1,344 results match your criteria Ewing Sarcoma and Primitive Neuroectodermal Tumors


Congenital Cutaneous Peripheral Primitive Neuroectodermal Tumor (pPNET) of Scalp: Youngest Case So Far.

J Indian Assoc Pediatr Surg 2021 Jan-Feb;26(1):60-62. Epub 2021 Jan 11.

Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi, India.

Congenital cutaneous peripheral primitive neuroectodermal tumor (pPNET) is very rare and also very rarely located in scalp. Only two cases of PNET as primary tumor in scalp are reported so far in the literature. Non mutilating surgical excision, combined with chemotherapy and radiotherapy are used in treating these rare tumors. Read More

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January 2021

Radiological and pathological diagnosis of an incidental Askin tumor.

Radiol Case Rep 2021 Jun 27;16(6):1245-1248. Epub 2021 Mar 27.

Department of Interventional Radiology, Kendall Regional Medical Center, 11750 SW 40th St, Miami, FL 33175, USA.

A 53-year-old male with no significant past medical history presented with an acute traumatic fracture of his thumb. Preoperative chest radiograph before K-wire fixation demonstrated an incidental 9 cm opacity of the left lung. Chest computed tomography revealed a 6. Read More

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Surviving cancer or future motherhood? Both are possible: an Ewing sarcoma case in an 8-year-old girl.

BMJ Case Rep 2021 Apr 8;14(4). Epub 2021 Apr 8.

Department of Obstetrics and Gynecology, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.

Ewing's sarcoma is an aggressive tumour, common in paediatric age, in which treatment often implies a decrease in reproductive potential. We describe a case of a woman who had a lumbar Ewing's Sarcoma in 1991, at the age of 8. She was submitted to extended tumourectomy, chemotherapy and local radiotherapy without preservation techniques. Read More

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Primary Spinal Epidural Extraosseous Ewing's Sarcoma with Brachial Plexus Infiltration.

Asian J Neurosurg 2020 Oct-Dec;15(4):1068-1071. Epub 2020 Oct 19.

Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.

The Ewing's sarcoma family of tumors are aggressive malignant small round blue cell tumors of undifferentiated mesenchymal origin. Skeletal Ewing's sarcoma is a common entity that classically involves the diaphysis of the long bones, pelvis, ribs, and sacrum. Extraosseous Ewing's sarcoma (EES) is rare, most commonly presenting as a paravertebral mass lesion. Read More

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October 2020

Primary gastric Ewing sarcoma/primitive neuroectodermal tumor.

J Int Med Res 2021 Feb;49(2):300060520986681

Department of Dermatology, Huangshi Central Hospital, Affiliated Hospital of Hubei Polytechnic University, Edong Health Care Group, Huangshi, Hubei, China.

A rare and highly malignant small round cell tumor, Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) usually occurs in the pelvis, long-axis bones, and femur. In contrast, extraosseous ES is more often found in the paraspinal region, limbs, and retroperitoneum, but is extremely rare in the stomach. We report a case of a 55-year-old woman who presented with fatigue, fever, and black stool. Read More

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February 2021

Congenital primary primitive neuroectodermal tumor of the orbit in a newborn.

Eur J Ophthalmol 2021 Jan 26:1120672121991047. Epub 2021 Jan 26.

Department of Pediatrics, Jawaharlal Postgraduate Medical Education and Research, Puducherry, India.

Introduction: Primitive neuroectodermal tumors arise from the progenitor cells of the neural crest, in the central nervous system or other peripheral locations.

Case Presentation: We report a rare case of a congenital malignant tumor, diagnosed as a primary orbital primitive neuroectodermal tumor on histopathological examination.

Conclusion: Multidisciplinary management with adjuvant chemotherapy needed for the management of these cases. Read More

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January 2021

Primary uterine Ewing sarcoma - A case report.

Taiwan J Obstet Gynecol 2021 Jan;60(1):142-144

Department of Obstetrics and Gynecology, Taipei Medical University Hospital, Taipei, Taiwan. Electronic address:

Objective: Ewing sarcoma is a type of neuroectodermal tumors (Ewing family of tumors-EFT) that mostly affect the bone or soft tissue. Primary uterine Ewing sarcoma is extremely rare.

Case Report: We report a case of a primary uterine Ewing sarcoma in a 46-year-old patient, treated with total abdominal hysterectomy, and bilateral salpingo-oophorectomy and following adjuvant chemotherapy with 6 cycles of vincristine, doxorubicin, and cyclophosphamide, achieving complete remission for one year. Read More

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January 2021

Primary Primitive Neuroectodermal Tumor (PNET) of the Spine With t(11;22): Report of 3 Cases and Review of Literature.

J Pediatr Hematol Oncol 2021 Jan 20. Epub 2021 Jan 20.

