neuroectodermic tumor treatment

Primary Ewing's kidney sarcoma is a rare and aggressive cancer with poor treatment outcomes. Furthermore, clinical presentations are nonspecific, such as abdominal pain, abdominal mass, cachexia, or hematuria. Currently, there is no consensus guideline for the treatment of this condition. We report on a 22-year-old man who presented with gross hematuria for 3 weeks. Computed tomography demonstrated a huge right renal mass with a thrombus in the inferior vena cava (IVC) without visceral metastasis. A percutaneous tissue biopsy revealed Ewing's sarcoma (ES) and the patient received neoadjuvant chemotherapy (doxorubicin and ifosfamide) for four cycles...

Carotid body tumors (CBTs) are rare neoplasms of the neuroectoderm accounting for 0.6% of head and neck tumors, with a 2%-12.5% risk of malignancy. While surgical resection has been associated with a high rate of neurologic and vascular complications, it remains the mainstay of treatment for malignant CBTs. We present the case of a 40-year-old female with a 5-year history of progressively enlarging right-sided neck mass, with MRI and MRA showing a Shamblin grade III CBT encasement of the internal carotid artery (ICA)...

Gastrointestinal neuroectodermal tumors (GNETs) are extremely rare and intriguing malignancies originating from neural crest cells in the digestive tract. The digestive tract's neural crest cells can give rise to incredibly unusual and interesting gastrointestinal neuroectodermal tumors (GNETs). GNETs present considerable hurdles in diagnosis and management because of their rarity and varied expression. In this case report, a 45-year-old male patient is described who had signs of GNET, such as exhaustion, weight loss, and abdominal pain...

OBJECTIVE: Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare, malignant tumor of neuroectodermal origin that arises from the olfactory neuroepithelium. In this study the authors present the first series in the literature on distant brain metastases (BMs) secondary to ENB that were treated with stereotactic radiosurgery (SRS), to evaluate the safety and effectiveness of SRS for this indication. METHODS: A retrospective analysis of clinical and radiological outcomes of patients with ENB who underwent CyberKnife (CK) SRS at a single center was conducted...

The 2016 WHO classification of tumors of the central nervous system affected importantly the group of CNS embryonal tumors. Molecular analysis on methylome, genome, and transcriptome levels allowed better classification, identification of specific molecular hallmarks of the different subtypes of CNS embryonal tumors, and their more precise diagnosis. Routine application of appropriate molecular testing and standardized reporting are of pivotal importance for adequate prognosis and treatment, but also for epidemiology studies and search for efficient targeted therapies...

Primitive neuroectodermal tumors (PNETs) are a type of malignant tumors made up of small neuroectodermal-derived round cells that affect soft tissue and bone, with a wide range of clinical symptoms and histological commonalities depending on the site of the tumor. PNETs account for 4% of all pediatric and adolescent cancers. Here we report a case of a peripheral primitive neuroectodermal tumor in a five-year-old boy. Two days before admission, he complained of multiple attacks of vomiting and one episode of hematemesis, associated with subjective fever, abdominal pain, and distention...

BACKGROUND: Neoplasms of the vermiform appendix are rare. They comprise a heterogeneous group of entities requiring different kinds of treatment. METHODS: This review is based on publications retrieved by a selective literature search in the PubMed, Embase, and Cochrane databases. RESULTS: 0.5% of all tumors of the gastrointestinal tract arise in the appendix. Their treatment depends on their histopathological classification and tumor stage...

Primitive neuroectodermal tumor of the periphery is a rare entity and belongs to Ewing sarcoma family of tumors. It comprises 1% of all sarcomas and majority of these cases occur in the age group of less than 35 years. It is highly aggressive and patient often presents with distant metastasis leading to poor prognosis. Its characteristic features include t(11:22) translocation. A young female patient presented with growth in the tip of the tongue. Biopsy was done and histopathology using H&E and PAS staining revealed primitive neuroectodermal tumor...

INTRODUCTION AND IMPORTANCE: Ewing's sarcoma (ES) is a malignant mesenchymal tumor that is categorized as a primitive neuroectodermal tumor (PNET) and is a member of the "small round-cell tumor" family. Spinal extraosseous extradural lesions are highly uncommon in PNETs. There are few clinical studies and information on extra-osseous Ewing tumor outcomes. CASE PRESENTATION: A 19-year-old woman, with progressive dull aching low back pain of a duration of one month presented...

BACKGROUND: Spinal atypical teratoid rhabdoid tumor (AT/RT) is an extremely rare tumor and represents less than 2% of all AT/RTs. METHODS: Available medical literature on spinal AT/RT in English was retrieved from PubMed and comprehensively reviewed. Clinical presentation, diagnosis, management, prognosis, and outcome in patients with spinal AT/RT have been elucidated by citing a case of extradural AT/RT of the cervicodorsal spine. RESULTS: The age at presentation is usually less than 3 years...

