Sarcoma&nbsp;&nbsp;&nbsp;

How do you treat metastatic, wild-type GIST?

Would you give liposomal doxorubicin to a patient with recurrent left thigh atypical spindle cell lipomatous tumor, low to intermediate grade, that is not a surgical or radiation candidate?

Pathology did not meet criteria for a well-differentiated nor dedifferentiated liposarcoma, nor a spindle cell sarcoma.

How do you approach treatment of recurrent histiocytic sarcoma in a young, healthy patient after surgical resection?

When should adjuvant chemotherapy be offered to patients with soft tissue sarcoma of the extremity?

With conflicting data from the EORTC RCT and the Sarcoma meta-analysis collaboration, what factors determine when you would offer adjuvant chemotherap...

When chemotherapy is indicated in early stage soft tissue sarcoma, would you prefer to use AD or AI?

AD: doxorubicin + dacarbazine AI: doxorubicin + ifosfamide

How do you incorporate denosumab into the surgical management of giant cell tumor of bone?

Does the degree of resectability affect your management?

What treatment would you recommend for a patient with multi-focal angiosarcoma involving the gastrointestinal tract?

How would you modify therapy for a younger, fit patient versus an older, frail patient? Lesions in stomach, small intestine, colon resulting in bleed...

What is your preferred first line treatment for metastatic follicular dendritic cell sarcoma?

Would you use gem/docetaxel, anthracycline-based regimens or targeted therapies/

What is the role of adjuvant chemotherapy in a desmoid tumor after R2 resection?

Would differentiating between embryonal vs alveolar rhabdomyosarcoma of the nasal cavity change your neoadjuvant chemotherapy regimen or make you consider neoadjuvant radiation in a locally advanced pleomorphic RMS?

40 yo adult patient with a~6cm mass with concern for skull base bony involvement (group III and stage III, no nodal involvement )

Is there any outpatient protocol for cisplatin/doxorubicin neoadjuvant chemotherapy for osteosarcoma?

Is there any role for neoadjuvant chemotherapy in renal leiomyosarcoma?

Very rare tumor with sparse literature

What is the optimal duration for denosumab use in the setting of recurrent, un-resectable giant cell tumor?

Received optimal pre-op denosumab; only option is now amputation

Would you give chemotherapy to a fit patient with myxoid liposarcoma of the knee that is resectable with adverse functional outcomes who will be undergoing neoadjuvant radiation and resection?

If yes, would you offer neoadjuvant or adjuvant, concurrent or sequential?

How would you approach unresectable cutaneous angiosarcoma of the scalp?

What would be your radiation volumes/dose and choice of chemotherapy?

What adjuvant therapy would you recommend for a T3N0, grade 3, undifferentiated sarcoma of the mandible with positive margins that is not amenable to re-resection?

Would you offer adjuvant RT or chemotherapy? If so, what chemotherapy would you prefer?

What is your preferred first-line therapy for metastatic extraskeletal osteosarcoma in a young, fit patient?

AIM or doxorubicin/cisplatin? Would you recommend adding methotrexate?

Outside of a clinical trial, what is your treatment of choice for metastatic melanoma of soft parts or clear cell sarcoma?

Would your treatment approach be similar for a patient with suboptimal performance status?

What is the utility of immunotherapy in management of malignant peripheral nerve sheath tumors?

In particular, is there any data on the efficacy of Ipilimumab/Nivolumab?

Do you routinely perform next-generation sequencing on otherwise "low-risk" GIST to guide adjuvant therapy?

Given that risk definitions are based on size, mitotic activity, location and not necessarily genetics/biology, would an otherwise low-risk GIST, who ...

Would you offer adjuvant chemotherapy to a young adult with undifferentiated embryonal sarcoma of the liver?

What factors make you more likely to offer or omit chemotherapy?

What is the optimal systemic therapy for dedifferentiated chondrosarcoma?

What is the role for adjuvant versus neoadjuvant chemotherapy?

