Raras
Buscar doenças, sintomas, genes...
Tumor neuroectodérmico primitivo periférico
ORPHA:370348CID-10 · C71.9DOENÇA RARA

Pequeno tumor de células redondas com diferenciação neural originado de tecidos moles ou ossos.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Pequeno tumor de células redondas com diferenciação neural originado de tecidos moles ou ossos.

Pesquisas ativas
14 ensaios
209 total registrados no ClinicalTrials.gov
Publicações científicas
362 artigos
Último publicado: 2026
Medicamentos
6 registrados
DEXRAZOXANE, DOXORUBICIN HYDROCHLORIDE, VINCRISTINE SULFATE

Tem tratamento?

6 medicamentos registrados
Ver detalhes, fases e interações →
DEXRAZOXANEDOXORUBICIN HYDROCHLORIDEVINCRISTINE SULFATEDOXORUBICINVINCRISTINEETOPOSIDE

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adolescent
+ adult, childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C71.9
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫃
Digestivo
5 sintomas
🧠
Neurológico
4 sintomas
📏
Crescimento
3 sintomas
🩸
Sangue
2 sintomas
🦴
Ossos e articulações
1 sintomas
👁️
Olhos
1 sintomas

+ 20 sintomas em outras categorias

Características mais comuns

100%prev.
Neoplasia neuroectodérmica primitiva periférica
17%prev.
Dor em membro superior
Ocasional (29-5%)
17%prev.
Prurido
Ocasional (29-5%)
17%prev.
Pancreatite
Ocasional (29-5%)
17%prev.
Neoplasia cerebral
Ocasional (29-5%)
17%prev.
Neoplasia ovariana
Ocasional (29-5%)
37sintomas
Muito frequente (1)
Ocasional (30)
Muito raro (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 37 características clínicas mais associadas, ordenadas por frequência.

Neoplasia neuroectodérmica primitiva periféricaPeripheral primitive neuroectodermal neoplasm
Muito frequente100%
Dor em membro superiorUpper limb pain
Ocasional (29-5%)17%
PruridoPruritus
Ocasional (29-5%)17%
PancreatitePancreatitis
Ocasional (29-5%)17%
Neoplasia cerebralBrain neoplasm
Ocasional (29-5%)17%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico362PubMed
Últimos 10 anos113publicações
Pico201515 papers
Linha do tempo
2026Hoje · 2026🧪 1987Primeiro ensaio clínico📈 2015Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.

FLI1Friend leukemia integration 1 transcription factorPart of a fusion gene inAltamente restrito
FUNÇÃO

Sequence-specific transcriptional activator (PubMed:24100448, PubMed:26316623, PubMed:28255014). Recognizes the DNA sequence 5'-C[CA]GGAAGT-3'

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (1)
Transcriptional regulation of granulopoiesis
MECANISMO DE DOENÇA

Ewing sarcoma

A highly malignant, metastatic, primitive small round cell tumor of bone and soft tissue that affects children and adolescents. It belongs to the Ewing sarcoma family of tumors, a group of morphologically heterogeneous neoplasms that share the same cytogenetic features. They are considered neural tumors derived from cells of the neural crest. Ewing sarcoma represents the less differentiated form of the tumors.

EXPRESSÃO TECIDUAL(Ubíquo)
Baço
90.1 TPM
Pulmão
43.0 TPM
Tecido adiposo
38.9 TPM
Linfócitos
37.4 TPM
Sangue
33.9 TPM
OUTRAS DOENÇAS (7)
bleeding disorder, platelet-type, 21Jacobsen syndromeParis-Trousseau thrombocytopeniaisolated delta-storage pool disease
HGNC:3749UniProt:Q01543

Medicamentos e terapias

DEXRAZOXANEPhase 3

Mecanismo: DNA topoisomerase II inhibitor

DOXORUBICIN HYDROCHLORIDEPhase 3

Mecanismo: DNA topoisomerase II alpha inhibitor

VINCRISTINE SULFATEPhase 3

Mecanismo: Tubulin inhibitor

DOXORUBICINPhase 3

Mecanismo: DNA topoisomerase II alpha inhibitor

VINCRISTINEPhase 3

Mecanismo: Tubulin inhibitor

ETOPOSIDEPhase 3

Mecanismo: DNA topoisomerase II inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

108 variantes patogênicas registradas no ClinVar.

