# Rhabdomyosarcoma — GCMD Library living collection

Updated: n/a · 8 episodes · 89 cited statements

## Episodes
### General Management
- [Critical elements of pediatric rhabdomyosarcoma surgery](https://library.globalcastmd.com/watch/critical-elements-of-pediatric-rhabdomyosarcoma-surgery-16275) — article · [machine version](https://library.globalcastmd.com/watch/critical-elements-of-pediatric-rhabdomyosarcoma-surgery-16275.md)
- [Management of rhabdomyosarcoma in pediatric patients](https://library.globalcastmd.com/watch/management-of-rhabdomyosarcoma-in-pediatric-patients-16298) — article · [machine version](https://library.globalcastmd.com/watch/management-of-rhabdomyosarcoma-in-pediatric-patients-16298.md)
- [Pediatric Sarcoma Surgical Margins: Rhabdomyosarcoma Treatment Guidelines w/ Dr. Roshni Dasgupta](https://library.globalcastmd.com/watch/pediatric-sarcoma-surgical-margins-rhabdomyosarcoma-treatment-guidelines-w-dr-ro-15896) — video · 0:25 · [machine version](https://library.globalcastmd.com/watch/pediatric-sarcoma-surgical-margins-rhabdomyosarcoma-treatment-guidelines-w-dr-ro-15896.md)
- [Clinical & Research Update: Sarcoma w/ Drs. Roshni Dasgupta, Joseph Pressey, Arthur Meyer, Luke Pater](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017) — video · 1:06:24 · [machine version](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017.md)
- [Technical approaches to rhabdomyosarcoma management: APSA Cancer Committee case series](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301) — video · 17:35 · [machine version](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301.md)

### Site-Specific Disease
- [Impact of local control and surgical lymph node evaluation in localized paratesticular rhabdomyosarcoma: A report from the Children's Oncology Group Soft Tissue Sarcoma Committee](https://library.globalcastmd.com/watch/impact-of-local-control-and-surgical-lymph-node-evaluation-in-localized-paratest-16300) — article · [machine version](https://library.globalcastmd.com/watch/impact-of-local-control-and-surgical-lymph-node-evaluation-in-localized-paratest-16300.md)
- [Outcomes for patients with perineal and perianal rhabdomyosarcoma: A report from the Children's Oncology Group Soft Tissue Sarcoma Committee](https://library.globalcastmd.com/watch/outcomes-for-patients-with-perineal-and-perianal-rhabdomyosarcoma-a-report-from-16297) — article · [machine version](https://library.globalcastmd.com/watch/outcomes-for-patients-with-perineal-and-perianal-rhabdomyosarcoma-a-report-from-16297.md)
- [Implications of Tumor Characteristics and Treatment Modality on Local Recurrence and Functional Outcomes in Children with Chest Wall Sarcoma: A Pediatric Surgical Oncology Research Collaborative Study](https://library.globalcastmd.com/watch/implications-of-tumor-characteristics-and-treatment-modality-on-local-recurrence-3529) — article · [machine version](https://library.globalcastmd.com/watch/implications-of-tumor-characteristics-and-treatment-modality-on-local-recurrence-3529.md)

## Chapters
- [4:13](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=253) Case 1 Introduction: 10-Year-Old with Chest Wall Ewing Sarcoma - Presentation and Imaging (Ep 4)
- [10:05](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=605) Case 1: Neoadjuvant Chemotherapy and Surgical Planning (Ep 4)
- [14:40](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=880) Case 1: Radiation Therapy and Completion of Treatment (Ep 4)
- [21:40](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1300) Case 2 Introduction: 12-Month-Old with Ovarian Mass - Presentation and Diagnosis (Ep 4)
- [25:53](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1553) Case 2: SCCOHT Biology, Staging, and Induction Chemotherapy (Ep 4)
- [36:06](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2166) Case 2: Second-Look Surgery with HIPEC and Consolidation (Ep 4)
- [45:28](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2728) Case 2: Germline SMARCA4, Radiation Decision, and Long-Term Outcomes (Ep 4)
- [51:36](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3096) Case 3 Introduction: 2-Year-Old with Chest Wall Rhabdomyosarcoma - Presentation and Surgical Approach (Ep 4)
- [58:42](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3522) Case 3: Radiation Decision and Li-Fraumeni Considerations (Ep 4)
- [60:46](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3646) Multidisciplinary Discussion: Germline Testing and Individualized Care (Ep 4)
