Sarcoma&nbsp;&nbsp;&nbsp;

Does the presence of anaplasia in embryonal rhabdomyosarcoma change your treatment approach?

How would you approach patients with primary cardiac sarcomas for adjuvant systemic treatment?

How do we approach patients with rare tumors given the paucity of data?

How do you sequence treatment for a patient following R2 resection of a large extremity leiomyosarcoma who has small lung nodules concerning for metastatic disease, but not yet biopsy proven?

How do you approach patients with SDH deficient advanced GIST tumor for systemic treatment?

Would you consider using repretinib as first line therapy given its novel mechanism of action versus other treatment options?

Would you recommend radiation therapy for an adult patient with rhabdomyosarcoma (embryonal) of the upper extremity s/p amputation with multiple lymph node involvement and ENE on pathology?

The patient is in their 40s with right arm rhabdomyosarcoma s/p amputation. Path shows embryonal rhabdomyosarcoma 6.5 cm, margins neg. 11 of 19 lymph ...

How do you approach patients with osteosarcoma of the maxilla for neoadjuvant chemotherapy?

How do you approach a young patient with undifferentiated pleomorphic sarcoma of the upper extremity who developed ifosfamide-induced nephrotoxicity after two cycles of adjuvant AIM chemotherapy?

Would you consider stopping therapy versus doxorubicin + dacarbazine/doxorubicin alone versus alternative chemotherapy ?

Would you offer dose reduction to 36 Gy pre-op to a lower extremity low grade myxoid liposarcoma based on results of the DOREMY non-randomized data?

Would you recommend imatinib 400 mg BID or stay with the usual dose of 400 mg/day for adjuvant therapy for a patient with intermediate-high risk exon 9 mutated GIST?

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 systemic treatment do you utilize for patients with metastatic fibrosarcomatous DFSP that have progressed on imatinib?

Would you prefer doxorubicin based chemotherapy or are alternative TKIs useful in this scenario?

Do you offer neoadjuvant radiation therapy for oral cavity sarcoma?

If so, what dose/fractionation? Pathology: High grade spindle cell sarcoma

Would you recommend post op radiation therapy for a primary pulmonary synovial sarcoma which underwent lobectomy?

Relevant pathology features: 7.5cm, grade 3 with a 5mm bronchial margin, negative nodes.

How do you approach a well controlled HIV patient with diffuse cutaneous kaposi sarcoma who failed to respond to radiation, paclitaxel and monthly liposomal doxorubicin?

When the initial diagnosis of stage I uterine leiomyosarcoma is made via a non-intact resection such as a myomectomy or supracervical hysterectomy, what factors, if any, might push you to consider adjuvant chemotherapy after completion surgery?

For primary angiosarcoma of the breast following mastectomy, what would your targets be for adjuvant radiation therapy?

Would you cover the chest wall alone or would it be chest wall plus regional nodes? Patient factors: no prior radiation therapy

What techniques and dose constraints do you utilize to limit lower extremity neuropathy when providing post op radiation following soft tissue sarcoma resection?

Relevant pathology features: positive margin Total dose prescription: 66 Gy

0 Answers available

When would you ever consider lifelong imatinib in adjuvant therapy for GIST?

Would non-gastric GISTs, high mitotic rate, large size, and young age of patient affect your decision? Especially knowing that TKIs suppress cell grow...

Is there a role for adj RT in a pleomorphic dermal sarcoma after WLE?

If there were negative margins, what other factors would you consider to add adjuvant RT? Size of the tumor or depth of invasion?

How do you approach a patient with newly diagnosed high-grade osteosarcoma who received maximum cumulative dose of doxorubicin for a soft tissue sarcoma 10 years earlier?

How do you integrate surgery in treatment of recurrent tenosynovial giant cell tumors (TGCT) when using Pexidartinib?

What is your local control approach to localized relapse of Ewing sarcoma in a site that was previously unresectable and received definitive radiation?

Do you ever extend adjuvant imatinib for patients with high-risk, localized GIST beyond 3 years?

If so, which patients would you extend treatment?

For unresectable radiation induced angiosarcoma, what dose and fractionation would you use?

Angiosarcoma is invading the brachial plexus and thoracic vertebral bodies. Prior radiation therapy: Radiation to the left chest wall and regional no...

