46 terms

MSK Soft-Tissue and Bone Tumors

___ is an intermediate filament common to mesenchymal tissues. ____ protein is also common to mesenchymal tissues, in particular sarcomas.
Vimentin, S-100
Myoglobin is specific to ___ ___ .
skeletal muscle
Mesenchymal tumors tend to be ___ rather than ___ . Nonetheless, they tend to be more frequently malignant among ___ than among ___ .
benign, malignant, children, adults
Usually risk for developing a sarcoma increases with ___ age and tends to be higher in ___ than in ___ . Signs usually include a ___ growing mass that often metastasizes by the time of clinical diagnosis.
advancing, men, women, rapidly
___ are the most common soft tissue mass in adulthood. Grossly it is a soft, yellow, and encapsulated mass.
___ are common among adults in their 50s or 60s. The most common type is a ___ ___ which is frequently associated with a ____ translocation.
Liposarcomas, myxoid liposarcoma, t(12;16)
All liposarcomas tend to recur unless they have been properly excised; however, ___ tumors are difficult to excise and even when they are, they tend to recur with subsequent involvement of vital organs.
Grossly, myxoid liposarcomas are ___ , ___ and have many ___ . Microscopically, myxoid liposarcomas contain small, uniform undifferentiated mesenchymal cells with a variable number of ___ , all set in an abundant ___ matrix and often seen with a branching network of ___ .
soft, mucoid, cysts, lipoblasts, mucoid, capillaries
___ ____ represents a reactive lesion that may arise secondary to ___ and may be mistaken for a sarcoma. However, because it is a reactive lesion the mass does not recur post ___ .
Nodular fasciitis, trauma, excision
Nodular fasciitis tends to occur in ___ adults, specifically on the __ side of the forearm or on surfaces superficial to either ___ or ___ .
young, volar, fascia, muscle
Grossly, nodular fasciitis masses have poorly defined ___ . Microscopically they appear as highly cellular lesions with plump, immature appearing ___ that are randomly arranged in whirls or fasicles with a richly vascular myxoid background showing evidence of extravasated ___ and ___ .
margins, fibroblasts, RBCs, lymphocytes
___ ____ are also known as ___ fibromatoses, and are most commonly seen in ___ up to adults in their ___ . These are usually ___ tumors - typically ____ - and tend to recur after excision but do not metastasize.
Desmoid tumors, desmoid, teenagers, 30s, deep, intramuscular
Desmoid tumors can be divided into 3 types: (1) __ (2) __ and (3) simply ___ . Grossly, demoid tumors appear ___ and ___ while microscopically, they appear as plump, long sweeping fascicles of ____ infiltrating ___ tissue.
extra-abdominal, intra-abdominal, abdominal, grey, rubbery, fibroblasts, adjacent
___ ___ ___ are the most common type of sarcoma in adults. These type of tumors may occur at any age but are more frequently diagnosed in ___ ___ .
Malignant fibrous histiocytomas usually arise de novo, but some may be __ to other conditions. They may present as either a ___ ___ malignancy or more rarely as an ___ lesion.
secondary, soft tissue, intraosseous
Malignant fibrous histiocytomas may occur anywhere in the body, but have a predilection for ___ planes of the ___ extremities and also the ___ .
deep, proximal, retroperitoneum
Malignant fibrous histiocytomas may be divided into ___ clinically and prognostically relevant histological types. The most common type is referred to as ______ which usually presents at ___ plus years of age and its prognosis is ___ ___ relative to the five year survival rate of patients with a liposarcoma.
five, storiform-pleomorphic, 60, less favorable
Grossly, MFHs appear as ___ masses with areas of ___ and or ___ . Intra-osseous lesions often demonstrate extensive destruction of the underlying ___ .
tan-white, hemorrhage, necrosis, bone
Microscopically, the storiform-pleomorphic type is composed of plump, pleomorphic ___ cells arranged in a ___ pattern. Some cells may resemble ___ laden histiocytes.
spindle-shaped, whirling, lipid
Treatment for patients with a malignant fibrous histiocytoma includes surgical ___ , and both ___ and ___ therapies.
excision, radiation, chemo
Rhabdomyosarcomas are the most common type of sarcoma in ___ and ___, but occur rarely in ___ .
children, adolescents, adults
Rhabdomyosarcomas can be divided into three clinically and prognostically relevant types with the most common being referred to as ___ which typically presents by the age of ___ and arises in either the (1) ___ (2) ___ region or (3) ___ .
embryonal, 10, head, genitourinary, retroperitoneum
Grossly, rhabdomyosarcomas are soft, gray white ___ masses that might demonstrate evidence of ___ and or ___ .
infiltrative, hemorrhage, necrosis
Microscopically, the embyronal subtype appears as a collection of ___ striated muscle cells with abundant ___ cytoplasm in a variably myxoid stroma.
undifferentiated, eosinophilic
Morphologically, characteristic elongated rhabdomyoblasts, also known as ___ cells, with cross-sections are a feature of ___ differentiated forms.
strap, better
A ___ is a relatively common, benign, intraosseous tumor characterized by the production of abundant mature hyaline cartilage.
