These are reviewed studies whose abstracts concern Renal Sarcoma. Each describes only what that study reported. This is not a claim by OncoForge that any compound helps or harms Renal Sarcoma. Most are early lab, animal, or small human studies, and findings often conflict.
ReviewInconclusiveLimited evidenceTier 3 · early human
Clinics in perinatology · Mar 2021
neonatal renal tumorsrenal leiomyosarcomarenal cell carcinomatransitional cell carcinomarenal sarcoma
This review summarizes how renal tumors present, are worked up, treated, and what outcomes are reported in the neonatal period. It notes that some lesions are detected prenatally but most present after birth, frequently as a palpable abdominal mass, and that cross-sectional imaging followed by radical nephrectomy is commonly used to obtain a specific histologic diagnosis. The abstract highlights that renal leiomyosarcoma is rare and aggressive with a high tendency for local recurrence and metastasis, and it reviews adult renal cell carcinoma epidemiology and subtypes for context.
Key findings
- Renal tumors are rare in the neonatal period; some may be detected prenatally but a greater proportion present after birth, most often with a palpable abdominal mass with or without other associated symptoms.
- Cross-sectional imaging is typically followed by radical nephrectomy to make a specific histologic diagnosis to determine the need for additional therapy.
- Renal leiomyosarcoma (LMS) is a rare and aggressive mesenchymal tumor that usually arises from smooth muscle cells of intrarenal blood vessels or the renal pelvis.
- Primary renal leiomyosarcomas represent 1%-2% of all malignant renal tumors, have a mean age at presentation of 50–60 years with female preponderance, show a high tendency of local recurrence, frequently metastasize via hematogenous spread, and carry an overall poor prognosis.
- Differentiation of primary renal leiomyosarcoma from sarcomatoid renal cell carcinoma is necessary because prognosis differs.
- Renal cell carcinoma (RCC) is the most common type of cancer arising in the kidney in adults, making up more than 9 out of 10 renal cancers in adults and accounting for over 3% of all adult malignancies; it is most commonly seen between ages 50 to 70 with an approximate 2:1 male to female ratio.
Limitations: Narrative review article; no new primary data or study-level results are presented in the abstract.; Abstract provides no sample sizes, quantitative neonatal outcome data, or details of methods used to identify or synthesize evidence.; Discussion includes adult epidemiology and age ranges that may not be applicable to neonatal patients..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
ReviewMechanismInconclusiveLimited evidenceTier 3 · early human
Acta neuropathologica · Apr 2020
pleuropulmonary blastomaovarian Sertoli-Leydig cell tumorcystic nephromamultinodular goiterthyroid carcinomaanaplastic sarcoma of the kidneyembryonal rhabdomyosarcomanasal chondromesenchymal hamartomametastases of pleuropulmonary blastoma to the cerebrumpituitary blastomapineoblastomaciliary body medulloepitheliomaprimary DICER1-associated CNS sarcomasETMR-like infantile cerebellar embryonal tumormacrocephaly (non-neoplastic phenotype)
This is a review of the central nervous system (CNS) manifestations of DICER1 syndrome, a rare tumor predisposition syndrome that mainly affects children and young adults. The authors summarize the genetic basis (germline loss-of-function DICER1 alterations with somatic RNase IIIb hotspot missense mutations), the wide spectrum of pleiotropic benign and malignant lesions with pleuropulmonary blastoma as the hallmark tumor, and previously defined CNS manifestations including several primary CNS tumors and macrocephaly as a non-neoplastic phenotype.
Key findings
- DICER1 syndrome is typically caused by heterozygous germline loss-of-function DICER1 alterations accompanied by somatic missense mutations at hotspots in the RNase IIIb domain.
- DICER1 encodes a component of the microRNA biogenesis machinery.
- The syndrome is highly pleiotropic and includes a constellation of benign and malignant neoplastic and dysplastic lesions.
- Pleuropulmonary blastoma (PPB) is the hallmark tumor of the syndrome.
- Other reported manifestations include ovarian Sertoli-Leydig cell tumor, cystic nephroma arising in childhood, multinodular goiter, thyroid carcinoma, anaplastic sarcoma of the kidney, embryonal rhabdomyosarcoma, and nasal chondromesenchymal hamartoma.
