These are reviewed studies whose abstracts concern Peritoneal Sarcoma. Each describes only what that study reported. This is not a claim by OncoForge that any compound helps or harms Peritoneal Sarcoma. Most are early lab, animal, or small human studies, and findings often conflict.
Case reportReported negativeLimited evidenceTier 3 · early humann = 1
International medical case reports journal · Jul 2026 · case report
intra-abdominal sarcomaprimary high-grade intraperitoneal sarcomapediatric abdominal tumor
This case report describes an 8-year-old girl in Somaliland who presented with a large abdominopelvic mass initially suspected to be a germ cell tumor. Empiric germ cell chemotherapy produced no response; surgery and histopathology revealed a high-grade intra-peritoneal sarcoma (desmin+, myogenin-), and the patient died about three months after treatment. The report emphasizes the diagnostic challenges when biopsy and molecular testing are limited.
Reported effects: cycles without response 2, n=1 · time to death 3 mo, n=1
Key findings
- Imaging suggested a germ cell tumor, and empiric germ cell tumor-directed chemotherapy was started.
- No clinical or radiological response was observed after two cycles of empiric chemotherapy.
- Exploratory laparotomy and cytoreductive surgery identified a large intra-peritoneal mass with omental and nodal involvement.
- Histopathology showed a high-grade malignant neoplasm with pleomorphic round-to-spindle cells and rhabdoid features; immunohistochemistry was diffusely desmin positive and myogenin negative; INI1 testing was unavailable.
- Despite postoperative chemotherapy the disease progressed and the patient died approximately 3 months after treatment.
Limitations: Single-patient case report (n=1).; Empiric therapy was given without pre-treatment histopathological confirmation.; Incomplete diagnostic work-up: INI1 testing and other molecular diagnostics were unavailable.; Short clinical follow-up (patient died ~3 months after treatment).; Findings from one case in a resource-limited setting may not generalize..
AI summary of the abstract, human-reviewed · Jul 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
ReviewMechanismInconclusiveLimited evidenceTier 3 · early human
Advances in anatomic pathology · Jan 2026 · narrative review
gynecologic neoplasmsembryonal rhabdomyosarcomaSertoli-Leydig cell tumorpleuropulmonary blastoma-like peritoneal sarcomaadenosarcomagynandroblastomajuvenile granulosa cell tumorSertoli cell tumorDICER1-related Wilms-like uterine tumor
This narrative review summarizes how germline and somatic DICER1 mutations are associated with a range of benign and malignant gynecologic neoplasms and describes their shared morphologic features. The authors note that a germline loss-of-function DICER1 mutation is often followed by a somatic hotspot (second-hit) mutation in tumors, and they recommend that recognition of characteristic morphology should prompt genetic testing and surveillance for patients and families. The review proposes the term "DICER1-related primitive polyphenotypic neoplasm" to encompass the diverse histologic features of these tumors.
Key findings
- DICER1 is crucial for microRNA biogenesis and maturation.
- Germline DICER1 mutations are associated with increased risk of a wide range of benign and malignant neoplasms; the same tumors can also arise sporadically via somatic DICER1 mutations.
- In syndromic patients, a germline loss-of-function DICER1 mutation is usually followed by a somatic hotspot mutation in the tumor as a second hit.
- DICER1-associated gynecologic neoplasms most commonly include embryonal rhabdomyosarcoma and moderately to poorly differentiated Sertoli-Leydig cell tumor, with several less frequent tumor types also described.
- DICER1-mutant gynecologic neoplasms frequently share characteristic morphology (primitive mesenchyme, fetal-type epithelium/cartilage, rhabdomyoblastic and/or neuroectodermal differentiation, osteoid formation, and anaplasia).
- Recognition of these distinctive morphologic features should prompt consideration of DICER1-associated neoplasm and genetic testing to facilitate surveillance for patients and families.
- The morphologic spectrum of most DICER1-mutant gynecologic neoplasms appears wider than that of any known type of sarcoma.
- The authors propose the term "DICER1-related primitive polyphenotypic neoplasm" to better capture the diverse histologic features.
Limitations: Narrative review without description of systematic search or methods — potential selection bias in included reports.; No primary data or quantitative synthesis (no new experimental or cohort data presented).; Extent of evidence, frequency estimates, and outcomes are not quantified in the abstract..