🧬 FLI1: NM_002017.5(FLI1):c.769C>T (p.Arg257Trp) ()
🧬 FLI1: GRCh38/hg38 11q24.1-25(chr11:123345328-135064169)x1 ()
🧬 FLI1: NM_002017.5(FLI1):c.969_973del (p.Arg324fs) ()
🧬 FLI1: NM_002017.5(FLI1):c.338dup (p.Pro114fs) ()
🧬 FLI1: NM_002017.5(FLI1):c.355A>C (p.Thr119Pro) ()
Ver todas no ClinVar

Vias biológicas (Reactome)

1 via biológica associada aos genes desta condição.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 39
2Fase 213
1Fase 12
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 6 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Tumor neuroectodérmico primitivo periférico

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

4 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

209 ensaios clínicos encontrados, 14 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
113 papers (10 anos)
#1

Neoadjuvant chemotherapy-enabled tumor conversion and surgical resection in pediatric primary pulmonary Ewing sarcoma: a case report.

Frontiers in oncology2026

Ewing sarcoma (EWS) is a highly aggressive malignant tumor that primarily affects the skeletal system in children and adolescents. Primary pulmonary Ewing sarcoma (PPEWS) is extremely rare, particularly in children, with very few cases reported in the literature. This report describes a 10-year-old girl who was admitted with an intermittent cough lasting over 7 months. Chest computed tomography (CT) revealed a mass in the right lower lung lobe. Bronchoscopic biopsy demonstrated a highly malignant undifferentiated small round cell tumor, with immunohistochemistry confirming EWS (positive for CD99, NKX2.2, and FLI-1). After 8 cycles of VDC/IE neoadjuvant chemotherapy, neoadjuvant therapy enabled tumor conversion from unresectable to resectable status. Postoperative pathology confirmed extraskeletal EWS/peripheral primitive neuroectodermal tumor (pPNET) following right middle and lower lobectomy, with good postoperative recovery. Following a multidisciplinary team (MDT) consensus, the patient initiated adjuvant chemotherapy utilizing the same VDC/IE regimen. As of the latest follow-up, she has successfully completed six cycles of adjuvant chemotherapy, and no clinical or radiological signs of recurrence have been observed. This case underscores the extreme rarity of PPEWS in children and the complexities of its diagnosis and treatment. Neoadjuvant chemotherapy can facilitate tumor conversion, while surgery plays a pivotal role in localized cases with incomplete chemotherapeutic response. Multidisciplinary management is essential for optimizing outcomes.

#2

Percutaneous Ultrasound-Guided Intratumoral Injection of Lauromacrogol and Pingyangmycin in the Treatment of Advanced pPNET in Children.

Journal of clinical ultrasound : JCU2025 Dec 03

To evaluate real-time ultrasound-guided intratumoral lauromacrogol + pingyangmycin for pediatric peripheral primitive neuroectodermal tumors (pPNET) refractory to chemotherapy. Five tumor lesions in three children from our institution were treated under real-time ultrasound guidance. Color Doppler and contrast-enhanced ultrasound assessed tumor hemodynamics and size pre- and post- injection. Pre-injection, all tumors showed rich blood flow (mixed arteriovenous signals). Post-injection contrast ultrasound revealed significantly reduced arterial-phase blood supply. At 4-week follow-up, all five tumors showed statistically significant volume reduction (p < 0.05) and reduced tumor-related pain. No adverse reactions such as bleeding and pain occurred during injection. Ultrasound-guided intratumoral lauromacrogol + pingyangmycin injection effectively reduces tumor blood supply, destroys local vasculature, and shrinks tumor volume in end-stage pediatric pPNET. It is a safe and effective local palliative treatment.

#3

Multimodal Treatment of a Peripheral Primitive Neuroectodermal Tumor Originating From the Thoracic Cavity in a Dog.

