Human · observationalReported positiveLimited evidenceTier 3 · early humann = 10
BMC cancer · Dec 2024 · retrospective cohort
Trastuzumab-deruxtecan-t-dxdTrastuzumab-deruxtecanendometrial neoplasmsovarian neoplasmscervical neoplasmsuterine carcinosarcomauterine leiomyosarcomauterine serous carcinomaovarian carcinosarcomahigh-grade serous ovarian carcinomamucinous ovarian carcinomasquamous cervical carcinoma This retrospective single-center study identified 10 patients with HER2-expressing (IHC 2+/3+) recurrent or metastatic gynecological cancers who received trastuzumab deruxtecan (5.4 mg/kg IV every 3 weeks). The cohort had a median progression-free survival of 5.4 months (95% CI 0.8-9.8). Five patients had a partial response, one had stable disease at 12 weeks, and four had disease progression at initial assessment. Clinical benefit was observed mainly in tumors with HER2 IHC 3+.
Reported effects: median PFS 5.4 mo [0.8–9.8], n=10 · partial responses 5, n=10 · +2 more
Key findings
- 10 patients with recurrent/metastatic HER2-expressing gynecological malignancies were treated with T-DXd.
- Histologies included uterine neoplasms (n=5), cervical squamous carcinoma (n=1) and ovarian cancers (n=4).
- Median age was 65.4 years (25th-75th percentile, 58.1-75.2 years).
- HER2 by IHC: 5 patients were 3+ and 5 patients were 2+.
- Median number of prior therapy lines was 4 (range 2-6); 2 uterine serous carcinoma patients were pretreated with trastuzumab and 4 patients had prior immunotherapy.
- Dose: T-DXd 5.4 mg/kg IV every 3 weeks until progression/toxicity.
- Median progression-free survival (PFS) in the cohort was 5.4 months (95% CI 0.8-9.8 months).
- Responses: 5 patients had partial response (including 2 previously treated with trastuzumab), 1 patient had stable disease at 12 weeks, 4 patients had disease progression at initial assessment.
- Most patients who derived clinical benefit had HER2 IHC 3+ expression.
Limitations: Retrospective, single-center design; Very small sample size (n=10); No control or comparator arm; Heterogeneous mix of gynecologic histologies; Heavily pre-treated population limits generalizability; Limited/absent reporting of safety or adverse event data in the abstract; Potential selection and reporting bias 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
ReviewMechanismMixed resultsLimited evidenceTier 4 · clinical
Cells · Jun 2024
uterine leiomyosarcoma
This is a narrative review summarizing current knowledge about uterine leiomyosarcoma (uLMS), including its poor prognosis and high rates of recurrence and metastasis. The authors discuss multiple biological pathways implicated in uLMS (DNA repair, immune checkpoints, protein kinases, hedgehog), the emerging roles of epigenetics and the epitranscriptome, biomarkers and diagnostic approaches (including AI and shear wave elastography), current medical management, and ongoing clinical trials. The abstract notes that drugs targeting abnormal pathway functions have been reported to improve survival but that chemotherapy resistance remains a major challenge.
Key findings
- uLMS has a poor prognosis with high rates of recurrence and metastasis; five-year survival reported between 25 and 76%, and survival approaches 10-15% for patients with metastatic disease at initial diagnosis.
- Multiple biological pathways are implicated in uLMS pathogenesis, including DNA repair defects, immune checkpoint pathways, protein kinases/intracellular signaling, and the hedgehog pathway.
- The review states that drugs that block abnormal functions of these pathways have been reported to remarkably improve survival in uLMS patients.
- Chemotherapy resistance remains a major unmet need, motivating the search for novel drugs that effectively target these pathways.
- The authors review emerging roles of epigenetics and the epitranscriptome, as well as serum markers, AI/machine learning approaches, shear wave elastography, current management options, and ongoing clinical trials.
Limitations: Narrative review rather than primary research — no original patient-level data presented.; Abstract does not state systematic review methods or a structured literature search, so selection bias in included literature is possible.; Broad scope synthesizes preclinical, early-phase, and clinical data together, which may mix evidence levels.; Survival ranges reported are wide and drawn from the literature rather than newly generated cohort data..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
Human · observationalMechanismReported positiveModerate evidenceTier 3 · early humann = 167
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · Apr 2023 · diagnostic test development with test and validation cohorts (molecular-based IHC correlated with targeted NGS)
uterine leiomyosarcomauterine smooth muscle tumorsuterine leiomyomasmooth muscle tumor of uncertain malignant potential (STUMP)low-grade endometrial stromal sarcoma
The authors sequenced 167 uterine leiomyosarcomas to identify common genomic alterations and developed an immunohistochemical (IHC) algorithm based on those genomic landmarks. In test and validation cohorts the IHC panel (p53, Rb, PTEN, ATRX, with follow-up markers DAXX, MTAP, MDM2) showed multiple abnormal markers in most LMS but not in leiomyomas. Agreement among pathologists and concordance between IHC and NGS were high.
