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Teratoma extragonadal
ORPHA:883CID-10 · D48.7DOENÇA RARA

Um teratoma extragonadal é um tipo de tumor muito raro que se desenvolve fora dos testículos (em homens) ou ovários (em mulheres). Ele surge de células germinativas (células que podem dar origem a vários tipos de tecido do corpo) e pode ser benigno (não canceroso) ou maligno (canceroso). Clinicamente, ele é identificado quando um teratoma aparece em locais como: na região atrás da barriga, na área entre os pulmões, na cabeça ou no rosto, na região do cóccix (o último osso da coluna), dentro dos ossos, ou em órgãos sólidos (como o fígado). Ao ser analisado no microscópio, esse tumor geralmente mostra uma mistura de tecidos bem formados e outros que ainda são imaturos. Os sintomas que a pessoa sente variam bastante, dependendo do tamanho e do lugar exato onde o tumor está.

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Introdução

O que você precisa saber de cara

📋

Um teratoma extragonadal é um tipo de tumor muito raro que se desenvolve fora dos testículos (em homens) ou ovários (em mulheres). Ele surge de células germinativas (células que podem dar origem a vários tipos de tecido do corpo) e pode ser benigno (não canceroso) ou maligno (canceroso). Clinicamente, ele é identificado quando um teratoma aparece em locais como: na região atrás da barriga, na área entre os pulmões, na cabeça ou no rosto, na região do cóccix (o último osso da coluna), dentro dos ossos, ou em órgãos sólidos (como o fígado). Ao ser analisado no microscópio, esse tumor geralmente mostra uma mistura de tecidos bem formados e outros que ainda são imaturos. Os sintomas que a pessoa sente variam bastante, dependendo do tamanho e do lugar exato onde o tumor está.

Publicações científicas
52 artigos
Último publicado: 2025 Jan
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D48.7
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Sinais e sintomas

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

Partes do corpo afetadas

🦴
Ossos e articulações
2 sintomas
🫃
Digestivo
1 sintomas
👁️
Olhos
1 sintomas
🧠
Neurológico
1 sintomas

+ 3 sintomas em outras categorias

Características mais comuns

Diplopia
Teratoma
Morfologia anormal do abdome
Poliúria
Hemiparesia
Nistagmo
8sintomas
Sem dados (8)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 8 características clínicas mais associadas, ordenadas por frequência.

Diplopia
Teratoma
Morfologia anormal do abdomeAbnormality of abdomen morphology
PoliúriaPolyuria
HemiparesiaHemiparesis

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico52PubMed
Últimos 10 anos29publicações
Pico20226 papers
Linha do tempo
2025Hoje · 2026🧪 1989Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
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3Fase 33
2Fase 21
1Fase 11
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 5 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

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Ensaios clínicos abertos e novidades científicas recentes

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Publicações mais relevantes

Timeline de publicações
28 papers (10 anos)
#1

Delayed presentation of mature teratoma with umbilical perforation in a 9-years old female without congenital abdominal wall defects: A rare case report of atypical teratoma location.

International journal of surgery case reports2025 May

Mature teratomas are benign germ cell tumors that predominantly occur in gonadal regions. Extra-gonadal teratomas, especially in the umbilical region, are rare and often present significant diagnostic challenges. A delayed presentation with umbilical perforation in absence of congenital abdominal wall defects is uncommon. A 9-year-old female presented with a four-year history of progressively enlarging umbilical mass with occasional discomfort. Examination revealed a firm, pedunculated mass protruding through a perforated umbilicus. A contrasted CT scan, revealed a well-encapsulated heterogeneous mass containing calcifications, with no evidence of deep intra-abdominal involvement. Complete surgical excision of the tumor was performed. Histopathological analysis confirmed a mature teratoma. Recovery was uneventful, and follow-up showed no recurrence. Umbilical teratomas are rare entities, particularly in children without congenital abnormalities. This case illustrates delayed presentation of umbilical perforation and diagnostic challenges in unusual located teratoma, emphasizing the need for high suspicious index when evaluating persistent umbilical masses. Radiology plays a critical role in differentiating these tumors from other conditions like granulomas, infections, or congenital anomalies. Complete surgical excision is the treatment of choice. This report highlights the rarity and diagnostic complexity of mature teratomas in atypical locations. Early recognition of this rare differential diagnosis in cases of persistent umbilical symptoms is essential to prevent complications such as infection and perforation. Awareness and early intervention are critical to achieving favorable outcomes in such cases. This case contributes to the limited literature on extragonadal teratomas with umbilical perforation and the need for timely diagnosis and management.