Journal of veterinary internal medicine2025

Peripheral primitive neuroectodermal tumor (pPNET) is a very rare, highly malignant tumor encountered in young dogs, with only four necropsy cases in the veterinary literature. A 1.5-year-old male intact French Bulldog presented for evaluation of progressive left forelimb lameness and ipsilateral Horner's syndrome. Whole body computed tomography identified a 12 cm mediastinal mass with extension into the vertebral canal and multifocal metastatic lesions. Histological and immunohistochemical results were consistent with a pPNET. Chemotherapy, radiation therapy, immunotherapy, and targeted therapy were administered. Precision-based tumor testing, including DNA sequencing and chemosensitivity assays, were performed to guide systemic treatment recommendations. A partial remission was observed 3.5 months from presentation based on imaging and improved clinical status. After 7 months of treatment, diffuse metastatic disease, including intracranial spread, was observed and the dog was euthanized. This PDQ cancer information summary has current information about the treatment of childhood Ewing sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change. The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

#4

Primary ovarian peripheral primitive neuroectodermal tumor presented with breast metastasis; Case report.

Rare tumors2025

Ewing sarcoma family tumors (ESFT) pose diagnostic challenges, which largely depend on the primary site of involvement and tumor stage. Despite advancements in treatment, metastatic ESFTs remain associated with poor outcomes. This case describes a 21-year-old woman who, in July 2022, presented with a left breast mass identified through ultrasound and CT scan, along with abdominal distention. A biopsy of the breast mass confirmed metastatic extraskeletal Ewing sarcoma. Further imaging revealed an ovarian mass, with subsequent biopsy confirming ovarian origin as extraskeletal Ewing sarcoma. The breast mass was identified as metastatic based on imaging features, including irregular margins and CT scan confirmation of widespread metastasis. Histopathology and immunohistochemistry confirmed Ewing sarcoma, consistent with the ovarian mass pathology that was the primary site. She underwent 15 cycles of VDC/IE chemotherapy ((vincristine, doxorubicin, and cyclophosphamide) for 2 days and 5 days IE (ifosfamide etoposide)), resulting in tumor cytoreduction. However, in less than 2 years, she developed metastases to the dura, spine, and bone, with optic nerve involvement. Despite treatment with radiotherapy and two cycles of high-dose Ifosfamide chemotherapy, her condition deteriorated, and she passed away in April 2024. This case underscores the complexity of managing metastatic ESFTs. Further research is needed to improve outcomes and establish treatment protocols for this malignancy.

#5

Challenges in Diagnosing Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report.

Current medical imaging2025

Primary intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) is exceedingly rare and easy to misdiagnose. We present a case involving a 23-year-old male who presented with headaches and vomiting. The preoperative brain imaging revealed an irregular mass in the left parietal lobe, initially misdiagnosed as meningioma. However, the surgical specimen was ultimately diagnosed as primary intracranial EWS/pPNET. The patient underwent a total tumor resection, followed by adjuvant chemotherapy and radiotherapy. No recurrence or distant metastasis was observed 18 months after the surgery. When the imaging features of young patients' lesions are solid, aggressive, and unevenly enhanced masses, physicians should be aware of the possibility of primary intracranial EWS/pPNET, and if possible, Gross Total Resection (GTR) and intensive chemotherapy and radiotherapy are recommended. This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood Ewing sarcoma and undifferentiated small round cell sarcomas of bone and soft tissue. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions. This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Publicações recentes

Ver todas no PubMed

📚 EuropePMC199 artigos no totalmostrando 111

2026

Neoadjuvant chemotherapy-enabled tumor conversion and surgical resection in pediatric primary pulmonary Ewing sarcoma: a case report.

Frontiers in oncology
2025

Percutaneous Ultrasound-Guided Intratumoral Injection of Lauromacrogol and Pingyangmycin in the Treatment of Advanced pPNET in Children.

Journal of clinical ultrasound : JCU
2025

Multimodal Treatment of a Peripheral Primitive Neuroectodermal Tumor Originating From the Thoracic Cavity in a Dog.

Journal of veterinary internal medicine
2025

Primary ovarian peripheral primitive neuroectodermal tumor presented with breast metastasis; Case report.