Division of Pediatric Oncology, Children's Hospital of Orange County, Orange Division of Hematology/Oncology and Bone Marrow Transplantation, Children's Hospital of Los Angeles, Los Angeles, CA.

Intradural extramedullary peripheral primitive neuroectodermal tumor (pPNET) with t(11;22) is a rare clinical finding in the pediatric population with few published cases in the literature. The authors report 3 cases of intradural primary pPNET and discuss the clinical presentation, treatment, and survival of the patients. Clinicians should be vigilant in considering pPNET in the differential diagnosis of extradural masses. Read More

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January 2021

Ovarian tissue cryopreservation and transplantation in patients with central nervous system tumours.

Hum Reprod 2021 Apr;36(5):1296-1309

Pôle de Gynécologie, Institut de Recherche Expérimentale et Clinique (IREC), Université Catholique de Louvain, Brussels, Belgium.

Study Question: Is there a possibility of reseeding cancer cells potentially present in frozen ovarian tissue from patients with central nervous system (CNS) tumours?

Summary Answer: Malignancy reseeding in cryopreserved ovarian tissue from 20 patients with CNS tumours was not detected by histology, immunohistochemistry (IHC), molecular biology or xenotransplantation.

What Is Known Already: Ovarian metastasis potential has been documented in patients with leukaemia, borderline ovarian tumours, advanced breast cancer and Ewing sarcoma. However, data on the safety of transplanting frozen-thawed ovarian tissue from cancer patients with CNS tumours are still lacking. Read More

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C19MC amplification and expression of Lin28A and Olig2 in the classification of embryonal tumors of the central nervous system: A 14-year retrospective study from a tertiary care center.

Childs Nerv Syst 2021 Apr 24;37(4):1067-1075. Epub 2020 Nov 24.

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India, 110029.

Introduction: CNS embryonal tumors (CET) other than medulloblastomas (MB) and atypical teratoid/rhabdoid tumors (AT/RTs), previously designated as 'central nervous system primitive neuroectodermal tumors' ('CNS PNETs'), are a heterogenous subset of tumors with poorly defined diagnostic criteria. Other than the subset of embryonal tumor with multilayered rosettes (ETMR) defined by C19MC amplification, most CETs are diagnosed by exclusion of other molecularly defined entities and histological mimics including MB, AT/RTs, and high-grade gliomas, and termed as CET, not otherwise specified (NOS) in the 2016 WHO classification.

Aim: To reclassify 'CNS PNETs' as per WHO 2016, and estimate the true proportion of CET, NOS in a tertiary healthcare setting, and to evaluate the diagnostic utility of C19MC amplification, Lin28A and Olig2 expression in the subclassification of CETs. Read More

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A rare entity of Primary Ewing sarcoma in kidney.

BMC Surg 2020 Nov 11;20(1):280. Epub 2020 Nov 11.

Department of Urology, Anhui Province, The First Affiliated Hospital of Wannan Medical College, Wuhu, People's Republic of China.

Background: Ewing sarcoma (ES) or primitive neuroectodermal tumors (PNET) represents a spectrum of poorly differentiated and aggressive malignancies. It rarely arises from the kidney and accounts for less than 1% of renal mass. Given the uncharacteristic clinical symptoms and imaging features, renal Ewing sarcoma (RES) is often diagnosed by postoperative pathology. Read More

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November 2020

Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Mimicking Meningioma: A Case Report and Literature Review.

Front Oncol 2020 6;10:528073. Epub 2020 Oct 6.

Department of Neurosurgery, First Hospital of Jilin University, Changchun, China.

Primary intracranial Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies, which arise in children and adolescents, with only 9 cases reported in patients over 30 years of age. Due to its rarity, MRI features and treatment strategies for primary intracranial ES/pPNETs remain unclear. The purpose of this study was to explore the clinical features, imaging findings, pathological characteristics, different diagnoses, treatment, and prognosis of cerebellar liponeurocytoma in adults. Read More

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October 2020

First report of a primitive neuroectodermal tumor of the bladder in a newborn.

Urol Case Rep 2021 Jan 16;34:101458. Epub 2020 Oct 16.

Research and Innovation Centre of the Faculty of Health Sciences, Universidad Peruana de Ciencias Aplicadas, Lima, Peru.

Primitive neuroectodermal tumor (PNET) is part of the Ewing sarcoma family of tumors. The present case reports a primitive neuroectodermal tumor (PNET) of rare location in the bladder in a newborn. It was evaluated with prenatal ultrasound and postnatal tomography that revealed a mass in the posterior wall of the bladder. Read More

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January 2021

Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: The MD Anderson Cancer Center Experience.

Cancers (Basel) 2020 Oct 11;12(10). Epub 2020 Oct 11.

Department of Pediatrics, the University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.

