Sarcoma Flashcards
Clinical behavior of desmoid tumors
Don’t metastasize but are locally aggressive
Precipitants of desmoid tumors
1) High estrogen states - during or following pregnancy (extra-abdominal or abdominal)
2) Trauma
3) FAP
Clinical course of desmoid tumors
highly variable clinical course (stable to spontaneously regressing to slow growing to rapidly progressive)
Management of desmoid tumor
- IF asymptomatic or minimally symptomatic, initial period of observation (Let disease biology declare itself. Significant rate of spontaneous regression and periods of growth arrest. Placebo arm had a 20% response rate in sorafenib trial).
- IF causing significant symptoms, multimodality evaluation
Management of gross residual disease after resection of a desmoid tumor
- Observation or RT rather than more surgery
Management of large intra-abdominal desmoid tumors in the setting of Gardner syndrome
- Surgery still preferred
Klinefelter presentation
- tall
- micropenis
- hypogonadism (small firm testes) and signs of androgen deficiency (gynecomastia, sexual dysfunction, osteoporosis)
- learning and developmental disabilities
New drug approved for RET translocated sarcoma
selpercatinib
New drug approved for desmoid tumors
nirogacestat
Drug to know for giant cell tumor of bone
denosumab
Drug recently approved for alveolar soft part sarcoma
atezolizumab
Drug to know for tenosynovial giant cell tumor + major SE to be aware of
- pexidartinib
- highly hepatotoxic, requiring REMS
Drug approved for advanced epithelioid sarcoma
tazometastat
Osteosarcoma age distribution
bimodal (smaller peak in 60-80)
Radiographic features of osteosarcoma
- sunbursting (see google image)
- codman triangle (see google image)
Biopsy required for osteosarcoma
- incisional or core always required (never FNA, need architecture in sarcoma, similar to lymphoma)
- always done by sarcoma expert (biopsy track has to be removed surgically)
Osteosarcoma preferred staging modalities
- MRI of primary location
- CT chest (sarcoma can have hematogenous seeding to lung)
- bone scan (can have skip mets along bones)
Major prognostic indicator in osteosarcoma
- response to neoadjuvant chemotherapy
*greater than 90% osteonecrosis after 3 cycles has highest relapse free survival
Management of high grade localized osteosarcoma (most are high grade)
neoadjuvant chemo
Surgery
+/- adjuvant
Management of low grade localized osteosarcoma
surgery alone
Neoadjuvant systemic therapy for osteosarcoma
3 cycles of doxorubicin + cisplatin
*high dose methotrexate can be added if young with good renal function
First line chemotherapy for metastatic osteosarcoma
Doxorubicin based
Cisplatin/doxorubicin without high dose methotrexate
Second line for metastatic osteosarcoma
Ifosfamide +/- etoposide
Oligometastatic paradigm exist in osteosarcoma
- Yes, for limited number of lung mets
Systemic therapy for localized ewing sarcoma
- cyclophosphamide + doxorubicin + vincristine alternating with ifosfamide + etoposide (VACIE)
12-14 cycles neoadjuvant and adjuvant
Radiographic finding of ewing sarcoma
- infiltrative pattern looking like onion skinning (see image online)
What to do when cumulative maximum of doxorubicin has been reached in osteosarcoma patient
Replace doxorubicin with actinomycin D
Management of localized bone sarcoma if surgery infeasible
Radiation
Management of metastatic ewing sarcoma
Same as osteosarcoma
Second line for ewing
topotecan combinations
temodar + irinotecan
topotecan + cytoxan
chondrosarcoma radiology buzzword
- scalloped edging
localized chondrosarcoma management
- surgery alone (chemo and radioresistant tumors)
metastatic chondrosarcoma systemic therapy options
- pazopanib
- dasatinib
targeted therapy options for chondrosarcoma
- IDH inhibitors
Management of dedifferentiated chondrosarcoma
- osteosarcoma chemo regimen
Management of mesenchymal chondrosarcoma
- Ewing’s regimen