How would you approach therapy for a patient with high-grade sarcoma who is unable to tolerate systemic cytotoxic chemotherapy, but whose tumor revealed NRAS G12A, BRCA1, and MET amplification?

Patient with hepC and advanced cirrhosis with multiple liver mets, but may be able to tolerate oral TKI or PARP. Would you offer this or focus on hosp...

Would you offer neoadjuvant chemotherapy for a large, but recurrent grade 1-2 myxoid chondrosarcoma of knee which is no longer amenable to limb salvage?

Performance status is excellent. Second surgical opinion has also confirmed need for amputation, as the recurrence is now breaching intra and extra ar...

In a patient who experienced less than 90% necrosis after neoadjuvant chemotherapy for localized, high-grade osteosarcoma, do you recommend adjuvant ifosfamide?

Osteosarcoma conventional type of the distal right femur. cT1N0M0 G3 Stage IIA on MAP protocol completed NACT followed by surgery on radical resection...

How would you manage a patient with isolated metastatic high grade sarcoma to inguinal and external nodes in a patient with BRCA+ mutation?

This patient had resection of the primary, requiring multiple resections to obtain an R0 resection, followed by adjuvant radiation to the primary. The...

Which adjuvant treatment(s) would you recommend for an incompletely resected, high grade osteoblastic osteosarcoma of the jaw?

Is chemoradiation a viable strategy in osteosarcoma?

4 Answers available

How do you approach contraception and pregnancy counseling in women of childbearing age with Desmoid tumors who may be contemplating future pregnancy?

Since desmoid tumors are associated with high estrogen states, is there an optimal contraception approach? Is there an optimal time for pregnancy? How...

For cases of foraminal and extraforaminal monophasic synovial sarcoma post excision with note of residual tumor, would your approach be a second look surgery with re-excision or delivery of adjuvant RT with boost to residual disease?

Would you consider doing a pre-op RT prior to possible re-excision? If yes, what dose would you give? If the location is between L4-L5 spine.

At what lower end of recurrence risk do you offer adjuvant imatinib for patients with resected GIST?

What is your preferred risk stratification model? There does not appear to be a consensus among experts as to what cutoff in recurrence risk ju...

What is your preferred first line systemic therapy for metastatic chordoma?

Mets to spine and s/p RT

How would you treat a patient with recurrent malignant perivascular epithelioid cell neoplasm (PEComa) arising from the kidney and metastatic to lungs?

How do you modify therapy for patients with severe intolerance to imatinib in the adjuvant setting for GIST?

Do you switch to alternative TKI? Dose reduce?

Is there a role for adjuvant systemic therapy (in addition to adjuvant radiation) in an early-stage, resected cutaneous angiosarcoma of the head/neck?

Post-auricular cutaneous angiosarcoma, pT1 (19 mm) with negative margins. Unclear if there is any indication for systemic therapy, given early stage a...

Would you offer immunotherapy for recurrent metastatic Ewing sarcoma if the tumor is MSI high or has high TMB?

What is the best treatment approach for fit, young patients with extraskeletal Ewing Sarcoma in the lung?

Assuming no access to clinical trial

How do you approach adjuvant therapy for a resected gastric GIST when the risk for recurrence is discordant between various prediction tools?

For example, gastric GIST, < 5 mitoses/hpf and size > 5 cm but < 10 cm is intermediate by NIH criteria but low by NCCN/CAP criteria. Th...

What is your typical approach for soft tissue sarcomas of the buttock?

If post-op with clear margins would you prefer adjuvant RT or close observation with surgery for optimal reduction of local recurrence? This particula...

Would you offer adjuvant chemotherapy for patient with a large, high-grade leiomyosarcoma of the colon who underwent an R0 resection with no lymph node involvement?

> 7cm, grade 3. If chemotherapy offered, what is your preferred adjuvant therapy?

What adjuvant therapy, if any, would you offer for a high-grade perivascular epithelioid cell (PEComa) tumor of the endometrium after R0 resection?

>10 cm, no metastatic disease identified on staging scans

What is the optimal neoadjuvant systemic therapy dosing and schedule in a patient with undifferentiated pleomorphic sarcoma of the extremity for whom up-front surgery would portend significant morbidity?

