From
Dr. Roshni Dasgupta
Technical approaches to rhabdomyosarcoma management: APSA Cancer Committee case series
With Dr. Roshni Dasgupta & Dr. Emily Christison-Lagay & Dr. Daniel Rhee · hosted by Dr. Tim Lautz
Chapter 1 of 9
Session introduction
Introduction to APSA Cancer Committee Rhabdomyosarcoma Session
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No expert statements were drawn from this page.
Host summaries · secondary, not cited in answers
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Incisional biopsy or image-guided core needle biopsy is recommended for extremity rhabdomyosarcoma, taking into account tumor location and relationship to neurovascular structures.
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.
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.
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.
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.
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.
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.
Patients with extremity rhabdomyosarcomas should have a sentinel lymph node biopsy regardless of what imaging shows.
Sentinel lymph node biopsy is more accurate than random lymph node sampling.
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.
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.
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.
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.
All lymph nodes in the nodal basin with counts greater than 10% of the first identified lymph node should be removed.
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.
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.
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.
If complete resection appears possible without compromising function or causing significant anatomic distortion after neoadjuvant chemotherapy, delayed primary excision can be performed.
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.
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.
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.
Paratesticular location for rhabdomyosarcoma is a favorable site with overall five-year survival exceeding 94% and progression-free survival exceeding 87%.
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.
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.
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.
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%.
Histological confirmation is required to determine regional lymph node involvement in paratesticular rhabdomyosarcoma.
Paratesticular rhabdomyosarcoma requires inguinal orchiectomy with early and high ligation of the spermatic cord and retroperitoneal lymph node assessment.
Historically, routine surgical evaluation of retroperitoneal nodes was not recommended under most European protocols.
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.
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.
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.
The optimal number of nodes for retroperitoneal lymph node dissection is 8 to 12 to accurately capture nodal disease.
There is a potential role for sentinel lymph node biopsy using ICG in paratesticular tumors.
Transcrotal biopsy or excision has been reported in up to 25% of paratesticular rhabdomyosarcoma cases.
Historically, scrotal violation would mandate a hemiscrotomy.
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.
Current COG recommendation is to avoid hemiscrotomy unless there is direct evidence of scrotal invasion.
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.