The role of surgery in recurrent local cerebral metastases: a multi-institutional retrospective analysis.

Background: Local recurrent brain metastases are defined as lesions that recur in the brain at the same site after a previous local therapy. In patients already submitted to surgery, a second operation may be potentially challenging due to scar formation, infiltration of cerebral vessels or eloquent brain areas and local effect of previous radiotherapy. The aim of this study is to retrospectively review the results and complications of a second surgical treatment in a series of local recurrent lesions and to review the literature on this topic.

Second surgery for relapsed glioblastoma: an observational study on criteria for patient selection in real life.

There is little consensus on salvage management of glioblastoma after recurrence, for lack of evidence. A retrospective study of treatments in patients with recurrent glioblastoma. Surgery at recurrence was related to better overall survival (OS) and progression-free survival (PFS).

Weakening or Structural Strengthening? An Evaluation of Bone Density after MRgFUS Ablation for Treatment of Benign Bone Lesions.

Previous studies suggest that interventional ablative procedures on bone lesions may weaken the bone, especially when performed through the needle approach. Our purpose was to evaluate, through Computed Tomography (CT), the effects of Magnetic Resonance guided Focused Ultrasound Surgery (MRgFUS) ablation on painful osteoid osteomas and osteoblastomas in terms of bone density and morphological changes. We retrospectively evaluated patients treated at our institution with MRgFUS for superficial, painful osteoid osteoma or osteoblastoma during the last 9 years.

Osteoblastoma: When the Treatment Is Not Minimally Invasive, an Overview.

Background: osteoblastoma is a bone-forming tumor accounting for about 1% of all primary bone tumors and 3% of benign bone tumors. The gold-standard treatment is surgical excision; nevertheless, minimally invasive radiological techniques such as thermoablation and, more recently, high intensity focused ultrasound are gaining more importance. The aim of the present paper is to analyze surgical indications based on our experience and on the evidences in the literature.

Correlation Between Immunohistochemistry and Sequencing in H3G34-Mutant Gliomas.

Recurrent glycine-to-arginine/valine alterations at codon 34 (G34R/V) within H3F3A gene characterize a subset of hemispheric high-grade gliomas (HGG) affecting children and young adults. These tumors, defined as G34R/V-mutant gliomas, are histologically heterogenous, with microscopic features of either HGG or embryonal tumors (primitve neuroectodermal tumor-like features). To assess the value of immunohistochemistry (IHC) to detect G34R/V-mutated cases, we tested anti-histone G34V (clone 329E5) and anti-histone G34R (clone RM240) antibodies in a series of 28 formalin-fixed and paraffin-embedded samples.

Histopathological grading affects survival in patients with IDH-mutant grade II and grade III diffuse gliomas.

Background: Diffuse grade II and grade III gliomas are actually classified in accordance with the presence of isocitrate dehydrogenase mutation (IDH-mut) and the deletion of both 1p and 19q chromosome arms (1p/19q codel). The role of tumour grading as independent prognostic factor in these group of tumours remains matter of debate. The aim of this study was to determine if grade is an independent prognostic factor and not somehow associated to IDH mutation and 1p/19q status of the tumour.

Loss of histone H3 trimethylation on lysine 27 and nuclear expression of transducin-like enhancer 1 in primary intracranial sarcoma, DICER1-mutant.

Aims: Primary intracranial sarcoma, DICER1-mutant is a recently described central nervous system tumour with specific genomic and DNA-methylation profiles. Although some of its histological features (focal spindle-cell morphology, intracytoplasmic eosinophilic granules, and focal heterologous differentiation) are common across most reported cases, the presence of significant histological variability and the lack of differentiation pose diagnostic challenges. We aim to further define the immunoprofile of this tumor.

Transethmoidal encephalocele endoscopically treated in a newborn: a case report.

Congenital encephalocele is a very rare entity, with herniation of normal brain or gliotic tissue through a defect in the skull. The objective is to present a newborn child diagnosed with transethmoidal encephaloceles at birth. She developed respiratory problems, feeding difficulties, and failure to thrive since the first days of life and so required early surgery at her 33th day of life, through an endoscopic nasal approach.

Primary cardiac synovial sarcoma: A review correlating outcomes with surgery and adjuvant therapy.

Background: Cardiac synovial sarcoma (CSS) is an extremely rare malignant tumor with a severe prognosis, due to frequent relapses and metastases. To obtain useful information for treatment protocols, we analyzed survival and therapy data from the cases reported in the literature.

Methods: A search of MEDLINE was performed throughout December 2018.

F-FDG PET/CT in the evaluation of cartilaginous bone neoplasms: the added value of tumor grading.

Objectives: Cartilaginous bone tumors represent a wide variety of neoplasms ranging from benign to extremely aggressive malignant lesions. Unlike other tumors, the biopsy cannot easily predict the histological grade, sometimes not allowing choosing the best therapeutic approach. The aim of the study was to evaluate the ability of F-FDG PET/CT to differentiate enchondroma from chondrosarcoma and to predict the histological grade as compared to biopsy.

HMGA1/E2F1 axis and NFkB pathways regulate LPS progression and trabectedin resistance.

Although the medical treatments of sarcoma have evolved in the last years, a significant portion of patients develops recurrence after therapies suggesting the need to identify novel targets to improve the treatments. By the use of patient-derived and established cell lines from liposarcoma, as well as specimens from patient biopsies, we found that HMGA1 is involved in the progression of dedifferentiated and myxoid liposarcoma. The immunohistochemical and RT-PCR analyses of 68 liposarcoma specimens revealed a significant high expression of HMGA1, at the protein and RNA levels, both in myxoid and dedifferentiated liposarcoma subtypes compared with differentiated ones.