How do you sequence systemic therapy with radiation and surgery? For e.g. how many cycles of ifos/doxorubicin do you administer in this context?

What is your approach to adjuvant systemic therapy for radiation-induced epithelioid angiosarcoma of the chest wall after successful margin-negative resection?

Nearly 11 cm in size

How would you approach therapy for a SMARCA4-deficient undifferentiated sarcoma with isolated involvement of a mediastinal lymph node?

No primary thoracic mass identified. Knowing these often following an aggressive clinical course, would you consider concurrent chemo/RT, incorporate ...

What is the maximum cumulative anthracycline dose you would administer in a patient with metastatic soft tissue sarcoma who is asymptomatic with good disease control?

Do you routinely offer chemotherapy after resection of solitary or oligometastatic pulmonary nodule/s in young patients with previously treated soft tissue sarcoma who are otherwise without any evidence of disease?

How does histology, time to distant Mets affect your decision?

Do you have a preferred systemic therapy for unresectable epithelioid hemangioendothelioma?

In patients with symptomatic disease that observation would not be appropriate, what is your preferred treatment?

How would you approach therapy for a solitary fibrous tumor of the orbit with residual disease (10%) after surgery?

Which Radiation modality, if any, would be preferred? Typical chemotherapy does not work for SFT. In which circumstance would you use VEGF ( Temozolo...

How would you approach therapy in a young adult with synovial sarcoma of the head and neck who is not amenable to surgery due to location on the face?

What systemic therapy would you recommend for metastatic pleomorphic dermal sarcoma with a large inoperable solitary lung metastasis that is too large for radiotherapy?

Potentially, if metastasis responds, could be treated with surgery or radiotherapy

How would you approach therapy for a young, fit patient with alveolar rhabdomyosarcoma involving the anterior nasal vault/sinuses in the absence of available clinical trials?

Presented with mass projecting into right/left anterior ethmoid complex

Would you offer adjuvant therapy following successful resection of a solitary lung recurrence of leiomyosarcoma if no other evidence of recurrent disease on PET CT?

Would you offer adjuvant avapritinib or imatinib for a high-risk, PDGFR D842V-mutated GIST after resection?

Data suggest patients with D842V-mutated or KIT/PDGFRA WT GIST do not respond to imatinib, but NCCN guidelines does not take into consideration mutati...

Would you offer adjuvant chemotherapy for a large, high-grade radiation-induced malignant peripheral nerve sheath tumor following R1 resection?

>5cm. If so, what is your preferred regimen?

Would you consider neoadjuvant ifosfamide-based chemotherapy for a large (>10cm) high grade soft tissue extremity sarcoma in an attempt to achieve limb sparing surgery in an otherwise healthy young patient?

Pleomorphic sarcoma

How would you approach therapy for an elderly patient with NF1-associated metastatic MPNST who remains asymptomatic?

Any strong considerations for infusional vs. bolus doxorubicin?

What is your approach to unresectable metastatic rhabdomyosarcoma transformed from an embryonal cell germ cell tumor?

Should treatment be based on current histology (rhabdomyosarcoma) or origin (germ cell)?

How would you approach adjuvant therapy for resected spindle cell rhabdosarcoma of the tongue?

Margins are negative (closest 4mm), grade 2, 3 of 23 lymph nodes positive with no extranodal extension. What is your preferred chemotherapy approach a...

What is your approach to systemic treatment of a rapidly progressing, symptomatic, unresectable MPNST arising from Plexiform Neurofibroma in a patient with NF1?

Would you consider using recently approved agent for symptomatic / unresectable PN, Selumetinib prior to Systemic Chemotherapy in this setting?

What chemotherapy would you recommend for a patient with Ewing Sarcoma who has relapsed after being initially treated with vincristine/doxorubicin/ifosfamide?

Outside of a clinical trial, what is your preferred first-line therapy for a fit patient with de novo metastatic spindle cell sarcoma?

What role, if any, does molecular testing play in diagnosis and management in the absence of available clinical trials?

