Sarcomas Flashcards
SDH deficient GIST
Insensitive to imatinib- use sunitinib or regorafenib
Localized, clinically resectable, extremity and superficial trunk soft tissue sarcoma
(Tx overview)
Grade 1: surgery +/- radiation
Grade 2-3: surgery +/- radiation and chemo before and after surgery
Localized, clinically unresectable, extremity and superficial trunk soft tissue sarcoma
(Tx overview)
Neoadjuvant chemo +/- RT
—-> surgery if now resectable, otherwise tx as advance dx
Advanced/metastatic clinically resectable soft tissue sarcoma
(Tx overview)
Isolated Mets: surgery +/- chemo
Mx Mets: tx as advance unresectable
Advanced/metastatic, unresectable soft tissue sarcoma
(Non-specific histology)
Chemo, options are:
1. Doxorubicin (better for less fit patients)
2. Doxorubicin + ifos + Mesna (AIM)
*give with g-CSF
Others:
-doxorubicin + Dacarbazine (AD)
- ifos + Epirubicin + Mesna
-liposomal dox
-Epirubicin
Advanced/metastatic, unresectable soft tissue sarcoma
(Non-specific histology)
And cannot tolerate doxorubicin
Gemcitabine + docetaxel (or Vinorelbine or dacarbazine)
infuse Gemcitabine at 10 mg/m2/min -increases efficacy has not been proven but this is how it was studied
Advanced/metastatic, unresectable soft tissue sarcoma. non-liposomal
(Non-specific histology)
SECOND LINE
Pazopanib
Advanced/metastatic, unresectable soft tissue sarcoma. Liposomal
(Non-specific histology)
Eribulin or trabectedin
(use 2 lines of therapy first including anthracycline)
Advanced/metastatic, unresectable soft tissue sarcoma. Leimyosarcoma.
(Non-specific histology)
Trebectedin
Soft tissue sarcoma neoadjuvant
Dox + ifos + Mesna (AIM)
(Or Epirubicin instead of dox)
*give with g-CSF
Soft tissue sarcoma neoadjuvant
Unable to do doxorubicin
Gemcitabine + docetaxel
Soft tissue sarcoma: Desmoid tumors (aggressive fibromatosis)
Observation!!!
-Sorafenib (cat 1)
-nirogacestat (cat 1)
Others:
-Mtx + vin
-imatinib
-liposomal dox
-dox + Dacarbazine
-Pazopanib
Associated with Gardner syndrome (APC gene mutation)
Soft tissue sarcoma: non-pleomorphic rhabdomyosarcoma
Vincristine + dactinomycin + cyclophosphamide (VAC)
Soft tissue sarcoma: alveolar soft part sarcoma
-sunitinib
-Pazopanib
-pembrolizumab
-pembro + axitinib
-atezolizumab
Soft tissue sarcoma: angiosarcoma
Paclitaxel
Or doxorubicin
Soft tissue sarcoma: dermatofibrosarcoma transformation (DFSP)
Imatinib
Soft tissue sarcoma: Epithelioid sarcoma
Early stage: surgical resection
Metastatic: Tazometostat 800 PO BID
Extra skeletal osteosarcoma
-ifos
- or doxorubicin + cisplatin
“I am a CD”
Dedifferentiated chordoma
Treat as general soft tissue sarcoma (anthracycline OR Gemcitabine based regimens )
Soft tissue sarcoma: inflammatory myofibroblastic tumor (IMT) with anaplastic lymphoma kinase (ALK) translocation
-alectinib
-Brigatinib
-ceritinib
-crizotinib
-lorlatinib
Soft tissue sarcoma: malignant perivascular epitheloid cell tumor (PEComa)
Albumin based sirolimus
Recurrent angiomyolipoma
OR
lymphangioleiomyomatosis
-sirolimus
-everolimus
-temsirolimus
Soft tissue sarcoma: solitary fibrous tumor
-bevacizumab + temozolomide
-sunitinib
-sorafenib
-pazopanib
Tenosynovial giant cell tumor/pigmented villonodular synovitis
Pexidartinib