How would you manage primary breast osteosarcoma?

How would you treat a patient with a locally recurrent myxofibrosarcoma, FNCLCC grade 2, that has recurred after multiple resections and radiotherapy?

What is the role of local control +/- whole lung irradiation in a patient with relapsed/refractory Ewing sarcoma to the hilum plus multiple lung nodules?

Patient initially achieved CR with VAC-IE with resolution of presumed lung mets and 100% necrosis in LLE primary tumor on BKA. Then was NED again afte...

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?

Would you give doxorubicin and cisplatin for localized chondroblastic osteosarcoma in a patient with prior childhood anthracycline exposure and resultant cardiomyopathy with EF 50%?

How would you approach an adult patient >50 years old with an intermediate risk extremity T2N0 fusion neg rhabdomyosarcoma who is progressing on neoadjuvant chemotherapy with VAC?

How would you add radiation in your treatment paradigm? No clinical trials available due to age.

What adjuvant therapy would you recommend for a patient with a sarcomatoid carcinoma of the submandibular gland after R1 resection?

No lymph node dissection. cN0

What is the optimal frequency and duration of post-operative radiological surveillance following resection of a solitary fibrous tumor?

Would you offer adjuvant radiation for a low-grade cutaneous leiomyosarcoma after R0 resection?

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?

How would you approach the adjuvant treatment of a stage IVA adenosarcoma of the ovary?

Would your systemic therapy choice be more similar to treatment of ovarian carcinoma or soft tissue sarcoma?

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 a sarcoma that was initially diagnosed as UPS of the distal femur with groin lymph node spread, resected, and found to have an EWSR1-FLI1 fusion diagnostic of Ewing's on NGS?

When would you consider adjuvant radiation therapy for tenosynovial giant cell tumors of the extremity?

Would you treat a knee?

What is your treatment approach for a pediatric patient with uterine embryonal rhabdomyosarcoma found after removal of a prolapsing uterine mass, with no evidence of distant metastasis on imaging?

Would you favor post op radiation therapy for soft tissue sarcoma if a positive margin were still expected despite pre-op radiation?

Tumor factor: Large axillary soft tissue sarcoma with involvement of the brachial plexus Other factor: Surgery would like to pursue upfront surgery t...

In light of the recent rEECur trial interim analysis, would you offer high-dose ifosfamide to an AYA patient with relapsed Ewing's sarcoma who initially had a complete response to up-front therapy?

J Clin Oncol 40, 2022 (suppl 17; abstr LBA2). https://meetings.asco.org/abstracts-presentations/209024

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...

How would you manage an adult patient status post subtotal resection of spinal osteoblastoma?

The patient presented with spinal cord compression, had subtotal resection and instrumentation with metallic hardware. Main concern is that post op su...

How would you approach the adjuvant management of thoracic SMARCA4-deficient undifferentiated tumor of the lung following lobectomy?

4 cm with negative LN

What treatment would you recommend for a patient with recurrent oligometastatic abdominal leiomyosarcoma, with two liver lesions?

No evidence of distant disease elsewhere. Surgical resection is not possible. Would you recommend metastasis direct therapy to the liver (i.e. SBRT or...

What treatment would you recommend to a patient with a locally recurrent sarcoma of the retrohepatic IVC (reconstruction of IVC with PTFE graft) resected over 2 years ago?

This was resected over 2 years ago with reconstruction of the IVC with a PTFE graft. No preop or postop RT given and the recurrence is in the area whe...

What constraints do you use for a non-weight bearing bone when treating a patient with sarcoma?

Do you offer adjuvant radiation therapy for sacral chordoma s/p gross total resection with close margins <0.5 cm?

What dose would you use? For negative margins do you offer 50-50.4 Gy/25-28 fractions? What target volume margins do you typically recommend on t...

Would you offer upfront radiation for a large painful keloid of the chest that has arisen from an irritated pyoderma gangrenosum lesion?

What dose and fractionation do you recommend for an unresectable epithelioid hemangioendothelioma of the rib?

What radiation fractionation scheme would you use to palliate a patient with metastatic paratesticular leiomyosarcoma limited mainly to soft-tissue sites as well as the scrotum?

