Sarcoma Flashcards
Types of sts
Translocation associated - young30-50
Complex karyotype- 50-60
TRANSLOCATION ASSOCIATED
SARCOMAS
Myxoid/ Round cell Liposarcoma Ewing's sarcoma . Synovial sarcoma Desmoplastic small round cell tumour Alveolar Rhabdomyosarcoma Alveolar soft part sarcoma DFSP Extraskeletal myxoid chondrosarcoma
Complex karyotype sts
Well Well differentiated and Dedifferentiated liposarcoma Pleomorphicliposarcoma Myxofibrosacoma Undifferentiated Pleomorphic sarcoma Leiomyosarcoma MPNST Angiosarcoma
What is the most common radiation induced sarcoma
HISTOLOGY Undifferentiated pleomorphic sarcoma 26% Angiosarcoma 21 Fibrosarcoma 12 LMS 12 MPNST 9
4. What is the life time risk of MPNST in Neurofibromatosis-I(17q 11.2) a. 10% b. 17% c. 31% d. 53%
10% of patients with neurofibromatosis are
thought to develop STS.
Most common histologic type in Li-Fraumeni
syndrome
RMS - 55%
Fibrosarcomas - 13%
• UPS - 10%.
Rb gene associated sts m/c
LMS
Chemical agents sts
Arsenic
Thorotrast
Vinyl chloride
Intermediate metastatic tumors
DFSP hemangiopericytoma Kaposi Myoepithelial ca Inflammatory myofibroblastic tr Infantile fibrosarcoma
The prevalence of pulmonary metastases among patients previously treated for extremity STS approximately
19%
Ledderhose disease implies
Plantar fibromatosis
——% of FAP patients develop desmoid tumors.
20
Dfsp
Adjuvant rt useful when
Cd34+ COL1A1 PDGFRB translocation Mononuclear spindle cells Margin 2 cm or more Art when vlose margins or rec disease
Bednar tumor
Melanin pigmented DFSP
RT IN DFSP
Radiation is useful in
a. Recurrent disease
Close or positive surgical margins.
Sts Ntrk inhibitor
Characterized by ETV6-NTRK3 fusion NTRK inhibitors such as larotrectinib. Surgery is the primary modality. Fibrosarcoma Herring bone pattern
Vhich of the following is part of the FNCLCC
grading of soft tissue sarcoma?
a. Differentiation score
b. Necrosis score
C.
Mitotic score
Regional lymph node involvement for STS is
uncommon (2% to 10%).
Angiosarcoma Rhabdomyosarcoma • MFH, •Epithelioid sarcoma Clear cell sarcoma • Liposarcoma. CLEAR MFH
Visceral sarcoma T stage
1- organ confined 2- extend into tissue beyond organ A- serosa or viscer peritoneum B- beyond serosa 3- another organ 4- a multiple -2 b- 3-5 c- >5 sites
Drug used in inflammatory fibrobalstic tumor
a. Crizotinib
b. Imatinib
c. Rituximab
- Doge- Potter syndrome occurs with
Patients present with a very large SOlitary Fibrous Tumor
and
hypoglycemia
(Doege-Potter
syndrome),
which is associated with production of a form
of IGF2 by these tumors.
Mri features of liposarcoma
Adipose<75%
Thick septa>2mm
No adipose areas
Size>10Cm
- Which type of liposarcoma is susceptible for
unusual sites of metastases?
- ALT/WDLS- supernumerary ring 12q14-15
- Dedifferentiated liposarcoma (DDLS)
- MYXOID ROUND CELL✅-40%-FUS DDIT3 transloaction in 90%
- Pleomorphic <5%
Which are the places leiomyoma can arise?
Blood vessels
Skin
Uterus
Gastrointestinal tract.
M/c sts in children
Rhabdomyosarcoma
Types of rhabdomyosarcoma
Embryonal -VAC Regime
Alveolar- PAX3-FOXO1 fusion transloction
And pleomorphic- m/c form in adults
What is true about extraskeletal
osteosarcoma?
> 50yrs
- Which of the tumours are PEComas?
a. Lymphangioleiomyomatosis
b. Angiomyolipomas
c. Clear cell “sugar” tumors of the lung
a& b Associated with tuberous sclerosis
Melanoma of soft parts
Clear cell sarcoma
Brain imaging in sts done in
Alveolar soft part sarcoma,
Clear cell sarcoma, and angiosarcoma.
Kawaguchi criteria
Thick barriers-3 cm lliotibial band Presacral fascia Joint capsule. Periosteum of an infant or young child. Thin- 2 cm muscle fascia, adult periosteum, vessel sheath, epineurium Growth plate : Infant or young child - Thick barrier. • Linear appearance -Thin barrier. JOINT CARTILAGE- 5 cm
Most common rp sarcoma
Liposarcoma 60%
Lms- 22%
Indications for post-operative radiotherapy in sts
All high grade lesions
All recurent lesions
• Low grade lesions if deep seated /or 2 Scm/or margin+
ClasicalTikhoff linberg resection
Type 4
Extra articular total scapulectomy and humeral head resection
Removing part of sacrum which type of hemipelvectomy
Type 4
Type1- iliac crest
2- ischium
3- pubis
Periosteal isteosarcoma m/c
Ant tibial shaft
Paraosteal os
Distal femur
Clear cell chondrosarcoma m\c site
Femora head
Ewsr1-FLI1 fusion
22 -11
VAC REGIME
Classification based on response to chemo in bone tr
Picci
Huvos
Bone tr with epithelial component
Adamantinoma Diaphys Tibia Pan cytokeratin + Soap bubble xray
