Ewing Sarcoma Family Tumors: Past, Present and Future Prospect

2020 ◽  
Vol 16 ◽  
Author(s):  
Adil Abbas ◽  
Alaa Mohammed Noor Samkari
Keyword(s):  
Soft Tissues ◽  
Fusion Gene ◽  
Surgical Techniques ◽  
Disease Free Survival ◽  
Clinical Aspects ◽  
Immunohistochemical Markers ◽  
The Central Nervous System ◽  
Blue Cell ◽  
Involved Field ◽  
Involved Field Radiotherapy

Abstract: Ewing’s sarcoma (ES), also known as mesenchymal primitive neuroectodermal tumor (PNET), is a malignant round blue cell tumour (MRBCT) with a varying degree of neuronal differentiation. PNET arise from the primitive nerve cells of the central nervous system (CNS) but they may occur in the bones of the extremities, pelvis, vertebral column and chest wall. Extraskeletal ES/PNET may affect the various soft tissues including those of the pelvis, paraspinal region and the thoraco - pulmonary regions Histopathological differentiation between ES, PNET and other related sarcomas is often difficult. On light microscopy, the same histopathological appearance of ES, has been termed PNET, Askin – Rosay (A – R) tumor and malignant neuropeithelioma by various other authors. Immunohistochemical distinction is also difficult due to poor tissue differentiation and poor intake of the various specific immunohistochemical markers. The most frequent translocation is t (11; 22) (q24; q12) resulting in the EWSR1-FLI1 fusion gene detected in nearly 90% of cases and is considered the whole mark of the diagnosis ES, PNET, atypical ES and A – R tumor. Therefore, ES, atypical ES, PNET and A – R tumor are currently regarded as one entity grouped together under the Ewing Family Tumors (EFT) and are treated in an identical way. EFT represent only about 3% of all pediatric malignancies. The annual incidence is between 2 and 5 cases per million children per year. The peak prevalence of the tumor is between the ages of 10 and 15 years. The incidence is higher in males than in females with a ratio of 1.3: 1. Newer groups of MRBCT are with great similarities to EFT are being recently described. These tumors; atypical EFT and Ewing’s like Sarcomas (ELS) bear similarities to EFT but has basic morphological and molecular differences. Optimal treatment requires the use of adjuvant and new-adjuvant chemotherapy (CTR), radical surgical resection and/or involved field radiotherapy (RT). The reported disease free survival (DFS) and overall survival (OS) ranges between 45 – 80% and 36 - 71% respectively. The overall prognosis for metastatic and recurrent disease remains poor. Use of newer conventional and targeted medications, improved RT delivery and surgical techniques may further improve the outcomes. The past few years has seen advances in genomics-based sarcoma diagnosis and targeted therapies. In this wide review article we provide comprehensive report of EFT and discuss the various clinical aspects and the recent advances used in the diagnosis and treatment.

scholarly journals Melanotic Neuroectodermal Tumor of Infancy

2018 ◽  
Vol 142 (11) ◽  
pp. 1358-1363 ◽  
Author(s):  
Brian S. Soles ◽  
Allecia Wilson ◽  
David R. Lucas ◽  
Amer Heider
Keyword(s):  
Differential Diagnosis ◽  
Local Recurrence ◽  
Soft Tissues ◽  
Malignant Neoplasm ◽  
Neck Region ◽  
Neuroectodermal Tumor ◽  
Growth Potential ◽  
Incomplete Resection ◽  
The Central Nervous System ◽  
Blue Cell

Context.— Melanotic neuroectodermal tumor of infancy, albeit rare and generally regarded as benign, is an important tumor to recognize because of its rapid growth, potential for local recurrence, and small round blue cell morphology, which can lead to misdiagnosis of a malignant neoplasm. Objective.— To review its clinical presentation and immunomorphologic findings, and discuss common entities in the differential diagnosis. Data Sources.— The study involved PubMed searches, including multiple review articles, case studies, retrospective studies, selected book chapters, and University of Michigan cases. Conclusions.— Melanotic neuroectodermal tumor of infancy most commonly occurs in the bones of the head and neck region during the first year of life, but it can also present in other locations, including the central nervous system, testes, ovaries, and subcutaneous soft tissues. Histologically, it is composed of a biphasic population of cells, consisting of epithelioid melanin-producing cells and primitive neurogenic cells in a fibrocollagenous stroma. These microscopic findings, especially in small biopsies, can lead to a broad differential diagnosis that includes malignant small round blue cell tumors and malignant melanoma. Melanotic neuroectodermal tumor of infancy commonly has an infiltrative growth pattern, and anatomic constraints often lead to incomplete resection and local recurrence, requiring multiple surgical operations. Because melanotic neuroectodermal tumor of infancy can mimic a more aggressive and aggressively treated malignancy, recognition of this rare tumor is very crucial for pathologists.

Curability of solitary bone plasmacytoma.