This patient is young and active but refuses palliative chemotherapy

When do you recommend post-operative radiation therapy for extracranial chondrosarcoma?

Is there a role for pre-operative RT?

In a patient with solitary metastasis skin/subcutaneous wrist, resected with graft, from a primary breast angiosarcoma, what would be your preferred fields and dose?

50+ yo F. Mastectomy, chemo, and RT in 2016 for primary breast angiosarcoma, not RT induced. Had longstanding cyst on wrist, biopsied, and positive fo...

When would you consider neoadjuvant RT for dermatofibrosacroma protuberans?

Is there any role for neoadjuvant chemotherapy in renal leiomyosarcoma?

Very rare tumor with sparse literature

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

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

How do you treat metastatic, wild-type GIST?

How would you manage a patient with epithelioid hemangioendothelioma?

How do you manage recurrent inoperable chest wall pleomorphic liposarcoma with prior radiation therapy?

Prior treatment: GTR in 2020, adjuvant radiation 63Gy Patient factors: Medically inoperable and not fit for chemotherapy The recurrence is within th...

Would you consider adjuvant chemotherapy in the management of retroperitoneal leiomyoscarcoma who did not respond to neoadjuvant doxorubicin/dacarbazine?

And if so which?

What, if any, adjuvant therapy would you offer a high risk (based on size, location, mitotic rate) GIST patient who is wild type or PDGFRA D842V mutated?

How would you treat a locally-recurrent extraosseous (pulmonary) Ewing sarcoma?

Ewing sarcoma of the right lung, status post bi-lobectomy and adjuvant VDC/IE, with recurrence 6 years in the right lung only. He has reached a cumula...

What dose-fractionation and clinical target volume do you use when treating tenosynovial giant cell tumor (TGCT), historically known as pigmented villonodular synovitis (PVNS), of the knee?

What is your preferred regimen for palliative treatment of unresectable retroperitoneal sarcoma?

How would you manage a high grade primary mediastinal leiomyosarcoma status-post excision with negative margins?

What is the role for post-operative irradiation?

Do you recommend adjuvant RT for positive margins of dermatofibrosarcoma protuberans?

Would you change your management based on location, such as a sensitive area like the groin? What volumes do you recommend? Does age influence y...

Would you offer preoperative radiation for an intrathoracic leiomyosarcoma in the posterior mediastinum?

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.

What dose and fractionation would you recommend for palliative-intent radiation therapy for cutaneous angiosarcoma in a patient with distant metastases?

Would you go beyond a traditional palliative regimen such as 30Gy in 10 fractions?

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

For post-operative radiation of resected high grade soft tissue sarcoma, how long of a delay do you tolerate before starting radiation?

This is particularly relevant for patients with extensive delays due to wound healing issues.

What is the clinical benefit of primary site postoperative radiation after neoadjuvant chemotherapy and oncologic surgery for an extremity soft tissue synovial sarcoma in an oligometastatic patient with pulmonary metastases ?

The pulmonary metastases are planned to be addressed with a combination of surgical resection and SBRT.

How do you manage near-field recurrence of a soft tissue sarcoma that was previously treated with pre-op RT?

Would you off more radiation if the prior pre-op radiation was within a few cm to the recurrence? If there a time interval you would wait before offer...

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 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?

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

What is your approach to extremity STS (G2-3) after an initial unplanned (non-oncologic) surgery who is planned for oncologic re-excision?

When do you offer preop RT (50Gy) before the re-excision? When do you offer post-op RT after the re-excision?

Would you consider definitive radiation therapy (EBRT + interstitial HDR) in lieu of pelvic exenteration for a vaginal spindle cell sarcoma?

How would you treat unresectable angiosarcoma of the extremity in a patient who is inoperable due to underlying comorbidities?

How would you approach unresectable synovial sarcoma of the heart ?

When do you offer adjuvant radiation therapy for high grade non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) in a skeletally mature teenager or young adult?

Individuals often cite ARST0332 to justify radiation omission in high grade R0 resections, but R0 was defined as > 5 mm margins.

Would you recommend prophylactic retro-peritoneal RT in an adolescent with paratesticular rhabdomyosarcoma, s/p radical inguinal orchiectomy without a lymph node dissection?