Reported effects: NGS cohort size 167, n=167 · test cohort LMS n 16, n=16 · +16 more
Key findings
- Overall, 94% of LMS showed ≥1 genomic alteration involving TP53, RB1, ATRX, PTEN, CDKN2A, or MDM2; 80% showed alterations in ≥2 of these genes.
- In the test cohort, abnormal IHC expression was seen in 75% (p53), 88% (Rb), 44% (PTEN), and 38% (ATRX) of LMS.
- Two or more abnormal IHC results among p53, Rb, PTEN, and ATRX were seen in 81% of LMS in the test cohort.
- STUMPs demonstrated only a single IHC abnormality involving these markers, and no IHC abnormalities were seen in leiomyomas in the test cohort.
- In the validation cohort abnormal p53, Rb, and PTEN IHC results were seen in LMS; rare STUMP or leiomyomas with bizarre nuclei showed abnormalities involving only 1 marker.
- Both diagnostically challenging smooth muscle tumors in the validation set had abnormalities in ≥2 markers, supporting a diagnosis of LMS.
- Concordance among pathologists for IHC interpretation was κ = 0.97, and concordance between IHC and NGS results was κ = 0.941.
Limitations: Test and validation cohorts were relatively small (test cohort: 16 LMS; validation cohort: 8 LMS and other tumor types).; No clinical outcome or prognostic correlation data reported in the abstract.; Not a prospective, multicenter diagnostic accuracy study; broader external validation is not reported in the abstract.; The abstract does not report sensitivity, specificity, or formal diagnostic accuracy statistics beyond concordance/kappa..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
ReviewMixed resultsLimited evidenceTier 3 · early humann = 80
International journal of environmental research and public health · Jul 2021 · Literature review of publications from 2010 to 2021 based on specific criteria; 80 papers selected.
ovarian carcinosarcomaovarian leiomyosarcomaprimary ovarian melanomaovarian carcinoid tumor
The authors reviewed the literature from 2010 to 2021 on four rare primary ovarian neoplasms and selected 80 papers for analysis. Most included articles were clinical case reports; surgery (radical or fertility-sparing) was identified as the mainstay of treatment. The review notes single mentions of potential modern pharmacological treatments and reports a variety of adjuvant therapies, but finds no current treatment guidelines. The authors recommend improved biological understanding and the creation of an international database to enable further research.
Reported effect: papers_selected 80
Key findings
- A literature review covering 2010–2021 resulted in 80 papers selected for analysis.
- The vast majority of the articles were clinical case reports.
- Surgery, either radical or fertility-sparing, is identified as the mainstay of treatment for these rare ovarian tumors.
- There are single mentions of new potential pharmacological treatments, and various adjuvant treatments have been reported in the literature.
- There are currently no treatment guidelines for these tumors.
- The authors recommend more detailed biological study of these tumors and the creation of an international database to gather data across centers.
Limitations: Review is based largely on clinical case reports (low-level evidence).; Heterogeneous and sparse data across rare tumor types limit generalizability.; No high-level controlled trials or guidelines identified in the reviewed literature.; Search limited to publications from 2010 to 2021 (time-limited)..
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
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
ReviewMixed resultsLimited evidenceTier 3 · early human
Current problems in cancer · Aug 2019
uterine sarcomaleiomyosarcomauterine neoplasms
This is a narrative review of uterine sarcomas, with emphasis on leiomyosarcoma. The author states that early-stage uterine LMS can be cured by complete hysterectomy, while patients with metastatic disease may have tumor responses and improved quality of life though long-term remissions are rare. The review outlines adjuvant therapies for resected early-stage LMS and treatment options for metastatic disease.
Key findings
- Uterine sarcomas comprise a small percentage of uterine malignancies; the most common is leiomyosarcoma (LMS).
- Early stage uterine LMS is curable with complete hysterectomy with removal of an intact uterus.
- Patients with metastatic disease may achieve tumor responses with improvements in quality of life, but long-term remissions are rare.
- The article outlines adjuvant therapies for early stage resected uterine LMS and treatment of metastatic disease.
Limitations: Narrative review; abstract provides no methods, selection criteria, or systematic search details.; No primary patient-level data or quantitative results are reported in the abstract.; Abstract does not specify particular therapies, doses, comparative outcomes, or strength of evidence for treatments..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
ReviewMixed resultsLimited evidenceTier 3 · early human
Oncology research and treatment · Jan 2018 · narrative review with a single case report
uterine leiomyosarcoma
This article is a narrative review of uterine leiomyosarcoma (uLMS) and includes a single case report of a 30-year-old patient. The authors state that surgery with removal of the uterus intact is the mainstay of management, that undiagnosed uLMS worsens prognosis when morcellation is used, and that systemic therapy and radiotherapy have no proven value in the adjuvant setting. They conclude there is a need for reliable pre-operative risk assessment tools to guide surgical approach.
