Health Condition 1: C498- Malignant neoplasm of overlappingsites of connective and soft tissue
Conditions
Interventions
Intervention1: Nil: Nil
Intervention2: Nil: Nil
Sponsors
Dr Badira Cheriyalinkal Parambil
Eligibility
Inclusion criteria
Inclusion criteria: Children less than or equal to 18 years of age with a diagnosis of sarcoma at first diagnosis or relapse or progression There is a diagnostic dilemma in defining the tumor type or the tumor is defined as difficult to treat by the available current modalities (chemotherapy, radiotherap, surgery) or has a described targetable mutation warranting sequencing as listed below Pediatric extracranial sarcomas which would be eligible for testing in the study (There will be central pathology review of blocks and slides from other institutes at the nodal centre to confirm eligibility) 1. Diagnosis (at first diagnosis or progression or relapse- tumors lacking definitive diagnosis or classical genomic findings after histologic review and standard molecular testing)- any of the below a. Undifferentiated round cell sarcomas (NKX 2.2 on IHC atypical or negative) b. Sarcomas with fibrosarcoma morphology or NTRK positive on Immunohistochemistry (IHC) c. Pediatric spindle cell sarcomas, Not Otherwise Specified (NOS) d. Tumors which are not categorizable by basic morphology, IHC or molecular testing e. Low-risk fusion negative Rhabdomyosarcomas (RMS), intraosseous RMS, Spindle cell sclerosing (SS) RMS 2. Therapeutic (any of the below) a. All those included above in the diagnostic category will be explored for therapeutic targets b. High grade sarcomas treated with a curative intent which are; metastatic at first diagnosis (All histological variants of RMS, synovial sarcomas, Non Rhabdomyomatous Soft Tissue Sarcomas) or unresectable NRSTS or At relapse or progression of sarcomas (All histological variants of RMS, synovial sarcomas, undifferentiated round cell sarcomas, sarcomas with IHC positive for NRTK, NRSTS, spindle cell sarcomas NOS) c. High-risk sarcomas with an expected 3-year EFS less than 30% at first diagnosis or progression or relapse with contemporary treatment modalities (chemotherapy, surgery or radiotherapy) 3. Availability of sufficient tumor specimen for genomic profiling from diagnosis, progression, or relapse (whichever is the indication at enrolment)
Exclusion criteria
Exclusion criteria: If the current diagnosis qualifies as a CNS tumor or hematolymphoid malignancy If the pathology is confirmed to be a benign histology
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The percentage of patients with a diagnostic or therapeutic decision based on sequencing will be calculated. Also, the incremental value above the decision based on conventional pathology techniques (morphology/ immunohistochemistry/ routinely used FISH or PCR wherever indicated) will be assessed in making diagnostic or therapeutic decisions as percentage.Timepoint: Diagnosis | — |
Secondary
| Measure | Time frame |
|---|---|
| Event free survival will be calculated from the time of diagnosis of the current malignancy to the date of event while on study. The events will be relapse or progression or second malignancy or death due to any cause. If no event, the patient will be censored at last follow-up. Relapse free survival will be calculated from the time of diagnosis of the current malignancy to the date of relapse or progression while on study, or censored at last follow-up if neither occurred. Overall Survival will be calculated from the time of diagnosis of the current malignancy to the date of death due to any cause while on study. The survival analysis of the patients will be done at the end of the three-year study period for the various subsets of sarcomas.Timepoint: Diagnosis, Relapse, Progression | — |
Countries
India
Contacts
Public ContactDr Badira Cheriyalinkal Parambil
Tata Memorial Hospital
Outcome results
None listed