- [0:10](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=10) Introduction to APSA Cancer Committee Rhabdomyosarcoma Session (Ep 5)
- [1:34](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=94) Faculty Introductions and Session Format (Ep 5)
- [3:14](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=194) Extremity Rhabdomyosarcoma: Initial Biopsy Approach (Ep 5)
- [4:46](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=286) Sentinel Lymph Node Biopsy Techniques and Indications (Ep 5)
- [8:42](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=522) Staging, Risk Stratification, and Local Control Strategies (Ep 5)
- [10:45](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=645) Paratesticular Rhabdomyosarcoma: Prognostic Factors (Ep 5)
- [11:56](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=716) Imaging and Lymph Node Assessment in Paratesticular Disease (Ep 5)
- [13:41](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=821) Retroperitoneal Lymph Node Dissection: Indications and Technique (Ep 5)
- [16:12](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=972) Management of Scrotal Violation (Ep 5)

## Statements
Every statement is attributed, typed, and timestamped; the link is the citation.
- Compressed-interval VDC/IE chemotherapy given every 2 weeks is now well proven to be of benefit over 3-week schedule for Ewing sarcoma — Joseph Pressey (clinical) [Ep 4 · 9:23](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=563)
- For chest wall sarcomas, the old adage of needing a rib above and a rib below for margins is not the case anymore; the goal is just to get negative margins if possible — Roshni Dasgupta (clinical) [Ep 4 · 11:23](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=683)
- When tumor is adherent to diaphragm or pericardium, you should not dissect these structures off the tumor because you don't know where the margin is; instead take a portion of the adherent structure with the tumor — Roshni Dasgupta (clinical) [Ep 4 · 12:18](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=738)
- Titanium rib reconstruction systems may need revision as the child grows, particularly during puberty with significant growth spurts — Roshni Dasgupta (clinical) [Ep 4 · 14:05](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=845)
- For Ewing sarcoma radiotherapy, treatment typically involves two phases: disease presentation volume and then more specific targeting of gross disease or positive margins — Luke Pater (clinical) [Ep 4 · 15:29](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=929)
- Protons are preferred over photons for focused boost volumes because they reduce low-dose exposure to non-target tissue beyond the target, though in lungs the benefit is compromised due to low-density tissue causing scatter — Luke Pater (clinical) [Ep 4 · 20:12](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1212)
- Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) was originally described around 1980 in young adult women presenting with ovarian tumors with nondescript morphology, often associated with hypercalcemia — Joseph Pressey (epidemiological) [Ep 4 · 23:23](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1403)
- SCCOHT tumors are actually malignant rhabdoid tumors; some have postulated they should be called malignant rhabdoid tumor of the ovary — Joseph Pressey (clinical) [Ep 4 · 23:47](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1427)
- In 2014, three labs simultaneously identified SMARCA4 mutation (usually single nucleotide variant) as the underlying genetic defect in SCCOHT — Joseph Pressey (clinical) [Ep 4 · 24:06](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1446)
- SMARCA4 encodes BRG1 and its loss unifies all SCCOHT tumors; like Ewing sarcoma, these tumors have very low tumor mutational burden with SMARCA4 usually the only mutation found — Joseph Pressey (clinical) [Ep 4 · 24:40](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1480)
- Many SCCOHT patients enter the OR without this diagnosis in the differential; they are usually thought to be germ cell tumors, resulting in incomplete initial surgery — Joseph Pressey (clinical) [Ep 4 · 26:39](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1599)
- SCCOHT is a chemo-sensitive tumor but only transiently so; it is very important to start intensive systemic chemotherapy quickly rather than sending patients back for immediate second-look operation — Joseph Pressey (clinical) [Ep 4 · 27:00](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1620)