Summarizes the association between DICER1 mutations and a spectrum of gynecologic tumors and highlights implications for pathologic recognition and genetic testing.
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Human · observationalSafetyReported positiveLimited evidenceTier 3 · early humann = 1120
Journal of clinical medicine · May 2023 · retrospective single-center analysis
leiomyosarcomasarcomaperitoneal sarcoma
This retrospective single-center study reviewed 1120 laparoscopic procedures using electromechanical in-bag morcellation to remove large benign uterine specimens and evaluated practicability and safety. Most specimens were large (78.7% >250 g; 9% >1000 g); bag manipulation was reported as practicable with only two detected bag punctures and no peritoneal debris on cytology. Histology found one retroperitoneal angioleiomyomatosis and three unexpected malignancies (two leiomyosarcomas, one sarcoma); one patient developed abdominal metastases in year three and was lost to follow-up. The authors conclude in-bag morcellation was feasible for large tumors and rarely associated with detectable bag perforation or visible tissue spread in this series.
Reported effects: myomectomies 804 · supracervical hysterectomies 242 · +13 more
Key findings
- A total of 804 myomectomies, 242 supracervical hysterectomies, 73 total hysterectomies, and 1 retroperitoneal tumor extirpation were performed.
- A total of 78.7% of specimens weighed more than 250 g (n = 881) and 9% more than 1000 g.
- The largest specimens, weighing 2933 g, 3183 g, and 4780 g, required two bags for complete morcellation.
- Neither difficulties nor complications related to bag manipulation were recorded.
- Small bag puncture was detected in two cases, but peritoneal washing cytology was free of debris.
- One retroperitoneal angioleiomyomatosis and three malignancies were detected in histology (leiomyosarcoma = 2; sarcoma = 1); those patients underwent radical surgery.
- All patients were disease-free at 3 years follow-up, but one patient presented multiple abdominal metastases of the leiomyosarcoma in the third year and was lost from follow-up.
Limitations: Retrospective, single-center design with no control group or randomization.; Observational safety/feasibility data without a comparator arm (e.g., uncontained morcellation or open surgery).; Low number of unexpected malignancies (n = 3) limits conclusions about oncologic safety.; Peritoneal washing cytology may not detect all disseminated cells; no molecular assays reported to confirm absence of dissemination.; Follow-up described to 3 years but longer-term oncologic outcomes remain uncertain.; Potential selection and reporting biases inherent to retrospective series..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
Case reportReported positiveLimited evidenceTier 3 · early humann = 2
Annali italiani di chirurgia · Jan 2023 · Case report (two cases)
retroperitoneal sarcomaleiomyosarcomasoft tissue neoplasm
This report describes two women with large right-sided retroperitoneal leiomyosarcomas that involved the inferior vena cava who underwent radical en bloc multivisceral resection including part of the IVC wall. The IVC wall defect was closed by direct suture, which reduced the lumen calibre but produced no hemodynamic problems or endoluminal thrombi; histologic margins were negative and postoperative courses were uneventful with good caval flow.
Reported effect: case_count 2, n=2
Key findings
- Both patients underwent radical en bloc resection of the tumor with surrounding tissues and part of the right wall of the subrenal inferior vena cava.
- The IVC wall defect was repaired with direct suture repair, resulting in reduced calibre but no hemodynamic sequelae or endoluminal thrombi.
- All resection margins, including the IVC wall, were histologically negative for tumor invasion.
- Postoperative courses were unremarkable and caval blood flow was reported as optimal.
Limitations: Very small sample size (two case reports).; No control or comparison group.; No long-term follow-up or oncologic outcome data (recurrence, survival) reported.; Findings are surgical observations and may not generalize to other patients or centers.; No quantitative hemodynamic measurements or imaging data provided in the abstract..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
ReviewMechanismReported positiveLimited evidenceTier 3 · early human
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · Jan 2022 · narrative review
pleuropulmonary blastomalung neoplasmsSertoli-Leydig cell tumorgynandroblastomaembryonal rhabdomyosarcomamultinodular goiterdifferentiated thyroid carcinomapoorly differentiated thyroid carcinomacervical-thyroid teratomacystic nephromaanaplastic sarcoma of kidneynasal chondromesenchymal hamartomaintestinal juvenile-like hamartomatous polypciliary body medulloepitheliomapituitary blastomapineoblastomaprimary central nervous system sarcomaembryonal tumor with multilayered rosettes-like cerebellar tumorPPB-like peritoneal sarcomapresacral malignant teratoid neoplasm
This narrative review summarizes DICER1 tumor predisposition syndrome, an autosomal dominant disorder caused by heterozygous germline DICER1 mutations, and describes pleuropulmonary blastoma (PPB) as the most common associated tumor in early childhood. The review lists a broad spectrum of extrapulmonary neoplasms linked to DICER1 (each typically showing a second somatic DICER1 mutation), highlights overlapping histopathologic features and cystic-to-solid progression, and recommends that such findings should prompt testing for DICER1 mutations.