How are you modifying your current sarcoma-directed therapy in the setting of the COVID-19 pandemic?

For example, would you consider switching to an outpatient regimen in lieu of AIM for metastatic soft tissue sarcoma?

What is your preferred next line of therapy for a fit patient with unresectable, refractory osteosarcoma who has progressed after MAP, local radiation, and IE?

Would you consider regorafenib based on the REGOBONE trial? Or would you consider other agents knowing that we do not have positive survival data with...

What therapy would you recommend for metastatic myxoid liposarcoma in a young patient with anthracycline cardiomyopathy with EF 25% who failed eribulin?

Would you even consider trabectedin in this case given its cardiotoxicity?

What is your preferred second-line therapy for metastatic follicular dendritic cell sarcoma after progression on anthracycline-based chemotherapy?

Sarcoma arose from Castleman disease

What is your preferred treatment for a resected composite hemangioendothelioma of the skin arising from a childhood hemangioma?

Tumor 13 cm on back with retiform and kaposiform features; all margins negative except one abuts inked margin; atypical cells with 4 mitosis/HPF. Add...

How do you treat recurrent dedifferentiated liposarcoma after initial complete resection if the recurrence is multifocal but surgically resectable?

Do you proceed to resection or palliative chemotherapy?

What is your preferred initial treatment for metastastic primary lung liposarcoma in a fit patient?

How do you choose between single-agent or combination chemotherapy versus newer treatment strategies such as palbociclib?

How do you approach treatment for solitary fibrous tumor with a multifocal pleural-based metastatic recurrence that is surgically inoperable in a patient with good performance status?

How, if any, do you utilize genomic testing to guide systemic therapy?

How do you approach pathologic review and genomic testing, if indicated, of a spindle cell neoplasm?

For example, if a biopsy reads: lesion comprised of spindle cells arranged in a fascicular pattern, elongated, hyperchromatic nuclei, focally positive...

Outside of a clinical trial, how do you approach upfront therapy for a PDGFRA D842V-mutant GIST?

Would your recommendations change in the neoadjuvant versus adjuvant or metastatic setting?

How do you approach G-CSF use when offering neoadjuvant chemoRT for lower extremity soft-tissue sarcoma?

i.e. doxorubicin, ifosfamide + RT. Do you worry about decreased locoregional control or increased toxicities with the use of G-CSF in combination with...

How do you treat unresectable localized desmoid tumor?

How do you counsel patients with localized Ewing's sarcoma who wish to stop or cannot complete the full course of 14 cycles of perioperative chemotherapy?

How do you approach non-surgical patients with GIST who are intolerant to imatinib 400 mg daily and unlikely to tolerate other TKIs?

Do you reduce the dose to <400 mg daily or employ dosing schedules?

What is your preferred neoadjuvant chemotherapy course for healthy adult patients with osteosarcoma?

Is there an age limit you use to consider MAP? Would you consider using AIM instead?

How do you choose your next line of therapy for stage 4 GIST after progression on imatinib?

How do you choose between local therapy (surgical debulking or ablation) v. 2nd line systemic therapy? Would you consider immunotherapy?

How do you treat GIST with de-differentiation to pleomorphic sarcoma on chronic therapy with imatinib?

How do you treat if adriamycin is not an option?

Are there any types of sarcomas that you use induction and/or concurrent chemotherapy with radiation prior to surgery?

Specifically concurrent ifosfamide? Induction AIM?

What is your preferred systemic therapy for metastatic malignant peripheral nerve sheath tumor with early relapse after adjuvant AIM?

Given metastatic disease and not a trial candidate, how would you approach this scenario? How does an underlying diagnosis of NF1 affect your decision...

How do you approach adjuvant treatment of non-uterine leiomyosarcoma?

When, if ever, do you utilize adjuvant RT or chemotherapy?

For patients who received neoadjuvant Imatinib for initially unresectable GIST tumours, do you routinely prescribe adjuvant therapy or rely on preoperative staging and preoperative mitotic index?

