Mazabraud Syndrome Associated with McCune-Albright Syndrome: A Case Report and Literature Review.

The Mazabraud syndrome is a rare form of bone fibrous dysplasia associated with intramuscular myxomas. The McCune-Albright syndrome is characterized by the association of dysplasia fibrous bone to one or more extra-osseous manifestations, including café-au-lait skin spots and endocrine disturbances. Here, we report on a new case of a 52-year-old man with sacroiliac polyostotic bone fibrous dysplasia associated with intramuscular myxomas of the left buttock and thigh and a café-au-lait skin spot.

[Sinus node dysfunction associated with cardiac sarcoidosis: A case report].

Introduction: Sarcoidosis is a multisystemic granulomatous disease of unknown cause occurring in young adults. Cardiac sarcoidosis patients are at increased risk for atrioventricular blocks and ventricular arrhythmias. Sinus node dysfunction is scarcely reported.

[Out-of-hospital sudden cardiac arrest and COVID-19 pandemic].

Since the appearance of the COVID-19 pandemic in 2020, the direct mortality related to COVID-19 infections has been monitored worldwide, with a daily count of the number of deaths due to COVID-19. Several measures have been undertaken in the societal and professional field, and the healthcare systems have been reorganized to limit the virus spread, and to cope with the surge of hospital admissions for COVID-19. Questions have been raised regarding the indirect effect of the pandemic, with uncertainties regarding the impact of delays in non-COVID diseases management, due to lockdown, postponement of non-urgent medical consultations and interventions, and decrease in screening.

Out-of-hospital cardiac arrest during the COVID-19 pandemic in Paris, France: a population-based, observational study.

Background: Although mortality due to COVID-19 is, for the most part, robustly tracked, its indirect effect at the population level through lockdown, lifestyle changes, and reorganisation of health-care systems has not been evaluated. We aimed to assess the incidence and outcomes of out-of-hospital cardiac arrest (OHCA) in an urban region during the pandemic, compared with non-pandemic periods.

Methods: We did a population-based, observational study using data for non-traumatic OHCA (N=30 768), systematically collected since May 15, 2011, in Paris and its suburbs, France, using the Paris Fire Brigade database, together with in-hospital data.

Successful Imatinib Treatment of an Abdominal Compartment Syndrome due to Huge Gastrointestinal Stromal Tumour.

Gastrointestinal stromal tumours (GISTs) are the most common digestive mesenchymal tumours, whose prognosis has been revolutionised by targeted therapies such as oral imatinib. Abdomen compartment syndrome (ACS) is associated with mortality superior to 50% in adults. ACS has never been reported to date in patients with GIST.

PARP1 expression in soft tissue sarcomas is a poor-prognosis factor and a new potential therapeutic target.

Soft tissue sarcomas (STSs) are aggressive tumors with few efficient systemic therapies. Poly(ADP-ribose) polymerase-1 (PARP1) inhibitors represent an emerging therapeutic option in tumors with genomic instability. The genomics of STSs is complex in more than half of cases, suggesting a high level of inherent DNA damage and genomic instability.

Giant Pedunculated Hepatic Hemangioma: A Case Report and Literature Review.

Hepatic hemangioma is the most common benign hepatic tumor, and most of them are small in size and asymptomatic. Giant hepatic hemangiomas are uncommon, but pedunculated giant hemangiomas are even rarer and often difficult to diagnose because of their exophytic development. We report here on a 48-year-old man with a symptomatic pedunculated giant hepatic hemangioma and hepatic angiomatosis, mimicking a gastric gastrointestinal stromal tumor with liver metastases.

Reversible rituximab-induced rectal Kaposi's sarcoma misdiagnosed as ulcerative colitis in a patient with HIV-negative follicular lymphoma.

Background: Kaposi's sarcoma is a low-grade mesenchymal angioproliferative tumor, most commonly observed in immunocompromised individuals, such as HIV-infected patients. Iatrogenic Kaposi's sarcoma occurs in patients undergoing immunosuppressive therapies. Rituximab is a chimeric monoclonal antibody targeted against the pan B cell marker CD20.

Metastatic Intimal Sarcoma of the Pulmonary Artery Sensitive to Carboplatin-Vinorelbine Chemotherapy: Case Report and Literature Review.

Pulmonary artery intimal sarcoma (PAIS) is a very rare tumour with a very poor prognosis. In advanced stages, chemotherapy and radiotherapy are poorly efficient, and no standard chemotherapy guideline is currently available. Here, we report on a 37-year-old woman with PAIS initially treated with surgical resection who developed metastatic relapse refractory to anthracycline-based chemotherapy, then trabectedin, then pazopanib.

The Genomic Grade Index predicts postoperative clinical outcome in patients with soft-tissue sarcoma.