#2

Fistula formation after excision of extraperitoneal paravaginal teratoma.

Taiwanese journal of obstetrics & gynecology2025 Jan

Mature extragonadal teratomas, particularly at the pelvic extraperitoneal site, are rare. Herein, we report a case of paravaginal teratoma and fistula formation five years post-operation. A 23-year-old woman (G4P3A1) presented with a left paravaginal cystic tumor. After transvaginal tumor excision, histopathology revealed a paravaginal dermoid cyst. Five years post-operation, she returned with recurrent symptoms, including fever and perineal pain. Pelvic magnetic resonance imaging showed abscess at the surgical site, with tract-like extensions through the levator ani and vaginal wall. Treatment was administered without surgical intervention. Seven years post-operation, the patient underwent debridement of the left paravaginal abscess via the left perineal route. The patient was diagnosed with an abscess and an epidermal keratinous cyst with a vaginoperineal fistula. No recurrent abscess or fistula formation was encountered four years after debridement and fistulectomy. Preoperative and postoperative counseling should be conducted with long-term follow-up for such patients.

#3

Malignant transformation of a mature teratoma in the duodenum: A case report.

International journal of surgery case reports2025 Jan

Mature teratomas of the duodenum are extremely rare, with only five cases reported in the English literature and none documented in Vietnam. Malignant transformation within a mature teratoma presents a challenging scenario for diagnosis and management, requiring a nuanced approach to treatment. We report the first documented case of malignant transformation in a duodenal mature teratoma in Vietnam. A 35-year-old male presented with persistent dull pain in the left flank, anorexia, and melena. Clinical examination revealed a palpable 10 × 14 cm mass in the left hypochondrium. Computed tomography (CT) imaging identified a tumor in the D4 segment of the duodenum with invasion into adjacent structures. The patient subsequently underwent surgical resection of the tumor, followed by adjuvant chemotherapy. Histopathological analysis of the resected mass confirmed a moderately differentiated squamous cell carcinoma arising from a mature teratoma. Malignant transformations within teratomas, especially in such rare locations as the duodenum, require a multidisciplinary approach for optimal patient outcomes. In this case, the combination of surgical and adjuvant therapies was critical to managing the disease. This case highlights the importance of early recognition and a multidisciplinary approach in managing malignant transformation in duodenal teratomas. The combination of surgery and adjuvant chemotherapy proved effective, with no recurrence or metastasis observed at five months follow-up.

#4

Mediastinal teratoma in children: A case series of misdiagnoses in a high-endemic tuberculosis setting.

International journal of surgery case reports2024 Mar

Mediastinal teratoma is a rare extragonadal teratoma that frequently manifests with non-specific symptoms and is commonly misdiagnosed, particularly in Indonesia, where tuberculosis (TB) is endemic. Herein, we present two cases of children aged 19 months and 17 years old who were referred with chronic nonspecific symptoms of cough, tachypnea, chest pain, and hemoptysis. Both children were initially diagnosed with TB and both completed anti-tuberculosis treatment. After visiting several healthcare facilities, a mediastinal mass was finally diagnosed and they all underwent thoracotomy without any further complication. The histopathology findings revealed mature teratoma. All patients had satisfactory outcomes at follow-up visits. Patients presented with non-specific symptoms are often clinically diagnosed with TB. However, primary healthcare doctors should consider other differential diagnoses when a child has non-specific respiratory symptoms, especially when chest X-ray findings are nonspecific and TB workup is negative. The diagnosis of mediastinal masses is still challenging in Indonesia, as the diagnosis requires a vast array of work-up examinations, therefore, early recognition and referral are crucial for management.

#5

Parasitic mature cystic ovarian teratoma: A rare case of autoimplantation of a twisted dermoid cyst.

Clinical case reports2023 Sep

Extragonadal parasitic dermoid cysts are rare. Diagnosis of such extragonadal parasitic teratoma is often done intraoperatively during surgical exploration of abdominal mass.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC15 artigos no totalmostrando 28

2025

Delayed presentation of mature teratoma with umbilical perforation in a 9-years old female without congenital abdominal wall defects: A rare case report of atypical teratoma location.

International journal of surgery case reports
2025

Fistula formation after excision of extraperitoneal paravaginal teratoma.

Taiwanese journal of obstetrics & gynecology
2025

Malignant transformation of a mature teratoma in the duodenum: A case report.

