DeCure for Extraskeletal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
DeCure's autonomous Cancer AI scientist is researching a drug-repurposing hypothesis for Extraskeletal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor — screening already-approved drugs against its 43-gene Open Targets disease module to publish open-access research. Research is fast; the path to publication is funded in milestone stages.
Disease moduleExtraskeletal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor maps to a 43-gene Open Targets module — the target space DeCure's AI scientist screens approved drugs against.
DeCure.ai methodSignature reversal (LINCS) plus network proximity (STRING) rank already-approved drugs likely to perturb this module — the same engine that produces DeCure.ai's repurposing hypotheses.
Repurposing thesisScreening approved medicines against this disease module, then publishing the evidence for the strongest candidate. Known pharmacology and human exposure data make the first question sharper — they do not establish safety or efficacy in a new indication.
Research record
01
ResearchComing soon
Candidate research + dossier — target rationale, drug-repurposing thesis and evidence pack.proof: Published dossier + on-chain hash
02
ValidationComing soon
In-vitro biological validation at a contract research org (CRO).proof: CRO contract + in-vitro report
03
Peer review & paperComing soon
Peer-reviewed paper published open-access (preprint + journal).proof: DOI + open-access link + on-chain hash
Current lead
No approved-drug candidate for extraskeletal ewing sarcoma/peripheral primitive neuroectodermal tumor is corroborated in the literature DeepSearch retrieved. Some conditions are managed with non-pharmacological care — a device, surgery or physical therapy — rather than a medicine; that may be the case here, or the literature we found may simply be too sparse yet to support a drug-repurposing angle.
Molecular view
NRAS proto-oncogene, GTPase (NRAS) — NRAS is one of the genes genetically linked to this disease in Open Targets — shown as context, not as a drug target we're pursuing: no approved-drug candidate for this disease is yet corroborated in the literature we found.
Loading structure…
helix sheet gdpdrag to rotate · scroll to zoom
RCSB Protein Data Bank · entry 6ZIO · 1.55 Å · ligand GUANOSINE-5'-DIPHOSPHATE (GDP). Experimental structure, not a prediction.
What the evidence adds up to
Extraskeletal Ewing sarcoma and peripheral primitive neuroectodermal tumour are treated as clinically similar entities. In a retrospective review of 34 patients treated between 1964 and 1991, 5-year overall survival was 50% for extraskeletal Ewing sarcoma (21 patients) and 44% for peripheral neuroectodermal tumour (13 patients); 5-year disease-free survival was 33% for both. Survival was higher for Stage III than Stage IV disease, and there was a tendency toward better outcome after complete surgical resection. Survivors tended to be younger at diagnosis. Adjunctive radiotherapy or chemotherapy was given in 95% of Ewing sarcoma patients and 85% of peripheral neuroectodermal tumour patients.
Prognosis is poor when the tumour arises around the spinal column. In a series of four patients, all received high-dose chemotherapy with or without radiotherapy after surgery; three died of disease. The single patient who remained alive and disease-free had undergone en bloc resection combined with multiagent chemotherapy followed by high-dose chemotherapy with peripheral blood stem cell transplantation. The authors state that multiagent chemotherapy combined with en bloc resection and radiation is the preferred treatment for this location.
Primary cutaneous extraskeletal Ewing sarcoma appears to follow a more indolent course with a favourable prognosis compared with deep soft-tissue disease, according to a 2014 case report and literature review. Primary renal Ewing sarcoma is described as a rare entity with aggressive behaviour and poor prognosis; diagnosis relies on histopathology, immunohistochemistry, and cytogenetic molecular studies, as clinical and imaging findings are non-specific.
What is missing are prospective trials large enough to stratify by anatomical site, age, and extent of resection; consistent molecular characterisation to distinguish Ewing sarcoma from other small round cell tumours; and funding to move beyond single-centre retrospective reports.
Evidence
Retrieved by DeepSearch across 234,678,978 indexed works and resolved on OpenAlex — ranked by citations, including the results that did not work.
Spine · 2003 · 75 citations
Primitive Neuroectodermal Tumor and Extraskeletal Ewing Sarcoma Arising Primarily Around the Spinal Column
AbstractSTUDY DESIGN: Report of four cases and a review of the literature. OBJECTIVES: To study the clinical features and prognosis of primitive neuroectodermal tumor or extraskeletal Ewing sarcoma arising around the spinal column. SUMMARY OF BACKGROUND DATA: Primitive neuroectodermal tumor or extraskeletal Ewing sarcoma that originates around the spinal column is very rare, and its prognosis is very poor. METHODS: Four patients were diagnosed and underwent treatment. RESULTS: Although all the patients received high-dose chemotherapy with or without radiotherapy after surgery, three patients died of the disease. Only one patient who received en bloc resection of the tumor combined with multiagent chemotherapy followed by high-dose chemotherapy with peripheral blood stem cell transplantation remains alive and continues to be disease free. CONCLUSION: The prognosis of the patients with primitive neuroectodermal tumor or extraskeletal Ewing sarcoma around the spinal column is very poor. Multiagent chemotherapy combined with en bloc resection and radiation therapy is the preferred treatment for patients with primitive neuroectodermal tumor or extraskeletal Ewing sarcoma around the spinal column.