- The VPCBAE regimen (vinorelbine, cisplatin, cyclophosphamide, bleomycin, doxorubicin, etoposide) with all six agents given every 21-day cycle is very intensive toxic therapy requiring meticulous supportive care — Joseph Pressey (clinical) [Ep 4 · 27:20](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1640)
- Four cycles of VPCBAE induction chemotherapy are given prior to second-look operation for SCCOHT; patients with measurable disease after initial surgery often respond well even by RECIST criteria — Joseph Pressey (clinical) [Ep 4 · 27:43](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1663)
- The 12-month-old SCCOHT patient is the youngest ever described with this tumor; the average age for diagnosis is 24 years — Joseph Pressey (epidemiological) [Ep 4 · 28:25](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1705)
- MRIs and PET scans are not great for detecting peritoneal disease and very small nodules (1mm); second-look surgery is necessary to identify disease that imaging cannot detect — Roshni Dasgupta (clinical) [Ep 4 · 29:18](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1758)
- For SCCOHT second-look surgery, laparoscopy is used initially to understand extent of disease and determine size of laparotomy incision needed; the operation is never done with laparoscopy alone — Roshni Dasgupta (clinical) [Ep 4 · 29:44](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1784)
- Complete cytoreduction for SCCOHT includes removing all visible disease, with particular attention to peritoneal reflection in pouch of Douglas and surface of rectum where tumor can hide after spill — Roshni Dasgupta (clinical) [Ep 4 · 30:40](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1840)
- HIPEC in pediatric patients requires a multidisciplinary team experienced in this procedure; it is not the same as adult HIPEC — Roshni Dasgupta (clinical) [Ep 4 · 32:25](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1945)
- HIPEC protocol includes preoperative hyperhydration (1-2x maintenance fluids), warming abdomen to 42°C, cisplatin given in 2/3 dose initially and 1/3 at 45 minutes, continuous shaking for 90-minute dwell time, sodium thiosulfate infusion at 30 minutes to scavenge cisplatin, and 4-5L washout — Roshni Dasgupta (clinical) [Ep 4 · 32:59](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=1979)
- Post-HIPEC patients receive 24 hours of hyperhydration in ICU; main counseled toxicities are ileus and kidney function issues, though the Cincinnati protocol has not seen significant renal toxicity — Roshni Dasgupta (clinical) [Ep 4 · 34:49](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2089)
- SCCOHT patients can resume next cycle of chemotherapy 4-7 days postoperatively after HIPEC once bowel function recovers — Roshni Dasgupta (clinical) [Ep 4 · 35:50](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2150)
- Maintaining interval compression as dense as possible (7-8 days to resume chemotherapy post-HIPEC) is very important to continue killing residual tumor cells — Joseph Pressey (clinical) [Ep 4 · 36:06](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2166)
- SCCOHT treatment uses 6 cycles of VPCBAE (usually 4 induction, 2 adjuvant); some stage 1 patients or those with considerable toxicity (especially renal) have stopped after 5 cycles to get to transplant in good health — Joseph Pressey (clinical) [Ep 4 · 36:39](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2199)
- Cincinnati uses busulfan-thiotepa conditioning for autologous stem cell transplant in SCCOHT, which is more toxic than the carboplatin-based regimen used by the French consortium but complements VPCBAE better with heavy alkylator exposure — Joseph Pressey (clinical) [Ep 4 · 37:08](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2228)
- Approximately 43% of SCCOHT patients carry germline SMARCA4 mutations; in Cincinnati's last 20 patients, 8 had germline SMARCA4 (40%) — Joseph Pressey (epidemiological) [Ep 4 · 37:56](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2276)
- For SCCOHT patients with germline SMARCA4, contralateral salpingo-oophorectomy should be performed at second-look operation because it would be nearly impossible to treat a metachronous SCCOHT tumor a second time with intensive therapy — Joseph Pressey (clinical) [Ep 4 · 38:21](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2301)
- Germline SMARCA4 mutations have been found in older SCCOHT patients in their late 30s with no prior personal or family history of cancer; all patients should be tested regardless of family history — Joseph Pressey (clinical) [Ep 4 · 38:55](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2335)