International journal of surgery case reports
2024

Mediastinal teratoma in children: A case series of misdiagnoses in a high-endemic tuberculosis setting.

International journal of surgery case reports
2023

Parasitic mature cystic ovarian teratoma: A rare case of autoimplantation of a twisted dermoid cyst.

Clinical case reports
2023

Primary immature teratoma of the liver in an adult: Case report.

Radiology case reports
2022

Mature cystic extragonadal teratoma in Douglas' pouch: Case report and literature review.

Frontiers in medicine
2023

An unusual case of oropharyngeal mature teratoma in a kitten.

Iranian journal of veterinary research
2022

Case report: Mature extragonadal teratoma at the proximal part of the tail in a kitten.

Frontiers in veterinary science
2022

Mature Jejunal Teratoma in adolescents: A case report.

The Medical journal of Malaysia
2022

Neuroendocrine Tumor Arising within Mature Cystic Teratoma of the Pancreas: Literature Review and Case Report.

Current oncology (Toronto, Ont.)
2022

Laparoscopic Management of a Large Extragonadal Teratoma.

Gynecology and minimally invasive therapy
2022

Gluteus as a rare localization of extragonadal teratoma.

Pediatric blood & cancer
2021

Extratesticular gliomatosis peritonei after mesenteric teratoma: a case report and literature review.

The Journal of international medical research
2021

Extragonadal teratomas in women and adolescent girls: A systematic review.

European journal of obstetrics, gynecology, and reproductive biology
2021

Mature cystic teratoma mimicking meningomyelocele.

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

Choroid plexus papilloma in extragonadal teratoma with predominantly neurogenic elements: a case report and review of the literature.

The Turkish journal of pediatrics
2019

Bladder teratoma in a maned wolf (Chrysocyon brachyurus).

Open veterinary journal
2019

Extragonadal yolk sac tumor following congenital buccal mature cystic teratoma.

Journal of oral and maxillofacial pathology : JOMFP
2019

Analysis of chromosome 12p over-representation and clinicopathological features in mediastinal teratomas.

Pathology
2019

Clinicopathologic Features and Chromosome 12p Status of Pediatric Sacrococcygeal Teratomas: A Multi-institutional Analysis.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2017

Split liver transplantation for retroperitoneal immature teratoma masquerading as hepatoblastoma.

Pediatric transplantation
2016

Gluteal teratoma: A rare site of extragonadal teratoma.

Journal of Indian Association of Pediatric Surgeons
2015

Mediastinal Teratoma with Neuroendocrine Features in 34-Year-Old Male with Syncope.

Case reports in surgery
2015

A Case of Extragonadal Teratoma in the Pouch of Douglas and Literature Review.

Journal of minimally invasive gynecology
2015

Mature Cystic Teratoma in Douglas' Pouch.

Case reports in pathology
2015

Neuroendocrine carcinoma arising in a mediastinal teratoma with pulmonary metastasis: a case report and the chemotherapy response.

Internal medicine (Tokyo, Japan)
2015

An extragonadal teratoma in a female cynomolgus monkey (Macaca fascicularis).

Journal of medical primatology

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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. Delayed presentation of mature teratoma with umbilical perforation in a 9-years old female without congenital abdominal wall defects: A rare case report of atypical teratoma location.
    International journal of surgery case reports· 2025· PMID 40228345mais citado
  2. Fistula formation after excision of extraperitoneal paravaginal teratoma.
    Taiwanese journal of obstetrics & gynecology· 2025· PMID 39794024mais citado
  3. Malignant transformation of a mature teratoma in the duodenum: A case report.
    International journal of surgery case reports· 2025· PMID 39756232mais citado
  4. Mediastinal teratoma in children: A case series of misdiagnoses in a high-endemic tuberculosis setting.
    International journal of surgery case reports· 2024· PMID 38308982mais citado
  5. Parasitic mature cystic ovarian teratoma: A rare case of autoimplantation of a twisted dermoid cyst.
    Clinical case reports· 2023· PMID 37744627mais citado
  6. Primary immature teratoma of the liver in an adult: Case report.
    Radiol Case Rep· 2023· PMID 37680664recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:883(Orphanet)
  2. MONDO:0019500(MONDO)
  3. GARD:18711(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)

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 extragonadal
Compêndio · Raras BR

Teratoma extragonadal

ORPHA:883 · MONDO:0019500
CID-10
D48.7 · Neoplasia de comportamento incerto ou desconhecido de outras localizações especificadas
MedGen
UMLS
C4708601
EuropePMC
Wikipedia
Papers 10a
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