Treatment of cardiac synovial sarcoma: experience of two cases.

Background: Primary heart sarcomas are exceedingly rare tumors. Among primary cardiac sarcomas, synovial sarcoma is one of the rarest, involving cardiac cavities or pericardium.

Case Presentation: Two cases of synovial sarcoma are presented with the clinical course and therapy.

Precision diagnostics of Ewing's sarcoma by liquid biopsy: circulating fusion transcripts.

Background: Limited information is available on the applicative value of liquid biopsy (LB) in rare tumors, including Ewing's sarcoma (ES). The accepted precision diagnostics standards would greatly benefit from a non-invasive LB test monitoring pathognomonic gene rearrangements in the bloodstream.

Methods: Tissue and blood samples were collected from six and four ES patients, respectively.

Intracranial neuromuscular choristoma: Report of a case with literature review.

Neuromuscular choristoma (NMC), also called neuromuscular hamartoma or nerve rhabdomyoma, is a rare lesion of the spinal and cranial nerves composed of skeletal muscle intimately associated with nerve fibers. Its origin has not been precisely clarified and a malformative event, resulting from aberrant differentiation or a true neoplastic growth, have been proposed by authors. We hereby present a cerebellopontine angle NMC enlarging the eighth cranial nerve in a 3-year-old child, that histologically appeared composed of a large amount of striated muscle mixed with nerve fibers.

Characteristics of ganglion cells in pituitary gangliocytomas.

The occurrence of ganglion cells in the sella turcica, in association or not with a pituitary adenoma, has been rarely reported. Various names have been employed for this rare entity, gangliocytoma being frequently used and recommended by WHO classification. Expression of cytokeratin in these ganglion cells has been previously occasionally reported, a very intriguing observation raising questions on the possible nature and derivation of these cells.

Spontaneous Regression of Pineal Lesions: Ghost Tumor or Pineal Apoplexy?

Background: Pineal apoplexy (either hemorrhagic or ischemic) may complicate the course of a tumor at this site. This event usually is characterized by an acute clinical onset and requires emergency surgical management whereas the regression of the lesion is a much rarer outcome.

Material And Methods: Three cases of pineal vanishing tumors in the pediatric population are reported and the pertinent literature is reviewed.

Recurrent adult-type fibrosarcoma of the frontal bone in a child.

Introduction: Primary, adult-type bone fibrosarcoma is an uncommon, malignant spindle-cell tumor of fibroblastic origin, rarely affecting children. Most frequently diagnosed among bone malignancies in the past, improved diagnostic techniques and further restrictive classification criteria have currently made the diagnosis of fibrosarcoma very unusual.

Case Report: We hereby report the case of a 7-year-old child with a right frontal swelling mass.

miR-181c associates with tumor relapse of high grade osteosarcoma.

High-grade osteosarcoma (OS) is characterized by low incidence, high aggressiveness and moderate 5-years survival rate after aggressive poly-chemotherapy and surgery. Here we used miRNA profiling as a tool to possibly predict and monitor OS's development and therapeutic outcome. First, we evaluated the altered expression of selected miRNAs from a case of Giant Cell Tumor (GCT) apparently evolved into an OS.

Is CRX protein a useful marker in differential diagnosis of tumors of the pineal region?

The cone-rod homeobox (CRX) is a gene that belongs to the member of the orthodenticle homeobox (Otx) family, with important function in development and differentiation of retinal and pineal cells. Moreover, CRX appears to be specifically expressed in pineal tumors and retinoblastomas. We performed an immunohistochemical study on 91 pediatric and adult central nervous system tumors, plus 2 normal brain samples.

Cartilage differentiation in ependymoma: histogenetic considerations on a new case.

The presence of cartilage in gliomas is a very unusual finding and has been mainly reported in ependymomas and in astrocytomas. A derivation of cartilage from neuroepithelial cells through a neuroepithelial-mesenchymal transition or directly from blood vessel-associated multipotent stromal elements has been proposed. We herein describe a further case of ependymoma with the presence of cartilage in a child affected by a tumor in the posterior fossa.

Giant cell tumor of bone in a patient with diagnosis of primary hyperparathyroidism: a challenge in differential diagnosis with brown tumor.

Giant cell lesions of bone share similar clinical, radiological, and histological features. The most challenging differential diagnosis is between giant cell tumor (GCT) and brown tumor (BT) secondary to hyperparathyroidism. Differential diagnosis is based on determining serum calcium concentration and other markers of calcium metabolism.

Myeloid sarcoma with megakaryoblastic differentiation mimicking a sellar tumor.

Myeloid sarcoma (MS) is a localized extra-medullary tumor mass of immature myeloid cells, arising de novo or related to acute myeloid leukemia, of which it can be a forerunner, a coinciding or late event. Less commonly, MS represents an acute blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms. This rare condition commonly consists of a proliferation of more or less immature cells with a myeloid immunophenotype, very exceptional cases showing a megakaryoblastic or erythroid differentiation.

Primary multicentric anaplastic pleomorphic xanthoastrocytoma with atypical features.

Pleomorphic xanthoastrocytoma (PXA) is a rare tumor with good prognosis after surgery. Few cases of anaplastic PXA (either de novo or secondary to transformation of a recurrent low grade PXA) have been reported. Moreover, primary anaplastic PXA with dissemination at diagnosis has been described only in two patients, to our knowledge.