Sarcomas are a diverse group of cancers with mesenchymal origin. Although sarcomas comprise less than 1% of cancers, there are more than 50 different subtypes that are quite different from one another in terms of both their biology and clinical behavior. Historically, the need for adequate patient numbers in clinical trials has pushed sarcoma researchers to lump these very different malignancies together and treat the patients using a "one-size-fits-all" approach. However, with improvements in our scientific understanding, we are finally ready for a histology-tailored therapeutic approach to these complex diseases. In this review, we discuss key advances in our understanding of the biology underlying selected sarcoma subtypes and how targeting these subtypes is relevant therapeutically with respect to both molecularly targeted agents as well as immunotherapy.

https://ascopubs.org/doi/pdfdirect/10.1200/EDBK_205423

More Than 50 Subtypes of Soft Tissue Sarcoma: Paving the Path for Histology-Driven Treatments.

Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms PI3K/AKT/mTOR pathway upregulation is critical for their pathogenesis and is often associated with TSC1/TSC2 inactivation Although first line mTOR inhibitors are an effective treatment, metastatic PEComas eventually progress A 53-year-old woman presented a 4-month history of post-menopausal vaginal bleeding Clinical and radiological examination detected a uterine mass and a single S1 bone lesion The patient underwent a radical hysterectomy and bone biopsy The anatomopathological evaluation concluded to an oligo-metastatic uterine PEComa The tumor harbored a heterozygous deletion of 9q34 that contains the TSC1 gene Concerning the primary lesion, the resection was complete and the single bone metastasis was treated with radiotherapy Three months later, the patient presented bone, lung and subcutaneous metastatic progression An everolimus and denosumab treatment was initiated After 2 years of treatment, a clinically significant bone, lung and subcutaneous progression was detected Following a literature review of the possible therapeutic options, we initiated a second line treatment by pazopanib This treatment resulted in regression of the subcutaneous lesions and stability of lung and bone metastases In this challenging, rare setting, our report suggests single agent, anti-angiogenic, tyrosine kinase inhibitor to be effective as second line treatment of metastatic uterine PEComa progressing on mTOR inhibitors

https://serval.unil.ch/resource/serval:BIB_0F71532CFC6B.P001/REF.pdf

VEGFR Inhibitors for Uterine Metastatic Perivascular Epithelioid Tumors (PEComa) Resistant to mTOR Inhibitors. A Case Report and Review of Literature.

/pdf/management-strategies-for-adults-with-locally-advanced-296a67mi.pdf

Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions

Introduction: Perivascular epithelioid cell tumor (PEComa) constitutes a family of rare mesenchymal soft-tissue tumors. These tumors pose a significant diagnostic challenge, given their cytomorphol...

The potential of emerging new therapeutics for the treatment of perivascular epithelioid cell tumors (PEComa)

Abstract PEComa (PEC tumor; perivascular epithelioid cell tumors) is a rare group of tumors of mesenchymal origin composed of perivascular epithelioid cells (PEC) with features of melanotic and smooth muscle differentiation. In this article, we would like to present the current treatment options for this group of tumors. PEComas are classified as tumors of uncertain malignant potential because recurrences occur after radical treatment. The primary treatment is surgical resection with negative margins. Due to the different locations of the tumors, often the cooperation of multispecialty surgeons is required during the operations. In locally advanced cases, cytoreduction and HIPEC may be effective but still are an experimental treatment. For nonresectable PEComa chemotherapy, mTOR inhibitors and VEGFR inhibitors are used.

1450psystemic therapy in perivascular epithelioid cell tumors (pecoma)

Limited real-world data exist on the effectiveness and safety of abiraterone acetate plus prednisone (abiraterone hereafter) in the treatment of patients with metastatic castration-resistant prostate cancer (mCRPC) naive to chemotherapy. Most of the few available studies had a retrospective design and included a small number of patients. In the interim analysis of the ABItude study, abiraterone showed good clinical effectiveness and safety profile in the chemotherapy-naive setting over a median follow-up of 18 months.We evaluated clinical and patient-reported outcomes (PROs) of chemotherapy-naive mCRPC patients treated with abiraterone as for clinical practice in the Italian, observational, prospective, multicentric ABItude study. mCRPC patients were enrolled at abiraterone start (February 2016-June 2017) and followed up for 3 years; clinical endpoints and PROs, including quality of life (QoL) and pain, were prospectively collected. Kaplan-Meier curves were estimated.Of the 481 patients enrolled, 454 were assessable for final study analyses. At abiraterone start, the median age was 77 years, with 58.6% elderly patients and 69% having at least one comorbidity (57.5% cardiovascular diseases). Visceral metastases were present in 8.4% of patients. Over a median follow-up of 24.8 months, median progression-free survival (any progression reported by the investigators), time to abiraterone discontinuation, and overall survival were, respectively, 17.3 months [95% confidence interval (CI) 14.1-19.4 months], 16.0 months (95% CI 13.1-18.2 months), and 37.3 months (95% CI 36.5 months-not estimable); 64.2% of patients achieved ≥50% reduction in prostate-specific antigen. QoL assessed by Functional Assessment of Cancer Therapy-Prostate, the European Quality of Life 5 Dimensions 3 Level, and European Quality of Life Visual Analog Scale remained stable during treatment. Median time to pain progression according to Brief Pain Inventory data was 31.1 months (95% CI 24.8 months-not estimable). Sixty-two patients (13.1%) had at least one adverse drug reaction (ADR) and 8 (1.7%) one serious ADR.With longer follow-up, abiraterone therapy remains safe, well tolerated, and active in a large unselected population. 

http://www.esmoopen.com/article/S2059702922000527/pdf

Real-world experience of abiraterone acetate plus prednisone in chemotherapy-naive patients with metastatic castration-resistant prostate cancer: long-term results of the prospective ABItude study

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1450psystemic therapy in perivascular epithelioid cell tumors (pecoma)

Real-world experience of abiraterone acetate plus prednisone in chemotherapy-naive patients with metastatic castration-resistant prostate cancer: long-term results of the prospective ABItude study