1992 ◽  
Vol 10 (4) ◽  
pp. 587-590 ◽  
Author(s):  
M A Dimopoulos ◽  
J Goldstein ◽  
L Fuller ◽  
K Delasalle ◽  
R Alexanian
Keyword(s):  
Disease Free Survival ◽  
Solitary Plasmacytoma ◽  
Survival Fraction ◽  
Myeloma Protein ◽  
Free Survival ◽  
Solitary Bone Plasmacytoma ◽  
Involved Field ◽  
Involved Field Radiotherapy ◽  
Extent Of Disease

PURPOSE The effects of involved-field radiotherapy were assessed in patients with a solitary plasmacytoma of bone (SBP). PATIENTS AND METHODS Forty-five consecutive patients with an SBP received megavoltage irradiation of at least 3,000 cGy. The median age was 53 years, 67% of patients showed a myeloma protein, and uninvolved immunoglobulins (Igs) were preserved in 93% of patients. RESULTS Permanent control of presenting disease was achieved in all but two patients, but 46% of patients developed multiple myeloma. When it occurred, progression of myeloma occurred within 3 years in two thirds of the patients, suggesting that the extent of disease was understaged at diagnosis. Myeloma protein disappeared in nine patients (30%) whose disease has not yet recurred. The median survival for all patients was 13 years and the myeloma-specific survival fraction at 10 years was 53%. CONCLUSION In patients with an SBP, the disappearance of myeloma protein with involved-field radiotherapy predicted long-term disease-free survival and possible cure. Nonsecretory disease and persistent myeloma protein after treatment were adverse prognostic factors for which adjuvant therapy with interferon alfa should be considered.

The Role of Arthroscopy in the Treatment of Gouty Arthritis of the Knee Case presentation

2018 ◽  
Vol 69 (8) ◽  
pp. 2236-2239
Author(s):  
Marius Moga ◽  
Mark Edward Pogarasteanu ◽  
Dumitru Ferechide ◽  
Antoine Edu ◽  
Chen Feng Ifrim
Keyword(s):  
Gouty Arthritis ◽  
Surgical Techniques ◽  
Term Treatment ◽  
Clinical Aspects ◽  
Associated Lesions ◽  
Number Of Patients ◽  
Long Term Treatment ◽  
Functional Scores ◽  
Urate Crystals

Gout is a metabolic disease involving the impregnation of joints and other tissues with urate crystals. The onset is often brutal, and it manifests itself with pain and inflammation in the affected joint. The treatment usually involves rest, ice, NSAIDs and anti-gout medication. The long-term treatment involves medication and dietary changes. In the joint, urate crystals are deposited in the synovial, in the cartilage and in the menisci. In the arthroscopic practice, the gouty knee is a rare occurrence. We present a relevant case, that of a 57 years old patient without a prior gout diagnosis where we found urate crystal deposits covering the synovium, cartilage and meniscus, and we discuss the current and recent year Pub Med indexed literature in order to evaluate the possibilities for arthroscopic treatment of this pathology. We looked at the number of patients involved, their characteristics, and the surgical techniques used. We also looked at the temporal relation of the arthroscopic intervention to the recent gout attacks, and at the described lesions that were found. Also, we evaluated the papers for joint liquid analysis, gout drug treatment, and description of clinical aspects involved and associated lesions. Finally, we looked at the follow-up, at the functional scores used to monitor the patient�s evolution, at the associated medication and at the long-term outcomes, if described. We have found opinions to vary. In the end, we draw conclusions pertaining to the practical short-term and long-term use of knee arthroscopy in the treatment of gout.

RESULTS OF TREATMENT OF LOCALLY RECURRING SOFT TISSUES SARCOMAS OF EXTREMITIES

2018 ◽  
Vol 64 (3) ◽  
pp. 408-413
Author(s):  
Grigoriy Zinovev ◽  
Georgiy Gafton ◽  
Sergey Novikov ◽  
Ivan Gafton ◽  
Yekaterina Busko ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Soft Tissues ◽  
Cox Regression ◽  
Disease Free Survival ◽  
Tumor Location ◽  
Long Term Results ◽  
Clinical Feature ◽  
National Medical ◽  
Results Of Treatment ◽  
Kaplan Meier

Background: The most striking clinical feature of soft tissues sarcomas (STS) is their ability to recur. At present disputes about the clinical and morphological factors of STS recurrence such as the degree of malignancy, size, location, depth of tumor location, patient’s age and the presence of previous relapses in the anamnesis do not subside. It also requires clarification of the effect of the volume of tissues removed on the long-term results of treatment of STS as well as indications for the application of various regimes of remote radiation therapy. Materials and methods: Of 1802 registered cases of STS of extremities at the N.N. Petrov National Medical Research Center of Oncology from 2004 to 2016 there were selected data on 213 patients who suffered from at least one relapse of the disease. There was performed an assessment of overall, non-metastatic and disease-free survival using a single-factor (the Kaplan-Meier method) and multivariate analysis (the Cox regression model). Conclusion: The detection of various prognostic factors of locally recurrent STS allows determining the necessary treatment tactics (the vastness and traumatism of surgery and the advisability of radiation therapy).

