Soft Tissue, Visceral and Bones Tumours Flashcards
What are the risk factors for soft tissue, and visceral tumours
- ionising radiation
- hereditary factors:
Li Fraumeni syndrome
NF type 1
Retinoblastoma
FAP
What diagnostics are used?
- 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
What is the TNM staging for STS?
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
Which STS is the most common?
- Liposarcoma (makes up 20% of all STS)
- mesenchyme neoplasm from lipoblasts
- 5 different subtypes
- occur subcutaneously in visceral fat and
retroperitoneum
What are the 5 types of liposarcoma?
- atypical lipomatous tumour (40-45%)
- de-differentiated liposarcoma
- myxoid liposarcoma
- pleomorphic liposarcoma
- pleomorphic myxoid liposarcoma
What is a atypical lipomatous tumour?
- 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
What is dedifferentiated lipossarcoma?
- 10 % of Liposarcoma
- mostly in elderly
- unusually slow growing
- painless mass
- commonly in retroperitoneum
- high degree of local relapse
- metastasises in lung
What is mxyoid liposarcoma?
- 30% of liposarcoma
- extremely rare aggressive type
- found in deep parts e.g. mediastinum, extremity of head/neck
What is a leiomyosarcoma?
- 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
What is undifferentiated pleomorphic sarcoma (UPS)?
- sarcoma of uncertain undifferentiation
- deep tissue sarcoma, painless but aggressive, fast growing
- highly metastatic
- Dx is by process of elimination of other sarcoma
What is rhabdomyosarcoma?
- sarcoma of mesenchymal
rhabdomyoblast cells
-very aggressive- chemosensitive
- metastasis in lungs and bones (lymph is rare)
What is a malignant peripheral neural shift tumour?
- malignant schwannoma, earlier neurogenic sarcoma
- highly aggressive
- highly metastatic
- high relaps
- poor prognosis due to chemo and radio resistant
What is synovial sarcoma
- Cell of origin is uncertain
- One of STS that has mild or high chemosensitivity (monophasic at first, while biphasic has poor response)
What are other rare types of STS?
- fibrosarcoma
- angiosarcoma
- sarcoma of uncertain undifferentiated
What is the treatment strategy for localised resectable STS?
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
What is the treatment strategy for localised unresectable STS?
- Cht +/- RT
- R0/R1 then surgery plus RT
- FU
if R0/R1 resection not possible then advanced stage treatment
What is the treatment strategy for advanced/metastatic resectable STS?
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
What is the treatment strategy for advanced/metastatic unresectable STS?
- 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)
What are the risk factors for bone sarcomas?
- Previous bone irradiation
- paget’s diease
- hereditary e.g. Li-Fraumeni syndrome, Werner’s syndrome or retinoblastoma
What is the diagnostic algorithm for bone sarcoma?
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
What is osteosarcoma?
- 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
What is Ewing sarcoma, CF and Lab findings?
-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
Where does Ewings metastasise to?
Primary predominantly begin in axial skeleton (pelvis most commonly)
- lungs
- other bones
- bone marrow
What is a chondrosarcoma?
- arises from chondroblasts being cartilaginous
- chemo and radio resistant, surgery is mostly indicated if resectable
- If metastatic, it grows slow
What is a chordoma?
- 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
What is the treatment plan for Ewings?
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
What is the treatment of osteosarcoma?
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
Types of Rhabdomyosarcoma?
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
What is angiosarcoma?
- 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
What is fibrosarcoma?
- 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
What are sarcomas of undifferentiated origins?
- clear cell sarcoma : cutaneous
- intimal cell sarcoma: initima of large blood vessels
- atypical teratoid: neonate CNS
- desmoplastic small blue round cell tumour: intraA
Examples of extraskeletal osteosarcoma and ewings?
- liver osteoS
- osteo of frontal mediastinum
- retrobulbar ewings
- ewings of thigh
