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MSS Weeks 3-5 > Soft Tissue Tumors > Flashcards

Soft Tissue Tumors Flashcards

1
Q

What is the cause of most soft tissue tumors?

Majority of tumors occur _________

A

Unknown (Documented association following rad therapy, and some rare burns( chemical and thermal) and trauma

Sporadically

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2
Q

Where do soft tissue tumors arise?

A

Any location, but 40% of sarcomas occur in lower extremity, esp. thigh

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3
Q

What is the relationship between soft tissue tumors and ages?

A

Incidence of sarcomas increases with age (15% arise in children)

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4
Q

What are the primary diagnostic features used? What ancillary techniques are used to aid in diagnosis?

A

Primary

  • Cell morphology
  • Architectural arrangement

Ancillary techniques:

  • Immunohistochemistry
  • Electron microscopy
  • Cytogenetics
  • Molecular genetics
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5
Q

What are prognostic factors for soft tissue tumors?

A
  • Grading (I-III): Degree of differentiation, avg. number of mitoses, extent of necrosis
  • Size (> or <5 cm)
  • Depth (above or under fascia)
  • Stage (I-IV) of tumor
  • Superficial locations have better prognosis than deep lesions
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6
Q

What is treatment for soft tissue tumors?

A

Wide surgical excision (frequently limb-sparing) usually
Irradiation and systemic therapy reserved for large high-grade tumors

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7
Q

What is the most common soft tissue tumor of adulthood?

A

Lipoma

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8
Q

How do lipomas usually present and how are they treated?

A

Most are solitary lesions, mobile slowly enlarging, painless (except angiolipoma) masses
Complete excision usually is curative

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9
Q

What is the morphology of lipoma?

A 
Suclassified according to particular morphological feature (angiolipoma, spindle cell, etc.)
Conventional lipomas (most common) are soft, yellow, well-encapsulated masses
Consist of mature white fat cells with no pleomorphism
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10
Q

Genetic variant for lipomas

A

12q14-q15

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11
Q

What is one of the most common sarcomas of adulthood (40-60s)?

A

Liposarcoma

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12
Q

Where do liposarcomas arise and what is their morphology?

A

Deep soft tissues of proximal extremities and retroperitoneum, may develop into large tumors
Well-differentiated, myxoid/round cell, and pleomorphic histological variants

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13
Q

What is the behavior of liposarcoma?

A

WD-LPS is relatively indolent
Myxoid/round cell type is intermediate
Pleomorphic variant usually agressive and may metastasize
All types recur locally unless adequately excised

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14
Q

What is the genetics of liposarcoma?

A

WD-LPS: Supernumerary ring chromosomes (amplification of 12q14-15, containing MDM2)
Myxoid/round LPS: t(12;16)(q13;p11)

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15
Q

What are pseudosarcomatous proliferations?

A

Reactive non-neoplastic lesions that develop in response to some form of local trauma or are idiopathic
Develop suddenly and grow rapidly
Hypercellularity, mitotic activity, and primitive appearance mimic sarcoma

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16
Q

What are the two types of pseudosarcomatous proliferations and their qualities?

A

Nodular fasciitis: deep dermis, subcutis or muscle
Several centimeters

Myositis Ossificans: Proximal extremities, young adults, trauma in >50% of cases
Presence of metaplastic bone; eventually the entire lesion ossifies and the inertrabecular spaces become filled with bone marrow
Must be distinguished from extraskeletal osteosarcoma

17
Q

Where do superficial fibromatoses arise?

A

Palmar (Dupuytren contracture)
Plantar
Penile (Peyronie disease)

18
Q

What is the prognosis of superficial fibromatoses?

A

May stabilize and resolve spontaneously, some recur

19
Q

Deep-Seated Fibromatosis (Desmoid Tumors)
Behavior?
Age?
Associated disease risk?
Gene association?

A

Behavior lies between benign fibrous tumors and low-grade fibrosarcomas; frequently recur after incomplete excision
Most frequent in the teens to 30s
Some associated with Gardner syndrome
Mutations in the APC or Beta-catenin genes

20
Q

What is fibrosarcoma?
What age does it target?
Where does it target?
Rates of recurrence and metastasize?

A
  • Malignant tumors composed of fibroblasts
  • Mostly adults
  • Deep tissues of the thigh, knee, and retroperitoneum
  • Aggressive tumors, recur in >50% of cases, metastasize in >25%
21
Q

What is the most common neoplasm in women?

A

Uterine leiomyomas

22
Q

Where do leiomyomas arise?
What is the size?
What is the prognosis?

A
  • Uterine, skin, deep soft tissues
  • <1-2 cm
  • Solitary: Easily cured, Multiple: May be difficult to remove
23
Q

What percentage of soft-tissue sarcomas are leiomyosarcoma?
Who is the most susceptible?
Where do they proliferate?
Prognosis?

A
  • 10-20% of soft-tiisue sarcomas
  • Adults, F>M
  • Skin and deep soft tissues of the extremities and retroperitoneum
  • Superficial leiomyosarcomas usually small and have good prognosis; retoperitoneum sarcomas are large cannot be entirely excised
24
Q

What is the age of incidence for rhabdomyosarcoma?
Where does rhabdomyosarcoma arise?
What are the subtypes?

A
  • Most common soft tissue sarcoma of childhood and adolescence (Before age 20)
  • Head, neck, GU tract, sites where little if any normal skeletal muscle
  • Embryonal, alveolar, pleomorphic
25
Q

What are the rates of embryonal RMS?
Age of RMS
Location of RMS
Variants of RMS

A
  • 49% of RMS
  • <10 years of age
  • Head/neck (orbit and parameninges), GU tract, deep soft tissues of the extremities, pelvis and retroperitoneum)
  • Variants: Sarcoma botryoides, spindle cell, anaplastic
26
Q

Alveolar RMS

  • Age
  • Location
  • Genetic variant
A
  • 10 and 25 years of age
  • Deep soft tissues of the extremities: head and neck, perineum, pelvis, retroperitoneum
  • t(2;13)/PAX3-FKHR or t(1;13)/PAX7-FKHR in most (80-85%) of alveolar RMS
27
Q

Synovial sarcoma

  • Rate
  • Who is at risk
  • Where do they develop
  • Genetic variant
  • Histology
  • Treatment
  • Prognosis
A
  • 5-10% of all soft tissue sarcomas
  • Young adults, more commonly in males
  • Over 80% in the deep soft tissue of extremities, especially around the knee, metastatic sites are lung, bone, and regional lymph nodes
  • Characteristic t(X;18) (p11;q11)
  • Histologically biphasic or monophasic
  • Aggressively with limb-sparing surgery and chemotherapy
  • 5-year surival rates from 25% to 62% and only 10-30% of patients live longer than 10 years

MSS Weeks 3-5 (22 decks)

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