Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23
Tumor-induced osteomalacia (TIO) is a rare disease characterized by an overproduction of fibroblast growth factor 23 (FGF 23) by tumors,hypophosphatemia secondary to phosphaturia, and an alteration in the active vitamin D synthesis. This causes osteomalacia, bone pain, fractures,muscle weakness, and...
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Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2019
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| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/25539 |
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I10-R327-article-25539 |
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| institution |
Universidad Nacional de Córdoba |
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I-10 |
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R-327 |
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Revista de la Facultad de Ciencias Médicas de Córdoba |
| language |
Español |
| format |
Artículo revista |
| topic |
tumor-induced osteomalacia hypophosphatemia fgf23 burosumab osteomalacia inducida por tumor hipofosfatemia fgf23 burosumab |
| spellingShingle |
tumor-induced osteomalacia hypophosphatemia fgf23 burosumab osteomalacia inducida por tumor hipofosfatemia fgf23 burosumab Salica, Daniel Salica, Daniel García, A García, A Jarchum, G Sánchez , A Sánchez , A Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23 |
| topic_facet |
tumor-induced osteomalacia hypophosphatemia fgf23 burosumab osteomalacia inducida por tumor hipofosfatemia fgf23 burosumab |
| author |
Salica, Daniel Salica, Daniel García, A García, A Jarchum, G Sánchez , A Sánchez , A |
| author_facet |
Salica, Daniel Salica, Daniel García, A García, A Jarchum, G Sánchez , A Sánchez , A |
| author_sort |
Salica, Daniel |
| title |
Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23 |
| title_short |
Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23 |
| title_full |
Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23 |
| title_fullStr |
Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23 |
| title_full_unstemmed |
Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23 |
| title_sort |
osteomalacia inducida por tumor en inicio de tratamiento específico con burosumab anticuerpo monoclonal anti fgf23 |
| description |
Tumor-induced osteomalacia (TIO) is a rare disease characterized by an overproduction of fibroblast growth factor 23 (FGF 23) by tumors,hypophosphatemia secondary to phosphaturia, and an alteration in the active vitamin D synthesis. This causes osteomalacia, bone pain, fractures,muscle weakness, and fatigue. When mesenchymal tumors secretory of FGF23 are completely resected, disease is cured. Medical treatment involvesphosphorus salts and calcitriol. Treatment with burosumab, monoclonal antibody anti-FGF23, is in phase II.Case report: Woman of 50 yrs. old, with evolution of the disease of more than 14 yrs., with progressive disabling symptomatology of musculoskeletalpain, functional muscle deficit, multiple fractures in spine, humerus, and shoulder, and total hip arthroplasty (right and left). Musculoskeletal pain isresistant to the analgesic. At every moment, hyperphosphaturia and hypophosphatemia (1,2 mg/dl average) did not produce a significant therapeuticresponse to the phosphorus salts and gastric intolerance to the salts, which led to interruptions and modifications of the dose depending on tolerance.The patient was also treated with calcium and calcitriol. Serum FGF-23 levels were 1102,56 pg/dl (VN: 0-134 pg/dl). Besides, a secondaryhyperparathyroidism with neither hypercalcemia nor hypercalciuria was confirmed. In 2008 a tumor was removed from left ischio-pubian region, whoseanatomopathological study showed fibromuscular and adipose fragments, and bone fragments with pathological diagnose compatible with fibrousdysplasia. After surgery, the patient remained asymptomatic for about 3 yrs. Afterwards, progressively and uninterruptedly, TIO reappeared. Numerousstudies were carried out to localize the tumor responsible for the TIO, which included biopsies of suspicious areas, with a negative result.Patient with serious tumor-induced osteomalacia, of long-term evolution, with an unsatisfactory response to conventional treatment at the beginning oftreatment with burosumab. Up to the present, this constitutes the first and only case of tumor-induced osteomalacia in the world, which has begun to betreated with burosumab, without an investigation protocol.