Limited information exists on the clinical behavior of the Ewing sarcoma family of tumors (ESFT) of the kidney. We reviewed the records of 30 patients (aged 8-69 years) with ESFT of the kidney seen at our institution between 1990 and 2013. We analyzed the event-free survival (EFS) and overall survival (OS) for associations with patient demographics, disease group, tumor size, tumor thrombus, and treatment. Read More

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October 2020

-associated metastatic abdominopelvic primitive neuroectodermal tumor with an rearrangement in a 16-yr-old female.

Cold Spring Harb Mol Case Stud 2020 10 7;6(5). Epub 2020 Oct 7.

Division of Pediatric Oncology, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, Maryland 21231, USA.

We report a case of a -associated -rearranged malignant primitive neuroectodermal tumor (PNET) arising in a patient with DICER1 tumor predisposition syndrome. A 16-yr-old female with a history of multinodular goiter presented with a widely metastatic abdominal small round blue cell tumor with neuroectodermal differentiation. gene rearrangement was identified in the tumor by fluorescence in situ hybridization (FISH). Read More

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October 2020

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor.

Sci Rep 2020 09 24;10(1):15656. Epub 2020 Sep 24.

Department of Radiation Oncology, Peking Union Medical College Hospital. Chinese Academy of Medical Sciences & Peking Union Medical College, No.1 Shuaifuyuan Wangfujing, Dongcheng District, Beijing, 100730, People's Republic of China.

Peripheral primitive neuroectodermal tumors (PNETs) constitute very rare and aggressive malignancies. To date, there are no standard guidelines for management of peripheral PNETs due to the paucity of cases arising in various body sites. Therapeutic approach is derived from Ewing sarcoma family, which currently remains multimodal. Read More

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September 2020

Pediatric vulvar malignancies: rare but important to know.

Semin Diagn Pathol 2021 Jan 5;38(1):99-109. Epub 2020 Sep 5.

Department of Pathology, University of California San Diego, San Diego, CA, USA. Electronic address:

Malignancies of the vulva in the pediatric population are exceptionally rare, which makes it difficult to gain any insight into their clinicopathologic profile. In this review, we summarize all published cases of a vulva malignancy in pediatric patients (≤21 years) reported in the English language literature for the 50-year period between 1970 and 2020. We estimate that less than 100 malignancies have been reported in total, approximately 50% of which were rhabdomyosarcomas. Read More

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January 2021

Structural Optimization and Enhanced Prodrug-Mediated Delivery Overcomes Camptothecin Resistance in High-Risk Solid Tumors.

Cancer Res 2020 10 24;80(19):4258-4265. Epub 2020 Aug 24.

Division of Oncology, Children's Hospital of Philadelphia, and the University of Pennsylvania/Perelman School of Medicine, Philadelphia, Pennsylvania.

Camptothecins are potent topoisomerase I inhibitors used to treat high-risk pediatric solid tumors, but they often show poor efficacy due to intrinsic or acquired chemoresistance. Here, we developed a multivalent, polymer-based prodrug of a structurally optimized camptothecin (SN22) designed to overcome key chemoresistance mechanisms. The ability of SN22 vs. Read More

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October 2020

Primary vulvar Ewing sarcoma/peripheral primitive neuroectodermal tumor with pelvic lymph nodes metastasis: A case report and review of literature.

J Obstet Gynaecol Res 2020 Oct 5;46(10):2185-2192. Epub 2020 Aug 5.

Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, American University of Beirut Medical Center, Beirut, Lebanon.

Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumors (pPNET) are soft tissue tumors that generally affect the bones. Extraosseous ES/pPNET has been rarely reported. Our patient presented with a 6 × 4 cm right subcutaneous solid vulvar lesion causing pain and discomfort. Read More

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October 2020

Primary intracranial Ewing sarcoma/ peripheral primitive neuroectodermal tumor, an entity of unacquaintance: a series of 8 cases.

Childs Nerv Syst 2021 Mar 6;37(3):839-849. Epub 2020 Aug 6.

Department of Radiation Oncology, Tata Memorial Hospital and ACTREC, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.

Purpose: The purpose is to highlight the primary intracranial (meningeal-based) occurrence of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET).

Methods: This report is a collation of clinicopathological features of eight cases of molecularly and clinicoradiologically confirmed primary (non-metastatic) intracranial (non-osseous) meningeal ES/PNET.

Results: The age range was 1 to 33 years with a median age of 9 years. Read More

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Comparative analysis of magnetic resonance imaging and pathological findings of microcystic meningioma and meningeal Ewing sarcoma/peripheral primitive neuroectodermal tumors.

Acta Neurol Belg 2020 Jul 15. Epub 2020 Jul 15.

Department of Radiology, Lanzhou University Second Hospital, Cuiyingmen No. 82, Chengguan District, Lanzhou, 730030, China.