Clinical Orthopaedics and Related Research · 1996 · 39 citations
Comparison of Soft Tissue Ewing's Sarcoma and Peripheral Neuroectodermal Tumor
AbstractThirty-four cases with either extraskeletal Ewing's sarcoma (21 patients) or malignant peripheral neuroectodermal tumor of the soft tissues (13 patients) were reviewed retrospectively. The patients were treated between 1964 and 1991. Followup periods averaged 7.2 years for patients with peripheral neuroectodermal tumors and 10.4 years for patients with Ewing's sarcoma (minimal followup of 2 years). There were no significant differences in patient's age, gender, tumor location, and stages on presentation between patients with Ewing's sarcoma and those with malignant peripheral neuroectodermal tumor of the soft tissue. All but 2 patients underwent surgery for tumor resection. Adjunctive therapy (radiation or chemotherapy or both) was administered in 95% of the patients with Ewing's sarcoma and in 85% of the patients with peripheral neuroectodermal tumors. The 5-year overall survival was 50% for Ewing's sarcoma and 44% for peripheral neuroectodermal tumor. The 5-year disease free survival was 33% for both types of tumors. Survival rates were higher for Stage III compared with Stage IV disease for both types of tumors. There was a tendency for better outcome after complete surgical tumor resection. Survivors tended to be of a younger age at the time of tumor diagnosis. The results suggest that from a clinical perspective, these 2 tumors are quite similar.
European Journal of Dermatology · 2014 · 5 citations
Primary cutaneous extraskeletal Ewing's sarcoma/PNET: possibility of better prognosis than deep ES/PNET
AbstractExtraskeletal Ewing sarcoma (ES) and Primitive Neuroectodermal Tumour (PNET) were first described by Angerval and Enzinger in 1975 [1]. Primary ES/PNET arising in skin is distinctly rare [2-6]. Unlike ES/PNET in deep soft tissue, which has a poor prognosis, cutaneous and subcutaneous ES seem to be associated with an indolent course and a favourable prognosis [3-5, 7]. We report a case of cutaneous ES (CES) in a young adult and additionally review the cases of CES which have been previously reported [...]
Primary Adult Renal Ewing’s Sarcoma: A Rare Entity
AbstractEwing's sarcoma/primitive neuroectodermal tumors are high-grade small round blue cell tumors traditionally found in children and adolescents.These tumors primarily affect the bone and soft tissue, with extraskeletal sites rarely being affected. The clinical presentation and imaging findings are non-specific and are not characteristic. The diagnosis is essentially based on the histopathologic findings assisted by immunohistochemistry and/or cytogenetic molecular studies. Proper diagnoses and timely management of this tumor are essential owing to the aggressive nature and poor prognosis of the disease.
Journal of Pediatric Orthopaedics · 1997 · 0 citations
COMPARISON OF SOFT TISSUE EWING'S SARCOMA AND PERIPHERAL NEUROECTODERMAL TUMOR
AbstractThirty-four cases with either extraskeletal Ewing's sarcoma (21 patients) or malignant peripheral neuroectodermal tumor of the soft tissues (13 patients) were reviewed retrospectively. The patients were treated between 1964 and 1991. Followup periods averaged 7.2 years for patients with peripheral neuroectodermal tumors and 10.4 years for patients with Ewing's sarcoma (minimal followup of 2 years). There were no significant differences in patient's age, gender, tumor location, and stages on presentation between patients with Ewing's sarcoma and those with malignant peripheral neuroectodermal tumor of the soft tissue. All but 2 patients underwent surgery for tumor resection. Adjunctive therapy (radiation or chemotherapy or both) was administered in 95% of the patients with Ewing's sarcoma and in 85% of the patients with peripheral neuroectodermal tumors. The 5-year overall survival was 50% for Ewing's sarcoma and 44% for peripheral neuroectodermal tumor. The 5-year disease free survival was 33% for both types of tumors. Survival rates were higher for Stage III compared with Stage IV disease for both types of tumors. There was a tendency for better outcome after complete surgical tumor resection. Survivors tended to be of a younger age at the time of tumor diagnosis. The results suggest that from a clinical perspective, these 2 tumors are quite similar.
Disease module: DeepOracle (Open Targets). Structures: RDKit from PubChem SMILES. Literature: retrieved by DeepSearch across 234,678,978 indexed works (targeted per-candidate search), resolved on OpenAlex.
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