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Clinical Oncology > Soft Tissue, Visceral and Bones Tumours > Flashcards

Soft Tissue, Visceral and Bones Tumours Flashcards

1
Q

What are the risk factors for soft tissue, and visceral tumours

A
  • ionising radiation
  • hereditary factors:
    Li Fraumeni syndrome
    NF type 1
    Retinoblastoma
    FAP
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2
Q

What diagnostics are used?

A
  • MRI of extremities, pelvis and abdomen is gold standard for STS
  • MDT approach for pleuropulmonary involvement
  • Core needle biopsy
  • CT/PET may also be an option
  • Complete lab work (LDH is high in STS)
  • Dx to be made by experienced pathologist at tertiary centre
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3
Q

What is the TNM staging for STS?

A

T1: < 5cm
a: superficial
b: deep

T2: > 5cm
a: superficial
b: deep

N0: LN negative
N1: LN positive

M0: no distant metastases
M1: distant metastases

G1, G2, G3 & G4
G1: low risk and G2-4: high risk

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4
Q

Which STS is the most common?

A
  • Liposarcoma (makes up 20% of all STS)
  • mesenchyme neoplasm from lipoblasts
  • 5 different subtypes
  • occur subcutaneously in visceral fat and
    retroperitoneum
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5
Q

What are the 5 types of liposarcoma?

A
  • atypical lipomatous tumour (40-45%)
  • de-differentiated liposarcoma
  • myxoid liposarcoma
  • pleomorphic liposarcoma
  • pleomorphic myxoid liposarcoma
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6
Q

What is a atypical lipomatous tumour?

A
  • 40-45%
  • rarely metastasise: considered benign/precancerous
  • may de-differentiate to more severe types
  • Presence in paratesticular, oral cavity, orbit etc.
  • genetic analysis for dx - esp for DDx from Lipoma
  • surgery is treatment of choice
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7
Q

What is dedifferentiated lipossarcoma?

A
  • 10 % of Liposarcoma
  • mostly in elderly
  • unusually slow growing
  • painless mass
  • commonly in retroperitoneum
  • high degree of local relapse
  • metastasises in lung
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8
Q

What is mxyoid liposarcoma?

A
  • 30% of liposarcoma
  • extremely rare aggressive type
  • found in deep parts e.g. mediastinum, extremity of head/neck
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9
Q

What is a leiomyosarcoma?

A
  • smooth cell origin neoplasm
  • often involves uterus, abdomen, small bowel
  • chemo and radio resistant
  • surgery is best tx when possible
  • can be dormant for long time then suddenly active
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10
Q

What is undifferentiated pleomorphic sarcoma (UPS)?

A
  • sarcoma of uncertain undifferentiation
  • deep tissue sarcoma, painless but aggressive, fast growing
  • highly metastatic
  • Dx is by process of elimination of other sarcoma
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11
Q

What is rhabdomyosarcoma?

A
  • sarcoma of mesenchymal
    rhabdomyoblast cells
    -very aggressive
    • chemosensitive
    • metastasis in lungs and bones (lymph is rare)
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12
Q

What is a malignant peripheral neural shift tumour?

A
  • malignant schwannoma, earlier neurogenic sarcoma
  • highly aggressive
  • highly metastatic
  • high relaps
  • poor prognosis due to chemo and radio resistant
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13
Q

What is synovial sarcoma

A
  • Cell of origin is uncertain
  • One of STS that has mild or high chemosensitivity (monophasic at first, while biphasic has poor response)
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14
Q

What are other rare types of STS?

A
  • fibrosarcoma
  • angiosarcoma
  • sarcoma of uncertain undifferentiated
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15
Q

What is the treatment strategy for localised resectable STS?

A

Grade 1
1) Surgery
R0 resection: optional RT if not given pre-op and FU
R1: see if R0 is feasible = resect, if not resectable give RT and FU

Grade 2/3:

High risk:
- optional Cht and RT
- surgery
- see if R0 or R1 and use Grade 1

Low/intermediate risk:
- optional RT
- surgery
- see if R0 or R1 and use Grade 1

*if high risk: optional adj Cht in grade 2/3

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16
Q

What is the treatment strategy for localised unresectable STS?

A
  • Cht +/- RT
  • R0/R1 then surgery plus RT
  • FU

if R0/R1 resection not possible then advanced stage treatment

17
Q

What is the treatment strategy for advanced/metastatic resectable STS?

