Raras
Buscar doenças, sintomas, genes...
Tumor não epitelial maligno do ovário
ORPHA:398940DOENÇA RARA

Câncer (português brasileiro) ou cancro (português europeu), também conhecido como neoplasia maligna é um grupo de doenças que envolvem o crescimento celular anormal, com potencial para invadir e espalhar-se para outras partes do corpo, além do local original. Há mais de cem diferentes cânceres conhecidos que afetam os seres humanos, mas nem todos os tumores são cancerosos (malignos); tumores benignos não se espalham pelo corpo. Sinais e sintomas possíveis incluem surgimento de uma massa cancerígena, sangramento anormal, tosse prolongada, perda de peso inexplicável, mudança nas funções intestinais, entre outros. Apesar de estes sintomas poderem indicar câncer, eles também podem ocorrer devido a outras doenças.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Tumor ovariano raro, geralmente de início na idade adulta, com subtipos como disgerminoma e gonadoblastoma. Pode cursar com hirsutismo, dor abdominal e alterações hormonais, como aumento de testosterona, associado a disgenesia gonadal.

Medicamentos
6 registrados
ETOPOSIDE, PACLITAXEL, LEUPROLIDE ACETATE

Tem tratamento?

6 medicamentos registrados
Ver detalhes, fases e interações →
ETOPOSIDEPACLITAXELLEUPROLIDE ACETATELEUPROLIDEDAROLUTAMIDEEXEMESTANE

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.43
Europe
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
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

📏
Crescimento
3 sintomas
🫃
Digestivo
2 sintomas
🧬
Pele e cabelo
1 sintomas
👂
Ouvidos
1 sintomas

+ 7 sintomas em outras categorias

Características mais comuns

Início na idade adulta
Gonadoblastoma
Anormalidade do metabolismo/homeostase
Dor abdominal
Disgerminoma
Hirsutismo
14sintomas
Sem dados (14)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 14 características clínicas mais associadas, ordenadas por frequência.

Início na idade adultaAdult onset
Gonadoblastoma
Anormalidade do metabolismo/homeostaseAbnormality of metabolism/homeostasis
Dor abdominalAbdominal pain
DisgerminomaDysgerminoma

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa11
Últimos 10 anos97publicações
Pico202514 papers
Linha do tempo
20202015Hoje · 2026🧪 1992Primeiro ensaio clínico📈 2025Ano 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

3 genes identificados com associação a esta condição.

DICER1Endoribonuclease DicerCandidate gene tested inAltamente restrito
FUNÇÃO

Double-stranded RNA (dsRNA) endoribonuclease playing a central role in short dsRNA-mediated post-transcriptional gene silencing. Cleaves naturally occurring long dsRNAs and short hairpin pre-microRNAs (miRNA) into fragments of twenty-one to twenty-three nucleotides with 3' overhang of two nucleotides, producing respectively short interfering RNAs (siRNA) and mature microRNAs. SiRNAs and miRNAs serve as guide to direct the RNA-induced silencing complex (RISC) to complementary RNAs to degrade them

LOCALIZAÇÃO

CytoplasmCytoplasm, perinuclear region

VIAS BIOLÓGICAS (5)
MicroRNA (miRNA) biogenesisSmall interfering RNA (siRNA) biogenesistRNA-derived small RNA (tsRNA or tRNA-related fragment, tRF) biogenesisM-decay: degradation of maternal mRNAs by maternally stored factorsRegulation of MITF-M-dependent genes involved in apoptosis
MECANISMO DE DOENÇA

Pleuropulmonary blastoma

A rare pediatric intrathoracic neoplasm. The tumor arises from the lung, pleura, or both, and appears to be purely mesenchymal in phenotype. It lacks malignant epithelial elements, a feature that distinguishes it from the classic adult-type pulmonary blastoma. It arises during fetal lung development and is often part of an inherited cancer syndrome. The tumor contain both epithelial and mesenchymal cells. Early in tumorigenesis, cysts form in lung airspaces, and these cysts are lined with benign-appearing epithelium. Mesenchymal cells susceptible to malignant transformation reside within the cyst walls and form a dense layer beneath the epithelial lining. In a subset of patients, overgrowth of the mesenchymal cells produces a sarcoma, a transition that is associated with a poorer prognosis. Some patients have multilocular cystic nephroma, a benign kidney tumor.

