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Heme / Onc > SM_273b: Sarcomas > Flashcards

SM_273b: Sarcomas Flashcards

1
Q

____ are a group of rare solid tumors arising from mesenchymal tissue

A

Sarcomas are a group of rare solid tumors arising from mesenchymal tissue

  • Soft tissue sarcomas
  • Bone sarcomas
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2
Q

Describe soft tissue sarcomas

A

Soft tissue sarcomas

  • Include fat, muscle, nerve and nerve sheath, blood vessel, and other connective tissue tumors
  • Most common subtypes include leiomyosarcoma, pleomorphic sarcoma / malignant fibrous histiocytoma, liposarcoma, dermatofibrosarcoma, rhabdomyosarcoma, angiosarcoma, and malignant peripheral nerve sheath tumors
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3
Q

Describe bone sarcomas

A

Bone sarcomas

  • Most common subtypes include osteosarcoma, chondrosarcoma, and Ewing’s sarcoma
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4
Q

____ is the most prevalent type of sarcoma

A

Leiomyosarcoma is the most prevalent type of sarcoma

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5
Q

Describe etiology of sarcomas

A

Sarcomas

  • Fat - liposarcoma
  • Smooth muscle - leiomyosarcoma
  • Skeletal muscle - rhabdomyosarcoma
  • Nerve - MPNST
  • Blood vessels - angiosarcoma
  • Connective tissues - fibrosarcoma
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6
Q

Sarcoma is associated with genetic syndromes: ____, ____, ____, and ____

A

Sarcoma is associated with genetic syndromes

  • Li-Fraumeni (TP53 mutation)
  • Retinoblastoma (RB1 gene deletion)
  • NF-1 (NF1 mutation)
  • Gardner (APC mutation)
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7
Q

Describe Li-Fraumeni syndrome

A

Li-Fraumeni syndrome

  • Patient has been diagnosed with a sarcoma at a young age: below 45
  • First-degree relative has been diagnosed with any cancer at a young age: below 45
  • Another first-degree or second-degree relative has been diagnosed with any cancer at a young age (below 45) or with a sarcoma at any age
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8
Q
A
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9
Q

Describe clinical presentation and diagnosis of sarcomas

A

Clinical presentation and diagnosis of sarcomas

  • Soft tissue masses are common and usually benign
  • Variable: heterogeneous based on sites of origin
  • Soft tissue of extremities and trunk wall: mass, generally painless or asymptomatic
  • Malignant if: increasing in size, > 5 cm in size, deep to deep fascia, painful
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10
Q

Superficial soft tissue lesions > 5 cm and all deep-seated masses have a high risk of being a ___

A

Superficial soft tissue lesions > 5 cm and all deep-seated masses have a high risk of being a sarcoma

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11
Q

Describe pathologic features and prognostic features of sarcoma

A

Pathologic features and prognostic features of sarcoma

  • Core needle biopsy
  • Fine needle aspiration inadequate
  • Tumor size, tumor size, tumor depth, tumor grade, histologic subtype, increasing age, metastatic disease at presentation, tumor site, and positive surgical margins
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12
Q

Most important factor for prognosis of sarcoma is ___

A

Most important factor for prognosis of sarcoma is grade of tumor

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13
Q

___ is primary treatment for sarcomas

A

Surgery is primary treatment for sarcomas

  • R0 resection is required
  • Adjuvant radiation to site of primary tumor for all soft tissue sarcomas of extremities / trunk > 5 cm
  • No clear role for radiation in abdominal or visceral sarcomas
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14
Q

Adjuvant chemo for sarcomas is ___

A

Adjuvant chemo for sarcomas is controversial

  • May shrink but not eliminate microscopic metastatic disease
  • Not beneficial for soft tissue sarcomas arising from visceral or abdominal sites
  • Patients with specific subtypes benefit: synovial sarcoma, high-grade myxoid / round cell liposarcoma
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15
Q

Describe treatment of metastatic soft tissue sarcomas

A

Treatment of metastatic soft tissue sarcomas

  • Anthracyclines and alkylating agents yield best response rates
  • Gemcitabine-docetaxel are synergistic combo
  • Single agent therapy may be useful as combo therapy because doxorubicin and ifosfamide are not synergistic
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16
Q

____ and ____ are classic chemo drugs for metastatic sarcoma with highest response rates

A

Doxorubicin and ifosfamide are classic chemo drugs for metastatic sarcoma with highest response rates