Reported effect: age_range_of_progression
Key findings
- DICER1 syndrome is an autosomal dominant tumor predisposition disorder caused by a heterozygous germline DICER1 mutation.
- Pleuropulmonary blastoma (PPB) is the most common tumor seen clinically in this syndrome and is classified into types (IR, I, II, III) with progression from cystic (type I) to solid (type III).
- A wide spectrum of extrapulmonary neoplasms (e.g., Sertoli-Leydig cell tumor, embryonal rhabdomyosarcoma, thyroid carcinomas, cystic nephroma, pineoblastoma, pituitary blastoma, others) have been associated with germline DICER1 mutations.
- Each of these neoplasms is characterized by a second somatic mutation in DICER1.
- Many of the associated tumors share overlapping histopathologic features, particularly primitive mesenchyme with rhabdomyoblastic and chondroid differentiation, and several show an initial cystic stage with progression to higher-grade neoplasms.
- Pathologic recognition of these features should alert pathologists and clinicians to consider DICER1-associated neoplasm testing.
Limitations: Narrative review: abstract does not report new primary patient-level data.; Abstract provides descriptive summary without systematic review methods or quantitative synthesis.; No sample sizes, incidence rates, or outcome metrics provided in the abstract.; Primarily descriptive/pathologic correlations; clinical management implications not detailed in abstract..
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 = 7
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · Oct 2020 · pathology review / case series
pleuropulmonary blastoma-like peritoneal sarcomaperitoneal sarcoma
The authors report a pathology review identifying seven pediatric cases of a primitive peritoneal sarcoma resembling pleuropulmonary blastoma. These tumors arose mainly from the fallopian tube or other peritoneal surfaces and exhibited histologic patterns similar to PPB; all seven cases had pathogenic DICER1 variation in germline and/or tumor DNA. The report proposes that abdominal or pelvic tumors with heterogeneous rhabdomyosarcomatous and/or cartilaginous differentiation should prompt germline and tumor DICER1 testing.
Reported effects: total_cases 7, n=7 · median_age 13, n=7 · +7 more
Key findings
- A total of seven cases were identified through pathology review in children presenting at a median age of 13 years (range 3-14 years).
- Primary sites of origin included the fallopian tube (four cases), serosal surface of the colon (one case), and pelvic sidewall (two cases).
- One case had pathologic features of type I PPB, another type Ir (regressed) PPB, and the remaining five had features of type II or III PPB with a mixed primitive sarcomatous pattern with or without cystic elements.
- All had a pathogenic DICER1 variation identified in germline and/or tumor DNA.
- Authors conclude that tumors arising from the fallopian tube or elsewhere in the abdomen/pelvis, especially those with heterogeneous rhabdomyosarcomatous and/or cartilaginous differentiation, should prompt consideration of germline and tumor DICER1 testing.
Limitations: Small sample size (seven cases).; Retrospective pathology review / case series design without a control group.; No clinical outcome, treatment, or long-term follow-up data reported in the abstract.; Potential selection and referral bias from cases identified via pathology review..
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 = 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
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
Animal studyReported positivePreclinical onlyTier 2 · animal
Pathology, research and practice · Sep 2018
peritoneal sarcomatosisperitoneal neoplasmssarcoma-180 ascites
In a mouse model of ascitic Sarcoma-180, the authors administered Ruta graveolens loco-regionally and examined tumour cells. They report increased anti-tumour immunity, tumour cell cytotoxicity, disruption of cellular energetics, induction of apoptosis and impairment of cell division. Expression of c-Myc and Aurora kinase A decreased while Chk-2 and CD95 increased in ascitic tumour cells. The authors conclude these findings indicate potential therapeutic utility of Ruta in managing malignant peritoneal ascites in this experimental model.
