A platform for rapid patient-derived cutaneous neurofibroma organoid establishment and screening.

Localized cutaneous neurofibromas (cNFs) are benign tumors that arise in the dermis of patients affected by neurofibromatosis type 1 syndrome. cNFs are benign lesions: they do not undergo malignant transformation or metastasize. Nevertheless, they can cover a significant proportion of the body, with some individuals developing hundreds to thousands of lesions.

Dedifferentiation in bone and soft tissue sarcomas: How do we define it? What is prognostically relevant?

Dedifferentiation traditionally is defined by descriptive criteria as a tumor showing an abrupt change in histology from a conventional, classic, low-grade appearing neoplasm to a tumor that is more cellular, pleomorphic and "high grade", with grading typically being performed by subjective criteria. The dedifferentiated areas range from areas with recognizable histologic differentiation which differs from the primary tumor (such as an osteosarcoma arising from a low-grade chondrosarcoma) to areas containing sarcomas without specific histologic differentiation (such as pleomorphic or spindle cell sarcoma). Many, but not all, dedifferentiated tumors are aggressive and associated with significantly shorter survival than their conventional counterparts, even grade 3 conventional tumors.

Leflunomide-induced collagenous colitis: a case report and literature review.

We describe a patient with rheumatoid arthritis and Hashimoto's thyroiditis who developed chronic diarrhea and subsequently diagnosed with collagenous colitis (CC) 5 years after leflunomide initiation. Cessation of leflunomide resulted in complete resolution of diarrhea within 2 months. Although rare, leflunomide-induced colitis should be considered in patients with otherwise unexplained chronic diarrhea.

IGF2BP3 as a Prognostic Biomarker in Well-Differentiated/Dedifferentiated Liposarcoma.

Background: Although IGF2BP3 has been implicated in tumorigenesis and poor outcomes in multiple cancers, its role in soft-tissue sarcoma (STS) remains unknown. Preliminary data have suggested an association with IGF2BP3 expression among patients with well-differentiated/dedifferentiated liposarcoma (WD/DD LPS), a disease where molecular risk stratification is lacking.

Methods: We examined the survival associations of IGF2BP3 via univariate and multivariate Cox regression in three unique datasets: (1) the Cancer Genome Atlas (TCGA), (2) an in-house gene microarray, and (3) an in-house tissue microarray (TMA).

Fibroblast Activation Protein Expression in Sarcomas.

Objectives: Fibroblast activation protein alpha (FAP) is highly expressed by cancer-associated fibroblasts in multiple epithelial cancers. The aim of this study was to characterize FAP expression in sarcomas to explore its potential utility as a diagnostic and therapeutic target and prognostic biomarker in sarcomas.

Methods: Available tissue samples from patients with bone or soft tissue tumors were identified at the University of California, Los Angeles.

The landscape of drug sensitivity and resistance in sarcoma.

Unlabelled: Sarcomas are a family of rare malignancies composed of over 100 distinct histological subtypes. The rarity of sarcoma poses significant challenges in conducting clinical trials to identify effective therapies, to the point that many rarer subtypes of sarcoma do not have standard-of-care treatment. Even for established regimens, there can be substantial heterogeneity in responses.

A pilot study evaluating the use of sirolimus in children and young adults with desmoid-type fibromatosis.

Deregulation of the mTOR pathway may play an important role in tumor biology when the APC/β-catenin pathway is disrupted in desmoid-type fibromatosis (DT). A pilot study was conducted to determine whether sirolimus can block the mTOR pathway (primary aim) as well as determine whether it can safely be given in the preoperative setting, decrease tumor size/recurrence, and decrease tumor-associated pain in children and young adults (secondary aims) with DT. Nine subjects ages 5-28 years were enrolled from 2014 to 2017 across four centers.

Does "Low-Grade" Dedifferentiated Liposarcoma Exist? The Role of Mitotic Index in Separating Dedifferentiated Liposarcoma From Cellular Well-differentiated Liposarcoma.

Background: Subjective, varying criteria identify "low-grade" dedifferentiation in well-differentiated/dedifferentiated liposarcoma (WD/DDLPS). The value of mitotic rate (MR) in defining DDLPS is not confirmed. We studied all patients with the resection of their primary or first recurrence retroperitoneal WD/DDLPS at our institution to determine the value of MR in diagnosing DDLPS and if MR associates with patient survival.