NCCN suggests to consider continuation of imatinib from some single/multi institution non-randomized trials for ~2 years, but does not suggest what fa...

Is there a preferred neoadjuvant chemotherapy regimen for a marginally-resectable, dedifferentiated, MDM2+ liposarcoma?

How would you treat someone with unknown primary metastatic SMARCA4 deficient tumor (high-grade tumor with rhabdoid morphology)?

What is a preferred regimen for metastatic leiomyosarcoma after failure of doxorubicin/olaratumab and gemcitabine/docetaxel?

No actionable mutations were identified on next generation genomic sequencing.

Would mild hemophilia A that does not require frequent factor replacement be an absolute contraindication to anti-VEGF medications when indicated?

How would you treat metastatic, unresectable, solitary fibrous tumor in a patient with mild hemophilia A? Each anti-VEGF medication comes with a warni...

What adjuvant therapy, if any, do you consider for high grade extraskeletal osteosarcomas after R0 resection?

Do you use chemotherapy or radiation?

How do you treat metastatic mesenchymal chondrosarcoma?

How does grade or performance status affect your treatment decisions?

How would you approach adjuvant chemotherapy for high grade pleomorphic/dedifferentiated liposarcoma?

Does anatomic location (eg retroperitoneal or thoracic) affect your treatment decision?

Do you recommend stopping olaratumab in patients receiving doxorubicin/olaratumab for treatment of advanced sarcoma?

Given the recent press release regarding the update on the phase 3 soft tissue sarcoma study of LARTRUVO (olaratumab) showing no benefit to the combin...

When do you give exogenous albumin in an attempt to reduce risk of ifosfamide induced encephalopathy?

What alternative adjuvant therapy do you recommend to an imatinib intolerant patient with high-risk GIST?

I have seen favorable clinical trial reports on dasatinib for metastatic disease, but no data on possible adjuvant use. NCCN guidelines suggest ...

How do you approach need for adjuvant therapy after R0 resection of breast angiosarcoma?

What other pathologic features may you use to guide therapy decisions (eg mitoses/grade or NGS)? If you decide on adjuvant therapy, what regimen...

What is the standard of care (if any) for pediatric desmoplastic round cell tumor?

Is there a role for immunotherapy outside of trial setting?

Would you offer imatinib to a patient with a high risk GIST (> 10 cm, low Ki-67, s/p complete resection) who initially declined adjuvant therapy, but is now interested 14 months post surgery?

Is there any data to suggest delayed initiation of adjuvant treatment would be beneficial? Or would close surveillance be reasonable? Patient is asymp...

How would you treat metastatic malignant peripheral nerve sheath tumor in a patient who received adjuvant Adriamycin ( 240 mg/m2) 10 years ago for breast cancer?

Patient currently has bone and lung metastasis.

Which systemic therapy would you use to treat a poorly-differentiated round cell sarcoma with CIC-DUX4 gene fusion?

CIC-DUX4 fusion sarcoma is a rare entity, the aggressiveness of which is comparable to Ewing's sarcoma. A major dilemma is whether to treat these pati...

What is your treatment approach to patients with extremity sarcoma with multiple positive regional nodes?

Do you offer neoadjuvant RT with resection and node dissection vs. definitive radiation? How extensive does the nodal disease have to be for you...

How do you treat a locally-invasive, keratin-positive gastrointestinal spindle-cell neoplasm?

Tumor involves stomach, liver, and lymph nodes. Pathology is suspicious for sarcomatoid carcinoma, possibly sarcomatoid cholangiocarcinoma.

What treatment options would you consider for a patient with an ECOG PS of 0 with metastatic non-uterine leiomyosarcoma who has progressed on all standard systemic therapies including anthracycline, gemcitabine, taxane, vinorelbine, pazopanib, temozolomide, and trabectidin?

Are there clinic trials that look promising for patients with leiomyosarcoma?

What third and fourth line options do you consider for patients with pleomorphic sarcoma and a good performance status who have progressed on standard anthracycline and gemcitabine/docetaxel?

Should all GISTs be sequenced?