Malignant triton tumor of the lower extremity with a history of fracture / Tumor «tritón» maligno de la extremidad inferior con antecedentes de fractura
Rev. esp. patol
; 57(1): 59-63, ene.-mar. 2024. ilus
Article
in English
| IBECS
| ID: ibc-EMG-543
Responsible library:
ES1.1
Localization: ES15.1 - BNCS
ABSTRACT
Introduction Malignant triton tumor (MTT) is a rare and aggressive subtype of malignant peripheral nerve sheath tumor consisting of a neurogenic tumor with rhabdomyoblastic differentiation. Only 170 cases have been reported to date, two-thirds occurring in young patients with neurofibromatosis type 1 and the remaining third presenting as a sporadic tumor. Case presentation We present the case of a 49-year-old man with a sporadic grade 2 MTT of the lower limb which had had a previous tibial fracture. The patient underwent an above-knee amputation. Five months post-operatively metastases were present in the liver and vertebral column causing compression of the spinal cord, so decompressive radiotherapy and palliative chemotherapy were initiated. Conclusion Due to the precocious spread of the disease, we would suggest that adjuvant chemotherapy be considered for the eradication of micrometastases. To our knowledge, this is only the second reported case of an MTT arising in a site with a history of previous severe trauma. (AU)
RESUMEN
Introducción El tumor tritón maligno (MTT) es un subtipo raro y agresivo de tumor maligno de la vaina del nervio periférico que consiste en un tumor neurogénico con diferenciación rabdomioblástica. Hasta la fecha solo se han descrito 170 casos, dos tercios de ellos en pacientes jóvenes con neurofibromatosis tipo 1 y el tercio restante como tumor esporádico. Presentación del caso Presentamos el caso de un varón de 49 años con un MTT esporádico de grado 2 de la extremidad inferior que había tenido una fractura tibial previa. El paciente fue sometido a una amputación por encima de la rodilla. A los 5 meses del postoperatorio presentaba metástasis en el hígado y en la columna vertebral que causaban compresión de la médula espinal, por lo que se inició radioterapia descompresiva y quimioterapia paliativa. Conclusión Debido a la diseminación precoz de la enfermedad, sugerimos que se considere la quimioterapia adyuvante para la erradicación de las micrometástasis. Hasta donde sabemos, este es solo el segundo caso descrito de un MTT surgido en un lugar con antecedentes de traumatismo grave previo. (AU)
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Full text:
Available
Collection:
National databases
/
Spain
Health context:
Sustainable Health Agenda for the Americas
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SDG3 - Health and Well-Being
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Neglected Diseases
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SDG3 - Target 3.3 End transmission of communicable diseases
Health problem:
Goal 1 Equitable access to health services
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Target 3.8 Achieve universal access to health
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Target 3.3: End transmission of communicable diseases
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Neglected Diseases
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Tuberculosis
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Tuberculosis
Database:
IBECS
Main subject:
Neurofibrosarcoma
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Orthopedic Procedures
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Anxiety
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Osteitis
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Patient Isolation
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Precancerous Conditions
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Health Personnel
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Coronavirus Infections
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Community Participation
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Health Status Disparities
Type of study:
Controlled clinical trial
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Observational study
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Prevalence study
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Prognostic study
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Risk factors
Aspects:
Social determinants of health
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Equity and inequality
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Patient-preference
Limits:
Humans
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Male
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Female
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Adult
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Aged
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Adolescent
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Child
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Child, preschool
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Infant
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Infant, Newborn
Country/Region as subject:
Europa
Language:
English
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Spanish
Journal:
Rev. esp. patol
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An. pediatr. (2003. Ed. impr.)
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Arch. bronconeumol. (Ed. impr.)
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Enferm. glob
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Gac. sanit. (Barc., Ed. impr.)
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Med. oral patol. oral cir. bucal (Internet)
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Rev. fitoter
Year:
2022
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2023
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2024
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2021
Document type:
Article
Institution/Affiliation country:
Centro Hospitalar Universitário de Santo António/Portugal
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Agència de Salut Pública de Barcelona/Spain
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Hospital General de Gran Canaria Dr. Negrín/Spain
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Hospital Regional Universitario de Málaga/España
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Hospital Universitario San Agustín/Spain
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Hospital Universitario Virgen del Rocío/Spain
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SEPAR (Sociedad Española de Neumología y Cirugía Torácica)/Spain
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Universidad César Vallejo/Perú
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Universidad Privada del Norte/Perú
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University of Palermo/Italy