do if you leave it alone? |
|
Definition
| may progress, stabilize, or even regress |
|
|
Term
| How do you treat superficial fibromatosis? |
|
Definition
| meds, verapamil, collagenase, steroids, radiation, needle, surgery |
|
|
Term
| What's another name for deep-seated fibromatoses? |
|
Definition
|
|
Term
| Are desmoid tumors benign or malignant? |
|
Definition
|
|
Term
| What are the benign characteristics of desmoid tumors? |
|
Definition
| well differentiated fibroblasts (cytologically bland) that do not metastasize |
|
|
Term
| What are the characteristics of malignant desmoid tumors? |
|
Definition
| large infiltrative masses (1-15 cm), increased mitotic rate, recur after incomplete excision |
|
|
Term
| How do you treat malignantish desmoid tumors? |
|
Definition
| tamoxifen, chemotherapy, radiation |
|
|
Term
| Why do patients with desmoid tumors come into clinic? |
|
Definition
| desmoid tumors can be disfiguring, disabling, painful |
|
|
Term
| How can you cure desmoid tumors? Does it recur? |
|
Definition
| adqequate excision is usually curative but tumor can recur locally if incompletely excised |
|
|
Term
| What is the age range for desmoid tumors? |
|
Definition
| any age but most frequent in teens thru 30s |
|
|
Term
| What are the three classifications of where desmoid tumors are most often found? |
|
Definition
| extra abdominal, abdominal, and intraabdominal |
|
|
Term
| Where are extra abdominal desmoid tumors found? What's the gender preference? |
|
Definition
shoulder, chest wall, back, thigh
M=F |
|
|
Term
| Where are abdominal desmoid tumors found? |
|
Definition
| musculoaponeurotic structures of abdominal wall in women during or after pregnancy |
|
|
Term
| Where are intra-abdominal desmoid tumors found? In what patients? |
|
Definition
| mesentery or pelvic wall; familial adenomatous polyposis (gardner syndrome) |
|
|
Term
| What are the characteristics of a fibrosarcoma? |
|
Definition
| unencapsulated, hypercellular/herringbone, infiltrative, pleomorphic, hemorrhage, necrosis, mitosis |
|
|
Term
| What is a fibrohistiocyte? |
|
Definition
| descriptive term describing the appearance of cells of tumors that look like both fibroblasts and histiocytes. Phenotypically, these tumors are composed of fibroblasts |
|
|
Term
| What's another name for benign fibrous histiocytoma? |
|
Definition
|
|
Term
| T/F Benign fibrous histiocytomas are common. |
|
Definition
|
|
Term
| What type of patient and where on that patient are typical of dermatofibroma? |
|
Definition
| legs of young/middle age women |
|
|
Term
| What are the characteristics of dermatofibromas? |
|
Definition
| painless, slow growing, pigmented, dimple inward on compression. |
|
|
Term
| How big are dermatofibromas? |
|
Definition
|
|
Term
| What are the histological characteristics of dermatofibromas? |
|
Definition
| benign spindle cells with overlying epithelial hyperplasia |
|
|
Term
| What are malignant fibrous histiocytomas? |
|
Definition
| no longer a legitimate diagnostic entity; most are reclassified as fibrosarcomas |
|
|
Term
| T/F Rhabdomyomas are common. |
|
Definition
|
|
Term
| Where are rhabdomyomas most commonly found and what is this associated with? |
|
Definition
| cardiac rhabdomyomas, associated with tuberous sclerosis |
|
|
Term
| What is the most common soft-tissue tumor of childhood |
|
Definition
|
|
Term
| What is the typical age range of pts with rhabdomyosarcomas? |
|
Definition
|
|
Term
| Where are rhabdomyosarcomas more often found? |
|
Definition
| in the head and neck or GU |
|
|
Term
| What types of cells are found in rhabdomyosarcoas? |
|
Definition
| rhabdomyoblasts "tadpole" or "strap cell" |
|
|
Term
| What molecules are found in rhabdomyosarcomas? |
|
Definition
|
|
Term
| What are the three different types of rhabdomyosarcomas? |
|
Definition
| pleomorphic, alveolar, embryonal |
|
|
Term
| What is a pleomorphic rhabdomyosarcoma? |
|
Definition
| rare variant that occurs in deep soft tissue in adults. Characterized by numerous large bizzarre, multinucleated cells. |
|
|
Term
| What are the two most common rhabdomyosarcomas? (%?) |
|
Definition
| embryonal (60%), alveolar (20%) |
|
|
Term
| In what age groups do the different types of rhabdomyosarcomas occur? |
|
Definition
| pleomorphic in adults; alveolar in early adolescence, embryonal in chidlren under 10 |
|
|
Term
| Where in the body do alveolar rhabdomyosarcomas occur? |
|
Definition
| deep muscles of extremities |
|
|
Term
| What do alveolar rhabdomyosarcomas look like histologically? |
|
Definition
| fibrous septae, resembling pulmonary alveolae |
|
|
Term
| Where are embryonal rhabdomyosarcomas found? |
|
Definition
| nasal cavity, orbit, middle ear, prostate, paratesticular |
|
|
Term
| What is sarcoma botryoides? |
|
Definition
| a subtype of embryonal rhabdomyosarcoma that occurs in the walls of hollow mucosae (nasopharynx, bladder, vagina). resembles a bunch of grapes |
|
|
Term
| Which type of rhabdomyosarcoma has the best prognosis? |
|
Definition
|
|
Term
| What is the most common neoplasm in women? |
|
Definition
|
|
Term
| What percent of all soft tissue sarcomas do leiomyosarcomas account for? |
|
Definition
| ~ 20% of soft tissue sarcomas |
|
|
Term
| Where are leiomyosarcomas found? |
|
Definition
| deep soft tissue of extremities, retroperitoneum |
|
|
Term
| What do leiomyosarcomas look like grossly? |
|
Definition
| painless, firm, bulky masses |
|
|
Term
| What proteins are typically found in leiomyosarcomas? |
|
Definition
| smooth muscle actin (SMA), desmin |
|
|
Term
| Where are synovial sarcomas located? |
|
Definition
| only 10% are intra-articular, deep soft tissue around knee, thigh |
|
|
Term
| Describe the growth of synovial sarcomas. |
|
Definition
biphasic (epithelial and mesenchymal)
usually present for several years at the time of diagnosis |
|
|
Term
| What do synovial sarcomas look like on xray? |
|
Definition
|
|
Term
| What immunohistochemistry targets can you identify in synovial sarcomas? |
|
Definition
|
|
Term
| What causes synovial sarcomas? |
|
Definition
|
|
Term
| How are synovial sarcomas treated? |
|
Definition
|
|
Term
| Where do synovial sarcomas metastasize to? |
|
Definition
|
|
Term
| What is another name for a glomus tumor? |
|
Definition
|
|
Term
|
Definition
| benign, painful tumor derived from modified smooth muscle cells that is located on distal digits often under fingernails |
|
|
Term
| How do you cure glomangiomas? |
|
Definition
|
|
Term
| What is a pyogenic granuloma? |
|
Definition
| rapidly growing capillary hemangioma that looks like a pedunculated red nodule and can ulcerate and or bleed |
|
|
Term
| Where are pyogenic granulomas most often located? |
|
Definition
| skin, gingival or oral mucosa |
|
|
Term
| What is a pregnancy tumor? |
|
Definition
| pyogenic granuloma on the gingiva |
|
|
Term
| List the painful soft tissues. |
|
Definition
| angiolipoma, neurofibroma, glomangioma, eccrine poroma, leiomyoma, schwannoma |
|
|