Rare tumors
2025

Challenges in Diagnosing Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report.

Current medical imaging
2024

Axillary lesion in a young adult ends up to a peculiar diagnosis: primitive neuro-ectodermal tumor (PNET) of ulnar nerve: a rare case report.

Annals of medicine and surgery (2012)
2024

Concurrent delayed recurrence of peripheral primitive neuroectodermal tumors in orbital and sellar/suprasellar regions in an older adult.

European journal of ophthalmology
2024

Primary Ewing's sarcoma of the intestine: case report and literature review.

Frontiers in oncology
2024

Askin's Tumor in the Chest Wall-a Rare Clinical Entity and Review of Literature.

Indian journal of surgical oncology
2024

Ewing sarcoma with intra thoracic and multiple extra thoracic metastases in a young adult male: A case report.

International journal of surgery case reports
2024

Primary intracranial peripheral primitive neuroectodermal tumor: lessons from an exceptionally rare neoplasm. Illustrative case.

Journal of neurosurgery. Case lessons
2024

Extraskeletal Ewing's sarcoma of supraglottis: A rare case report.

Oral oncology
2024

Clinical and histopathological spectrum of cranial small round cell tumors: An experience from a tertiary care center.

Journal of cancer research and therapeutics
2023

Primary Intracranial Ewing Sarcoma With EWSR1-FLI1 Gene Translocation Mimicking a Meningioma and a Multidisciplinary Therapeutic Approach: A Case Report and Systematic Review of Literatures.

Brain tumor research and treatment
2023

Detection of various fusion genes by one-step RT-PCR and the association with clinicopathological features in 242 cases of soft tissue tumor.

Frontiers in cell and developmental biology
2023

Askin's Tumor a Rare Clinical Entity of Thoracopulmonary Region Cresenting as a Case of Paraparesis: Findings on 18F-FDG PET/CT.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2023

Peripheral Primitive Neuroectodermal Tumor: A Rare Case in Pediatrics.

Cureus
2023

Askin Tumor - Presenting as a Case of Paraparesis: A Rare Clinical Entity. Case Report and Findings on 18F-Fluorodeoxyglucose-Positron Emission Tomography/Computed Tomography.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2023

Peripheral primitive neuroectodermal tumor of the orbit in Graves' ophthalmopathy - A rare presentation.

Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological Society
2023

Intracranial peripheral primitive neuroectodermal tumor presenting as neurosurgical emergency: A report of two cases.

Journal of neurosciences in rural practice
2022

Peripheral primitive neuroectodermal tumor orbit in an elderly woman.

Oman journal of ophthalmology
2022

Primary intracranial extraosseous Ewing's sarcoma of the skull base in an elderly adult: illustrative case.

Journal of neurosurgery. Case lessons
2022

The prognostic factors of Ewing sarcoma/peripheral primitive neuroectodermal tumor: A retrospective analysis of 67 patients at a single center.

Medicine
2022

[Peripheral primitive neuroectodermal tumor in the oropharynx of a child: a case report].

Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery
2022

Primary Ewing's sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) of the tongue in a child: A case report.

Oral oncology
2022

Peripheral primitive neuroectodermal tumor: a case report.

Journal of medical case reports
2022

Primary intracranial peripheral primitive neuroectodermal tumor in an adult patient with aphasia: A rare case report.

Clinical case reports
2021

Primary intradural extramedullary extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumor (PIEES/PNET) of the thoracolumbar spine: A case report and literature review.

Open medicine (Warsaw, Poland)
2021

Primary Mediastinal Ewing sarcoma presenting as a massive lung lesion with a mediastinal shift.

Clinical case reports
2021

Primary Spinal Extradural Extraosseous Primitive Neuroectodermal Tumor/Ewing's Sarcoma: A Critical Analysis and Review.

Asian journal of neurosurgery
2021

Primitive neuroectodermal tumor of the pericardium: a case report and literature review.

BMC cardiovascular disorders
2021

Prostate Ewing Sarcoma/PNET: A case of long survival in a highly aggressive malignancy.