Key findings
- uLMS is a rare malignant gynecologic tumor with an unfavorable prognosis, most prevalent in pre- and peri-menopause.
- uLMS is often diagnosed accidentally and can be difficult to distinguish pre-operatively from benign uterine fibroids.
- Surgery aimed at removing the uterus intact is the basis of therapy.
- Pre-operatively undiagnosed uLMS that undergoes morcellation (vaginal, abdominal, endoscopic) will have a worsened prognosis.
- Systemic treatment and radiotherapy have no proven value in the adjuvant setting according to the authors.
- There is a stated strong need for a reliable pre-operative risk score for leiomyosarcoma to inform diagnostic work-up and surgical pathway selection.
- The clinical problems are illustrated with a single case report of a 30-year-old childless patient.
Limitations: Narrative review and single case report rather than systematic review or controlled study.; No new quantitative or comparative data presented in the abstract.; Conclusions about lack of proven adjuvant benefit are general statements without presented trial data in the abstract.; Single case report cannot establish generalizable outcomes..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
ReviewMechanismInconclusiveLimited evidenceTier 4 · clinical
International journal of immunopathology and pharmacology · Jun 2015 · narrative review / short guideline
vulvar sarcomaleiomyosarcomaepithelioid sarcomarhabdomyosarcomamalignant rhabdoid tumorvulvar neoplasms
This paper is a short guideline/review summarizing the most common forms of vulvar sarcomas and their histopathological recognition and clinical management. It reports that vulvar tumors are uncommon, most are benign, and that vulvar sarcomas are rare but aggressive with high metastatic potential, frequent recurrences, and high mortality.
Reported effects: Proportion of vulvar tumors among gynecological neoplasms 4% · Percent benign among vulvar tumors 98% · +4 more
Key findings
- Vulvar tumors represent only 4% of all gynecological neoplasms and are fourth in frequency after tumors of the cervix, uterus, and ovary.
- Ninety eight percent of all vulvar tumors are benign and only 2% are malignant.
- The overall incidence of tumors with vulvar location is between two and seven cases per 100,000 women; the death rate is estimated at 0.7 per 100,000 women.
- Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers; the most common sarcoma subtypes are leiomyosarcomas, epithelioid sarcomas, and rhabdomyosarcomas.
- Vulvar sarcomas are described as having rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality.
- The paper emphasizes a broad differential diagnosis, rare appearance, and a non-specific clinical picture for vulvar sarcomas.
Limitations: Narrative review/short guideline with no original patient-level data reported in the abstract.; Abstract does not describe systematic search methods or evidence grading (no methods reported).; Findings are descriptive and rely on aggregated literature; no new clinical outcomes or comparative analyses presented.; Rarity of vulvar sarcomas limits generalizability and the strength of evidence available..
Provides a concise review and histopathological guidance on vulvar sarcomas, summarizing incidence, common subtypes, and clinical behavior.
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
ReviewInconclusiveModerate evidenceTier 4 · clinical
Hematology/oncology clinics of North America · Oct 2013
leiomyosarcomauterine leiomyosarcomainferior vena cava leiomyosarcomacutaneous leiomyomasangioleiomyomaspiloleiomyomasgenital leiomyomas
This is a narrative review summarizing the biology, pathology, and clinical management of leiomyosarcoma. It discusses systemic treatment options, from cytotoxic chemotherapy to targeted therapies, and addresses particular subtypes (for example uterine and inferior vena cava leiomyosarcoma). The abstract also describes benign cutaneous smooth muscle tumors (cutaneous leiomyomas) and notes that multiple piloleiomyomas may be associated with an underlying genetic mutation that increases risk of renal cell carcinoma.
Studied with: cytotoxic chemotherapy, targeted therapies.
Key findings
- Provides an overview of current literature on biology, pathology, and clinical management of leiomyosarcoma.
- Emphasizes and discusses current systemic treatment options available for leiomyosarcoma, including cytotoxic chemotherapy and targeted therapies.
- Discusses particular leiomyosarcoma subtypes (uterine leiomyosarcoma and inferior vena cava leiomyosarcoma) separately.
- Notes that cutaneous leiomyomas are benign smooth muscle tumors with subtypes (angioleiomyomas, piloleiomyomas, genital leiomyomas).
- Reports that patients with multiple piloleiomyomas may harbor an underlying genetic mutation increasing susceptibility to renal cell carcinoma.
Limitations: Narrative review article rather than original research—no new primary data reported in abstract.; Abstract does not report methods for literature search or selection, so risk of selection/publication bias is unknown.; No quantitative synthesis or pooled estimates presented in the abstract.; Clinical implications and comparative effectiveness of treatments are not quantified in the abstract..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed