O tumor maligno de Triton (MTT) é um subtipo raro e agressivo de tumor maligno da bainha dos nervos periféricos (MPNST), caracterizado histopatologicamente por diferenciação rabdomioblástica focal.
Introdução
O que você precisa saber de cara
O tumor tritão maligno é um tipo raro e agressivo de câncer que atinge a camada que envolve os nervos periféricos. Ele é caracterizado por misturar elementos celulares comuns a tumores de nervos com diferenciação de tecido muscular sob o microscópio. A doença pode se manifestar em pessoas de todas as idades e surgir como uma massa em diferentes partes do corpo. No Brasil, a condição não tem um PCDT específico do Ministério da Saúde, sendo acompanhada diretamente pela rede de atenção oncológica do SUS.
O tumor maligno de Triton (MTT) é um subtipo raro e agressivo de tumor maligno da bainha dos nervos periféricos (MPNST), caracterizado histopatologicamente por diferenciação rabdomioblástica focal.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
2 genes identificados com associação a esta condição.
Variantes genéticas (ClinVar)
160 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
3 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Tumor tritão maligno
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Publicações mais relevantes
Heterologous Rhabdomyoblastic Differentiation in Dedifferentiated Liposarcoma Is Significantly Associated With Loss of H3K27 Trimethylation.
We identified that H3K27me3 loss appears to be a recurrent event underlying tumor dedifferentiation and heterologous rhabdomyoblastic differentiation in dedifferentiated liposarcoma. Therefore, the loss of H3K27me3 by itself does not provide definitive evidence of malignant peripheral nerve sheath tumor or malignant triton tumor when the morphology and clinical presentation of the tumor align with dedifferentiated liposarcoma.
Malignant triton tumor of the common bile duct.
Malignant triton tumor (MTT) is a rare subset (5%) of malignant peripheral nerve sheath tumors (MPNSTs), classified as soft-tissue sarcomas. MTT is an orphan disease characterized by rhabdomyoblastic differentiation, therapeutic resistance, and a sinister prognosis. The neoplasms classically arise at the trunk, head and neck region, and extremities. In 50% of the cases, MTT is associated with neurofibromatosis type 1 (NF1), a relatively common autosomal dominant cancer-prone disorder of the central nervous system. Few cases of MTT in the gastrointestinal tract have been published, including esophagus, duodenum, and rectum. In this article, we present what we believe to be the first report of MTT in the common bile duct. A multidisciplinary approach was the key in establishing this particular diagnosis, and workup included endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, pathological staining, and genetic testing. Literature focusing on MTT remains scarce, and patients with MTT are often included with other subtypes in broader studies of MPNST. Therefore, our literature review covers MPNST and focusses on MTT where appropriate. It provides the current understanding of tumor epidemiology, genetics, and diagnostic workup, and discusses therapeutic challenges and future perspectives. Our case report underlines the value of cholangioscopy-guided biopsies, and honoring patient's autonomy in end-of-life setting.
Pathological and Immunohistochemical Features of Malignant Peripheral Nerve Sheath Tumor With Heterologous Rhabdomy Oblastic Differentiation (Malignant Triton Tumor) in the Context of the Malignant Peripheral Nerve Sheath Tumor Spectrum.
Malignant peripheral nerve sheath tumors (MPNST) are aggressive sarcomas arising from Schwann cells or neurofibromas, comprising ∼10% of soft tissue sarcomas. Found in 8% to 13% of neurofibromatosis type 1 patients, they typically show spindle cells expressing S100 and SOX10. Some display heterologous differentiation (bone, cartilage, vessels, or glands). When rhabdomyosarcomatous features are present, they are called malignant peripheral nerve heath tumor with heterologous rhabdomyoblastic differentiation (malignant triton tumors), which are more prone to recurrence, metastasis, and worse outcomes. Epithelial differentiation does not affect prognosis. Despite advances in understanding MPNST biology, many pathophysiological mechanisms remain unclear. This review highlights their histopathology, focusing on malignant triton tumor.
Imaging Progress and Treatment of Malignant Triton Tumor of the Skull Base.
Reporting the clinical course and management of a skull base malignant triton tumor in a child. An 11-year-old female patient initially presented at the age of 7 with left parotid gland swelling, exhibiting symptoms similar to parotitis. Magnetic resonance imaging (MRI) of the head performed at an external hospital suggested a neoplastic lesion in the left parotid gland and the left parasellar region. Six months later, a parotid gland biopsy was conducted. Postoperative pathology confirmed the diagnosis of embryonal rhabdomyosarcoma. Following the biopsy, the patient underwent one year of radiotherapy and chemotherapy. A follow-up head MRI examination revealed that the lesions in the parotid gland and the left parasellar region had completely resolved. The patient did not adhere to a regular schedule of follow-up examinations. At the age of 10, a follow-up head MRI revealed recurrence of the lesions in the left parotid gland and the left parasellar region. The patient subsequently underwent resection of the left parotid gland lesion at an external hospital. Postoperative pathologic analysis once again indicated embryonal rhabdomyosarcoma. The patient underwent genetic testing, which revealed a germline pathogenic mutation: NF1 p.L952Ffs*2, suggesting an inherited form of NF1. Chemotherapy was subsequently continued. A follow-up MRI performed 5 months after the second surgery showed no recurrence of the left parotid gland lesion; however, the left parasellar region lesion remained unchanged in size. A follow-up head MRI at 9 months postsecond surgery indicated an increase in the size of the left parasellar region tumor. By 10 months postsecond surgery, a follow-up head MRI showed a reduction in the left parasellar region lesion, with no recurrence of the left parotid gland lesion, leading to the discontinuation of chemotherapy. At 14 months postsecond surgery, a follow-up MRI revealed an enlargement of the left parasellar region lesion. The patient received 2 courses of chemotherapy, but a subsequent MRI showed continued growth of the left parasellar region lesion, accompanied by abnormal enhancement of the parotid gland lesion. The patient was then transferred to our department, where they underwent a left temporo-occipital craniotomy for tumor resection. Postoperative pathologic examination confirmed the diagnosis of a malignant triton tumor. Molecular pathologic testing was also performed. The patient recovered well and was discharged successfully. The patient died from cachexia caused by widespread dissemination of the parotid tumor 4 months after discharge. Pediatric skull base malignant triton tumors lack specific imaging manifestations. Radiotherapy and pharmacotherapy are insufficient to control tumor growth. Surgical resection remains the primary treatment method, yet the overall prognosis is poor.
Malignant triton tumor of the bladder with fatal outcome: a rare case report.
Malignant triton tumors (MTTs) are a rare subtype of malignant peripheral nerve sheath tumors characterized by rhabdomyoblastic differentiation, often associated with neurofibromatosis type 1 (NF-1). Primary MTTs of the bladder are exceptionally uncommon, with few documented cases. We report a case of a 75-year-old male without neurofibromatosis type 1 history presenting with hematuria and urinary obstruction. Imaging revealed a 6.5 × 4 × 2 cm invasive bladder mass, and histopathology confirmed a high-grade spindle cell neoplasm with biphasic neurogenic (S100-positive) and rhabdomyoblastic (desmin- and myogenin-positive) differentiation, consistent with a diagnosis of MTT. Despite radical cystoprostatectomy, the patient succumbed to postoperative complications. This case highlights the aggressive behavior, diagnostic challenges, and poor prognosis of bladder MTTs, emphasizing the critical role of immunohistochemistry in distinguishing them from other spindle cell neoplasms. The rarity of this presentation underscores the need for further research to elucidate its pathogenesis and optimize therapeutic strategies.
Publicações recentes
Malignant triton tumor with thoracic region as the initial presentation: a case report.
Heterologous Rhabdomyoblastic Differentiation in Dedifferentiated Liposarcoma Is Significantly Associated With Loss of H3K27 Trimethylation.
Pathological and Immunohistochemical Features of Malignant Peripheral Nerve Sheath Tumor With Heterologous Rhabdomy Oblastic Differentiation (Malignant Triton Tumor) in the Context of the Malignant Peripheral Nerve Sheath Tumor Spectrum.
Imaging Progress and Treatment of Malignant Triton Tumor of the Skull Base.
Malignant triton tumor of the bladder with fatal outcome: a rare case report.
📚 EuropePMC128 artigos no totalmostrando 73
Heterologous Rhabdomyoblastic Differentiation in Dedifferentiated Liposarcoma Is Significantly Associated With Loss of H3K27 Trimethylation.
Pathology internationalPathological and Immunohistochemical Features of Malignant Peripheral Nerve Sheath Tumor With Heterologous Rhabdomy Oblastic Differentiation (Malignant Triton Tumor) in the Context of the Malignant Peripheral Nerve Sheath Tumor Spectrum.
International journal of surgical pathologyImaging Progress and Treatment of Malignant Triton Tumor of the Skull Base.
The Journal of craniofacial surgeryMalignant triton tumor of the bladder with fatal outcome: a rare case report.