Urology
2021

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

Journal of Indian Association of Pediatric Surgeons
2021

Radiological and pathological diagnosis of an incidental Askin tumor.

Radiology case reports
2020

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

Asian journal of neurosurgery
2021

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

Journal of pediatric hematology/oncology
2020

Primary pulmonary Ewing sarcoma/peripheral primitive neuroectodermal tumor.

Proceedings (Baylor University. Medical Center)
2020

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

Frontiers in oncology
2020

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor.

Scientific reports
2020

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

The journal of obstetrics and gynaecology research
2021

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

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Intracranial Ewing Sarcoma - A case report.

Surgical neurology international
2021

A case report of neonatal orbital peripheral primitive neuroectodermal tumor and literature review.

European journal of ophthalmology
2020

Primary Peripheral Primitive Neuroectodermal Tumor of the Prostate on 18F-DCFPyL PET/CT.

Clinical nuclear medicine
2019

Ewing's sarcoma/peripheral primitive neuroectodermal tumor with extraskeletal myxoid chondrosarcoma-like areas: a case report.

International journal of clinical and experimental pathology
2019

Ewing's Sarcoma of the Sinonasal Tract: A Report of Two Challenging Cases.

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
2019

Retroperitoneal extraosseous peripheral primitive neuroectodermal tumor in a Formosan serow: case report and literature review.

Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
2019

Primary Intracranial Dural-Based Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor with Extracranial Extension: An Uncommon Case.

Asian journal of neurosurgery
2019

Congenital cutaneous peripheral primitive neuroectodermal tumor/Ewing sarcoma: A rare case in a male neonate.

Pediatric blood &amp; cancer
2019

Extraskeletal Ewing's sarcoma/peripheral primitive neuroectodermal tumor of the small bowel presenting with gastrointestinal perforation.

Clinical and experimental gastroenterology
2019

Bone marrow examination in patients with Ewing sarcoma/peripheral primitive neuroectodermal tumor without metastasis based on 18F-fluorodeoxyglucose positron emission tomography/computed tomography.

Medical oncology (Northwood, London, England)
2019

Ewing's sarcoma of the sinonasal tract with orbital extension: a rare case report and literature review.

Journal of surgical case reports
2018

Peripheral Primitive Neuroectodermal Tumor of the Ovary: The Report of Two Rare Cases.

Iranian journal of pathology
2020

Primary Intra-abdominal Ewing's Sarcoma in Adults: A Multimodality Imaging Spectrum.

Current problems in diagnostic radiology
2019

Peripheral primitive neuroectodermal tumor associated with paraneoplastic Cushing's syndrome: The rare case.

Annals of medicine and surgery (2012)
2018

Ultrasensitive quantification of tumor mRNAs in extracellular vesicles with an integrated microfluidic digital analysis chip.

Lab on a chip
2018

A Rare Case of Dumbbell-shaped Primary Intraspinal Peripheral Primitive Neuroectodermal Tumor Involving Thoracic Spinal Epidural Space.

Asian journal of neurosurgery
2018

Scrotal Peripheral Primitive Neuroectodermal Tumor.

Current urology
2019

Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumors in Bronchus.

The American journal of the medical sciences
2018

From Ataxia to Diagnosis of Askin Tumor - a Case Report.

Acta medica (Hradec Kralove)
2018

Intracranial Peripheral Primitive Neuroectodermal Tumor Arising from the Clivus with Intracranial Metastasis in an Elderly Woman: Case Report and Review of the Literature.

World neurosurgery
2019

Primary Ewing Family of Tumor Arising in the Ovary: A Case Report.

International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists
2018

Peripheral primitive neuroendocrine tumor of the chest wall-A case report with pathological correlation.

Radiology case reports
2018

Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor in the Adrenal Gland of a Child.

Iranian journal of kidney diseases
2018

Peripheral primitive neuroectodermal tumor of cauda equina: A report and review of literature.

Neurology India
2018

Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.

World neurosurgery
2018

Extraosseous Primary Intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor: Series of Seven Cases and Review of Literature.

Asian journal of neurosurgery
2018

Primary primitive neuroectodermal tumor of the kidney.