- Hysterectomy should be considered for germline SMARCA4 carriers due to risk of SMARCA4-deficient uterine sarcoma, which peaks around age 50 (later than SCCOHT which peaks at 23-24 years) — Joseph Pressey (clinical) [Ep 4 · 39:16](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2356)
- Two patients who survived SCCOHT developed metachronous SCCOHT tumors in the contralateral ovary and subsequently died, demonstrating this is a real risk not worth taking — Joseph Pressey (clinical) [Ep 4 · 39:37](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2377)
- Penetrance of germline SMARCA4 is estimated at 62% based on UK Biobank study comparing males vs females over age 40, assuming the deficit of females was explained by SCCOHT deaths — Joseph Pressey (epidemiological) [Ep 4 · 40:10](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2410)
- Cincinnati's methodology estimates SMARCA4 penetrance closer to 20% studying family members of SCCOHT patients; even at 20% this is considered considerable risk for otherwise healthy female carriers — Joseph Pressey (epidemiological) [Ep 4 · 41:08](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2468)
- About 20% of SCCOHT patients have de novo SMARCA4 mutations with neither parent carrying the mutation — Joseph Pressey (epidemiological) [Ep 4 · 41:35](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2495)
- Major long-term toxicity from SCCOHT treatment is ototoxicity requiring hearing aids; otherwise patients grow and develop well — Joseph Pressey (clinical) [Ep 4 · 42:11](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2531)
- Whole abdomen radiation for SCCOHT was used historically but is excessively toxic in patients who receive autologous stem cell transplant and HIPEC; Cincinnati has walked away from this approach — Luke Pater (clinical) [Ep 4 · 43:16](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2596)
- Whole abdomen radiation could still be considered for SCCOHT patients who do not get HIPEC or autologous bone marrow transplant — Luke Pater (opinion) [Ep 4 · 43:46](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2626)
- Protons are ideal for SCCOHT lymph node radiation to minimize bowel, liver, kidney exposure and in similar scenarios to minimize dose to uterus, ovaries, pancreas, and spleen (low-dose pancreatic/splenic exposure is problematic long-term for metabolic syndrome and immune function) — Luke Pater (clinical) [Ep 4 · 44:30](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=2670)
- Cincinnati's SCCOHT series of 20 consecutive patients (2018-2024) with median follow-up of 58 months shows 60% overall survival, about 20 percentage points higher than the French study published in 2020 — Joseph Pressey (epidemiological) [Ep 4 · 50:21](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3021)
- For rhabdomyosarcoma, surgery should only be attempted if negative margins are achievable; if only debulking is possible, local control should be done with radiation only — Roshni Dasgupta (clinical) [Ep 4 · 55:18](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3318)
- The goal of delayed primary excision (DPE) for rhabdomyosarcoma is to achieve at least R1 disease (microscopic residual only) — Roshni Dasgupta (clinical) [Ep 4 · 55:41](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3341)
- Scapular disarticulation provides excellent access for high chest wall tumors, allowing the chest to be opened like a book — Roshni Dasgupta (clinical) [Ep 4 · 56:33](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3393)
- For tumors near subclavian vessels and brachial plexus, a neck incision (similar to first rib operation for thoracic outlet syndrome) allows isolation of these structures from above before thoracotomy — Roshni Dasgupta (clinical) [Ep 4 · 56:47](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3407)
- For high chest wall defects covered by scapula, bony reconstruction is not needed; Gore-Tex mesh with trapezius flap rotation provides adequate coverage — Roshni Dasgupta (clinical) [Ep 4 · 57:34](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3454)
- After delayed primary excision for presenting R3/group 3 rhabdomyosarcoma, radiotherapy is typically recommended — Luke Pater (clinical) [Ep 4 · 59:48](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3588)