If a staging RPLND is NOT performed, is there a role for prophylactically irradiating the lymph nodes, even in the setting of a negative PET/CT at dia...

How would you approach an intrathoracic solitary fibrous tumor in a patient who is not a surgical candidate?

Would you consider SBRT?

How would you approach unresectable cutaneous angiosarcoma of the scalp?

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

How would you approach subtotal resection of a sarcoma of the scrotum/groin with grossly positive margin at the base of the penis?

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?

Would you offer post-operative radiation therapy for a pseudoendocrine sarcoma with a CTNNB mutation of the spine that has been resected with a positive margin?

If so, what dose and treatment fields would you use?

How do you manage a soft tissue sarcoma arising from neurofibromas (NF1)?

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?

How would you manage metastatic alveolar soft part sarcoma in an young patient failing TKI?

How do you incorporate PD-L1 blockade?

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?

Do you routinely perform bilateral oophorectomy at the time of hysterectomy for a premenopausal woman with a uterine leiomyosarcoma that appears to be limited to the uterus?

Does ER/PR status help guide your decision?

Would you give adjuvant therapy after diagnosis of presumed stage IA, 5cm, ER/PR+ uterine adenosarcoma on a total hysterectomy with bilateral salpingoopheretomy without staging?

No sarcomatous overgrowth seen.

Would you consider boost radiation for focally positive margins after 50 Gy and surgery in undifferentiated pleomorphic sarcoma of chest wall?

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...

Do you typically incorporate radiotherapy into the management of extraskeletal myxoid chondrosarcoma?

These tumors are often low grade, but have a propensity for local recurrence.

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 treat osteosarcoma that has relapsed with unresectable pulmonary metastatic disease?

Would you offer adjuvant radiation for a grade 1 osteosarcoma of the mandilbe with close margins following surgical resection?

What margin would you consider to be necessary to omit radiation?

How would you treat refractory unresectable pulmonary metastases in high grade synovial sarcoma?

Patient received Pazopanib upfront with Ifos/Dox. Pulmonary metastatectomy is not an option as there are multiple mets.

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?

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 a patient with cardiac sarcoma, is there a minimum ejection fraction to proceed with adjuvant radiation therapy?

What is your approach to adjuvant therapy for a fully resected (contained morcellation and laparoscopic removal), isolated retroperitoneal leiomyosarcoma in a patient with a remote history of a hysterectomy?

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

When do you recommend preop RT for retroperitoneal sarcomas?

How do you interpret the results from the recently published EORTC-62092: STRASS study that showed no benefit to preop RT+surgery vs surgery alone and...

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

What is the appropriate dose/fractionation when treating a dermatofibrosarcoma protuberans of the spine following debulking surgery in a patient with a good performance status?

In this patient dermatofibrosarcoma protuberans of the thoracic spine, which originally caused a cord compression and has since been debulked, gross r...

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

How would you incorporate whole abdomen radiation therapy after cytoreduction in an AYA patient with desmoplastic small round cell tumor?

What if the patient had also received HIPEC prior?

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?

How would you manage a recurrent uterine leiomyosarcoma, now status post secondary cytoreduction, with no gross residual disease?

Would you consider adjuvant chemotherapy, radiation therapy, hormone therapy, or surveillance?

Would you offer adjuvant radiation to a patient with a vaginal spindle cell sarcoma s/p modified radical hysterectomy with bilateral sapingoopherectomy with negative margins?

Final pathology showed grade 3 spindle cell sarcoma of thee vagina involving the anterior lateral cervix 5.6cm x 4.5 x 3.4cm, <0.1cm left lateral m...

How would you modify adjuvant radiation for oral cavity SCC based on the presence of sarcomatoid features?

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 treat recurrent unresectable sarcoma in previously irradiated maxillary region, with no distant metastatic disease?

What is your treatment approach to a young woman with widely metastatic low grade endometrial stromal sarcoma not amenable to complete cytoreduction?

Would you consider "neoadjuvant" hormonal therapy to shrink tumors and potentially make more amenable to complete cytoreduction at a later date? Woul...

Would you offer preoperative radiation therapy when treating retroperitoneal sarcomas?