Indian journal of pathology &amp; microbiology
2018

Ewing sarcoma of the adrenal gland: a case report and review of the literature.

Journal of medical case reports
2019

Spinal Peripheral Primitive Neuroectodermal Tumors: A Radiological Analysis of Ten Cases.

Turkish neurosurgery
2017

Adrenal mass of unusual etiology: Ewing sarcoma in a young man.

Radiology case reports
2018

[Paranasal peripheral primitive neuroectodermal tumor: a case report].

Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery
2017

Antiangiogenic therapy for primitive neuroectodermal tumor with thalidomide: A case report and review of literature.

Medicine
2017

[The treatment and prognosis of peripheral primitive neuroectodermal tumor].

Zhonghua zhong liu za zhi [Chinese journal of oncology]
2017

Skull base bony lesions: Management nuances; a retrospective analysis from a Tertiary Care Centre.

Asian journal of neurosurgery
2017

Primary intracranial dural-based Ewing sarcoma/peripheral primitive neuroectodermal tumor mimicking a meningioma: A rare tumor with review of literature.

Asian journal of neurosurgery
2017

Systemic Therapy Outcomes in Adult Patients with Ewing Sarcoma Family of Tumors.

Case reports in oncology
2017

Primary primitive neuroectodermal tumor of the cervix: A report of two cases and review of the literature.

Molecular and clinical oncology
2017

Cytogenetically confirmed primary Ewing's sarcoma of the pancreas.

BMJ case reports
2017

Ewing's Sarcoma Family Tumors in the Jaws: Case Report, Immunohistochemical Analysis and Literature Review.

In vivo (Athens, Greece)
2017

Triple-phase 99mTc-3P-RGD2 imaging of peripheral primitive neuroectodermal tumor in the hip muscle group with bone metastasis.

Molecular and clinical oncology
2017

Isolated cardiac peripheral primitive neuroectodermal tumor: A case report.

Cancer biology &amp; therapy
2017

Computed Tomography and Magnetic Resonance Imaging Characteristics of Peripheral Primitive Neuroectodermal Tumor: A Retrospective Analysis of 16 Cases.

Journal of computer assisted tomography
2016

Huge peripheral primitive neuroectodermal tumor of the small bowel mesentery at nonage: A case report and review of the literature.

World journal of clinical cases
2016

Peripheral primitive neuroectodermal tumors of the spine: a case report and review of the literature.

BMC research notes
2017

Meningeal Ewing Sarcoma/Peripheral PNET: Clinicopathological, Immunohistochemical and FISH study of four cases.

Neuropathology : official journal of the Japanese Society of Neuropathology
2016

Primary Brachial Plexus Tumors: Clinical Experiences of 143 Cases.

Clinical neurology and neurosurgery
2016

Peripheral Primitive Neuroectodermal Tumor of Breast.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
2016

Synthetic treatment of intracranial peripheral primitive neuroectodermal tumor with multiple metastasis: a case report.

OncoTargets and therapy
2016

Cervicomedullary intramedullary peripheral primitive neuroectodermal tumor with intratumoral bleed: Report of one case and review of literature.

Journal of craniovertebral junction &amp; spine
2016

A peripheral primitive neuroectodermal tumor in the larynx: A case report and literature review.

Oncology letters
2016

Peripheral Primitive Neuroectodermal Tumor and Neurofibromatosis Type 1 in an Adolescent Male.

Pediatric blood &amp; cancer
2015

[Peripheral Primitive Neuroectodermal Tumor of the Small Bowel Mesentery - Report of a Case].

Srpski arhiv za celokupno lekarstvo
2016

A unique cause of neck pain: peripheral primitive neuroectodermal tumor.

The spine journal : official journal of the North American Spine Society
2015

Peripheral Primitive Neuroectodermal Tumor (pPNET) of the Parotid: Report of a Rare Case.

Archives of Iranian medicine
2015

Efficacy of trabectedin in advanced soft tissue sarcoma: beyond lipo- and leiomyosarcoma.

Drug design, development and therapy
2016

Sinonasal small round blue cell tumors: An approach to diagnosis.