- In the rhabdomyosarcoma case with germline TP53 mutation, radiation was deferred due to the mutation combined with excellent chemotherapy response and complete surgical resection, despite this being non-standard for group 3 disease — Luke Pater (clinical) [Ep 4 · 60:13](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3613)
- Radiation-induced sarcomas in Li-Fraumeni patients are typically more aggressive and less chemosensitive than primary sarcomas — Joseph Pressey (clinical) [Ep 4 · 60:53](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3653)
- The incidence of secondary malignancy from radiation is assumed to be higher the younger the patient, both because of more years to develop it and an inherent increased risk with young age — Luke Pater (clinical) [Ep 4 · 61:31](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3691)
- Radiation decisions in young children involve nuanced discussions of toxicities including pulmonary function, cardiac exposure, wound healing, and chest wall development, individualized case by case — Luke Pater (opinion) [Ep 4 · 61:48](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3708)
- The risk of hereditary predisposition among all sarcoma patients may be as high as 15% — Joseph Pressey (epidemiological) [Ep 4 · 64:17](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3857)
- Cincinnati now performs universal germline testing for all newly diagnosed solid tumor patients and many benign tumors like desmoid tumors (with FAP testing) — Joseph Pressey (clinical) [Ep 4 · 64:27](https://library.globalcastmd.com/watch/clinical-research-update-sarcoma-w-drs-roshni-dasgupta-joseph-pressey-arthur-mey-14017?t=3867)
- Incisional biopsy or image-guided core needle biopsy is recommended for extremity rhabdomyosarcoma, taking into account tumor location and relationship to neurovascular structures. — Emily Christison-Lagay (guideline) [Ep 5 · 3:26](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=206)
- Small superficial tumors may undergo primary excision provided negative margins can be achieved and lymph function will not be compromised, but this represents a minority of situations. — Emily Christison-Lagay (guideline) [Ep 5 · 3:39](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=219)
- The biopsy tract should be incorporated into any subsequent resection, and incorrect or poorly planned biopsy may contaminate uninvolved compartments and compromise local control, limb salvage likelihood, and overall outcome. — Emily Christison-Lagay (clinical) [Ep 5 · 3:56](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=236)
- Multiple large caliber cores verified by real-time involvement of a pathologist are recommended for core needle biopsy to ensure adequate viable tissue for complete assessment of biological markers. — Emily Christison-Lagay (guideline) [Ep 5 · 4:24](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=264)
- Nodal involvement occurs in nearly a quarter of patients with rhabdomyosarcoma and is most common in fusion positive patients, those with tumors greater than 5 cm, those with extremity or trunk tumors, and those with paratesticular tumors who are older than 10 years of age. — Emily Christison-Lagay (epidemiological) [Ep 5 · 5:03](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=303)
- Current COG protocols recommend cross-sectional imaging and FDG PET to detect distant metastatic and local regional nodal disease, with lymph nodes greater than 1 cm in short axis or PET SUV max greater than 2.5 times baseline suggesting nodal involvement. — Emily Christison-Lagay (guideline) [Ep 5 · 5:24](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=324)
- Both sensitivity and specificity of PET for nodal metastasis is less than sentinel lymph node biopsy, with imaging failing to detect nodal disease in approximately half of cases for extremity and other sites, and demonstrating false positivity in other instances. — Emily Christison-Lagay (clinical) [Ep 5 · 5:24](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=324)
- Patients with extremity rhabdomyosarcomas should have a sentinel lymph node biopsy regardless of what imaging shows. — Emily Christison-Lagay (guideline) [Ep 5 · 6:04](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=364)
- Sentinel lymph node biopsy is more accurate than random lymph node sampling. — Emily Christison-Lagay (clinical) [Ep 5 · 6:14](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=374)