scholarly journals Immunohistochemical Analysis of DNA Repair- and Drug-Efflux-Associated Molecules in Tumor and Peritumor Areas of Glioblastoma

2021 ◽  
Vol 22 (4) ◽  
pp. 1620
Author(s):  
Cristiana Angelucci ◽  
Alessio D’Alessio ◽  
Silvia Sorrentino ◽  
Filippo Biamonte ◽  
Umberto Moscato ◽  
...  
Keyword(s):  
Breast Cancer Resistance Protein ◽  
Surgical Techniques ◽  
Inverse Correlation ◽  
Immunohistochemical Analysis ◽  
Cancer Resistance ◽  
Chemotherapeutic Regimen ◽  
The Central Nervous System ◽  
Peritumoral Tissue ◽  
Resistance Protein ◽  
Methylguanine Methyltransferase

Glioblastoma (GBM), the most commonly occurring primary tumor arising within the central nervous system, is characterized by high invasiveness and poor prognosis. In spite of the improvement in surgical techniques, along with the administration of chemo- and radiation therapy and the incessant investigation in search of prospective therapeutic targets, the local recurrence that frequently occurs within the peritumoral brain tissue makes GBM the most malignant and terminal type of astrocytoma. In the current study, we investigated both GBM and peritumoral tissues obtained from 55 hospitalized patients and the expression of three molecules involved in the onset of resistance/unresponsiveness to chemotherapy: O6-methylguanine methyltransferase (MGMT), breast cancer resistance protein (BCRP1), and A2B5. We propose that the expression of these molecules in the peritumoral tissue might be crucial to promoting the development of early tumorigenic events in the tissue surrounding GBM as well as responsible for the recurrence originating in this apparently normal area and, accordingly, for the resistance to treatment with the standard chemotherapeutic regimen. Notably, the inverse correlation found between MGMT expression in peritumoral tissue and patients’ survival suggests a prognostic role for this protein.

scholarly journals All That Coughs Is Not Covid-19: A Delayed Diagnosis of Disseminated Coccidioidomycosis Following SARS-CoV-2

2021 ◽  
Author(s):  
Joshua C Chen ◽  
Darren Wong ◽  
Sina Rabi ◽  
Scott Worswick ◽  
Brittney DeClerck ◽  
...  
Keyword(s):  
Soft Tissues ◽  
Delayed Diagnosis ◽  
Severe Pneumonia ◽  
Coccidioides Immitis ◽  
Cutaneous Lesions ◽  
Additional Risk ◽  
Northern Mexico ◽  
The Central Nervous System ◽  
Endemic Fungi ◽  
Respiratory Complaints

Abstract Coccidioides immitis (and C. posadasii) are endemic fungi of the southwestern United States and northern Mexico. Uncomplicated, symptomatic Coccidioides infection most commonly causes a self-limited pneumonia; however, immunocompromised patients can manifest severe pneumonia with an additional risk of dissemination to bone, joints, soft tissues, and in the most severe the cases, the central nervous system. In the year 2020 clinicians are challenged with a previously unseen volume of acute respiratory complaints as a result of the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) pandemic. We present a patient with respiratory failure secondary to SARS-CoV-2 who experienced prolonged hypoxia and neurologic deterioration, eventually leading to a diagnosis of occult disseminated coccidiomycosis involving meningitis, miliary-pattern pneumonia, and cutaneous lesions.

scholarly journals Primitive Neuroectodermal Tumor of the Liver: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Eduardo Cambruzzi ◽  
Enilde Eloena Guerra ◽  
Hamilton Cardoso Hilgert ◽  
Herbert Jorge Schmitz ◽  
Vinícius Lopes Silva ◽  
...  
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Soft Tissues ◽  
Young Patients ◽  
Neuroectodermal Tumor ◽  
Solid Mass ◽  
Resected Liver ◽  
The Central Nervous System ◽  
Right Lobe ◽  
Upper Abdominal ◽  
The Right

Primary liver sarcomas represent a rare group of neoplasias, with angiosarcoma being the most common histological type. Primitive neuroectodermal tumor (PNET) represents a high malignant neoplasia that usually affects the central nervous system and soft tissues. An 18-year-old male patient was admitted with clinical complains of pain in the right upper abdominal quadrant. The clinical evaluation revealed a solid mass in the right hepatic lobe. On the gross examination of the resected liver specimen, the right lobe of the liver was replaced by a yellow-red solid mass measuring 21 cm in its largest dimension. On the histopathology, a tumor composed of small round blue cells with little cytoplasm and round nuclei was identified. The lesion revealed positive immunoexpression for vimentin and CD99 and negative immunostaining for desmin, CD45, cytokeratin, and neuroblastoma protein, suggesting, then, the diagnosis of PNET. Although it is an unusual tumor, it should be considered in the differential diagnosis of liver masses, especially in young patients.

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