Seminars in diagnostic pathology
2015

Incidences of Primary Soft Tissue Sarcoma Diagnosed on Extremities and Trunk Wall: A Population-Based Study in Taiwan.

Medicine
2015

Primitive neuroectodermal tumor of the prostate: Case report from China.

Journal of cancer research and therapeutics
2015

Renal primitive neuroectodermal tumor as a second malignancy after chemotherapy and radiation for Non-Hodgkin's Lymphoma--treatment-related or just poor old bad luck?: A case report.

Journal of cancer research and therapeutics
2015

A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature.

Surgical case reports
2015

Cyclin D1 and Ewing's sarcoma/PNET: A microarray analysis.

Acta histochemica
2015

Radiological and clinical findings of osseous peripheral primitive neuroectodermal tumors.

Oncology letters
2015

Application of electron microscopic analysis and fluorescent in situ hybridization technique for the successful diagnosis of extraskeletal Ewing's sarcoma.

The Journal of dermatology
2015

Mandibular peripheral primitive neuroectodermal tumor: a rare case report with review of literature.

Journal of maxillofacial and oral surgery
2015

Peripheral primitive neuroectodermal tumor of chest wall in young adult.

Radiologia brasileira
2015

Cyclin D1 is a useful marker for soft tissue Ewing's sarcoma/peripheral Primitive Neuroectodermal Tumor in children and adolescents: A comparative immunohistochemical study with rhabdomyosarcoma.

Acta histochemica
2015

Primary intracranial Ewing's sarcoma with unusual features.

International journal of clinical and experimental pathology
2014

Peripheral primitive neuroectodermal tumor of seminal vesicles: is there a role for relatively aggressive treatment modalities?

Archivio italiano di urologia, andrologia : organo ufficiale [di] Societa italiana di ecografia urologica e nefrologica
2015

Pancreatic peripheral primitive neuroectodermal tumor diagnosed by endoscopic ultrasound.

Endoscopy
2014

Peripheral primitive neuroectodermal tumor: dynamic CT, MRI and clinicopathological characteristics--analysis of 36 cases and review of the literature.

Oncotarget
Ver todos os 199 no EuropePMC

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Comunidades

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Neoadjuvant chemotherapy-enabled tumor conversion and surgical resection in pediatric primary pulmonary Ewing sarcoma: a case report.
    Frontiers in oncology· 2026· PMID 41777644mais citado
  2. Percutaneous Ultrasound-Guided Intratumoral Injection of Lauromacrogol and Pingyangmycin in the Treatment of Advanced pPNET in Children.
    Journal of clinical ultrasound : JCU· 2025· PMID 41339291mais citado
  3. Multimodal Treatment of a Peripheral Primitive Neuroectodermal Tumor Originating From the Thoracic Cavity in a Dog.
    Journal of veterinary internal medicine· 2025· PMID 40095239mais citado
  4. Primary ovarian peripheral primitive neuroectodermal tumor presented with breast metastasis; Case report.
    Rare tumors· 2025· PMID 39790869mais citado
  5. Challenges in Diagnosing Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report.
    Current medical imaging· 2025· PMID 39757659mais citado
  6. Correction to "Multimodal Treatment of a Peripheral Primitive Neuroectodermal Tumor Originating From the Thoracic Cavity in a Dog".
    J Vet Intern Med· 2025· PMID 40387184recente
  7. Ewing Sarcoma Treatment (PDQ®): Patient Version.
    · 2002· PMID 26389350recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:370348(Orphanet)
  2. MONDO:0018271(MONDO)
  3. GARD:17601(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q56014124(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Tumor neuroectodérmico primitivo periférico
Compêndio · Raras BR

Tumor neuroectodérmico primitivo periférico

ORPHA:370348 · MONDO:0018271
Prevalência
Unknown
Herança
Not applicable
CID-10
C71.9 · Neoplasia maligna do encéfalo, não especificado
Ensaios
14 ativos
Medicamentos
6 registrados
Início
Adolescent, Adult, Childhood
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0684337
EuropePMC
Wikidata
Papers 10a
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