Key findings
- Loco-regional administration of Ruta graveolens in Sarcoma-180 ascites mice was associated with boosting of anti-tumour immunity and generation of tumour cell cytotoxicity.
- Ruta administration produced disruption of cellular energetics, induction of apoptosis and simultaneous impairment of cell division in tumour cells.
- Expressional decline of c-Myc oncoproteins and Aurora kinase A, together with upregulation of tumour suppressor Chk-2 and apoptosis inducer CD95, was observed in ascitic tumour cells after Ruta treatment.
Limitations: Animal study in mice only—no human data.; Abstract provides no sample size, dosing regimen, duration, or control/comparator details.; Endpoints reported are molecular and cytopathological/surrogate outcomes, not clinical outcomes such as survival or symptom relief.; Loco-regional administration in mice may not translate to human clinical settings..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Animal studyReported positivePreclinical onlyTier 2 · animal
Experimental oncology · Jul 2012
peritoneal sarcoma-related malignant ascitessarcoma-180
In mice bearing peritoneal sarcoma-180 malignant ascites, intraperitoneal 17-AAG was given in divided doses over 15 days. Treatment reduced malignant-ascites cell proliferation and viability, was associated with downregulation of Hsp90 client proteins including TERT, cyclin D1 and PCNA, induced micronucleus-containing (error-prone) cells, reduced GM-CSF–associated peripheral neutrophilia, and improved median survival time. The study reports these effects in an animal model and does not provide human data.
Key findings
- 17-AAG was administered intraperitoneally at 330 µg/kg/day for 5 days followed by 166 µg/kg/day for 10 days in mice with full-grown peritoneal sarcoma-180 ascites.
- Treatment led to drastic downregulation of TERT and cyclin D1 at the point of cell-cycle entry and reduced PCNA, attributed to modulation of Hsp90 folding machinery.
- Malignant ascitic cells exhibited mitotic errors and micronucleus formation and showed low viability after treatment.
- Peripheral neutrophilia driven by overexpression of GM-CSF from the ascites was controlled by 17-AAG treatment.
- Overall, the treatment modality improved median survival time in the treated mice.
Limitations: Animal study only (mouse model); no human data.; Abstract does not report sample sizes or statistical values.; No numeric magnitude or statistical significance reported for survival improvement or other endpoints in the abstract.; Comparator/control group details are not provided in the abstract.; Toxicity, safety, and dose-ranging data are not presented in the abstract..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Animal studyReported positivePreclinical onlyTier 2 · animal
Journal of pharmaceutical sciences · Sep 2005 · preclinical comparative study in mice (dose and survival comparisons)
In a mouse peritoneal cancer model, investigators produced anti-methotrexate Fab fragments (AMF), measured their pharmacokinetics, and tested whether systemic AMF could permit higher intraperitoneal methotrexate (MTX) doses and improve survival. AMF had a mean terminal half-life of 10.9 +/- 3.3 h and 28% +/- 7% s.c. bioavailability (at 2.2 g/kg). Co-administration of s.c. AMF (4.2 g/kg) increased the maximally tolerated i.p. MTX dose from 1.9 mg/kg to 10 mg/kg and increased median survival in some combination groups (e.g., to 17 and 14 days for MTX 7.5 or 10 mg/kg plus AMF).
Reported effects: mean terminal half-life of AMF 10.9 · s.c. bioavailability at 2.2 g/kg 28% · +7 more
Studied with: methotrexate.
Key findings
- The mean terminal half-life of AMF was found to be 10.9 +/- 3.3 h and was not dose-dependent, and s.c. bioavailability was 28% +/- 7% at 2.2 g/kg.
- In mice bearing peritoneal tumors, the maximally tolerated dose of i.p. MTX increased from 1.9 mg/kg (following i.p. MTX alone) to 10 mg/kg (with co-administration of s.c. AMF).
- Median survival times for saline-treated control animals and animals receiving i.p. MTX (1.9, 2.8, 3.8 mg/kg) were 9, 12, 10, and 7 days, respectively.
- For animals receiving combination therapy with i.p. MTX 7.5 or 10 mg/kg and 4.2 g/kg s.c. AMF, median survival time increased to 17 and 14 days, respectively.
Limitations: Animal (murine) study only — results may not translate to humans.; Single tumor model (Sarcoma 180) tested.; Sample sizes per group are not reported in the abstract.; No detailed toxicity or long-term outcome data reported in the abstract..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed