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.