Recent results from the STRASS trial suggest limited benefit from preoperative radiation therapy for retroperitoneal sarcomas. https://pubmed.n...

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

Do you recommend salvage radiation therapy to limited recurrent Ewing's sarcoma bony lesions?

If you do what dose and fractionation do you recommend?

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

How would you manage relapsed desmoplastic small round cell tumor?

The patient previously received the P6 regimen.

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 progesterone therapy to a young woman with low grade endometrial stromal sarcoma who had elected ovarian conservation?

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...

What region would you treat in a patient with high grade leiomyosarcoma localized to the spermatic cord with close margins (<1mm) s/p inguinal orchiectomy?

More specifically there is no testicular invasion - would you cover the entire scrotal sac? What lymph node stations if any would you cover?

Would you recommend pre-op radiation for an operable recurrent well differentiated retroperitoneal liposarcoma with abutment of the right kidney?

The interval since prior resection is between 3-5 years. Additionally, there is some evidence of nodal involvement but no evidence of metastatic disea...

What adjuvant treatment would you offer a young woman with stage IB uterine mullerian adenosarcoma with sarcomatous overgrowth?

Would you give full post-operative dose radiation for an undifferentiated high grade pleomorphic sarcoma of the extremity with close surgical margins if the patient had previously received <20Gy preoperatively?

Re-excision would result in amputation. And the patient had attempted pre-operative RT but quit at 16Gy due to toxicity/social reasons. Would you trea...

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?

What CTV expansion volumes would you recommend for a large, unresectable, symptomatic desmoid tumor of the neck?

Would you use general sarcoma expansions or would smaller expansions like the ones in head and neck would be reasonable?

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

Would you consider preoperative radiation for a retroperitoneal sarcoma in a patient with Li Fraumeni syndrome where upfront surgery is likely to yield a positive margin?

Would chemotherapy be a consideration? If yes, would you adjust margins or dose?

In the setting of unresectable Ewing's sarcoma in an adult, would you consider boosting to a higher dose?

Specifically, when treating a tumor located in the head and neck region, would you consider treating closer to 64-66 Gy? Or would you be consistent wi...

Would you treat a pleomorphic sarcoma of the pelvis post-operatively?

When deciding to treat what would be your dose/volumes? And would a re-staging scan play a role in your treatment planning?

What do you consider a curative radiation dose for a sarcoma?

If a patient with a high grade sarcoma has gross residual/recurrent disease after resection and cannot undergo further surgery - would you boost this ...

5 Answers available

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)?

Are there any hypofractionated RT regimens that could be considered in a post-op setting for extremity soft tissue sarcoma?

What is your regimen? And long-term follow-up in these patients?

How would you approach a supraclavicular high grade monophonic synovial sarcoma?

What if it was status post partial resection? And had bilateral level 2 lymph nodes?

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?

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...

How do you treat plantar fibromatosis with radiation?

What dose schedule would you use to treat plantar fibromatosis? How soon after resection would you begin treatment? And how happy were you with the re...

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 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 intracavitary brachytherapy regimen would you use after EBRT for a 4cm vaginal leiomyosarcoma the mid vagina with positive margins?

MRI with intravaginal gel shows no gross residual disease.

What radiation dose is appropriate for unresectable spindle cell sarcoma of the spine?

The case in question is a radiation-induced spindle cell sarcoma at the thoracic spine previously treated for a plasmacytoma to a dose of 60 Gy in 30 ...

In the setting of a recurrent Grade 1 liposarcoma of the spermatic cord that extended to the scrotum and was previously treated with surgery alone, what volume and dose would you use in following re-resection?

For both the primary and re-resection surgeries, resection was achieved via both scrotal and inguinal incisions. And margins were reported as negative...

Is there a maximum time delay in offering adjuvant radiation to patients with wound healing issues for sarcoma and skin cancer patients with aggressive histology and positive margins?

Sometimes wound healing can delay adjuvant radiotherapy by several months. For aggressive histology, such as high grade sarcomas or skin cancers with&...

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?

Would you offer adjuvant radiotherapy for a malignant fibromyxoid tumor of soft tissue with margin < 1 cm on re-excision?

Adverse features include increased nuclear size, nuclear atypia, and mitotic activity up to 10/50 HPF.