EXPRESSÃO TECIDUAL(Ubíquo)
Nervo tibial
46.1 TPM
Tireoide
31.6 TPM
Cervix Endocervix
31.4 TPM
Útero
31.2 TPM
Ovário
30.2 TPM
OUTRAS DOENÇAS (10)
pleuropulmonary blastomaglobal developmental delay - lung cysts - overgrowth - Wilms tumor syndromegoiter, multinodular 1, with or without Sertoli-Leydig cell tumorsrhabdomyosarcoma, embryonal, 2
HGNC:17098UniProt:Q9UPY3
FOXL2Forkhead box protein L2Candidate gene tested inTolerante
FUNÇÃO

Transcriptional regulator. Critical factor essential for ovary differentiation and maintenance, and repression of the genetic program for somatic testis determination. Prevents trans-differentiation of ovary to testis through transcriptional repression of the Sertoli cell-promoting gene SOX9 (By similarity). Has apoptotic activity in ovarian cells. Suppresses ESR1-mediated transcription of PTGS2/COX2 stimulated by tamoxifen (By similarity). Is a regulator of CYP19 expression (By similarity). Par

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (1)
SUMOylation of transcription factors
MECANISMO DE DOENÇA

Blepharophimosis, ptosis, and epicanthus inversus syndrome

A disorder characterized by eyelid dysplasia, small palpebral fissures, drooping eyelids and a skin fold curving in the mediolateral direction, inferior to the inner canthus. In type I BPSE (BPES1) eyelid abnormalities are associated with female infertility. Affected females show an ovarian deficit due to primary amenorrhea or to premature ovarian failure (POF). In type II BPSE (BPES2) affected individuals show only the eyelid defects.

EXPRESSÃO TECIDUAL(Tecido-específico)
Ovário
102.0 TPM
Cervix Endocervix
31.4 TPM
Cervix Ectocervix
26.6 TPM
Fallopian Tube
25.6 TPM
Pituitária
19.0 TPM
OUTRAS DOENÇAS (6)
premature ovarian failure 3blepharophimosis, ptosis, and epicanthus inversus syndromemaligant granulosa cell tumor of ovaryblepharophimosis-ptosis-epicanthus inversus syndrome type 1
HGNC:1092UniProt:P58012
BMP15Bone morphogenetic protein 15Candidate gene tested inTolerante
FUNÇÃO

May be involved in follicular development. Oocyte-specific growth/differentiation factor that stimulates folliculogenesis and granulosa cell (GC) growth

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (2)
Post-translational protein phosphorylationRegulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
MECANISMO DE DOENÇA

Ovarian dysgenesis 2

A disorder characterized by lack of spontaneous pubertal development, primary amenorrhea, uterine hypoplasia, and hypergonadotropic hypogonadism as a result of streak gonads.

OUTRAS DOENÇAS (3)
ovarian dysgenesis 2immature ovarian teratoma46 XX gonadal dysgenesis
HGNC:1068UniProt:O95972

Medicamentos e terapias

ETOPOSIDEPhase 3

Mecanismo: DNA topoisomerase II inhibitor

PACLITAXELPhase 3

Mecanismo: Tubulin inhibitor

LEUPROLIDE ACETATEPhase 2

Mecanismo: Gonadotropin-releasing hormone receptor agonist

LEUPROLIDEPhase 2

Mecanismo: Gonadotropin-releasing hormone receptor agonist

DAROLUTAMIDEPhase 2

Mecanismo: Androgen Receptor antagonist

EXEMESTANEPhase 2

Mecanismo: Cytochrome P450 19A1 inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

1,612 variantes patogênicas registradas no ClinVar.

🧬 BMP15: Single allele ()
🧬 BMP15: GRCh38/hg38 Xp11.22(chrX:50770594-51083187)x2 ()
🧬 BMP15: GRCh38/hg38 Xp22.33-q28(chrX:2757837-156030895)x2 ()
🧬 BMP15: GRCh37/hg19 Xp22.33-11.1(chrX:168546-58364114) ()
🧬 BMP15: GRCh37/hg19 Xp22.33-11.21(chrX:168546-55653170) ()
Ver todas no ClinVar

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 33
2Fase 26
1Fase 12
Medicamentos catalogadosEnsaios clínicos· 6 medicamentos · 5 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Tumor não epitelial maligno do ovário

🗺️

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

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

0 ensaios clínicos encontrados.

