Ultrasound-guided core-needle biopsy for extra-ocular orbital soft tissue tumours: a minimally invasive alternative to surgical biopsy.

Objective: To assess the feasibility, accuracy, and safety of ultrasound (US)-guided coaxial core-needle biopsy (CNB) for histomolecular diagnosis of extra-ocular orbital soft tissue tumours as a minimally invasive alternative to surgical biopsy.

Methods: This retrospective study was conducted at a single Comprehensive Cancer Center and included all consecutive patients referred to our center between 2015 and 2023 for the diagnosis and treatment of orbital soft tissue tumours. All patients underwent US-guided transconjunctival coaxial CNB using a semiautomatic 18-gauge biopsy gun.

Cellular origin and clonal evolution of human dedifferentiated liposarcoma.

Dedifferentiated liposarcoma (DDLPS) is the most frequent high-grade soft tissue sarcoma subtype. It is characterized by a component of undifferentiated tumor cells coexisting with a component of well-differentiated adipocytic tumor cells. Both dedifferentiated (DD) and well-differentiated (WD) components exhibit MDM2 amplification, however their cellular origin remains elusive.

Alveolar soft part sarcomas in young patients: The French national NETSARC+ network experience.

Background And Aims: Alveolar soft part sarcoma (ASPS) is an ultra-rare chemo-resistant sarcoma in children, occurring preferentially in young adults. We aimed to describe and compare its clinical presentation and behaviour in children and young adults to determine whether the same therapeutic strategy should be addressed for both populations.

Methods: National retrospective multicentre study of children (0-18 years) vs.

Impact of guideline adherence and expert center referral on the early management and outcomes of uterine sarcoma patients: A retrospective analysis from the French NETSARC network.

Objective: Uterine sarcomas are rare tumors with a poor prognosis. Their diagnosis is often incidental, following surgery. Our goal was to examine the early management strategies for uterine sarcomas, and to assess the impact of guideline adherence and expert center referral on both the management approaches and the clinical outcomes in patients with uterine sarcomas.

Fusion-negative rhabdomyosarcoma 3D organoids to predict effective drug combinations: A proof-of-concept on cell death inducers.

Rhabdomyosarcoma (RMS) is the main form of pediatric soft-tissue sarcoma. Its cure rate has not notably improved in the last 20 years following relapse, and the lack of reliable preclinical models has hampered the design of new therapies. This is particularly true for highly heterogeneous fusion-negative RMS (FNRMS).

Diagnostic accuracy and safety of percutaneous core needle biopsy of retroperitoneal tumours.

Background: Histologic subtype of cancer guides treatment sequencing and the extent of surgery for retroperitoneal tumours (RPTs) but concerns persist regarding percutaneous core needle biopsy (CNB).

Objective: Endpoints were the incidence of early complications, needle tract seeding (NTS) after CNB, diagnostic accuracy.

Methods: Between 2015 and 2022, data from patients with RPT who underwent a CNB and who operated on at Institut Curie were collected.

Significant Predictors of Postoperative Morbidity After Radical Resection of Retroperitoneal Sarcoma in a Tertiary Center.

Background: The safety of multivisceral resection of retroperitoneal sarcoma is an issue. Previous reports have investigated its associations with the pattern of resection and factors recognized mostly per operatively.

Methods: All consecutive RPS resections from May 2015 to April 2022 were studied retrospectively with respect to adverse events.

MDM4 amplification in atypical lipomatous tumors/well-differentiated liposarcoma: Private event or alternative oncogenic mechanism?

Adipocytic tumors are the most common mesenchymal tumors in soft tissues. Among them, a diagnostic challenge relies in the distinction between lipoma and atypical lipomatous tumor (ALT)/well differentiated liposarcoma (WDLPS), as both entities are often undistinguishable not only from a radiological point of view, but also at the microscopic level and particularly when dealing with small tumor specimen. Thus, detection of recurrent MDM2 amplifications may be the only criteria to discriminate malignant tumors from lipomas.

Percutaneous Uterine Needle Biopsy with Microscopic and Array-CGH Analyses for Preoperative Sarcoma Diagnosis in Patients with Suspicious Myometrial Tumors on MRI: A Prospective Pilot Study (SARCGYN).