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...

Would you offer adjuvant radiation to a patient with uterine undifferentiated sarcoma s/p resection, vaginal cuff recurrence, and re-resection?

If so, would you treat the whole pelvis, cuff brachytherapy, or both?

How do you treat unresectable localized desmoid tumor?

How would you approach local recurrence of scalp angiosarcoma during the course of adjuvant radiotherapy after a widely R0 resection?

Recurrence is in the radiation field right at the border of graft and scalp, and started to surface at about 20-24 Gy. It is visibly enlarging over se...

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?

What is the role of postoperative radiation therapy after complete resection of a high grade spermatic cord sarcoma?

68 y.o. Male underwent inguinal orchiectomy and spermatic cord resection of a 5 cm malignant fibrous histiocytoma of the spermatic cord. Margins of re...

How would you suggest to radiate a Kaposi's sarcoma?

Would certain patient/disease characteristics make you choose RT therapy over chemotherapy or other locally ablative therapies? What dose/fraction wou...

5 Answers available

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?

How do you define close and negative margins for soft tissue sarcoma?

Did the publication earlier this year by Gundle et al. JCO 2018 (http://ascopubs.org/doi/pdf/10.1200/JCO.2017.74.6941) alter your concept of close mar...

Do you offer adjuvant radiation therapy for a patient with a > 5 cm resected malignant peripheral nerve sheath tumor with 1 cm negative margin?

What margins (if any) do you consider appropriate to not require adj RT? And would you ever consider neoadj RT?

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)?

Are there any anatomical locations that you would consider omiting preoperative radiation for sarcoma due to toxicity concerns?

Specifically the groin region? Or are there techniques or a change in the management style of radiation at these sites you would employ to minimize to...

Do you routinely offer post-operative radiotherapy for resected retroperitoneal sarcomas?

If so, how do you choose your region of treatment? Dose? And elective nodal volume?

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...

Is postoperative radiotherapy indicated for a 5 cm grade 2 synovial sarcoma of the knee joint?

If margins are clear, would this tumor necessitate adjuvant radiotherapy? Synovial sarcomas often occur in a young patient subset, would this cause yo...

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

Do you use chemotherapy or radiation?

Do you treat retroperitoneal sarcomas with a boost to the rind (or suspected close surgical margin)?

If so, what patients do you select for the boost? And what data do you give to adequately satisfy the insurance review and win approval?

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?

Is there a benefit to EBRT for recurrent solitary fibrous tumor/ hemangiopericytoma in patient with multiple sites of intrapelvic recurrence?

In a patient, with multiple small volume lesions (5-25mm), would you offer pelvic RT?

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...

How would you manage a recurrent chest wall desmoid tumor?

After and extensive en bloc resection, is there further surgery that would be recommended? Is radiation alone sufficient?

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

How do you manage sarcomas of the hand or the feet?

Would you treat these sites to definitive or post-operative doses? If not, is there a dose constraint you use at these sites? Do you use tighter margi...

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...

How would you manage an osteosarcoma patient with local disease only and an R0 resection who could not tolerate neoadjuvant chemotherapy?

Is there a role for adjuvant radiation? Other chemotherapy options?

How would you optimize patient set-up and planning for the post-operative treatment of a distal lower extremity sarcoma?

What things would you take into consideration for the simulation set-up? Specific instructions for treatment planning? If the PTV is confined to only ...

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.

In the setting of an atypical lipoma/well-differentiated liposarcoma of the extremity with a small component of dedifferentiated liposarcoma, is it necessary to treat the entire mass with neoadjuvant radiotherapy OR just the dedifferentiated component that is enhancing on contrast MRI?

How would you treat a high grade sarcoma of the breast?

Is breast conserving surgery followed by radiation therapy an option?

Would you consider SBRT for a retroperitoneal sarcoma with an R2 resection near critical structures precluding re-resection?

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 would your RT volumes be for a high grade sarcoma of a joint (such as the knee) after a gross total resection?

How do you treat pleomorphic rhabdomyosarcoma in an adult?

Do you follow pediatric protocols or treat this more like a high grade soft tissue sarcoma? Specifically for paratesticular pleomorphic RMS, do you ad...