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

Timeline de publicações
0 papers (10 anos)
#1

Correlation between ultrasound and gross pathology examination of malignant ovarian tumors: a pictorial review for pattern recognition.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society2026 Feb 24

This narrative review integrates recent literature and representative, non-consecutive, cases from the International Ovarian Tumor Analysis (IOTA) database (University Hospitals Leuven) to illustrate macroscopic-sonographic correlations in epithelial and non-epithelial ovarian malignancies. The main objective was to explore the correlation between ultrasound and gross pathologic features of malignant ovarian tumors, highlighting histotype-specific patterns using IOTA terminology. All patients provided informed consent. Key tumor features (solid components, necrosis, septations, exophytic growth, and papillary projections) were evaluated using standardized IOTA terms. Ultrasound images were compared with matched gross pathology photographs. The integration of matched ultrasound and pathology images may represent an effective educational tool, improving diagnostic accuracy among sonographers. Knowledge of the ultrasound features more frequently reported for specific histotypes in the existing literature, interpreted using IOTA terminology and supported by gross-ultrasound image correlation, may aid the differential diagnosis of borderline and primary invasive ovarian tumors and support subsequent clinical management. This approach may inform surgical decision-making and guide pre-operative planning. A non-validated diagnostic flowchart is proposed to assist in the differential diagnosis of malignant ovarian tumors.

#2

Combination of transvaginal ultrasound with cervical cancer screening contributes to early detection of ovarian cancer: Clinical trial.

International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics2026 Apr

Early detection of ovarian cancer at stage I is important to improve patients' prognosis. The goal of this study was to examine if transvaginal ultrasound (TVU) performed at the same time as cervical cancer screening can facilitate early detection of ovarian cancer. From 2014 to 2022, 483 269 women underwent TVU examinations during cervical cancer screening. The criteria for abnormal findings on TVU were ovarian enlargement ≥3 cm in long diameter (≥2 cm in postmenopausal women). Of the 483 269 women who underwent TVU, 3294 (0.68%) were selected for detailed examination. Of these women, 550 underwent surgery and 80 cases of ovarian cancer were found (positive predictive value: 2.43%). Pathologic review in 76 of these cases showed 70 cases of epithelial ovarian cancer (type I: 54 [77.1%], Type II: 16 [22.9%]) and six cases of non-epithelial malignant tumors. Clinical staging analysis showed that 81.6% (62 cases) were at stage I. Significantly more type I than type II tumors were detected at stage I (87.3% (n = 46) vs. 56.3%, P = 0.0068 (n = 9)). Notably, 95.7% (22/23) of clear cell carcinoma cases were detected at stage I. The high rate of early detection of type I ovarian cancer might be due to its slow progression. In Asia, where type I is common, the benefits of screening for ovarian cancer are particularly great. However, screening with TVU has generally been considered to have little benefit. The results of this study suggest a need for reassessment of this view.

#3

Cancer Antigen-125 and Carcinoembryonic Antigen in Determining Malignancy Risk in Epithelial Ovarian Tumor: An Observational Study.

JNMA; journal of the Nepal Medical Association2025 Sep

Epithelial ovarian cancers make up about 90% of ovarian malignancies and are often diagnosed late due to its vague symptoms. Cancer Antigen-125 (CA-125) and Carcinoembryonic Antigen (CEA), play a pivotal role in differentiating benign from malignant ovarian tumors and aid in assessing malignancy risk. The study was hospital-based cross-sectional study conducted at Paropakar Maternity and Women's Hospital among 53 women diagnosed with ovarian tumors scheduled for surgery. Data were collected between July to December 2023. Non-epithelial tumors were excluded after obtaining final histopathology report. Preoperative CA-125 and CEA levels were correlated with epithelial ovarian tumors. Data analysis was performed using Statistical Package for Social Sciences software version 29. Among 53 cases, 13 (33.30%) cases belonged to 40-49 years age group, while 5 (45.40%) cases of malignant tumors were in the 50-59 years age group. There were 5 (45.50%) cases of malignant tumors in women with parity two, while 11 (28.20%) cases of benign tumors in women with parity two. There were 39 (73.60%) benign cases, 3 (5.65%) borderline, and 11 (20.75%) malignant tumors. There were 36 (67.90%) serous tumors and 15 (28.30%) cases were mucinous tumors. Cancer Antigen-125 (>35 IU/mL) had high sensitivity 90.90%) and specificity (87.20%) for malignancy, while Carcinoembryonic Antigen (>3 ng/mL) had sensitivity 36.40% and high specificity 89.70% for malignancy. Cancer Antigen-125 showed high sensitivity and specificity in distinguishing malignant from benign epithelial ovarian tumors. Carcinoembryonic Antigen, while less sensitive, provided high specificity.

#4

Targeting Ovarian Neoplasms: Subtypes and Therapeutic Options.

Medicina (Kaunas, Lithuania)2025 Dec 18

The ovary, as the primary organ responsible for reproduction and new life, plays a central role in female development, maturation, and health. Neoplasms arising from the ovary and its associated tissues exhibit substantial heterogeneity in their histopathological and molecular profiles, many of which remain poorly understood. This review aims to summarize recent advances in the understanding of genetic alterations underlying ovarian neoplasms and to explore therapeutic strategies informed by molecular biomarkers and tumor microenvironmental factors. A comprehensive literature search was performed, focusing on genomic alterations, biomarker-guided therapies, and tumor microenvironmental modulation in ovarian cancers. Emphasis was placed on studies addressing lipid mediator pathways and their roles in immune regulation and therapeutic response. Based on diagnostic classifications, recurrent alterations in TP53, MYC, PIK3CA, and KRAS are consistently observed across epithelial and germ cell ovarian tumors, whereas non-epithelial subtypes such as sex cord-stromal tumors (SCSTs) and small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT), are predominantly associated with ARID1A and SMARCA4 mutations, respectively. These findings highlight distinct pathogenic mechanisms linked to specific genetic alterations and reveal potential therapeutic vulnerabilities. Moreover, lipid metabolism has been closely implicated in immune surveillance through STING signaling cascades within innate immune cells, suggesting that lipid mediators and their associated genes may represent promising therapeutic targets in ovarian cancers (OCs). Targeting lipid mediators could be particularly effective in relapsed OCs, as modulating innate immune cells within the tumor microenvironment (TME) may enhance immune surveillance and improve antitumor responses. Integrating genetic and microenvironmental insights offers a promising direction for developing more effective and personalized therapeutic strategies in OC.

#5

Long-term quality of life in non-epithelial ovarian cancer survivors: TMRG-GINECO-Vivrovaire rare tumors case-control study.

BMC medicine2025 Dec 12

Patients treated for rare ovarian germ cell tumors (GCT) and sex cord stromal tumors (SCST) often experience long-term survival. Treatments include surgery with or without chemotherapy (CT), which can cause late side effects that negatively impact quality of life (QoL). This study assessed long-term QoL among GCT and SCST survivors compared to age-matched healthy women (HW), focusing on patients treated with CT. Cancer-free GCT and SCST survivors (≥ 2 years post-treatment) were recruited from the INCa French Network TMRG. HW were recruited via the "Seintinelles" platform. Participants completed validated questionnaires assessing QoL (FACT-G/FACT-O), fatigue (MFI), anxiety/depression (HADS), insomnia (ISI), neurotoxicity (FACT/GOG-NTX), cognition (FACT-COG), and sexuality (FACT-O OCS). Clinically relevant differences were defined as ≥ 5% between groups. A total of 144 survivors (99 GCT and 45 SCST) were included and compared with 284 matched HW (median age: 35.2 years). The median time from the end of treatment was 6 years [Q1 3.5-Q3 9.3]. At inclusion, 42% of patients were menopausal compared to 17% of HW (p < 0.001), with reduced sexual QoL. Survivors reported more cognitive impairment (32% vs. 21%, p = 0.025) and severe neurotoxicity (23% vs. 8%, p < 0.001). QoL, fatigue, anxiety/depression, and insomnia scores were similar for patients and HW. With a median of 6 years after CT, most GCT and SCST survivors reported similar overall QoL compared to HW but faced premature menopause, reduced sexual QoL, and persistent cognitive and neurotoxic effects. NCT03418844.