Keywords: tumor-induced osteomalacia; hypophosphatemia; fgf23; burosumab |
| publisher |
Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2019 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/25539 |
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I10-R327-article-255392024-08-27T18:25:51Z Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23 Salica, Daniel Salica, Daniel García, A García, A Jarchum, G Sánchez , A Sánchez , A tumor-induced osteomalacia hypophosphatemia fgf23 burosumab osteomalacia inducida por tumor hipofosfatemia fgf23 burosumab Tumor-induced osteomalacia (TIO) is a rare disease characterized by an overproduction of fibroblast growth factor 23 (FGF 23) by tumors,hypophosphatemia secondary to phosphaturia, and an alteration in the active vitamin D synthesis. This causes osteomalacia, bone pain, fractures,muscle weakness, and fatigue. When mesenchymal tumors secretory of FGF23 are completely resected, disease is cured. Medical treatment involvesphosphorus salts and calcitriol. Treatment with burosumab, monoclonal antibody anti-FGF23, is in phase II.Case report: Woman of 50 yrs. old, with evolution of the disease of more than 14 yrs., with progressive disabling symptomatology of musculoskeletalpain, functional muscle deficit, multiple fractures in spine, humerus, and shoulder, and total hip arthroplasty (right and left). Musculoskeletal pain isresistant to the analgesic. At every moment, hyperphosphaturia and hypophosphatemia (1,2 mg/dl average) did not produce a significant therapeuticresponse to the phosphorus salts and gastric intolerance to the salts, which led to interruptions and modifications of the dose depending on tolerance.The patient was also treated with calcium and calcitriol. Serum FGF-23 levels were 1102,56 pg/dl (VN: 0-134 pg/dl). Besides, a secondaryhyperparathyroidism with neither hypercalcemia nor hypercalciuria was confirmed. In 2008 a tumor was removed from left ischio-pubian region, whoseanatomopathological study showed fibromuscular and adipose fragments, and bone fragments with pathological diagnose compatible with fibrousdysplasia. After surgery, the patient remained asymptomatic for about 3 yrs. Afterwards, progressively and uninterruptedly, TIO reappeared. Numerousstudies were carried out to localize the tumor responsible for the TIO, which included biopsies of suspicious areas, with a negative result.Patient with serious tumor-induced osteomalacia, of long-term evolution, with an unsatisfactory response to conventional treatment at the beginning oftreatment with burosumab. Up to the present, this constitutes the first and only case of tumor-induced osteomalacia in the world, which has begun to betreated with burosumab, without an investigation protocol. Keywords: tumor-induced osteomalacia; hypophosphatemia; fgf23; burosumab ntroducción: La Osteomalacia Inducida por Tumor (OIT) es una enfermedad rara, caracterizada por excesiva producción de factor de crecimiento fibroblástico 23 (FGF 23) de origen tumoral, hipofosfatemia secundaria a fosfaturia y alteración de la síntesis de la vitamina D en su forma activa. Esto produce osteomalacia, dolor óseo, fracturas, debilidad muscular y fatiga. Cuando los tumores mesenquimatosos secretores de FGF23 son resecados totalmente, se logra la curación. El tratamiento médico radica en sales de fósforo y calcitriol. Se encuentra en fase II el tratamiento con burosumab, anticuerpo monoclonal anti-FGF23. Caso Clínico: mujer de 50 años de edad, con evolución de más de 14 años, con sintomatología progresiva invalidante de dolores osteomusculares, déficit funcional muscular, fracturas múltiples a nivel vertebral, húmero y hombro, con reemplazo total de ambas caderas Los dolores osteomusculares son refractarios a la medicación analgésica. En todo momento hiperfosfaturia e hipofosfatemia (promedio 1,2 mg/dl), con escasa respuesta terapéutica a las sales de fósforo, e intolerancia gástrica a las mismas, lo que provocó interrupciones y modificaciones de dosis según tolerancia. También tratada con calcio y calcitriol. El FGF 23, mostró valores séricos de 1102,56 pg/dl (VN: 0-134 pg/dl). Además se constató respuesta hiperparatiroidea secundaria sin hipercalcemia ni hipercalcuria. En el 2008 se extirpó tumor de región isquiopubiana izquierda, cuyo estudio anatomopatológico informó fragmentos fibromusculoadiposos y fragmentos óseos con diagnóstico patológico compatible con displasia fibrosa. Después de la cirugía la paciente permaneció asintomática y recuperó valores a la normalidad de laboratorio, durante unos 3 años, tras lo cual reapareció progresiva e ininterrumpidamente el cuadro de OIT, y permanece así hasta el presente. Se realizaron numerosos estudios de localización del tumor generador del cuadro, incluyendo biopsias de sitios sospechosos, con resultado negativo. Conclusión: paciente con severa OIT en inicio terapéutico con burosumab, resultando el primer caso en el mundo de OIT tratado con burosumab fuera de un protocolo de investigación. Palabras Clave: osteomalacia inducida por tumor; hipofosfatemia; fgf23; burosumab Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2019-10-01 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/25539 Revista de la Facultad de Ciencias Médicas de Córdoba.; 2019: Suplemento JIC XX Revista de la Facultad de Ciencias Médicas de Córdoba; 2019: Suplemento JIC XX Revista da Faculdade de Ciências Médicas de Córdoba; 2019: Suplemento JIC XX 1853-0605 0014-6722 10.31053/1853.0605.v76.nSuplemento spa https://revistas.unc.edu.ar/index.php/med/article/view/25539/27347 Derechos de autor 2019 Universidad Nacional de Córdoba https://creativecommons.org/licenses/by-nc/4.0 |