Tumor immune microenvironment-associated prognostic and mifamurtide-response gene signatures for localized osteosarcoma: a correlative study of the ISG/OS2 trial.

Purpose: A risk-adapted treatment strategy, the ISG/OS-2 trial, evaluated the use of mifamurtide in P-glycoprotein (Pgp)-positive localized osteosarcoma patients. The primary objective was the identification of prognostic classifiers based on tumor immune microenvironment (TME) gene profiling in all patients and in those undergoing mifamurtide.

Experimental Design: RNA from pre-treatment FFPE non-decalcified tissues of 62 patients was analyzed with the PanCancer Immune profiling panel (NanoString Technologies, Seattle, WA, US).

Primary leiomyosarcoma of bone: a clinicopathologic and immunohistochemical study of 142 cases.

Leiomyosarcoma is a rare malignant mesenchymal tumor characterized by smooth muscle differentiation. It typically arises in both visceral and somatic soft tissues, while involvement of bone being exceptionally uncommon. Although primary leiomyosarcoma of bone has been a subject of ongoing debate, the advent of immunohistochemistry has reduced the misclassification of other sarcomas, such as fibrosarcoma and undifferentiated pleomorphic sarcoma, facilitating the accurate identification of true primary LMSB cases.

Genomic profiling of a collection of patient-derived xenografts and cell lines identified ixabepilone as an active drug against chemo-resistant osteosarcoma.

Background: Osteosarcoma (OS) shows a multitude of genetic and chromosomal abnormalities together with large biological heterogeneity. These features limited the identification of novel drugs to treat patients with metastases and/or chemo-resistant tumors. The purpose of this study was to create additional resources for drug screening by generating patient-derived xenograft (PDXs) and PDX-derived cell lines that reflect the spectrum of OS heterogeneity.

Diffuse-Type Tenosynovial Giant Cell Tumor of the Knee: Clinical Course After Anterior Open Synovectomy.

Diffuse type tenosynovial giant cell tumor (D-TGCT) of the knee presents with stiffness, pain and swelling with surgery being the mainstay treatment. However, the literature lacks data on clinical course of range of motion, pain (ROM), and swelling after open synovectomy for D-TGCT. Therefore, this study aims to evaluate clinical course after open anterior synovectomy.

Comparison of histological response across patients with Ewing sarcoma treated with preoperative chemotherapy with or without preoperative radiotherapy.

Purpose: This study aimed to compare the histological response, a surrogate preoperative treatment efficacy marker, of patients treated with preoperative chemotherapy with or without preoperative radiotherapy. Moreover, local recurrence in both groups was investigated.

Methods: Surgical specimens from 210 patients with Ewing sarcoma were assessed between 2003 and 2020 to evaluate their histological response to preoperative treatment; 181 patients received preoperative chemotherapy, while 29 received both preoperative chemotherapy and radiotherapy.

Skull Base Osteosarcoma: An Analysis of 9 Cases and Literature Review.

Purpose: Skull base osteosarcoma is an exceedingly rare malignancy. Unlike maxillofacial osteosarcomas, there are only few cases of skull base tumors reported in literature, and their clinical behavior and treatment outcome are not well defined. This study aims to characterize the clinical features and outcomes of primary skull base osteosarcoma based on our experiences of 9 cases and to review available literature data.

Clear Cell Sarcoma of Soft Tissues: Radiological Analysis of 14 Patients-MRI Findings Related to Metastatic Disease.

Clear cell sarcoma (CCS) is a very uncommon, aggressive soft-tissue sarcoma (STS) with a dismal prognosis. In the current literature, there are very limited data focused on the radiological features of CCS. Our study's objective was to describe CCS pre-treatment's peculiar imaging characteristics (MRI above all) and to assess if some radiologic features may predict patients' outcomes with regard to the occurrence of distant metastases.

Histomorphometric and Immunohistochemical Evaluation of Bone Healing Around Implants Placed Using Piezosurgery vs Conventional Drills: A Split-Mouth Pilot Study.

This pilot study evaluated and compared histomorphometric and immunohistochemical characteristics of peri-implant bone tissue after implant site preparation using piezoelectric tips versus conventional drills. Six patients with bilateral partial edentulism underwent a split-mouth protocol. Twelve alveolar ridges were randomized into 6 control implant sites prepared using conventional drills (drill group) and 6 test implant sites prepared using piezoelectric implant inserts (piezo group).

Conventional radiography for the assessment of focal bone lesions of the appendicular skeleton: fundamental concepts in the modern imaging era.

