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