Background: Soft-tissue sarcomas (STSs) are a group of rare, heterogeneous, and aggressive tumors, with high metastatic risk and relatively few efficient systemic therapies. We hypothesized that the Genomic Grade Index (GGI), a 108-gene signature previously developed in early-stage breast cancer, might improve the prognostic assessment of patients with early-stage STS.

Patients And Methods: We collected gene expression and clinicopathological data of 678 operated STS, and searched for correlations between the GGI-based classification and clinicopathological variables, including the metastasis-free survival (MFS).

Quality assessment of pelvic ultrasound for uterine myoma according to the CNGOF guidelines.

Introduction: French guidelines regarding the minimum criteria for gynaecological ultrasound were given in a recent report in 2016, by the French National College of Obstetricians and Gynaecologists (CNGOF). An accurate report is essential for the optimal care of women, especially those presenting myomas. The goal of this study was to evaluate the quality of gynaecological ultrasound reports for women with type 0 to 2 uterine myomas, referring to the items contained in the French guidelines.

A scoring system to guide the decision for a new systemic treatment after at least two lines of palliative chemotherapy for metastatic cancers: a prospective study.

Purpose: A four-parameter score has been identified as associated with overall survival (OS) in patients with advanced cancer with an estimated survival inferior to 6 months. Here, we tested its prognostic value for OS in patients who had received more than two lines of systemic therapy.

Methods: We prospectively enrolled patients with advanced cancer who were going to receive a third or more therapeutic line outside classical clinical guidelines.

Gastrointestinal Stromal Tumour with Synchronous Bone Metastases: A Case Report and Literature Review.

Gastrointestinal stromal tumours (GISTs) are mesenchymal tumours of the digestive tract, derived from Cajal interstitial cells. Bone metastases are very rare, and there is no consensus regarding their treatment. Here, we present the unusual case of a 66-year-old man with a gastric GIST with synchronous bone and liver metastases, fully documented at the pathological and molecular levels with a exon 11 mutation.

High-grade soft tissue sarcoma arising in a desmoid tumor: case report and review of the literature.

Desmoid tumors are rare benign monoclonal fibroblastic tumors. Their aggressiveness is local with no potential for metastasis or dedifferentiation. Here we report on a 61-year-old patient who presented a locally advanced breast desmoid tumor diagnosed 20 years after post-operative radiotherapy for breast carcinoma.

Primary synovial sarcoma of the thyroid gland: case report and review of the literature.

Synovial sarcoma (SVS) of the thyroid gland is exceedingly rare. We report the case of a 55-year-old man with a rapidly growing 7-cm neck mass. Because of suspicion of anaplastic thyroid carcinoma, a total thyroidectomy was planned, without preoperative cytology.

Correlation between overall survival and growth modulation index in pre-treated sarcoma patients: a study from the French Sarcoma Group.

Background: Growth modulation index (GMI), the ratio of two times to progression measured in patients receiving two successive treatments (GMI = TTP2/TTP1), has been proposed as a criterion of phase II clinical trials. Nevertheless, its use has been limited until now.

Patients And Methods: We carried out a retrospective multicentre study in soft tissue sarcoma patients receiving a second-line treatment after doxorubicin-based regimens to evaluate the link between overall survival and GMI.

Dramatic and delayed response to Doxorubicin-dacarbazine chemotherapy of a giant desmoid tumor: case report and literature review.

Desmoid tumors are benign, slow-growing mesenchymal tumors. Aggressiveness is local with no potential for metastasis or dedifferentiation. The treatment is challenging, particularly in the case of huge intra-abdominal locations.

Solitary fibrous tumor in the round ligament of the liver: a fortunate intraoperative discovery.

Solitary fibrous tumors (SFTs) are mesenchymal neoplasms of fibroblastic origin, most commonly found in the pleura. Numerous extrathoracic locations have been reported during the last 2 decades. Herein, we report the first case of an SFT in the round ligament of the liver.

Evaluating ecohydrological theories of woody root distribution in the Kalahari.

The contribution of savannas to global carbon storage is poorly understood, in part due to lack of knowledge of the amount of belowground biomass. In these ecosystems, the coexistence of woody and herbaceous life forms is often explained on the basis of belowground interactions among roots. However, the distribution of root biomass in savannas has seldom been investigated, and the dependence of root biomass on rainfall regime remains unclear, particularly for woody plants.

Solitary fibrous tumor of the prostate: case report and review of the literature.

Solitary fibrous tumor (SFT), usually described in the pleura, is exceedingly rare in the prostate. We report a 60-year-old man with prostatic SFT revealed by obstructive urinary symptoms, and detected by ultrasonography. Computed tomography (CT) and magnetic resonance imaging suggested a prostatic origin.

Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review.

Background: Chordomas are very rare low-grade malignant bone tumors that arise from the embryonic rests of the notochord. They are characterized by slow growth and long history with frequent local relapses, and sometimes metastases. While chemotherapy is not efficient, imatinib has shown antitumor activity.