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Teratoma nasofaríngeo
ORPHA:141107CID-10 · D10.6CID-11 · LA70.YDOENÇA RARA

Hipófise é uma glândula endócrina com cerca de 1 cm de diâmetro alojada na sela túrcica ou fossa hipofisária do osso esfenoide na base do cérebro. Está localizada abaixo do hipotálamo e posteriormente ao quiasma óptico, sendo ligada ao hipotálamo pela haste pedúnculo hipofisário ou infundíbulo, é envolvida pela dura-máter. A hipófise é considerada uma "glândula mestra", pois secreta hormônios que controlam o funcionamento de outras glândulas, sendo grande parte de suas funções reguladas pelo hipotálamo.

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Introdução

O que você precisa saber de cara

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Teratoma nasofaríngeo é um tumor congênito raro composto por diversos tipos de tecido, como cabelo, dentes e osso, localizado na nasofaringe. Pode causar obstrução respiratória, dificuldade de deglutição e outros sintomas dependendo do tamanho e localização.

Publicações científicas
107 artigos
Último publicado: 2025 Feb
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SUS: Sem cobertura SUSScore: 0%
CID-10: D10.6
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Anos de pesquisa1desde 2025
Total histórico107PubMed
Últimos 10 anos19publicações
Pico20156 papers
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2025Hoje · 2026🧪 2004Primeiro ensaio clínico📈 2015Ano de pico
Publicações por ano (últimos 10 anos)

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Publicações mais relevantes

Timeline de publicações
19 papers (10 anos)
#1

[The application of low-temperature plasma minimally invasive therapy in children with pharyngeal neoplasm].

Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery2025 Feb

Objective:Analyze the clinical features in children with pharyngeal neoplasm, and explore the clinical efficacy of low-temperature plasma minimally invasive treatment in children with pharyngeal neoplasm. Methods:A total of 46 pediatric cases of pharyngeal neoplasms admitted to the Otolaryngology Department of Wuhan Children's Hospital from January 2016 to December 2023 were included. There were 23 males and 23 females, with admission ages ranging from 5 days to 9 years and 4 months, and a median age of 2 years and 2 months. The clinical manifestations, examinations, treatments, and outcomes were evaluated. Results:Among the 46 pediatric patients, 21 were admitted with throat wheezing, 7 with masses in the oropharynx or head and neck, 5 with dyspnea, 4 with snoring during sleep as the main symptom, 3 with hoarseness, 2 with sore throat, 2 with swallowing discomfort, and 2 with difficulty feeding as the first symptom. Comorbidities included 5 cases of combined laryngomalacia, 4 of snoring, 4 of congenital heart disease, 4 of severe pneumonia, 3 of myocardial damage, 1 of multiple deformities, and 1 after hematopoietic stem cell transplantation. All patients underwent CT or MRI examination, with 26 cases undergoing local ultrasound examination and 39 undergoing electronic laryngoscopy examination. A total of 19 patients were transferred to the ICU after surgery, all of whom were patients with pharyngeal cysts. All patients underwent low-temperature plasma minimally invasive surgery, and all patients were diagnosed through pathological examination, including 1 case of nasopharyngeal teratoma, 5 of pharyngeal non-Hodgkin's lymphoma, 1 of oropharyngeal rhabdomyosarcoma, 1 of laryngeal perivascular epithelioid cell tumor (PEComa), and 38 of pharyngeal cysts (2 cases of uvula, 2 of oropharynx, 7 of epiglottic valley, 19 of tongue base, 2 of branchial cleft cyst, and 6 of throat). Conclusion:Pharyngeal neoplasms in children are prone to upper airway obstruction, including some rare or malignant tumors. Imaging and pathological diagnosis can assist in early diagnosis. Depending on the nature of the tumor, early detection and treatment can improve the quality of life and survival rate of children. Low-temperature plasma radiofrequency ablation is safe, minimally invasive, precise, and provides a clear field of vision, making it a valuable treatment option for children with pharyngeal neoplasms. 目的:分析儿童咽喉部新生物的临床特点,探讨低温等离子微创治疗在儿童咽喉部新生物中的临床疗效。 方法:总结2016年1月—2023年12月武汉儿童医院耳鼻咽喉科收治的46例咽喉部新生物患儿的临床资料,其中男23例,女23例;年龄5 d~9岁4个月,中位年龄2岁2个月。对其临床特点、辅助检查及治疗情况进行分析。 结果:46例患儿中,21例以喉喘鸣入院,7例因发现口咽部或头颈部肿物入院,5例以呼吸困难为首发症状,4例以睡眠打鼾为主要症状,3例声嘶,2例咽痛,2例吞咽不适,2例喂养困难。合并喉软化5例,鼾症4例,先天性心脏病4例,重症肺炎4例,心肌损害3例,多处畸形1例,另有1例为造血干细胞移植术后。所有患儿均行CT或MRI检查,26例行局部超声检查,39例行电子喉镜检查。共19例患儿术后转入ICU,均为咽喉囊肿患儿。所有患儿均行低温等离子微创手术,均经病理检查确诊,包括鼻咽部畸胎瘤1例,咽部非霍奇金淋巴瘤5例,口咽部横纹肌肉瘤1例,喉部血管周上皮样细胞肿瘤(PEComa)1例,咽喉部囊肿38例(悬雍垂2例,口咽2例,会厌谷7例,舌根19例,鳃裂囊肿2例,喉部6例)。 结论:儿童期咽喉部占位易致气道梗阻,包括一些罕见或者恶性肿瘤,影像学检查及病理学检查可辅助诊断及尽早确诊,根据肿物性质不同,早发现早治疗,提高患儿生活质量及生存率。低温等离子射频消融术安全、微创、精准,且视野清晰,值得在儿童咽喉部新生物切除中推广。.

#2

[Analysis of 141 cases with benign upper airway occupying in infant].

Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery2024 Apr

Objective:To analyze and summarize the clinical characteristics, diagnosis, treatment and prognosis of benign upper airway space occupancy in infants. Methods:The clinical data of 141 cases with begin upper airway space from January 2012 to January 2022 were analyzed. Among them, 101 were male and 68 were female, the age is 0-3 years old. In which there were 24 newborns. The clinical characteristics, auxiliary examination and treatment results were summarized and analyzed. Results:The main clinical manifestations of 141 infants were dyspnea and/or laryngeal wheezing, including 116 cases of congenital cyst of tongue, 15 cases of hair polyps, 4 cases of nasopharyngeal second pharyngeal fissure cysts, 2 cases of congenital laryngeal cysts, 2 cases of pharyngeal bronchial cyst, 1 case of nasopharyngeal teratoma and 1 case of myofibroma. All the infants had completed the corresponding examination and treatment. The diagnosis was clear, and there was no missed diagnosis or misdiagnosis. Among them, 19 infants with congenital cyst of tongue were given cyst puncture to relieve dyspnea. 2 cases of congenital cyst of tongue recurred half a year after operaion, and then they underwent reoperation. The prognosis of the remaining infants were good. Conclusion:The most common occupying of benign upper airway space occupancy is cyst, and low-temperature plasma resection under endoscope is the main treatment method. Timely puncture therapy is also a safe and effective treatment for infants who are dyspnea and life threatening. 目的:分析和总结婴幼儿上气道良性占位的临床特点、诊断、治疗及预后。 方法:回顾性分析2012年1月—2022年1月我院诊治的141例上气道良性占位患儿的临床资料。其中男85例,女56例;年龄0~3岁,其中新生儿24例。对其临床特点、辅助检查、治疗结果进行总结和分析。 结果:141例患儿主要临床表现为呼吸困难和(或)喉喘鸣,其中先天性舌根囊肿116例,毛息肉15例,鼻咽部第二鳃裂囊肿4例,先天性喉小囊囊肿2例,咽部支气管源性囊肿2例,鼻咽部畸胎瘤1例,肌纤维瘤1例。所有患儿都完成相应检查和治疗,诊断明确,无漏诊或误诊,其中19例舌根囊肿患儿行囊肿穿刺术,术后随访2例先天性舌根囊肿患者,因其术后半年复发故行二次手术,其他患儿预后良好。 结论:婴幼儿上气道良性占位,以囊性肿物多见,内镜辅助下低温等离子切除病变为主要治疗方式,对于呼吸困难严重且生命危急的患儿及时的穿刺治疗也是一种安全有效的治疗方法。.

#3

Immature nasopharyngeal teratoma with prenatal diagnosis: Case report and review of the literature.

Revista colombiana de obstetricia y ginecologia2023 Mar 30

To report the case of a pregnant woman with prenatal diagnosis of fetal immature nasopharyngeal teratoma, and to conduct a review of the literature describing the prognosis of this condition. We report the case of a 27-year-old pregnant woman who received care at the Obstetrics and Gynecology Unit of a reference hospital in Bogotá (Colombia) because of a finding during a prenatal visit of evidence of polyhydramnios, secondary to a nasopharyngeal teratoma. A literature search was conducted in the Medline vía PubMed, Scopus, SciELO and ScienceDirect databases, restricted by language (English and Spanish) and date of publication (January 2001 to January 2021). Case reports and case series covering the prognosis of this condition were included. Overall, 168 titles were retrieved, 55 of which met the inclusion criteria. Perinatal outcomes for a total of 58 fetuses with a diagnosis of immature nasopharyngeal teratoma detected during the prenatal stage were reported. In the identified cases, perinatal mortality was 25.4 % and the percentage of fetal demise was close to 3.6 %. Immature nasopharyngeal teratoma is an infrequent condition. The available literature suggests that fetal prognosis depends on the degree of compromise of intracranial structures and the possibility of resecting the lesion. Further studies are needed to assess the prognosis of fetuses with immature nasopharyngeal teratoma. reportar el caso de una gestante con diagnóstico de feto con teratoma nasofaríngeo inmaduro y realizar una revisión describiendo el pronóstico de esta patología. se reporta el caso de una gestante de 27 años, atendida en la Unidad de Ginecología y Obstétrica de un centro de referencia en Bogotá (Colombia), por hallazgo durante el control prenatal de un feto con evidencia de polihidramnios secundario a teratoma nasofaríngeo. Se realizó una búsqueda bibliográfica en las bases de datos Medline vía PubMed, Scopus, SciELO y ScienceDirect, restringiendo tipo de idioma (inglés y español) y por fecha de publicación (enero de 2001 a enero de 2021). Se incluyeron reportes de caso y series de casos que abarcaran el pronóstico de esta patología. la búsqueda recuperó 168 títulos, de los cuales 55 cumplieron con los criterios de inclusión. En total se reportó el resultado perinatal de 58 fetos con diagnóstico de teratoma nasofaríngeo inmaduro detectado en etapa prenatal. En los casos identificados, la mortalidad perinatal fue del 25,4 % y el porcentaje de óbito fue cercano al 3,6 %. el teratoma nasofaríngeo inmaduro es una patología poco frecuente. La literatura disponible sugiere que el pronóstico fetal depende del grado de compromiso de las estructuras intracraneales y de la posibilidad de resección de la lesión. Se requieren más estudios que evalúen el pronóstico de los fetos con teratoma nasofaríngeo inmaduro. To report the case of a pregnant woman with prenatal diagnosis of fetal immature nasopharyngeal teratoma, and to conduct a review of the literature describing the prognosis of this condition. We report the case of a 27-year-old pregnant woman who received care at the Obstetrics and Gynecology Unit of a reference hospital in Bogotá (Colombia) because of a finding during a prenatal visit of evidence of polyhydramnios, secondary to a nasopharyngeal teratoma. A literature search was conducted in the Medline via PubMed, Scopus, SciELO and ScienceDirect databases, restricted by language (English and Spanish) and date of publication (January 2001 to January 2021). Case reports and case series covering the prognosis of this condition were included. Overall, 168 titles were retrieved, 55 of which met the inclusion criteria. Perinatal outcomes for a total of 58 fetuses with a diagnosis of immature nasopharyngeal teratoma detected during the prenatal stage were reported. In the identified cases, perinatal mortality was 25.4 % and the percentage of fetal demise was close to 3.6 %. Immature nasopharyngeal teratoma is an infrequent condition. The available literature suggests that fetal prognosis depends on the degree of compromise of intracranial structures and the possibility of resecting the lesion. Further studies are needed to assess the prognosis of fetuses with immature nasopharyngeal teratoma.

#4

An operation on placental support in a fetus with a nasopharyngeal teratoma.

Anaesthesia reports2023

Airway obstruction diagnosed antenatally in the fetus due to congenital high airway obstruction syndrome or nasopharyngeal masses are the primary indications for performing an operation on placental support. Prenatally diagnosed nasopharyngeal masses pose the risk of total airway obstruction upon delivery. Placental support utilises uteroplacental blood flow to facilitate an airway intervention on the fetus while maintaining oxygenation. These interventions must be completed in under 20 min due to amniotic fluid loss and uterine contractions. This case report describes the anaesthetic management of a fetus with a nasal teratoma. In this report, we discuss the clinical indications and anaesthetic considerations of the operation on placental support procedure for nasopharyngeal masses.

#5

Duplication of the Pituitary Gland (DPG)-Plus Syndrome Associated With Midline Anomalies and Precocious Puberty: A Case Report and Review of the Literature.