- CNS manifestations defined in the literature include PPB metastases to the cerebrum, pituitary blastoma, pineoblastoma, ciliary body medulloepithelioma, primary DICER1-associated CNS sarcomas, and ETMR-like infantile cerebellar embryonal tumor.
- Macrocephaly has been reported as a non-neoplastic, haploinsufficient phenotype associated with DICER1.
Limitations: Review article: no new primary patient-level data presented in the abstract.; DICER1 syndrome is rare, so the literature is likely composed of small case series and individual reports, limiting generalizability.; Abstract does not indicate systematic review methods or quantitative synthesis.; Heterogeneous and pleiotropic manifestations make it difficult to derive uniform clinical conclusions from a narrative review..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Human · observationalMechanismReported positiveLimited evidenceTier 3 · early humann = 3
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · Jan 2020 · case series (3 cases) with comprehensive literature review
ovarian sarcomaperitoneal sarcomaintracranial sarcomapleuropulmonary blastomagenitourinary embryonal rhabdomyosarcomaanaplastic sarcoma of the kidney
The authors report three pediatric sarcoma cases (ovarian with germline DICER1 mutation; metastatic peritoneal and primary intracranial with somatic DICER1 mutations) and performed a literature review of DICER1-associated sarcomas. The review (including 83 cases) shows a consistent heterogeneous histologic pattern similar to pleuropulmonary blastoma across sites. They recommend that this distinctive histology should prompt review of family history and DICER1 mutation testing to enable genetic counseling and imaging surveillance.
Reported effects: cases_reported 3, n=3 · literature_review_count 83, n=83
Key findings
- Reported three pediatric sarcomas associated with DICER1 mutations: a germline DICER1-associated ovarian sarcoma (5-year-old female), a somatic DICER1-associated metastatic peritoneal sarcoma (16-year-old female), and a somatic DICER1-associated primary intracranial sarcoma (4-year-old male).
- Comprehensive literature review including 83 DICER1-associated sarcomas demonstrates a consistent histologic pattern that mimics pleuropulmonary blastoma regardless of site.
- Characteristic histologic features include undifferentiated small round blue cells, poorly differentiated spindle cells, and large bizarre pleomorphic (anaplastic) cells, often with rhabdomyoblastic and/or chondroid differentiation and occasional bone/osteoid formation.
- The authors state that this heterogeneous histologic pattern should prompt detailed family-history review and DICER1 mutation analysis, facilitating genetic counseling, caregiver education, and imaging-based surveillance.
Limitations: Small case series (n=3) reported from a retrospective/case-report design; Descriptive literature review without systematic meta-analysis or pooled quantitative synthesis; No functional experiments in this report to demonstrate biological causality between DICER1 variants and the described histology; Findings may be subject to reporting/selection bias and limited generalizability.
Expands the phenotypic spectrum of DICER1-associated tumors and highlights a characteristic histology that may indicate underlying DICER1 mutations.
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
ReviewMechanismInconclusiveLimited evidenceTier 3 · early human
Archives of pathology & laboratory medicine · Aug 2019
clear cell sarcoma of the kidney
This is a concise review summarizing recent molecular and diagnostic advances in clear cell sarcoma of the kidney. The authors report that BCOR exon 15 internal tandem duplications are present in most cases, YWHAE-NUTM2 fusions occur in a few, and other alterations (including BCOR-CCNB3 fusion and EGFR mutations) account for remaining cases; several markers (cyclin D1, nerve growth factor receptor, BCOR) may help diagnosis. The review highlights diagnostic challenges due to histologic diversity.
Key findings
- BCOR exon 15 internal tandem duplications have been uncovered in most cases of clear cell sarcoma of the kidney.
- YWHAE-NUTM2 fusion is present in a few cases.
- Remaining cases have other genetic mutations, including BCOR-CCNB3 fusion and EGFR mutations.
- Clear cell sarcoma of the kidney has no specific immunophenotype, but markers including cyclin D1, nerve growth factor receptor, and BCOR have emerged as potential diagnostic aides.