Background: Unlike for soft tissue sarcomas, percutaneous biopsy is not validated for uterine myometrial tumors, leading to leiomyosarcoma inadvertent morcellation and overtreatment in childbearing patients. This study aimed to evaluate preoperative percutaneous uterine needle biopsy (PUB) with microscopic examination (M-PUB) and array-comparative genomic hybridization (MCGH-PUB).

Methods: This was a prospective single-center cohort study including all consecutive patients who were candidates for hysterectomy because of suspected uterine leiomyosarcoma on magnetic resonance imaging (MRI) who received PUB.

Desmoid Tumors Arising on the Mesenteric Surgical Scar of Abdominal Sarcomas.

A sporadic desmoid tumor (DT) is a rare type of tumor of the mesenchymal connective tissues is now considered an intermediate disease or locally aggressive. They may develop on scars or after traumatism, favored by growth factors released during the initial phase of wound healing. Most of the abdominal DT arising on a scar is described on the wall incision.

Novel EWSR1::UBP1 fusion expands the spectrum of spindle cell rhabdomyosarcomas.

Over the last decade, the development of next-generation sequencing techniques has led to the molecular dismantlement of adult and pediatric sarcoma, with the identification of multiple gene fusions associated with specific subtypes and currently integrated into diagnostic classifications. In this report, we describe and discuss the identification of a novel EWSR1-UBP1 gene fusion in an adult patient presenting with multi-metastatic sarcoma. Extensive pathological, transcriptomic, and genomic characterization of this tumor in comparison with a cohort of different subtypes of pediatric and adult sarcoma revealed that this fusion represents a novel variant of spindle cell rhabdomyosarcoma with features of TFCP2-rearranged subfamily.

Verrucous Venous Malformation-Subcutaneous Variant.

Background: Verrucous venous malformation (VVM), previously called "verrucous hemangioma," typically involves the dermis and the subcutaneous fat. We have encountered patients with VVM confined to the hypodermis.

Materials And Methods: During a nearly 20-year period, 13 patients, aged 2-17 years, presented with a subcutaneous mass in the limb without clinically obvious epidermal alterations.

Neoadjuvant Everolimus for Adult Giant Mesenteric Cystic Lymphangioma with mTOR Pathway Activation.

Cystic lymphangioma are rare benign vascular or lymphatic tumors, diagnosed mostly in newborns or children, that may become life-threatening because of local invasiveness. Surgical "en-bloc" resection with negative margins is the only curative treatment, but some patients are diagnosed with unresectable tumors. We describe the case of a young adult with giant unresectable mesenteric lymphangioma.

The histopathology of congenital haemangioma and its clinical correlations: a long-term follow-up study of 55 cases.

Aims: Congenital haemangiomas (CHs) can be subdivided into different subtypes [rapidly involuting CHs (RICHs), non-involuting CHs (NICHs), and partially involuting CHs (PICHs)]. During the first few days of life, RICHs may be associated with transient but sometimes marked thrombocytopenia. We sought to assess the histological aspects and clinicopathological correlations of the three subtypes.

Evaluating the prognostic potential of the Ki67 proliferation index and tumour-infiltrating lymphocytes in olfactory neuroblastoma.

Aims: Olfactory neuroblastomas (ONBs) are rare malignant tumours that arise in the nasal vault. To date, the Hyams grade remains the only widely used histological grading system. However, it is based only on morphological criteria, and has not been updated since 1988.

Surgery and protontherapy in Grade I and II skull base chondrosarcoma: A comparative retrospective study.

Objective: Skull base chondrosarcoma is a rare tumour usually treated by surgery and proton therapy. However, as mortality rate is very low and treatment complications are frequent, a less aggressive therapeutic strategy could be considered. The objective of this study was to compare the results of surgery only vs surgery and adjuvant proton therapy, in terms of survival and treatment adverse effects, based on a retrospective series.

Olfactory Epithelial Hamartoma: A New Subtype of Sinonasal Hamartoma.

Sinonasal epithelial hamartomas occurring in adults are classified as seromucinous hamartoma (SMH) or respiratory epithelial adenomatoid hamartoma (REAH). We describe herein a novel subtype of adult sinonasal hamartoma that contains olfactory epithelium, a histologic feature not previously reported in the literature. Our pathology department database was retrospectively searched for sinonasal hamartomas containing areas of olfactory epithelium.