Frontiers in endocrinology2021

Duplication of the pituitary gland (DPG)-plus syndrome is a very rare developmental disorder with few cases described in the literature and characterized by multiple midline and central nervous system malformations. The hypothalamus and hypophysis involvement may be clinically associated with endocrine abnormalities. A 5.9-year-old female child was admitted to our Clinic for premature thelarche and acceleration of growth. DPG-plus syndrome with paired infundibula and pituitary glands was diagnosed after birth, when she appeared small for gestational age and she presented with lingual hypoplasia, cleft palate, right choanal stenosis, nasopharyngeal teratoma, and facial dysmorphisms. Neuroimaging revealed a duplication of the infundibula, the pituitary gland, and the dens of the epistropheus despite surgical removal of a rhino-pharyngeal mass performed at the age of two months. An array-CGH revealed a 2p12 deletion. At our evaluation, bone age assessment resulted advanced and initial pubertal activation was confirmed by Gonadotropin-Releasing Hormone stimulation test. Hormonal suppression treatment was started with satisfactory results. This case shows that DPG-plus syndrome must be considered in presence of midline and craniofacial malformations and endocrinological evaluations should be performed for the prompt and appropriate management of pubertal anomalies.

Publicações recentes

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📚 EuropePMC84 artigos no totalmostrando 19

2025

[The application of low-temperature plasma minimally invasive therapy in children with pharyngeal neoplasm].

Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery
2024

[Analysis of 141 cases with benign upper airway occupying in infant].

Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery
2023

Immature nasopharyngeal teratoma with prenatal diagnosis: Case report and review of the literature.

Revista colombiana de obstetricia y ginecologia
2023

An operation on placental support in a fetus with a nasopharyngeal teratoma.

Anaesthesia reports
2021

Duplication of the Pituitary Gland (DPG)-Plus Syndrome Associated With Midline Anomalies and Precocious Puberty: A Case Report and Review of the Literature.

Frontiers in endocrinology
2019

Mature Nasopharyngeal Teratoma in a Neonate: Case Report and a Review of Literature.

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

Case of nasopharyngeal teratoma: challenges in the management.

BMJ case reports
2019

Management of nasopharyngeal teratomas associated with cleft palate.

International journal of oral and maxillofacial surgery
2018

History and highlights of the teratological collection in the Museum Anatomicum of Leiden University, The Netherlands.

American journal of medical genetics. Part A
2017

Congenital nasal obstruction in infants: A retrospective study and literature review.

International journal of pediatric otorhinolaryngology
2016

Nasopharyngeal teratoma, congenital diaphragmatic hernia and Dandy-Walker malformation - a yet uncharacterized syndrome.

Clinical genetics
2015

Congenital nasopharyngeal teratoma causing airway obstruction in the newborn.

Journal of cancer research and therapeutics
2016

A Rare Nasopharyngeal Teratoma Arising From the Vomer.

The Journal of craniofacial surgery
2015

DPG-plus syndrome: new report of a rare entity.

BMJ case reports
2015

[The clinical characters and surgical management of nasopharyngeal teratoma in infants].

Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery
2015

Mature Nasopharyngeal Teratoma in a Child.

Case reports in otolaryngology
2014

Nasopharyngeal mature teratoma in the newborn.

Turk pediatri arsivi
2015

Radiofrequency coblation of congenital nasopharyngeal teratoma: a novel technique.

Case reports in otolaryngology
2015

Soft palatine mass with diagnosis of mature teratoma.

International journal of surgery case reports
Ver todos os 84 no EuropePMC

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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. [The application of low-temperature plasma minimally invasive therapy in children with pharyngeal neoplasm].
    Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery· 2025· PMID 39924323mais citado
  2. [Analysis of 141 cases with benign upper airway occupying in infant].
    Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery· 2024· PMID 38563179mais citado
  3. Immature nasopharyngeal teratoma with prenatal diagnosis: Case report and review of the literature.
    Revista colombiana de obstetricia y ginecologia· 2023· PMID 37093940mais citado
  4. An operation on placental support in a fetus with a nasopharyngeal teratoma.
    Anaesthesia reports· 2023· PMID 36936736mais citado
  5. Duplication of the Pituitary Gland (DPG)-Plus Syndrome Associated With Midline Anomalies and Precocious Puberty: A Case Report and Review of the Literature.
    Frontiers in endocrinology· 2021· PMID 34122353mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:141107(Orphanet)
  2. MONDO:0015391(MONDO)
  3. GARD:19948(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55785443(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

Teratoma nasofaríngeo
Compêndio · Raras BR

Teratoma nasofaríngeo

ORPHA:141107 · MONDO:0015391
CID-10
D10.6 · Neoplasia benigna da nasofaringe
CID-11
MedGen
UMLS
C4531264
EuropePMC
Wikidata
Papers 10a
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