Publicações recentes

Ver todas no PubMed

📚 EuropePMCmostrando 97

2026

Correlation between ultrasound and gross pathology examination of malignant ovarian tumors: a pictorial review for pattern recognition.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2025

Cancer Antigen-125 and Carcinoembryonic Antigen in Determining Malignancy Risk in Epithelial Ovarian Tumor: An Observational Study.

JNMA; journal of the Nepal Medical Association
2025

Targeting Ovarian Neoplasms: Subtypes and Therapeutic Options.

Medicina (Kaunas, Lithuania)
2025

Long-term quality of life in non-epithelial ovarian cancer survivors: TMRG-GINECO-Vivrovaire rare tumors case-control study.

BMC medicine
2026

Combination of transvaginal ultrasound with cervical cancer screening contributes to early detection of ovarian cancer: Clinical trial.

International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics
2025

"Is the incidence of non-epithelial ovarian tumors on the rise? insights from a Tunisian tertiary center".

Frontiers in oncology
2025

Primary treatment and survival in malignant ovarian germ cell tumors: A nationwide population-based Swedish Gynecologic Cancer Group (SweGCG) study.

Gynecologic oncology
2025

An overview of malignant ovarian tumors in children.

Seminars in pediatric surgery
2025

Polycystic ovary syndrome and the risk of ovarian tumours.

Gynecologic oncology
2025

Incidence and survival of rare female genital tract cancers in Europe: The EUROCARE-6 study.

European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
2025

Comprehensive analysis of calcification frequency and patterns in ovarian tumours using non-contrast CT.

Japanese journal of radiology
2025

Diagnosis and Treatment of Gestational Non-Epithelial Ovarian Cancer: A Systematic Review.

Anticancer research
2025

Primary versus interval cytoreductive surgery in patients with rare epithelial or non-epithelial ovarian cancer.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2025

Analysis of the global burden of ovarian cancer in adolescents.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2025

Patterns of care and development of quality indicators in patients with non-epithelial and rare ovarian tumors in Australia: insights from the National Gynae-Oncology Registry.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2025

Oncological safety of fertility preservation treatment in ovarian cancer: A Spanish multicenter study.

International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics
2024

Role of Pap Smear Cervical Cytology in the Diagnosis of Extrauterine Malignancies: Largest Study of 104 Cases from Tertiary Care Cancer Centre in India.

Acta cytologica
2024

Salpingectomy for ectopic pregnancy reduces ovarian cancer risk-a nationwide study.

JNCI cancer spectrum
2024

Predictive value of the Adult Comorbidity Evaluation 27 on adverse surgical outcomes and survival in elderly with advanced epithelial ovarian cancer undergoing cytoreductive surgery.

European journal of medical research
2024

Review on Sertoli-Leydig Cell Tumours of the Ovary.

Discovery medicine
2024

Laparoscopic versus laparotomic surgical treatment in apparent stage I ovarian cancer: a multi-center retrospective cohort study.

World journal of surgical oncology
2024

The Cukurova score in the prediction of primary cytoreduction in ovarian cancer.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2023

Clinicopathological Features and Survival Trends of Non-Epithelial Ovarian Cancer: Analysis of the Surveillance, Epidemiology, and End Results (SEER) Database.

Oncology research and treatment
2023

Characterization of ovarian cancer survival by histotype and stage: A nationwide study in Norway.

International journal of cancer
2023

A prospective randomized multicenter trial for lymphadenectomy in early-stage ovarian cancer: LOVE study.

Journal of gynecologic oncology
2023

Platelet RNA enables accurate detection of ovarian cancer: an intercontinental, biomarker identification study.

Protein &amp; cell
2023

Oncological outcomes among young women with non-epithelial ovarian cancer: the YOC-Care study (Young Ovarian Cancer - Care).

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2023

Spindle-nucleated cells in cervical liquid-based cytology: Difficulties in distinguishing between epithelial and non-epithelial tumours based on morphology.