- There is no clinical benefit to completion nodal dissection for patients found to have positive sentinel nodes, as nodal basins found to be positive will receive radiation therapy. — Emily Christison-Lagay (clinical) [Ep 5 · 6:20](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=380)
- Dual use of technetium-99m and blue dye (isosulfan blue/lymphazurin or methylene blue) is preferred for sentinel lymph node biopsy, with technetium injected 2-4 hours prior in four quadrants around the tumor, followed by lymphoscintigraphy. — Emily Christison-Lagay (clinical) [Ep 5 · 6:32](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=392)
- Intraoperatively, 2-3 milliliters of blue dye should be injected at the beginning using the same four quadrant technique, with handheld gamma probe used to identify maximal counts and visual identification of blue nodes. — Emily Christison-Lagay (clinical) [Ep 5 · 7:06](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=426)
- Simultaneous use of both technetium and blue dye improves the success rate of sentinel node biopsy by providing both auditory and visual clues for lymph nodes. — Emily Christison-Lagay (clinical) [Ep 5 · 7:32](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=452)
- All lymph nodes in the nodal basin with counts greater than 10% of the first identified lymph node should be removed. — Emily Christison-Lagay (guideline) [Ep 5 · 7:45](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=465)
- Indocyanine green (ICG) is a water-soluble green dye with near-infrared fluorescent properties that binds to plasma proteins and tracks through the lymphovascular system, allowing sentinel node mapping with laser-assisted imaging after dermal injection in four quadrants with real-time lymphangiography. — Emily Christison-Lagay (clinical) [Ep 5 · 7:53](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=473)
- Studies in adult tumors have demonstrated excellent sensitivity and specificity using ICG with equivalent or superior results to other methods of sentinel lymph node detection, though this is still being actively investigated in children. — Emily Christison-Lagay (clinical) [Ep 5 · 8:27](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=507)
- A patient with extremity rhabdomyosarcoma at an unfavorable site with node-positive disease and upfront biopsy only is classified as stage 3, group 3, and if fusion positive, represents intermediate risk disease treated with VAC, VI plus or minus temus for 42 weeks. — Emily Christison-Lagay (guideline) [Ep 5 · 8:51](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=531)
- If complete resection appears possible without compromising function or causing significant anatomic distortion after neoadjuvant chemotherapy, delayed primary excision can be performed. — Emily Christison-Lagay (clinical) [Ep 5 · 9:41](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=581)
- Delayed primary excision with negative margins allows reduction of radiation therapy to 36 gray for R0 resection, while microscopic margins allow reduction to 41.4 gray. — Emily Christison-Lagay (guideline) [Ep 5 · 9:53](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=593)
- If delayed primary excision would not be possible with negative margins on re-imaging, there is no role for debulking and the patient would receive definitive radiation therapy with 50.4 gray. — Emily Christison-Lagay (guideline) [Ep 5 · 10:08](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=608)
- In patients who receive definitive radiation therapy, there is no role for excision of residual mass at the end of planned therapy, as these excisions frequently fail to find viable tumor, are incomplete, and engender significant morbidity. — Emily Christison-Lagay (clinical) [Ep 5 · 10:26](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=626)
- Paratesticular location for rhabdomyosarcoma is a favorable site with overall five-year survival exceeding 94% and progression-free survival exceeding 87%. — Daniel Rhee (epidemiological) [Ep 5 · 11:13](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=673)
- Children greater than 10 years of age, those with tumors greater than 5 cm in size, and those with retroperitoneal lymph node involvement comprise a subset of paratesticular rhabdomyosarcoma patients with poor prognosis. — Daniel Rhee (clinical) [Ep 5 · 11:31](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=691)
- Cross-sectional imaging of chest, abdomen, and pelvis is needed for paratesticular rhabdomyosarcoma, with chest CT to evaluate for pulmonary metastasis and abdomen/pelvis imaging with CT or MRI. — Daniel Rhee (guideline) [Ep 5 · 12:00](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=720)