Microcystic meningiomas (MCMs) and meningeal Ewing sarcoma/peripheral primitive neuroectodermal tumours (pPNETs) are difficult to differentiate because of the similarity in their image manifestation on magnetic resonance imaging (MRI). Differential diagnosis of these two tumours before surgery could contribute to ameliorating clinical decision-making, and predicting prognosis. Here, we aimed to comparatively analyse the difference between MRI and pathological findings of these two tumours. Read More

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Childhood Therapy-Related Acute Myeloid Leukemia with t(16;21)(q24;q22)/RUNX1-CBFA2T3 After a Primitive Neuroectodermal Tumor of the Chest Wall.

Clin Lymphoma Myeloma Leuk 2020 10 29;20(10):e660-e666. Epub 2020 May 29.

Hematology-Oncology and Stem-Cell Transplantation Unit, Department of Hematology and Developmental Therapeutics, Istituto Nazionale Tumori, IRCCS, Fondazione G. Pascale, Naples, Italy.

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October 2020

Gastric Ewing Sarcoma identified on a Meckel's scan.

Radiol Case Rep 2020 Aug 15;15(8):1235-1237. Epub 2020 Jun 15.

Department of Radiology, Phoenix Children's Hospital, 1919 E Thomas Rd. Phoenix, AZ 85016 USA.

Ewing Sarcoma is a malignant small round blue cell tumor most commonly found in bones and soft tissues of the axial skeleton and extremities. The Ewing family of tumors, including peripheral neuroectodermal tumor, represent the second most common malignancy in the pediatric population and second most common primary bone tumor after osteosarcoma. In a majority of Ewing Sarcoma cases, there is a translocation between chromosomes 11 and 22. Read More

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Challenges in the management of localized Ewing sarcoma in a developing country.

Pediatr Hematol Oncol 2020 Oct 19;37(7):610-619. Epub 2020 Jun 19.

Paediatric Haematology Oncology unit, Department of Paediatrics.

Survival in pediatric Ewing sarcoma (ES) lags in low- and middle-income countries (LMICs). This study analyzed factors contributing to a lower outcome in an LMIC center. A retrospective case review of children with localized ES treated from January 2011 till December 2017 was performed. Read More

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October 2020

Phase II results from a phase I/II study to assess the safety and efficacy of weekly nab-paclitaxel in paediatric patients with recurrent or refractory solid tumours: A collaboration with the European Innovative Therapies for Children with Cancer Network.

Eur J Cancer 2020 08 15;135:89-97. Epub 2020 Jun 15.

Gustave Roussy, Villejuif, France.

Background: The phase I component of a phase I/II study defined the recommended phase II dose and established the tolerability of nab-paclitaxel monotherapy in paediatric patients with recurrent or refractory solid tumours. The activity and safety of nab-paclitaxel monotherapy was further investigated in this phase II study.

Patients And Methods: Paediatric patients with recurrent or refractory Ewing sarcoma, neuroblastoma or rhabdomyosarcoma received 240 mg/m of nab-paclitaxel on days 1, 8 and 15 of each 28-day cycle. Read More

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Intracranial Ewing Sarcoma - A case report.

Surg Neurol Int 2020 30;11:134. Epub 2020 May 30.

Department of Neurosurgery, St. George Public Hospital, Kogarah, New South Wales, Australia.

Background: Intracranial Ewing's sarcoma (ES) is a rare entity with <15 cases reported in the literature. It belongs to a family of round-cell neuroectodermally derived tumors bearing many similarities to peripheral primitive neuroectodermal tumor (pPNET). There is currently no established treatment protocol. Read More

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Giant primitive neuroectodermal pelvic tumour: a case report and literature review.

J Int Med Res 2020 Jun;48(6):300060520906747

Department of Orthopaedics, Jiangxi Provincial People's Hospital Affiliated to Nanchang University, Nanchang, Jiangxi, China.

Primitive neuroectodermal tumours (PNETs) are rare malignant small round cell tumours. Notably, despite widespread reports of PNET in multiple parts of the body, it is extremely rare in the pelvis. Here, a rare case of giant PNET of the pelvis, that was treated with surgical intervention comprising hemipelvectomy and amputation, is reported. Read More

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A rare case of pelvic primitive neuroectodermal tumor with misleading symptoms: A case report.

Ann Med Surg (Lond) 2020 Jul 16;55:93-96. Epub 2020 May 16.

Department of Surgery, Aleppo University Hospital, University of Aleppo, Syria.

Peripheral Primitive NeuroEctodermal Tumors (pPNETs) are rare highly malignant tumors; originating from the neuroectoderm. Although PNETs may arise in various locations (most commonly in the extremities), very few cases have been reported in the pelvis. There is still poor evidence about the management of these tumors in the literature. Read More

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