Cytopathology : official journal of the British Society for Clinical Cytology
2022

Ovarian Sertoli Cell Tumor with Immature Prepubertal-like Sertoli Cell Component: A Case Report and Literature Review.

Medicina (Kaunas, Lithuania)
2022

Hormone replacement in premenopausal women treated with bilateral oophorectomy for ovarian cancer - a nationwide population-based study.

Gynecologic oncology
2022

[Préservation de la fertilité avant traitement d'un cancer de l'ovaire].

La Revue du praticien
2023

Characterization of rare histological subtypes of ovarian cancer based on molecular profiling.

Cancer medicine
2022

Clinical and pathological patterns of non-epithelial malignant ovarian tumours in Western Saudi Arabia.

The Nigerian postgraduate medical journal
2022

Ultrasonographic diagnosis and surgical outcomes of adnexal masses in children and adolescents.

Scientific reports
2022

Non-Epithelial Ovarian Cancers: How Much Do We Really Know?

International journal of environmental research and public health
2022

Demographic features, clinical characteristics, and prognostic factors of non-epithelial ovarian tumors at Princess Noorah Oncology Center, National Guard Hospital, Jeddah, Saudi Arabia.

Saudi medical journal
2021

A pedunculated small bowel gastrointestinal stromal tumour (GIST) masquerading as an ovarian tumour.

Journal of surgical case reports
2021

"Chronic fatigue, quality of life and long-term side-effects of chemotherapy in patients treated for non-epithelial ovarian cancer: national case-control protocol study of the GINECO-Vivrovaire rare tumors INCa French network for rare malignant ovarian tumors".

BMC cancer
2021

GYNAECOLOGICAL ONCOLOGIC DISEASES AND PREGNANCY.

Wiadomosci lekarskie (Warsaw, Poland : 1960)
2022

Adjuvant and post-surgical treatment in non-epithelial ovarian cancer.

Best practice &amp; research. Clinical obstetrics &amp; gynaecology
2021

The association of reproductive factors with risk of non-epithelial ovarian cancer and comparison with serous ovarian cancer.

Gynecologic oncology
2021

The insolvable problem; survival effect of lymphadenectomy in advanced stage ovarian cancer.

Ginekologia polska
2021

Prognostic Value and Therapeutic Implications of Pleural Carcinosis and Malignant Pleural Effusion in Advanced Epithelial Ovarian Cancer.

Anticancer research
2021

Early stage epithelial ovarian cancer metastasis through peritoneal fluid circulation.

Journal of ovarian research
2021

Hereditary ovarian tumour syndromes: current update on genetics and imaging.

Clinical radiology
2021

Ultrasound, macroscopic and histological features of malignant ovarian tumors. Non-epithelial ovarian carcinomas: tubal choriocarcinoma and granulosa cell tumor.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2020

Single-cell analysis of copy-number alterations in serous ovarian cancer reveals substantial heterogeneity in both low- and high-grade tumors.

Cell cycle (Georgetown, Tex.)
2020

Preoperative Differentiation of Benign and Malignant Non-epithelial Ovarian Tumors: Clinical Features and Tumor Markers.

Revista brasileira de ginecologia e obstetricia : revista da Federacao Brasileira das Sociedades de Ginecologia e Obstetricia
2020

Survival and recurrence after intraperitoneal chemotherapy use: Retrospective review of ovarian cancer hospital registry data.

Cancer medicine
2020

Study of upfront surgery versus neoadjuvant chemotherapy followed by interval debulking surgery for patients with stage IIIC and IV ovarian cancer, SGOG SUNNY (SOC-2) trial concept.

Journal of gynecologic oncology
2020

The clinical impact of intra- and extracellular miRNAs in ovarian cancer.

Cancer science
2020

Non-epithelial ovarian carcinoma: what is the optimal staging surgery?

Chinese clinical oncology
2020

ESGO-SIOPE guidelines for the management of adolescents and young adults with non-epithelial ovarian cancers.

The Lancet. Oncology
2020

Role of high-dose salvage radiotherapy for oligometastases of the localised abdominal/pelvic lymph nodes: a retrospective study.

BMC cancer
2021

Differential blood count as triage tool in evaluation of pelvic masses.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2020

Pregnancy-related risk factors for sex cord-stromal tumours and germ cell tumours in parous women: a registry-based study.