A chondroma is referred to as a ___ when the tumor arises in the medullary cavity.
Chondromas/enchondromas typically present in patients ___ to ___ years of age with an ___ sex distribution. Chondromas typically present as ___ masses located in the ___ region of ___ bones, particularly in the small bones of the ___ and ___ .
20, 50, equal, solitary, metaphyseal, tubular, hands, feet
A syndrome in which multiple enchondromas arise, either in one or several bones, is called ____ or ___ disease.
enchondromatosis, Olier
If endochromatosis is associated with soft tissue hemangiomas, the disorder is referred to as ___ syndrome. Patients with this syndrome are at risk for other ____ .
Maffucci, malignancies
Patients with a chondroma or enchondroma tend to be ___ , although sometimes patients may have associated ___ and even pathological ___ .
asymptomatic, pain, fractures
Grossly, chondromas appear as masses that are smaller than ___ centimeters, colored ___ , are ___ when raised to light, well circumscribed with focal areas of ___ .
3, grey-blue, translucent, calcification
Microscopically, chondromas appear as nodules of well-circumscribed cartilage with a ___ matrix and irregularly scattered neoplastic ___ - cartilage undergoes enchondral ossification at the nodular ___ while the center of the lesion tends to ___ and ___- .
hyaline, chondrocytes, periphery, calcify, die
A ___ is a malignant tumor of cartilage cells that tends to affect __ more commonly than __ and typically presents in a patient's __ to __ .
chondrosarcoma, men, women, 40s, 60s
Chondrosarcomas can be __ or __ grade, and are subclassified according to their __ (e.g. __ or __ ) and histology. The most common type is __ __ chondrosarcoma which has a typical distribution that involves the ___ portion of the skeleton including the ___ , __ long bones, and __ . They are typically __ and progressively __ masses.
low, high, site, intramedullary, juxtacortical, intramedullary conventional, central, pelvis, proximal, ribs, painful, enlarging
Grossly, an intramedullary conventional chondrosarcoma is __ , has a __ __ color, and varying degrees of __ intermixed with firmer, ossified areas.
bulky, gray-white, translucence
Microscopically, grade 1 and 2 chondrosarcomas demonstrate innocuous __ __ with __ cells containing __ , pyknotic nuclei and usually __ mitoses.
hyaline cartilage, sparse, dark, no
Treatment for chondrosarcomas includes __ __ and ___; the five year survival rate for grades 1 and 2 are generally ___ ___ .
surgical excision, chemotherapy, very good
Both osteoid osteomas and osteoblastomas are ___ tumors with __ histological features, but ___ presentations.
benign, identical, different
Osteoid osteomas are ___ common than osteoblastomas and are less than __ centimeters in diameter. They tend to occur more frequently in ___ than in __ particularly amongst ___ and patients in their __ , frequently arise in the ___ extremity and are painful which is caused by excess production of ____ __ .
more, 2, males, females, teenagers, 20s, lower, prostaglandin E
Osteoblastomas are ___ common than osteoid osteomas, are larger than __ centimeters, and typically occur in patients in their ___ to ___ with an __ sex distribution. Osteoblastomas arise in the __ or __ as well as in the ___ , are ___ , but unlike osteoid osteoblastomas, they do not respond well to ___ .
less, 2, teens, 30s, equal, hands, feet, spine, painful, aspirin
Osteosarcomas are the __ __ malignant bone tumors with a ___ distribution. __ of __ cases occur during adolescence while the remainder occur among the elderly. More __ than __ are affected.
most, common, bimodal, 3, 4, men, women
Osteosarcomas are strongly associated with a mutation in the __ gene.
Osteosarcomas tend to arise in the __ of __ bones and are sub-classified based on where they arise, for example ___ vs. ___ .
metaphyses, long, intramedullary, extramedullary
Approximately 1 in 2 patients that present with an osteosarcoma tend to also have an elevated __ __ __ level.
serum, alkaline, phosphatase
Grossly, osteosarcomas appear as bulky, ___ , gritty masses with varying degrees of __ , __ , and __ degeneration. Microscopically, osteosarcomas vary, however, osteoid is commonly seen as laid down in a ___ fashion.
grey-white, hemorrhage, necrosis, cystic, haphazard