Limitations: This publication is a review article and does not present new primary patient-level data.; The tumor is described as histologically diverse, which the authors state makes accurate diagnosis challenging in some cases.; No single, specific immunophenotype exists for this tumor type; reported molecular alterations occur in subsets of cases..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
ReviewMechanismInconclusiveModerate evidenceTier 3 · early human
Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnosti · Jul 2019
pleuropulmonary blastomamultinodular goiterovarian Sertoli-Leydig cell tumorcystic nephromamedulloepithelioma (ciliary body/iris)embryonal rhabdomyosarcoma (botryoid type)nasal epithelial/chondromesenchymal hamartomapituitary blastomapineoblastomadifferentiated thyroid carcinomapulmonary blastomawell-differentiated fetal lung adenocarcinomaanaplastic sarcoma of the kidneyprimary ovarian sarcomaPPB-like peritoneal sarcomamulticystic liver neoplasmsWilms tumor
This article summarizes the clinical features, genetic diagnosis, management, and surveillance recommendations for DICER1 syndrome, an inherited disorder caused by germline DICER1 pathogenic variants that predispose to a spectrum of benign and malignant tumors. It lists the most common associated tumors (e.g., pleuropulmonary blastoma, thyroid nodules, ovarian Sertoli-Leydig cell tumors) and gives recommended surveillance schedules and guidance on genetic testing and cascade testing for relatives. The authors note autosomal dominant inheritance with reduced penetrance and state diagnosis is by identification of a heterozygous germline DICER1 pathogenic variant.
Key findings
- DICER1 syndrome is caused by pathogenic variants in the DICER1 gene (located at chromosome 14q32.13) and is associated with increased risk of a spectrum of malignant and benign tumors.
- The most common clinical features include lung cysts and thyroid nodules; common neoplasms include pleuropulmonary blastoma, Sertoli-Leydig cell tumor, pediatric cystic nephroma, and differentiated thyroid carcinoma.
- A broad and variable tumor spectrum is reported, with many tumors occurring before age 40 and PPB typically presenting in children younger than seven years.
- Diagnosis is established by identification of a heterozygous germline DICER1 pathogenic variant presumed to cause loss of function.
- Management of DICER1-associated tumors is tumor-type dependent and may include surgery, chemotherapy, and in some cases radiation; surveillance recommendations (based on the 2016 International PPB Register) are provided for chest imaging, thyroid ultrasound, pelvic and abdominal ultrasound, ophthalmologic assessment, and neurologic monitoring.
- Genetic counseling is recommended, with cascade testing of at-risk first-degree relatives and consideration of testing soon after birth because screening often begins in infancy.
Limitations: This is a review/clinical overview rather than original primary quantitative research.; Surveillance recommendations are presented but the abstract does not provide quantitative evidence of their effectiveness.; Recommendations appear to be based on existing guidance (2016 International PPB Register) and may reflect expert consensus rather than prospective trial data.; Variable penetrance and broad tumor spectrum limit precise risk prediction for individual carriers..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
ReviewMechanismReported positiveModerate evidenceTier 3 · early human
Epigenomics · May 2019
clear cell sarcoma of the kidneyprimitive mesenchymal myxoid tumor of infancysmall round blue cell sarcomaendometrial stromal sarcomaCNS-HGNET-BCOR (histologically heterogeneous CNS neoplasms group)mesenchymal tumorsepithelial tumorsneural tumorshematological tumors
This is a review summarizing evidence that BCOR, an epigenetic regulator in the noncanonical polycomb repressive complex 1, is involved in cancer. The authors report that specific BCOR internal tandem duplications and recurrent gene fusions (notably BCOR-CCNB3, BCOR-MAML3 and ZC3H7B-BCOR) act as driver alterations in several sarcomas and a CNS tumor group, and that other BCOR loss-of-function alterations recur across many tumor types.
Key findings
- BCOR encodes an epigenetic regulator involved in specification of cell differentiation and participates in the noncanonical polycomb repressive complex 1.
- Internal tandem duplications of the PCGF Ub-like fold discriminator domain and several gene fusions (mainly BCOR-CCNB3, BCOR-MAML3 and ZC3H7B-BCOR) represent driver elements of various sarcomas and a CNS tumor group (CNS-HGNET-BCOR).
- BCOR alterations, often loss-of-function mutations, recur across a wide variety of mesenchymal, epithelial, neural and hematological tumors, suggesting a central role in cancer evolution.
