Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
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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, Clinical & Research Update: Sarcoma w/ Drs. Roshni Dasgupta, Joseph Pressey, Arthur Meyer, Luke Pater · 1:01:48
Critical elements of pediatric rhabdomyosarcoma surgery
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Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in children, requires multimodal therapy which is determined by risk group stratification. Local control may be achieved by surgical resection, radiation, or both. Resection may oc
article · Oct 2026
Management of rhabdomyosarcoma in pediatric patients
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… Adult patients with RMS have a poorer overall survival than pediatric patients: 43% for … The clinical efficacy of CAR (chimeric antigen receptor) T-cell therapy to pediatric solid tumors has …
article · Oct 2026
Pediatric Sarcoma Surgical Margins: Rhabdomyosarcoma Treatment Guidelines w/ Dr. Roshni Dasgupta
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Dr. Roshni Dasgupta, a Pediatric Surgeon at Cincinnati Children's, discusses the evolving approach to surgical margins for pediatric sarcoma, specifically rhabdomyosarcoma. She explains that the old adage of wide margins is no longer the st
video0:25 · Oct 2026
Clinical & Research Update: Sarcoma w/ Drs. Roshni Dasgupta, Joseph Pressey, Arthur Meyer, Luke Pater
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This video presents a detailed clinical and research update on pediatric sarcomas from Cincinnati Children's, featuring a multidisciplinary team: with Drs. Roshni Dasgupta, Joseph Pressey, Arthur Meyers, & Luke Pater. It covers two complex
video1:06:24 · Sep 2026
Technical approaches to rhabdomyosarcoma management: APSA Cancer Committee case series
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Panel discussion on rhabdomyosarcoma with Cancer committee members David Rodeberg, Roshni Dasgupta, Timothy Lautz, Emily Christison-Lagay and Daniel Rhee from the APSA 2020 virtual meeting.
video17:35 · Oct 2026
Genitourinary1 item
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
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Abstract Paratesticular rhabdomyosarcoma (PT‐RMS) carries a favorable prognosis, but questions persist regarding optimal management. Our goal was to determine the importance of primary tumor resection and surgical assessment of retroperiton
article · Oct 2026
Perineal & Perianal1 item
Outcomes for patients with perineal and perianal rhabdomyosarcoma: A report from the Children's Oncology Group Soft Tissue Sarcoma Committee
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Abstract Purpose To describe clinical features, risk factors, and outcomes of patients with perineal and perianal rhabdomyosarcoma. Methods The records of 51 patients (38 perineal and 13 perianal) enrolled on Children's Oncology Group clini
article · Oct 2026
Paraspinal1 item
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
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Objective: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development and (3) patient-reported quality of life in children with sarcoma of the chest wall.Summary background dat
article · Jan 2021
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Clinical & Research Update: Sarcoma w/ Drs. Roshni Dasgupta, Joseph Pressey, Arthur Meyer, Luke Pater
Compressed-interval VDC/IE chemotherapy given every 2 weeks is now well proven to be of benefit over 3-week schedule for Ewing sarcoma
clinicalJoseph Pressey9:23 ↗
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
clinicalRoshni Dasgupta11:23 ↗
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
clinicalRoshni Dasgupta12:18 ↗
Titanium rib reconstruction systems may need revision as the child grows, particularly during puberty with significant growth spurts
clinicalRoshni Dasgupta14:05 ↗
For Ewing sarcoma radiotherapy, treatment typically involves two phases: disease presentation volume and then more specific targeting of gross disease or positive margins
clinicalLuke Pater15:29 ↗
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
clinicalLuke Pater20:12 ↗
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
epidemiologicalJoseph Pressey23:23 ↗
SCCOHT tumors are actually malignant rhabdoid tumors; some have postulated they should be called malignant rhabdoid tumor of the ovary
clinicalJoseph Pressey23:47 ↗
In 2014, three labs simultaneously identified SMARCA4 mutation (usually single nucleotide variant) as the underlying genetic defect in SCCOHT
clinicalJoseph Pressey24:06 ↗
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
clinicalJoseph Pressey24:40 ↗
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
clinicalJoseph Pressey26:39 ↗
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
clinicalJoseph Pressey27:00 ↗
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
clinicalJoseph Pressey27:20 ↗
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
clinicalJoseph Pressey27:43 ↗
The 12-month-old SCCOHT patient is the youngest ever described with this tumor; the average age for diagnosis is 24 years
epidemiologicalJoseph Pressey28:25 ↗
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
clinicalRoshni Dasgupta29:18 ↗
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
clinicalRoshni Dasgupta29:44 ↗
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
clinicalRoshni Dasgupta30:40 ↗
HIPEC in pediatric patients requires a multidisciplinary team experienced in this procedure; it is not the same as adult HIPEC
clinicalRoshni Dasgupta32:25 ↗
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
clinicalRoshni Dasgupta32:59 ↗
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
clinicalRoshni Dasgupta34:49 ↗
SCCOHT patients can resume next cycle of chemotherapy 4-7 days postoperatively after HIPEC once bowel function recovers
clinicalRoshni Dasgupta35:50 ↗
Maintaining interval compression as dense as possible (7-8 days to resume chemotherapy post-HIPEC) is very important to continue killing residual tumor cells
clinicalJoseph Pressey36:06 ↗
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
clinicalJoseph Pressey36:39 ↗
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
clinicalJoseph Pressey37:08 ↗
Approximately 43% of SCCOHT patients carry germline SMARCA4 mutations; in Cincinnati's last 20 patients, 8 had germline SMARCA4 (40%)
epidemiologicalJoseph Pressey37:56 ↗
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
clinicalJoseph Pressey38:21 ↗
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
clinicalJoseph Pressey38:55 ↗
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)
clinicalJoseph Pressey39:16 ↗
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
clinicalJoseph Pressey39:37 ↗
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