Datasets for reporting of soft-tissue sarcoma: recommendations from the International Collaboration on Cancer Reporting (ICCR).

Aims: Soft-tissue tumours are rare and both accurate diagnosis and proper treatment represent a global challenge. Current treatment guidelines also recommend review by specialised pathologists. Here we report on international consensus-based datasets for the pathology reporting of biopsy and resection specimens of soft-tissue sarcomas.

Lifelong Imaging Surveillance is Indicated for Patients with Primary Retroperitoneal Liposarcoma.

Background: Surveillance imaging of patients with retroperitoneal liposarcoma (RP-LPS) after surgical resection is based on a projected risk of locoregional and distant recurrence. The duration of surveillance is not well defined because the natural history of RP-LPS after treatment is poorly understood. This study evaluated the long-term risk of recurrence and disease-specific survival (DSS) for a cohort of patients with at least 10 years of progression-free survival (10yr-PFS) from their primary resection.

NCCN Guidelines® Insights: Gastrointestinal Stromal Tumors, Version 2.2022.

Gastrointestinal stromal tumors (GIST) are the most common type of soft tissue sarcoma that occur throughout the gastrointestinal tract. Most of these tumors are caused by oncogenic activating mutations in the KIT or PDGFRA genes. The NCCN Guidelines for GIST provide recommendations for the diagnosis, evaluation, treatment, and follow-up of patients with these tumors.

Soft Tissue Sarcoma, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology.

Soft tissue sarcomas (STS) are rare malignancies of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors.

Rare malignant glomus tumor of the esophagus with pulmonary metastasis: a case report.

Background: Glomus tumors are typically benign soft tissue neoplasms that arise in peripheral cutaneous structures. Visceral organ involvement is exceedingly rare.

Case Description: Here we present a case of malignant glomus tumor of the esophagus with pulmonary metastases in a 57-year-old woman presenting with three weeks of progressive dysphagia, epigastric pain, and 35-pound weight loss.

Electronic Health Record-Integrated Tumor Board Application to Save Preparation Time and Reduce Errors.

Purpose: Multidisciplinary oncology meetings, or tumor boards (TBs), ensure and facilitate communication between specialties regarding the management of cancer cases to improve patient care. The organization of TB and the preparation and presentation of patient cases are typically inefficient processes that require the exchange of patient information via e-mail, the hunting for data and images in the electronic health record, and the copying and pasting of patient data into desktop presentation software.

Methods: We implemented a standards-based electronic health record-integrated application that automated several aspects of TB organization and preparation.

An Integrated, Scalable, Electronic Video Consent Process to Power Precision Health Research: Large, Population-Based, Cohort Implementation and Scalability Study.

Background: Obtaining explicit consent from patients to use their remnant biological samples and deidentified clinical data for research is essential for advancing precision medicine.

Objective: We aimed to describe the operational implementation and scalability of an electronic universal consent process that was used to power an institutional precision health biobank across a large academic health system.

Methods: The University of California, Los Angeles, implemented the use of innovative electronic consent videos as the primary recruitment tool for precision health research.

Correction: Electronic Video Consent to Power Precision Health Research: A Pilot Cohort Study.

[This corrects the article DOI: 10.2196/29123.].

F-FLT PET/CT as a Prognostic Imaging Biomarker of Disease-Specific Survival in Patients with Primary Soft-Tissue Sarcoma.

The purpose of this study was to evaluate F-FLT PET/CT as an early prognostic imaging biomarker of long-term overall survival and disease-specific survival (DSS) in soft-tissue sarcoma (STS) patients treated with neoadjuvant therapy (NAT) and surgical resection. This was a 10-y follow-up of a previous single-center, single-arm prospective clinical trial. Patients underwent F-FLT PET/CT before treatment (PET1) and after NAT (PET2).

A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues.

Only 14 cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been documented in the literature. We report a case of a 63-year-old woman with EMC of the vulva confirmed by both and fluorescence in situ hybridization, the latter of which is a more specific probe for this entity. The unusual location of this tumor of prominent myxoid morphology gave rise to a wide differential diagnosis, which necessitated thorough histologic evaluation and confirmatory ancillary testing in the form of immunohistochemistry and cytogenetic studies.