- The absence of PET positivity does not rule out nodal involvement and positive findings on PET CT do not guarantee nodal involvement in paratesticular rhabdomyosarcoma. — Daniel Rhee (clinical) [Ep 5 · 12:42](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=762)
- In studies comparing PET to sentinel lymph node biopsy, of seven patients with biopsy-proven disease, three had normal FDG PET scans; of 14 patients with PET findings concerning for disease, only four had proven nodal disease, yielding positive predictive value of 30%, sensitivity of 57%, and specificity of 52%. — Daniel Rhee (clinical) [Ep 5 · 12:53](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=773)
- Histological confirmation is required to determine regional lymph node involvement in paratesticular rhabdomyosarcoma. — Daniel Rhee (guideline) [Ep 5 · 13:33](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=813)
- Paratesticular rhabdomyosarcoma requires inguinal orchiectomy with early and high ligation of the spermatic cord and retroperitoneal lymph node assessment. — Daniel Rhee (guideline) [Ep 5 · 13:47](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=827)
- Historically, routine surgical evaluation of retroperitoneal nodes was not recommended under most European protocols. — Daniel Rhee (clinical) [Ep 5 · 14:13](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=853)
- Current COG and North American approach is to perform ipsilateral retroperitoneal lymph node assessment in all patients greater than 10 years of age and for patients less than 10 who have enlarged lymph nodes on cross-sectional imaging, based on high known percentages of nodal involvement in older patients. — Daniel Rhee (guideline) [Ep 5 · 14:20](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=860)
- In SIOP studies using non-surgical strategy for evaluating retroperitoneal lymph node involvement based on cross-sectional imaging, a third of patients greater than 10 years of age experienced retroperitoneal recurrence compared to only 8% in patients less than 10. — Daniel Rhee (epidemiological) [Ep 5 · 14:40](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=880)
- Pooled analysis of European and North American studies found that patients greater than 10 years of age with tumors larger than 5 cm who underwent retroperitoneal lymph node assessment had superior outcomes compared to those who did not undergo surgical assessment. — Daniel Rhee (clinical) [Ep 5 · 15:09](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=909)
- The optimal number of nodes for retroperitoneal lymph node dissection is 8 to 12 to accurately capture nodal disease. — Daniel Rhee (clinical) [Ep 5 · 15:41](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=941)
- There is a potential role for sentinel lymph node biopsy using ICG in paratesticular tumors. — Daniel Rhee (opinion) [Ep 5 · 15:48](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=948)
- Transcrotal biopsy or excision has been reported in up to 25% of paratesticular rhabdomyosarcoma cases. — Daniel Rhee (epidemiological) [Ep 5 · 16:20](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=980)
- Historically, scrotal violation would mandate a hemiscrotomy. — Daniel Rhee (clinical) [Ep 5 · 16:24](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=984)
- Recent small data sets found that event-free survival for fusion negative patients who underwent transcrotal biopsy with subsequent inguinal radical orchiectomy was equivalent to those who underwent inguinal orchiectomy, challenging the notion that hemiscrotomy is needed to prevent increased risk of local recurrence. — Daniel Rhee (clinical) [Ep 5 · 16:31](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=991)
- Current COG recommendation is to avoid hemiscrotomy unless there is direct evidence of scrotal invasion. — Daniel Rhee (guideline) [Ep 5 · 16:53](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=1013)
- In the relatively rare group of fusion positive patients with paratesticular rhabdomyosarcoma, there may be a role for more aggressive management of scrotal violation, with the hemiscrotum potentially receiving radiation treatment and requiring contralateral transposition of the testicles. — Daniel Rhee (clinical) [Ep 5 · 17:04](https://library.globalcastmd.com/watch/technical-approaches-to-rhabdomyosarcoma-management-apsa-cancer-committee-case-s-16301?t=1024)

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