A

if multiple metastatic: treat as unresectable metastatic STS

if isolated metastases (one of the following)
1) resectable -> surgery
2) high risk and resectable -> surgery + adj cht
3) complete unresectable then treat as unresectable metastatic sts

18
Q

What is the treatment strategy for advanced/metastatic unresectable STS?

A
  • Doxorubicin
  • Doxorubicin + Ifosfamide
  • Doxorubicin + dacarbazine

Partial response/stable disease:
continue until max dose progression

No partial response/stable disease:
histological-driven Cht
(if after partial response -> treat accordingly)

19
Q

What are the risk factors for bone sarcomas?

A
  • Previous bone irradiation
  • paget’s diease
  • hereditary e.g. Li-Fraumeni syndrome, Werner’s syndrome or retinoblastoma
20
Q

What is the diagnostic algorithm for bone sarcoma?

A

1) X ray
2) MRI +/- CT
3) Biopsy
4) Histology and Molecular assessment
5) definitive diagnosis

*staging based on the information above and MRI of whole body via MDT

21
Q

What is osteosarcoma?

A
  • most common type of bone sarcoma
  • mostly in children, but can affect every age
  • mostly localised in knee, can include hip
  • Puffiness and pain in region
  • Dramatic spread to lungs and other bones
  • Increased LDH and ALP : prognostic markers
22
Q

What is Ewing sarcoma, CF and Lab findings?

A

-highly malignant tumour arising from neurectodermal cells
- due to translocation of EWSR1 gene on chr. 22

CF:
- fever
- sweats
- localised pain (worse at night)

Labs:
- leukocytosis
- raised ESR, CRP and fibrinogen

*genetic test is mandatory

23
Q

Where does Ewings metastasise to?

A

Primary predominantly begin in axial skeleton (pelvis most commonly)

  • lungs
  • other bones
  • bone marrow
24
Q

What is a chondrosarcoma?

A
  • arises from chondroblasts being cartilaginous
  • chemo and radio resistant, surgery is mostly indicated if resectable
  • If metastatic, it grows slow
25
Q

What is a chordoma?

A
  • notochorda origin, most frequent in the pelvic bones, skull base, chest

Slow evolutive massive tumors,

-radio and chemoresistant
- low metastatic potential except if high-grade

  • Only surgical removal has benefit if performed on time
  • In a case of R1 resection RT may be used
26
Q

What is the treatment plan for Ewings?

A

Localised or metastatic:

Induction involves: VIDE
Vincristine, Ifosfamide, Doxorubicin, Etopozide

Neoadjuvant tx

Surgery/RT

Adjuvant Tx

*High Dose Cht or whole lung RT for some cases

Unfavourable cases: salvage therapy followed by auto stem cell transplant

27
Q

What is the treatment of osteosarcoma?

A

Cisplatine+Doxorubicine+Etopozide+
Ifosfamide+ high dosemethotrexate (leucovorine given for protective effects against MTX)

Low grade: straight surgery

High grade: unresectable= Cht RT
Resectable Localised
Neoadj, Surgery, Adj

Resectable Pulmo Metastatic:
Neoadj, Surgery, Adj and Metastectomy

28
Q

Types of Rhabdomyosarcoma?

A

Types:
- embryonal (H&N)

  • botryoid (urogenital): favourable
  • alveolar (extremities, peritoneum): aggressive
  • pleomorphic (deep tissue - most aggressive) poor prog.
  • spindle/sclerosing (paratesticular): similar to leiomyo and favourable prog
29
Q

What is angiosarcoma?

A
  • vascular tumour of blood/lymph vessels
  • endothelial origin
  • mostly elderely on skin e.g.
  • visceral forms e.g. liver, breast, heart angioS
  • Deep tisse angioS and primary brain angioS
  • Poor prognosis
30
Q

What is fibrosarcoma?

A
  • rare tumouor of fibroblasts or anaplastic spindle cells
  • infantile or adult forms
  • resembles other STS = difficult to Dx
  • can appear anywhere = osseal or extraosseal
  • metastasis is possible
  • grade determines aggressiveness
31
Q

What are sarcomas of undifferentiated origins?

A
  • clear cell sarcoma : cutaneous
  • intimal cell sarcoma: initima of large blood vessels
  • atypical teratoid: neonate CNS
  • desmoplastic small blue round cell tumour: intraA
32
Q

Examples of extraskeletal osteosarcoma and ewings?

A
  • liver osteoS
  • osteo of frontal mediastinum
  • retrobulbar ewings
  • ewings of thigh
33
Q
A

Clinical Oncology (11 decks)

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