Non Rhabdo Soft Tissue Sarcomas Flashcards
Infantile fibrosarcoma translocation and fusion protein
T(12;15) fusion protein- ETV-NTRK3
Synovial sarcoma translocation and fusion protein
T(X;18) SYT-STX
Mutation associated with Desmond type fibromatosis
APC or CTNNB1- associated with FAP
Alveolar soft part sarcoma translocation and fusion
T(X;17) and ASPL-TFE3
Clear cell sarcoma translocation and fusion
T(2;22) or
t(12;22)-ATF1- EWSR1
Both EWSR1 rearranged
Desmoplastic small round cell tumors translocation
T(11;22)
EWSR1-WT1
What type of soft tissue sarcoma is associated with hypoglycaemia and hypophosphatemia?
Solitary fibrous tumor ( previously hemangioperocytoma)
Genetic syndromes associated with NRSTS
Li frameuni syndrome RB1 ( leiomyosarcoma) FAP- Desmoid type fibromatosis Werner syndrome NF1- MPNST SMARCB1 TSC- angiomtolipomas ADA deficiency- dermatofibrosarcoma protruberans
In which NR soft tissue sarcomas is sentinel LN biopsy recommended
Epitheliod sarcoma
Clear cell sarcoma
Prognostic factors
Size >5 cm Tumor histology Tumor grade Resectability Older age- worse Stage
Risk stratification of NRSTS
Low risk- non metastatic low grade tumor that is completely resected OR
Non metastatic small (<5cm) high grade tumor that is completely resected
( R1 resection allowed)
The latter RT is recommended
Intermediate risk
A larger ( >5 cm) non metastatic high grade tumor that is resected or non resected non metastatic tumor of any grade
Chemo And RT recommended
High risk
Metastatic
Chemo and RT recommended for all high grade
Low grade that is completely resected observation
Inflammatory myofobroblastic tumor cytogenetics
ALK rearrangements
Two types of NTRK inhibitors
Larotrectinib
Entrectinib
Chemo Treatment of RMS?
y
Prognosis
Low risk - 90%
Intermediate- 50-70
High 20