Sinonasal cancers (SNCs) are rare and heterogeneous in histology and biological behavior. The prognosis is generally unfavorable, especially in inoperable cases. In recent years, for some histologies, such as undifferentiated sinonasal carcinoma (SNUC), multimodal treatment with a combination of induction chemotherapy, surgery, and chemo/radiotherapy (RT) has improved the prognosis. Nevertheless, still about half of the patients treated incur a recurrence, in most of the cases at the local site. Surgery with and without RT is usually the treatment choice in cases of recurrence after previous RT in combination with systemic therapy or RT in a histology-driven fashion...

INTRODUCTION: Melanotic Neuroectodermal tumour of Infancy (MNTI) is a rare entity of pigmented neoplasms of head and neck region. It predominantly occurs within the first year of life. The authors present enucleation as the definitive surgical treatment, with reference to the five departmental cases of MNTI with no recurrence at 5years and 1year of follow-up of other 4 cases. CASE PRESENTATION: Five cases of MNTI (age group of 2.5months-7 months) presented to our department as a large bluish-brown non tender swelling protruding into the oral cavity...

Melanoma is one of the most dangerous types of cutaneous neoplasms, which are pigment-producing cells of neuroectodermal origin found all over the body. A great deal of research is focused on the mechanisms of melanoma to promote better diagnostic and treatment options for melanoma in its advanced stages. The progression of melanoma involves alteration in different levels of gene expression. With the successful implementation of next-generation sequencing technology, an increasing number of long noncoding RNAs (lncRNAs) sequences have been discovered, and a significant number of them have phenotypic effects in both in vitro and in vivo studies, implying that they play an important role in the occurrence and progression of human cancers, particularly melanoma...

BACKGROUND: Ewing sarcoma has attracted more attention in recent years but has yet to be bibliometrically analyzed. Hence, this study investigated the trend of Ewing sarcoma over the past 30 years with bibliometric analysis. METHODS: Original publications related to Ewing sarcoma were obtained from the Science Citation Index Extension (SCI-E), Social Sciences Citation Index (SSCI), and Web of Science Core Collection (WoSCC) between 1993 and 2022. CiteSpace and VOSviewer were used to extract the countries/regions, institutions, authors, journals, references, and keywords involved in this topic to identify and analyze the research hotspots and trends in this field...

BACKGROUND: Ectomesenchymomas (EMs) are extremely rare neoplasms composed of malignant mesenchymal components and neuroectodermal derivatives. They are described in a wide variety of locations, with the head and neck region being one of the most frequently involved areas. EMs are usually managed as high-risk rhabdomyosarcomas and have similar outcomes. METHODS: We present the case of a 15-year-old female with an EM that arose in the parapharyngeal space and extended into the intracranial space...

Gangliosides are expressed in nervous systems and some neuroectoderm-derived tumors at high levels and play pivotal roles. However, mechanisms for the regulation of glycosyltransferase genes responsible for the ganglioside synthesis are not well understood. In this study, we analyzed DNA methylation patterns of promoter regions of GD3 synthase (ST8SIA1) as well as mRNA levels and ganglioside expression using human glioma cell lines. Among 5 cell lines examined, 4 lines showed changes in the expression levels of related genes after treatment with 5-aza-dC...

Primary diffuse leptomeningeal primitive neuroectodermal tumor is a rare meningeal neoplasm which can masquerade as chronic meningitis. While the clinical presentation and radiological features may provide a clue to this condition, meningeal biopsy is essential to clinch the diagnosis. A high index of suspicion and a low threshold for re-evaluating cases of neuroinfection that do not respond to empirical therapy are essential in this scenario. We present the case of a nine year old boy who was initiated on antituberculous treatment for chronic meningitis with hydrocephalus...

The development of somatic-type malignancy (SM) in testicular germ cell tumor represents a major challenge in the diagnosis and treatment of testicular cancer. Most SMs are derived from teratoma, and the remainder is associated with yolk sac tumor. They occur more frequently in metastases than in primary testicular tumors. SMs demonstrate a variety of histologic types, including sarcoma, carcinoma, embryonic-type neuroectodermal tumor, nephroblastoma-like tumor, and hematologic malignancy. Sarcoma, particularly rhabdomyosarcoma, accounts for the majority of SMs in the primary testicular tumors, whereas carcinoma, particularly adenocarcinoma, is the most common SM in metastases...

Melanotic neuroectodermal tumors of infancy (MNTI) are a rare type of benign tumor that affects the head and neck region. MNTI represents (68-80%) of the tumors in the maxillary region. This tumor is usually located in the alveolar crest, skull, mandible, and brain. Although this tumor is considered benign, it can grow rapidly, with a high risk of recurrence and interference with functions of infancy, such as feeding and breathing. It is also frequently harmful to the surrounding soft and hard tissues or adjacent sensitive vital structures...

Advanced osteosarcomas (OSs) and Ewing sarcomas (ESs) tend to have poor prognosis with limited therapeutic options beyond first-line therapy. Aberrant angiogenesis and MET signaling play an important role in preclinical models. The anti-angiogenic drug cabozantinib was tested in a phase 2 trial of advanced OS and ES and was associated with clinical benefits. We retrospectively analyzed the off-label use of cabozantinib in adult patients with advanced OS and ES/primitive neuroectodermal tumors (PNETs) in three centers of the Hellenic Group of Sarcoma and Rare Cancers (HGSRC)...