Bone lesions of the appendicular skeleton can be caused by primary benign or malignant tumors, metastases, osteomyelitis, or pseudotumors. Conventional radiography plays a crucial role in the initial assessment of osseous lesions and should not be underestimated even in this era of modern complex and advanced imaging technologies. Combined with patient age, clinical symptoms and biology, and lesion features including location, solitary versus multiplicity, density, margin (transitional zone evaluated with Lodwick-Madewell grading score), and, if present, the type of periosteal reaction and matrix mineralization can narrow the differential diagnosis or offer a likely diagnosis.

p53 as a Potential Actionable Target in Myxofibrosarcoma: A Molecular and Pathologic Review of a Single-Institute Series.

Myxofibrosarcoma (MFS) is a common adult soft tissue sarcoma characterized by high-local recurrence rate, poorly understood molecular pathogenesis, lack of specific prognostic markers, and effective targeted therapies. To gain further insights into the disease, we analyzed a well-defined group of 133 primary MFS cases. Immunohistochemical (IHC) staining for p53, MET, RET, and RB was performed.

Peripheral nerve tumors.

The chapter is focused on the neoplastic peripheral nerve lesions, which primarily involve "cranial and paraspinal nerves," as outlined in the CNS volume (WHO_Classification_of_Tumours_Editorial_Board, 2021). These include classic peripheral nerve sheath tumors such as schwannoma, neurofibroma, intraneural perineurioma, and malignant peripheral nerve sheath tumors, with their variants as well as new and more precisely defined entities, including hybrid nerve sheath tumors and malignant melanotic nerve sheath tumor (previously melanotic schwannoma).

The utility of FISH analysis in the diagnosis of BCOR-rearranged sarcomas.

Background: A BCL6 corepressor (BCOR) gene alteration is a genetic signature of rare subsets of sarcomas. The identification of this alteration has recently contributed to the definition of new entities in the current WHO (2020) classification of soft tissue and bone tumours. We retrospectively examined cases of BCOR-rearranged sarcoma (BRS) to assess the reliability of the BCOR FISH analysis using an IVD (in vitro diagnostic) probe.

Secondary peripheral chondrosarcoma in multiple osteochondromas: a retrospective single-institution case series.

Background: Multiple osteochondromas is genetic disorder characterized by the formation of multiple benign cartilage-capped bone tumors, named osteochondromas, during skeletal development. The most feared complication is the secondary peripheral chondrosarcoma, a malignant cartilaginous neoplasm that arises from the chondroid cap of pre-existent osteochondromas. We conducted a retrospective cohort study on patients diagnosed and followed up from 1960 to 2019 to describe the clinical and pathological features of individuals affected by peripheral chondrosarcoma in multiple osteochondromas, to evaluate follow up information and individual outcome and to compare the results with literature.

A Systematic Review of Adjuvant Chemotherapy in Localized Dedifferentiated Chondrosarcoma.

Dedifferentiated chondrosarcoma (DDCS) is a high-grade subtype of chondrosarcoma with the bimorphic histological appearance of a conventional chondrosarcoma component with abrupt transition to a high-grade, non-cartilaginous sarcoma. DDCS can be radiographically divided into central and peripheral types. Wide resection is currently the main therapeutic option for localized DDCS.

Diagnostic challenges in low-grade central osteosarcoma.

Aims: Low-grade central osteosarcoma (LGCOS), a rare type of osteosarcoma, often has misleading radiological and pathological features that overlap with those of other bone tumours, thereby complicating diagnosis and treatment. We aimed to analyze the clinical, radiological, and pathological features of patients with LGCOS, with a focus on diagnosis, treatment, and outcomes.

Methods: We retrospectively analyzed the medical records of 49 patients with LGCOS (Broder's grade 1 to 2) treated between January 1985 and December 2017 in a single institute.

Imaging Features of Alveolar Soft Part Sarcoma: Single Institution Experience and Literature Review.

Alveolar soft part sarcoma (ASPS) is an extremely rare and aggressive soft-tissue sarcoma (STS) subtype with poor prognosis and limited response to radiation therapy and chemotherapy. Prompt recognition and referral to sarcoma centers for appropriate management are crucial for patients' survival. The purpose of this study was to report ASPS pre-treatment imaging features and to examine the existing literature on this topic.

NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report.

Neurotrophic tyrosine receptor kinase (NTRK) gene-fusion targeted molecules revolutionized the paradigm of treatment of a limited subgroup of cancers of various histologies. Entrectinib and larotrectinib obtained unprecedented response rates in patients with cancer harboring NTRK rearrangements. This evidence recently led to the agnostic approval of these drugs, and evidence (confirmation) of their activity in a broader disease setting is emerging.