British journal of cancer
2020

A phase II trial of cytoreductive surgery combined with niraparib maintenance in platinum-sensitive, secondary recurrent ovarian cancer: SGOG SOC-3 study.

Journal of gynecologic oncology
2020

Non-epithelial ovarian cancer in Denmark - Incidence and survival over nearly 40 years.

Gynecologic oncology
2020

[The pathology of hereditary ovarian tumors].

Annales de pathologie
2020

Detailed overview on rare malignant ovarian tumors.

Bulletin du cancer
2019

Fertility-sparing surgery for treatment of non-epithelial ovarian cancer: Oncological and reproductive outcomes in a prospective nationwide population-based cohort study.

Gynecologic oncology
2019

TRUST: Trial of Radical Upfront Surgical Therapy in advanced ovarian cancer (ENGOT ov33/AGO-OVAR OP7).

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2019

Overview of non-epithelial ovarian tumours: Incidence and survival in the Netherlands, 1989-2015.

European journal of cancer (Oxford, England : 1990)
2019

Development and validation of a model that includes two ultrasound parameters and the plasma D-dimer level for predicting malignancy in adnexal masses: an observational study.

BMC cancer
2019

Diagnostic Performance of Serum Human Epididymis Protein 4 (HE4) for Prediction of Malignancy in Ovarian Masses.

Asian Pacific journal of cancer prevention : APJCP
2019

Clinical characteristics and outcomes after bowel surgery and ostomy formation at the time of debulking surgery for advanced-stage epithelial ovarian carcinoma.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2019

Role of epithelial-mesenchymal transition factors in the histogenesis of uterine carcinomas.

Virchows Archiv : an international journal of pathology
2018

Cytopathological Study of the Circulating Tumor Cells filtered from the Cancer Patients' Blood using Hydrogel-based Cell Block Formation.

Scientific reports
2018

Validity and prognostic significance of sperm protein 17 as a tumor biomarker for epithelial ovarian cancer: a retrospective study.

BMC cancer
2019

Challenges and controversies in the conservative management of uterine and ovarian cancer.

Best practice &amp; research. Clinical obstetrics &amp; gynaecology
2018

Predicting non-home discharge in epithelial ovarian cancer patients: External validation of a predictive model.

Gynecologic oncology
2019

Clinically inappropriate post hoc exclusion of study participants from test accuracy calculations: the ROMA score, an example from a recent NICE diagnostic assessment.

Annals of clinical biochemistry
2018

Non-epithelial ovarian cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.

Annals of oncology : official journal of the European Society for Medical Oncology
2018

Possibility of women treated with fertility-sparing surgery for non-epithelial ovarian tumors to safely and successfully become pregnant-a Chinese retrospective cohort study among 148 cases.

Frontiers of medicine
2017

Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects.

Critical reviews in oncology/hematology
2018

Paediatric ovarian tumours and their associated cancer susceptibility syndromes.

Journal of medical genetics
2018

MicroRNA expression profiles in non‑epithelial ovarian tumors.

International journal of oncology
2017

[Significance and expression of PAX8, PAX2, p53 and RAS in ovary and fallopian tubes to origin of ovarian high grade serous carcinoma].

Zhonghua fu chan ke za zhi
2019

Epidemiology of rare cancers and inequalities in oncologic outcomes.

European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
2017

Better Predictive Value of Cancer Antigen125 (CA125) as Biomarker in Ovary and Breast Tumors and its Correlation with the Histopathological Type/Grade of the Disease.

Medicinal chemistry (Shariqah (United Arab Emirates))
2017

Optimal primary management of bulky stage IIIC ovarian, fallopian tube and peritoneal carcinoma: Are the only options complete gross resection at primary debulking surgery or neoadjuvant chemotherapy?

Gynecologic oncology
2016

[Sertoli-Leydig cell tumor of the ovary: case study of a 22-year old woman].

The Pan African medical journal
2017

Ovarian cancer in pregnancy.

Best practice &amp; research. Clinical obstetrics &amp; gynaecology
2017

The histology of ovarian cancer: worldwide distribution and implications for international survival comparisons (CONCORD-2).