What are the recommended treatment fields for desmoid tumors?

Is a margin of 5 cm on the GTV still standart?

What evidence is there for the benefit of adjuvant radiation therapy for a large primary thoracic synovial sarcoma that was completely resected?

What are some guidelines or principles that you use to recommend adjuvant radiation after complete resections of sarcoma?

How do you define your post-operative treatment volumes for a patient with a resected high-grade soft tissue sarcoma of the soft tissues of the upper back?

For example, in the setting of a lack of pre-operative imaging, what CTV margin(s) would you utilize for the initial and boost phases of treatment?

Do you treat radiation-induced sarcomas similarly to de novo sarcomas?

How does grade, margin status, and previous radiation dose factor into the recommendation?

What are your indications for treating a pelvic desmoid tumor in a young woman concerned with fertility?

When do you consider is too late after resection to offer adjuvant therapy for high grade osteosarcoma?

What time delay would make the benefit of adjuvant therapy likely no longer significant?

Do you recommend imaging surveillance vs adjuvant RT for a large low grade sarcoma of the lower extremity with multiple positive margins?

Would it differ if the tumor was high grade at the same location?

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.

How do you manage an adult primary rhabdomyosarcoma of the lung/mediastinum?

Is there any role for consolidative RT/CRT to the lung and mediastinum after initial chemo? What dose and fractionation would be most appropriate for ...

Would you recommend adjuvant radiation for a completely resected large well differentiated sarcoma (i.e. liposarcoma) involving the retropharyngeal area and mediastinum?

Should such a case be treated like retroperitoneal sarcoma?

Do you routinely treat postoperative extremity soft tissue sarcoma with IMRT?

If so, how to you address the scar? Do you use bolus? Do you place a 2mm retraction from skin for the target contour?

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?

For retroperitoneal sarcomas, do you consider neoadjuvantly boosting the "posterior rind" or area at high risk of positive margin?

If so, to what dose?

What treatment fields and dose do you use in the treatment of postoperative angiosarcoma of the scalp?

With the high rates of recurrence postoperatively with angiosarcomas of the scalp, adjuvant radiation is often recommended. What margins would y...

When pre-operatively treating high grade soft-tissue sarcomas involving the chest, abdomen, or pelvis what margins do you use for CTV and PTV?

Most guidelines discuss target delineation and contouring recommendations for extremity sarcomas, which generally suggest larger longitudinal than rad...

In what settings would you offer adjuvant radiation for a R1 resected desmoid tumor?

Do you routinely boost focally positive margins after preoperative 50 Gy and surgery in patients with extremity sarcomas?

For example, a patient with liposarcoma of the lower extremity status post preoperative radiation to 50 Gy who then underwent resection and had focall...

Do you boost residual lung disease after chemotherapy after whole lung irradiation for patients with Ewings, Rhabdomyosarcoma or Wilms?

Can you use SBRT for boost?

What dose should be used for a G3 retroperitoneal sarcoma with R1 resection?

Would the dose change based if there was tumor adherent to bowel versus abdominal wall or muscle?

What dose would be appropriate for large intra-abdominal or retroperitoneal sarcomas that are unresectable?

Would the histological grade or histology affect the dose? Would you consider dose escalating to the center of the tumor if this would not affect orga...

When treating a patient with a high grade sarcoma in the post op setting, do I need to get full dose to the incision and drain site (assuming all margins negative)?

When treating a soft tissue sarcoma close to humeral head, what humeral head dose constraint should be used?

I am treating a resected high grade liposarcoma with positive surgical margins to 66Gy. I would prefer to use IMRT to spare the humerus, but ...

For extremity rhabdomyosarcomas (hand) with positive epitrochlear and axillary lymph nodes, do you treat the lymph nodes or just the primary?

What is the risk of joint issues if you treat the epitrochlear nodes?

What dose do you feel comfortable treating the entire circumference of an extremity to before you are concerned about chronic toxicity?

When the disease (in this case, lymphoma) involves almost all of the entire muscle compartment of the distal lower extremity, what is a safe dose? I'm...

Should all GISTs be sequenced?

What dose do you use in the adjuvant treatment of desmoid tumors?

And does your dose changes with positive margins? I can't seem to find a good answer in the literature.