17
Q

Combo therapy is often ___ for metastatic sarcoma

A

Combo therapy is often beneficial for metastatic sarcoma

18
Q

Sarcoma can benefit from ___

A

Sarcoma can benefit from targeted therapy

19
Q

____ is beneficial for soft tissue sarcoma

A

Olaratumab is beneficial for soft tissue sarcoma

  • Promotes greater survival
20
Q

Liposarcomas are treated with ___ if well-differentiated

A

Liposarcomas are treated with surgery if well-differentiated

21
Q

High grade myxoid liposarcomas have ___

A

High grade myxoid liposarcomas have t(12;16) or t(12;22) translocations

  • TLS or ERSR1 coupled with CHOP
  • Most chemotherapy sensitive liposarcoma subtype: doxorubicin, ifosfamide, trabectedin, eribulin
22
Q

Leiomyosarcoma is treated with ___, ___, ___, or ___

A

Leiomyosarcoma is treated with doxorubicin, DTIC, gemcitabine, or gemcitabine-docetaxel

  • Also trabectedin or eribulin
  • May represent a distinct phenotype
23
Q

Synovial sarcoma involves ____ or ____

A

Synovial sarcoma involves t(X;18) SYT-SSX1 (biphasic) or SYT-SSX2 (monophasic)

  • Biphasic has better prognosis
  • Among most sensitive adult sarcoma to chemotherapy especially to ifofsfamide regimens
  • High grade
24
Q

Describe treatment for soft tissue sarcoma

A

Soft tissue sarcoma treatment

  • Stage I: surgery w/ wide excision ± radiation
  • II and III: surgery with wide excision ± pre or postop RT ± chemo, or RT or chemo/RT or chemo if unresectable
  • IV: metastectomy for limited disease, single or combination anthracycline-based chemotherapy
25
Q

GI stromal tumors often involve ___

A

GI stromal tumors often involve gene mutations that cause malignant transformation

  • KIT, PDGFRA
  • Gain of function mutation result in abnormal constitutively activated RTKs: ligand independent mitogenic activity, stimulation of downstream signaling pathways
26
Q

____ and ____ are common in GI stromal tumors and are the best predictors of clinical response to imatinib mesylate

A

KIT and PDGFRA are common in GI stromal tumors and are the best predictors of clinical response to imatinib mesylate

  • KIT exon 11: favorable
  • KIT exon 9: intermediate response
  • Wild-type or PDGFRRA D842V: low response
  • Exon 9 mutations are biologically more aggressive relative to other genotypes
27
Q

Describe mechanism of imatinib mesylate for GI stromal tumors

A

Imatinib mesylate for GI stromal tumors

  1. Occupies ATP binding pocket of the KIT kinase domain
  2. Prevents substrate phosphorylation and signaling
  3. Inhibits proliferation and survival
28
Q

___ is primarily modality for GI stromal tumors with adjuvant ___

A

Surgery is primarily modality for GI stromal tumors with adjuvant imatinib

29
Q

___ is main treatment for metastatic GI stromal tumors

A

Imatinib is main treatment for metastatic GI stromal tumors

  • Cytotoxic chemo is ineffective
30
Q

Describe osteosarcoma

A

Osteosarcoma

  • Peaks of incidence at age 10-20 and age 60-80: associated with Paget disease
  • Arise in metaphysis of the bone
  • Codman triangle: periosteal reaction
  • Characterized by lytic and blastic features
  • Neoadjuvant and/or adjuvant chemotherapy is standard care
31
Q

Osteosarcoma is treated with ___

A

Osteosarcoma is treated with doxorubicin and cisplatin

32
Q

Describe chondrosarcoma

A

Chondrosarcoma

  • Second most common tumor of brain
  • > 60 years of age
  • Typically low or intermediate grade
  • No role for chemo
  • Multinodular growth pattern
33
Q

Ewing’s sarcoma and PNET arise in ___ and ___

A

Ewing’s sarcoma and PNET arise in bones of children and commonly soft tissues in adults

  • Always considered high grade
  • Infiltrative onion skinning pattern in affecting shaft of bone
  • Surgery, chemo, and radiation multimodal is standard of care
34
Q

Describe chemo for Ewing’s sarcoma

A

Chemo for Ewing’s sarcoma

  • Primary disease: vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide
  • Metastatic disease: vincristine / doxorubicin / cyclophospamide followed by ifosfamide / etoposide at progression
35
Q

Giant cell tumor is ___

A

Giant cell tumor is tumor composed of malignant stromal cells that secrete receptor activator of NFkB RANKL ligand and recruit multinucleated osteoclast like cells that result in bone lysis

36
Q

____ is used to treat giant cell tumor of bone

A

Denosumab is used to treat giant cell tumor of bone

  • Inhibitory monoclonal antibody
37
Q

Describe survivorship of sarcomas

A

Sarcomas survivorship

  • 1-2% treated with doxorubicin and ifosfamide develop symptomatic cardiomyopathy and 5-10% develop renal tubular / glomerular dysfunction with risk related to cumulative dose received
  • Infertility is most often related to aklylating agent exposure and affects post-pubescent males more often than females

Heme / Onc (41 decks)

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