Gynecologic oncology
2017

Worldwide comparison of ovarian cancer survival: Histological group and stage at diagnosis (CONCORD-2).

Gynecologic oncology
2016

Non-epithelial Ovarian Cancer: Elucidating Uncommon Gynaecological Malignancies.

Anticancer research
2016

Recently characterized molecular events in uncommon gynaecological neoplasms and their clinical importance.

Histopathology
2016

A case report of a young girl with mucinous borderline tumor of the ovary.

Obstetrics &amp; gynecology science
2016

[Grading of gynecological tumors : Current aspects].

Der Pathologe
2017

Clinical and pathologic features of patients with non-epithelial ovarian cancer: retrospective analysis of a single institution 15-year experience.

Clinical &amp; translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico
2016

Differential expression of E-cadherin and catenins in ovarian sex cord stromal tumours.

Histopathology
2016

Rare non-epithelial ovarian neoplasms: Pathology, genetics and treatment.

Gynecologic oncology
2016

Comparing the Copenhagen Index (CPH-I) and Risk of Ovarian Malignancy Algorithm (ROMA): Two equivalent ways to differentiate malignant from benign ovarian tumors before surgery?

Gynecologic oncology
2015

[A Case of Mucinous Cystadenocarcinoma of the Retroperitoneum Supposed to Be Derived from Ectopic Ovarian Tissue].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2016

Diverse expression patterns of the EMT suppressor grainyhead-like 2 (GRHL2) in normal and tumour tissues.

International journal of cancer
2015

MicroRNAs in Ovarian Cancer.

Human pathology
2015

[Rare ovarian tumors: report of a series of 11 cases of malignant non-epithelial ovarian tumors].

The Pan African medical journal

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Doenças relacionadas

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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. Correlation between ultrasound and gross pathology examination of malignant ovarian tumors: a pictorial review for pattern recognition.
    International journal of gynecological cancer : official journal of the International Gynecological Cancer Society· 2026· PMID 41865621mais citado
  2. Combination of transvaginal ultrasound with cervical cancer screening contributes to early detection of ovarian cancer: Clinical trial.
    International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics· 2026· PMID 41178497mais citado
  3. Cancer Antigen-125 and Carcinoembryonic Antigen in Determining Malignancy Risk in Epithelial Ovarian Tumor: An Observational Study.
    JNMA; journal of the Nepal Medical Association· 2025· PMID 41783428mais citado
  4. Targeting Ovarian Neoplasms: Subtypes and Therapeutic Options.
    Medicina (Kaunas, Lithuania)· 2025· PMID 41470247mais citado
  5. Long-term quality of life in non-epithelial ovarian cancer survivors: TMRG-GINECO-Vivrovaire rare tumors case-control study.
    BMC medicine· 2025· PMID 41382138mais citado
  6. Mast cell mediators in hereditary angioedema.
    Orphanet J Rare Dis· 2026· PMID 41832580recente
  7. Prenatal Molecular Diagnosis of COL2A1-Associated Stickler Syndrome: Genotype-Phenotype Correlation in a Resource-Limited Healthcare Setting.
    Int J Mol Sci· 2026· PMID 41828453recente
  8. Platelet gene signatures detecting pulmonary artery stenosis in patients with pulmonary hypertension.
    Orphanet J Rare Dis· 2026· PMID 41827036recente
  9. The global impact of imiglucerase therapy in children with Gaucher disease types 1 and 3: a real-world analysis from the International Collaborative Gaucher Group Gaucher Registry.
    Orphanet J Rare Dis· 2026· PMID 41821052recente
  10. Monogenic lupus with SLC7A7 mutations: a retrospective study from a Chinese center.
    Orphanet J Rare Dis· 2026· PMID 41821046recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:398940(Orphanet)
  2. MONDO:0018365(MONDO)
  3. GARD:21650(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q55787996(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 não epitelial maligno do ovário
Compêndio · Raras BR

Tumor não epitelial maligno do ovário

ORPHA:398940 · MONDO:0018365
Prevalência
1-9 / 1 000 000
Medicamentos
6 registrados
Início
Adult
Prevalência
0.43 (Europe)
MedGen
UMLS
C5680039
Wikidata
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