Journal of surgical case reportsMalignant triton tumor of the common bile duct.
Clinical journal of gastroenterologyMalignant peripheral nerve sheath tumor in early childhood: a case report of a diagnostic challenge.
Frontiers in oncologyMalignant Triton Tumor of the Ileocolic Mesentery with Neurofibromatosis Type 1: A Case Report and Literature Review.
Surgical case reportsAdvanced Metastatic Malignant Triton Tumor in Neurofibromatosis Type 1: A Case Report and Management Challenges.
Cancer diagnosis & prognosisMalignant Transformation of a Vestibular Schwannoma Without Previous Radiation Exposure: Illustrative Case and Literature Review.
Journal of neurological surgery reportsLoss of H3K27me3 Is Not Specific to Malignant Triton Tumor: Immunohistochemical Analysis of 23 Cases of Embryonal Rhabdomyosarcoma.
Archives of pathology & laboratory medicineDifficulties in the diagnosis and treatment of axillary malignant triton tumors: A case report.
Oncology lettersMalignant Triton Tumor of the Distal Femur: A Case Report and Review of the Literature.
In vivo (Athens, Greece)A Rare Case of Malignant Triton Tumor Without Associated Neurofibromatosis.
CureusGenomic profiling of pleomorphic rhabdomyosarcoma reveals a genomic signature distinct from that of embryonal rhabdomyosarcoma.
Genes, chromosomes & cancerNF1-Driven Rhabdomyosarcoma Phenotypes: A Comparative Clinical and Molecular Study of NF1-Mutant Rhabdomyosarcoma and NF1-Associated Malignant Triton Tumor.
JCO precision oncologyMalignant triton tumor in the abdominal wall: A case report.
World journal of clinical casesGranulomatous Myocarditis Caused by Candida Spp Infection in a Spontaneously Diabetic Torii Rat.
Medical archives (Sarajevo, Bosnia and Herzegovina)Sporadic Malignant Triton Tumor of Shoulder: a Case Report.
Medical archives (Sarajevo, Bosnia and Herzegovina)Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature review.
Frontiers in medicineA rare case of malignant triton tumor in the common peroneal nerve.
The American journal of the medical sciencesMalignant "triton" tumor of the lower extremity with a history of fracture.
Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de CitologiaMalignant triton tumor of uterus: A case report and literature review.
Journal of clinical ultrasound : JCUSurgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review.
World journal of surgical oncologyClinical diagnostic and radiographic features of recurrent intracranial malignant triton tumor in an adult: illustrative case.
Journal of neurosurgery. Case lessonsRecurrent sporadic malignant triton tumor in the carotid sheath in the absence of neurofibromatosis.
The neuroradiology journal'Triton' Tumor of the Lower Alveolus: An Aggressive Variant of Malignant Peripheral Nerve Sheath Tumour.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaCase report: Concurrent malignant triton tumor and relapsed pituitary adenoma in the sellar region.
Frontiers in surgeryHow to Treat Metastatic Malignant Triton Tumor in an Adolescent.
Oncology (Williston Park, N.Y.)Case report: Primary intracranial EWs/PNET in adults: Clinical experience and literature review.
Frontiers in oncologyComputed tomography imaging features of malignant 'triton' tumor to facilitate its clinical diagnosis: report of two cases.
BMC medical imagingMalignant Triton tumor of the retroperitoneum in a male unaffected by neurofibromatosis 1: A case report and literature review.
Asian journal of surgeryLiver metastasis 13 years after the resection of a malignant triton tumor originating in the duodenum.
International cancer conference journalCarotid artery ligation via sternotomy as a palliative surgery: Case report of advanced intramediastinal malignant soft tissue tumor.
Clinical case reportsClinicopathological and prognostic significance of H3K27 methylation status in malignant peripheral nerve sheath tumor: correlation with skeletal muscle differentiation.
Virchows Archiv : an international journal of pathologyMalignant triton tumor of the kidney in a child: A case report.
International journal of surgery case reportsA Rare Case of Progressive Malignant Triton Tumor With Rare Somatic Mutation in TSC2 Gene.
Anticancer researchMalignant triton tumor of the anterior mediastinum: a rare tumor in a rare location.
Radiology case reportsMalignant Glandular Triton Tumor Arising in the Radial Nerve with Prolonged Survival: A Case Report and Review of the Literature.
Case reports in pathologyLoss of H3K27me3 occurs in a large subset of embryonal rhabdomyosarcomas: Immunohistochemical and molecular analysis of 25 cases.
Annals of diagnostic pathologyMalignant triton tumor of the rectum - A case report and review of the literature.
International journal of surgery case reportsRetroperitoneal malignant triton tumor in an infant: a case report and literature review.
Translational pediatricsElbow flexion reconstruction after arm-sparing excision for high-grade triton sarcoma: a case report.
Journal of medical case reportsMalignant triton tumor below the peritoneal reflection: a case report.
Journal of surgical case reports[Focus on oncologic safety and function: Primary defect reconstruction of a malignant Triton tumor of the sciatic nerve sheath with myocutaneous free flap surgery].
Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Mikrochirurgie der Peripheren Nerven und Gefasse : Organ der V...Malignant Triton Tumor in a Child: Case Report and Literature Review.
Cancer management and researchTriton tumor of the orbit.
Orbit (Amsterdam, Netherlands)Malignant Triton Tumor Diagnosed on Fine Needle Aspiration Cytology.
Journal of cytologyMalignant triton tumor of the left thoracic cavity: a case report.
Journal of surgical case reportsMediastinal malignant triton tumor: A rare case series and review of literature.
International journal of surgery case reportsA series of 10 malignant triton tumors in one institution.
MedicineWorsening Hemoptysis in a Rare Intrapulmonary Triton Tumor Mimicking Lung Cancer.
The Annals of thoracic surgeryMalignant triton tumor diagnosed twelve years after radiosurgically treated vestibular schwannoma.
Clinical neurology and neurosurgeryTeaching NeuroImages: Intracranial malignant triton tumor: An uncommon location of a rare tumor.
NeurologyA rare case of intracranial malignant triton tumor arising in the middle cranial fossa: a case report and review of the literature.
Folia neuropathologicaExpert's comment concerning Grand Rounds case entitled "Malignant triton tumor: Grand Round presentation of a rare aggressive case thoracolumbar spine tumor" by S. Ghailane et al. (Eur Spine J; 2017: DOI 10.1007/s00586-017-5277-2).
European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research SocietyMalignant triton tumor of the duodenum: report of a case.
International cancer conference journal[A Rare Case of Abdominal Malignant Triton Tumor].
Gan to kagaku ryoho. Cancer & chemotherapyMalignant triton tumor of trigeminal nerve-case report.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryMalignant triton tumor of the gluteal region in a patient unaffected by neurofibromatosis: A case report.
Acta orthopaedica et traumatologica turcicaA Case of Retroperitoneal Malignant Triton Tumor in a Nigerian Boy.
Nigerian journal of surgery : official publication of the Nigerian Surgical Research SocietyMalignant triton tumor: Grand Round presentation of a rare aggressive case thoracolumbar spine tumor.
European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research SocietyMalignant Triton tumor: a rare cause of sciatic pain and foot drop.
Revista brasileira de ortopediaInsidious malignant triton tumor of the chest wall with late flare-up.
Tzu chi medical journalMalignant triton tumor of the lung, infiltrating the left atrium and left ventricle, with metastasis to the small intestine.
Kardiochirurgia i torakochirurgia polska = Polish journal of cardio-thoracic surgeryMalignant Triton Tumor (Malignant Peripheral Nerve Sheath Tumor With Rhabdomyoblastic Differentiation) Occurring in a Vascularized Free Flap Reconstruction Graft.
International journal of surgical pathologyA case of recurrent malignant triton tumor successfully treated with radiotherapy.
Auris, nasus, larynxA rare case of recurrent malignant triton tumor in a male with NF1: Case report and mini-review.
International journal of surgery case reportsAnalysis of clinical features and prognosis of malignant triton tumor: A report of two cases and literature review.
Oncology lettersPrimary epididymis malignant triton tumor: case report and review of the literature.
European journal of medical researchNovel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation.
The American journal of surgical pathologyMalignant Triton Tumor of the Kidney. New Location Not Previously Reported.
Urologia internationalisMalignant Peripheral Nerve Sheath Tumor With Rhabdomyosarcomatous and Glandular Elements: Rare Epithelial Differentiation in a Triton Tumor.
International journal of surgical pathologyUnusual histological variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation.
World journal of clinical casesAssociações
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Doença com base genética
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Doenças relacionadas
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Perguntas frequentes
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O tumor tritão maligno é um subtipo histopatológico raro e agressivo de tumor maligno da bainha dos nervos periféricos. Ele apresenta histologicamente uma diferenciação rabdomioblástica focal.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
6 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (s.d.). Tumor tritão maligno. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/tumor-tritao-maligno
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Tumor tritão maligno
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Dado público estruturado
- fonte: Wikidata
