Sarcoma Flashcards
(11 cards)
Osteosarcoma path
Tumor osteoid
A 15-year-old boy presents with a paratesticular mass. A surgeon already has completed an inguinal orchiectomy with removal of the mass, and pathology confirms embryonal rhabdomyosarcoma. Scrotal skin was not involved with the tumor. You order a CT of the chest, abdomen, and pelvis and a PET scan. No other abnormalities are seen.
It is recommended that all boys aged 10 years or older undergo retroperitoneal nodal assessment.
Paratesticular rhabdomyosarcoma rarely involves inguinal lymph nodes unless scrotal skin is involved
Regional node sampling in RMS
Regional node sampling is generally recommended for rhabdomyosarcoma of the extremity.
Radiotherapy for RMS
Radiotherapy for rhabdomyosarcoma is usually based on group assignment.
Group 1 –> No RT
Group IIB; the primary tumor has been completely resected, but there is evidence for regional lymph node involvement without remaining gross disease. —-> give RT for LN only.
Group IIA or III–> The patient needs radiotherapy to the primary site only.
group IIC ( residual disease) or III if there is evidence of nodal involvement.–> RT to primary site and LN
infantile fibrosarcoma (IF),
a high-grade soft tissue sarcoma occurring in early infancy and molecularly characterized by NTRK gene fusions, most commonly ETV6-NTRK3.
Treatment with upfront complete surgical resection may be pursued, but only if functional and cosmetic outcomes are acceptable, and approximately 50% are considered unresectable at diagnosis.
Historically, neoadjuvant chemotherapy with VA or VAC has been used to facilitate surgical resection.
Larotrectinib and entrectinib are targeted inhibitors of TRK kinases and are FDA approved in all ages for TRK-fusion positive malignancies such as IF.
They should be considered as upfront therapy for unresectable IF.
Radiation and doxorubicin-ifosfamide would not be considered for initial therapy of this disease.
BCOR round cell sarcoma.
BCOR round cell sarcomas are thought to be slightly less aggressive than many of the other EWS-like sarcomas.
They are more typically treated with doxorubicin and ifosfamide, without interval compression.
Synovial sarcoma
Synovial sarcoma is both the most common pediatric NRSTS, and the most chemotherapy-sensitive pediatric NRSTS.
The Children’s Oncology Group clinical trial ARST0332 established a risk-based treatment strategy for NRSTS according to pretreatment risk factors and achieved favorable outcomes for patients with both low and intermediate risk disease.
non-metastatic grossly resected or unresected tumors >5 cm in size and of high grade are stratified as intermediate risk and treated with chemoradiotherapy.
Patients with synovial sarcoma had excellent outcomes with this multimodal approach.
TX with chemo/ radiation
A 10-month-old girl with localized vaginal rhabdomyosarcoma and no family cancer history
is there genetic testing needed ?
YES
Data suggest that a relatively high proportion of children younger than 3 years with rhabdomyosarcoma have TP53 mutations. Some of these are germline. This has implications for monitoring of the affected child, because screening according to the Toronto Protocol is associated with improved survival.
You are seeing a 15-year-old patient with newly diagnosed conventional osteosarcoma of the right distal femur. Chest CT shows seven 1+-cm nodules with a bilateral distribution. All are intraparenchymal, and some have calcifications.
Surgery will be staged, with one surgery to remove the primary tumor and a later surgery or surgeries to remove any remaining lung nodules. Chemotherapy will include ifosfamide among other agents and will be given at the start of treatment as well as interspersed between surgeries.
CTNNB1 mutation.
Desmoid tumors are locally invasive tumors that do not metastasize.
Although they can cause substantial morbidity, as well as mortality (particularly in cases of intra-abdominal disease), they typically behave in indolent fashion.
In cases of minimally symptomatic disease where progression does not pose an immediate risk, active surveillance is the recommended initial management strategy, as many desmoids will remain dormant or undergo spontaneous regression.
Surgical debulking is not recommended, particularly given the high rate of disease recurrence following both complete and partial resection.
Vinblastine-methotrexate is an active chemotherapy regimen for desmoid tumors but is not indicated in this case and tyrosine kinase inhibitors such as sorafenib are often used before conventional chemotherapy.
The neonatal intensive care unit (NICU) consults you regarding a 7-day-old boy who was born with a rapidly enlarging mass on his tongue. Plastic surgery performed a biopsy of the mass on day of life 2. Bleeding post biopsy was controlled, but the child has remained in the NICU for observation. The mass continues to enlarge. The patient has a normal chest x-ray and normal platelet count. Molecular pathology calls you to report the mass has a NTRK-ETV6 fusion. Diagnosis?
NTRK-ETV6 is a fusion oncoprotein diagnostic for infantile fibrosarcoma. Larotrectinib is a NTRK inhibitor that is FDA approved for patients of any age with NTRK fusion positive tumors and is available in a liquid formulation. It is safe and effective in achieving rapid and durable complete remission. Entrectinib, an NTRK, ALK, and ROS inhibitor, is FDA approved for patients older than 12 years and is available only in capsule form. NTRK-ETV6 is not associated with vascular malformations.