Limitations: Narrative review without primary data generation; methods for literature selection are not described in the abstract.; No quantitative synthesis or new experimental results presented in the abstract.; Heterogeneity of tumor types cited limits specificity about prevalence, effect size, or clinical impact.; Causality and clinical implications are not established by a review summary alone..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
ReviewInconclusiveLimited evidenceTier 3 · early human
Clinical cancer research : an official journal of the American Association for Cancer Research · May 2018
pleuropulmonary blastoma (PPB)ovarian sex cord-stromal tumorsSertoli-Leydig cell tumorlung cystscystic nephromarenal sarcomaWilms tumornodular hyperplasia of the thyroidnasal chondromesenchymal hamartomaciliary body medulloepitheliomagenitourinary embryonal rhabdomyosarcomapineoblastomapituitary blastomagastrointestinal polyps
This paper reports an expert consensus from the inaugural International DICER1 Symposium summarizing genetic testing and surveillance recommendations for people with pathogenic germline DICER1 variants. It lists the spectrum of associated tumors and lesions, recommends education and imaging-based surveillance (with emphasis that risks are highest in early childhood and decline in adulthood), and states that guidelines will be updated as research expands.
Key findings
- Pathogenic germline DICER1 variants cause a hereditary cancer predisposition syndrome with a variety of manifestations.
- Associated neoplasms and lesions include pleuropulmonary blastoma (PPB), ovarian sex cord-stromal tumors (particularly Sertoli-Leydig cell tumor), lung cysts, cystic nephroma, renal sarcoma and Wilms tumor, nodular hyperplasia of the thyroid, nasal chondromesenchymal hamartoma, ciliary body medulloepithelioma, genitourinary embryonal rhabdomyosarcoma, and brain tumors including pineoblastoma and pituitary blastoma.
- The International PPB Registry convened a multidisciplinary international symposium to develop consensus testing, surveillance, and treatment recommendations.
- Recommendations are provided for genetic testing, prenatal management, and surveillance for DICER1-associated pulmonary, renal, gynecologic, thyroid, ophthalmologic, otolaryngologic, and central nervous system tumors and gastrointestinal polyps.
- Risk for most DICER1-associated neoplasms is highest in early childhood and decreases in adulthood.
- Individual and caregiver education and judicious imaging-based surveillance are the primary recommended approaches.
- These recommendations reflect a consensus of expert opinion and current literature and will be updated as DICER1 research expands.
Limitations: Recommendations are based on expert consensus and existing literature rather than new primary data reported in this article.; Abstract does not present quantitative risk estimates, systematic evidence grading, or methodological details of literature review.; Guidance may change as further research in DICER1-associated conditions emerges..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
Human · observationalMechanismReported positiveLimited evidenceTier 3 · early humann = 16
Pediatric radiology · Sep 2017 · retrospective analysis
pleuropulmonary blastomapineoblastomaovarian Sertoli-Leydig cell tumorembryonal rhabdomyosarcomarenal sarcomacystic nephromathyroid nodule/cystrenal cystpineal cyst
This retrospective review analyzed imaging from 16 pediatric patients (≤18 years) with germline DICER1 variants seen from January 2004 to July 2016. The authors report a spectrum of DICER1-associated malignant tumors (in 68.8% of patients) and benign lesions (in 37.5%), and describe a common imaging appearance they call the "cracked windshield" sign. They conclude that early surveillance of at-risk patients is important while minimizing ionizing radiation exposure.
Reported effects: patients included 16, n=16 · female patients 12, n=16 · +13 more
Key findings
- Sixteen patients were included (12 females; mean age at presentation: 4.2years, range: 14days to 17years), with surveillance imaging encompassing chest X-ray and CT; abdominal, pelvic and neck US; and brain and whole-body MRI.
- Malignant lesions (68.8% of patients) included pleuropulmonary blastoma (5), pineoblastoma (3), ovarian Sertoli-Leydig cell tumor (1), embryonal rhabdomyosarcoma (1) and renal sarcoma (1).
- Benign lesions (37.5% of patients) included thyroid cysts (2), thyroid nodules (2), cystic nephroma (2), renal cysts (1) and pineal cyst (1).
- A common lesional appearance observed across modalities and organs was defined as the "cracked windshield" sign.
- Authors suggest early surveillance of at-risk patients while minimizing exposure to ionizing radiation.
Limitations: Retrospective design; Small sample size (n=16); Single tertiary pediatric centre (potential limited generalizability); Descriptive imaging study without control group or assessment of outcomes of surveillance; Possible selection bias from keyword-driven imaging database search and cross-referencing with institutional genetics database.
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed