CII REUNION DE LAS ASOCIACIONES TERRITORIALES DEL … · 2. Perry A, Kurtkaya-Yapicier O,...
Transcript of CII REUNION DE LAS ASOCIACIONES TERRITORIALES DEL … · 2. Perry A, Kurtkaya-Yapicier O,...
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MASA CORTICAL FRONTAL DERECHADr. Pilar I. González Márquez
Nuria Terán
F. Javier Figols
CII REUNION DE LAS
ASOCIACIONES TERRITORIALES DEL
NOROESTE DE LA PENINSULA
IBERICA
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HISTORIA CLINICA
• Varón de 20 años de edad
• Sin AP destacables
• No alergias medicamentosas conocidas
• No hábitos tóxicos
• Enfermedad actual:
“Crisis tónico-clónica generalizada con disestesias en hemicara derecha”
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RM CRANEAL: “lesión mixta intraaxial frontal derecha, intraparenquimatosa, córtico-subcortical con poco edema perilesional y sin efecto expansivo; bien delimitada, con pseudoquistes en su interior
que plantea el diagnóstico diferencial entre tumor disembrioplásico neuroectodérmico (DNET) y displasia cortical focal.”
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CRANIECTOMIA FRONTAL DERECHA + POLECTOMIA
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VIMENTINA
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EMA
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GFAP
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NFGFAP
AML S-100
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DIAGNOSTICO
• Biopsia cerebral (frontal derecha):
– MENINGIOANGIOMATOSIS
– GLIOSIS REACTIVA ACOMPAÑANTE
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MENINGIOANGIOMATOSIS
• Lesión intracraneal rara
• Benigna
• Afecta a la leptomeninge y el córtex
• Aumento de la vascularización con proliferación celular perivascular y gliosisreactiva
• Niños y adultos jóvenes
• Masa temporal / frontal
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MENINGIOANGIOMATOSIS• ESPORÁDICOS:
– Lesión focal, única
– Síntomas: epilepsia, dolor de cabeza, parálisis facial
• NEUROFIBROMATOSIS TIPO 2:– Multifocales (cerebelo)
– Asintomáticos (autopsia)
• OTRAS ASOCIACIONES:– Meningioma
– Malformaciones vasculares
– Oligodendroglioma
– Encefalocele
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MENINGIOANGIOMATOSIS• MACROSCOPICA:
– Leptomeninge y cortical-subcortical– Aumento de la vascularización– Firme, blanquecino– Calcificación, hemorragia, quistes
• HISTOPATOLOGIA:
Engrosamiento leptomeningeo en placa con aumento de la vascularización y proliferación celular fusada perivascular– Celulares (celulares y vasculares: hemorragia)– Fibrocalcificadas
• Cuerpos de psamoma• Áreas fibrocartilaginosas (osificación)
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MENINGIOANGIOMATOSIS
• PATOGENESIS DESCONOCIDA:– Hamartoma (degeneración)– Malformación vascular (induce proliferación
celular)– Meningioma
• Naturaleza celular:– Fibroblástico– Meningotelial– C.pluripotenciales (capuchones aracnoideos)
VIMENTINAEMACD 34
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CD34
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MENINGIOANGIOMATOSIS
• LESIONES ACOMPAÑANTES:– Gliosis reactiva– Degeneración granulovacuolar– Ovillos neurofibrilares (sin depósito de
Amiloide)– Inclusiones neuronales atípicas
• No atipia• No mitosis• No necrosis
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DIAGNOSTICO DIFERENCIAL• MENINGIOMA ANAPLASICO
– Atipia– Mitosis– Necrosis – Crecimiento en nidos
• SDM. STURGE-WEBER– Calcificación distrófica– Angioma venoso leptomeningeo
• MALFORMACION VASCULAR• HAMARTOMA
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MENINGIOANGIOMATOSIS• Lesión benigna
• Baja recurrencia
• TRATAMIENTO: exéresis quirúrgica
SU RECONOCIMIENTO ES IMPORTANTE POR LAS SU RECONOCIMIENTO ES IMPORTANTE POR LAS IMPLICACIONES PRONOSTICAS Y TERAPEUTICASIMPLICACIONES PRONOSTICAS Y TERAPEUTICAS
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BIBLIOGRAFIA1. Omeis I, Hillard VH, Braun A, Benzil DL, Murali R, Harter DH. Meningioangiomatosis associated with
neurofibromatosis: report of two cases in a single family and review of the literature. Surg Neurol. 2006 Jun;65(6):595-603. Review.
2. Perry A, Kurtkaya-Yapicier O, Scheithauer BW, Robinson S, Prayson RA, Kleinschmidt-DeMasters BK, Stemmer-Rachamimov AO, Gutmann DH. Insights into meningioangiomatosis with and without meningioma: a clinicopathologic and genetic serias of 24 cases with review of the literature. Brain Pathol. 2005 Jan;15(1):55-65.
3. Kim WY, Kim IO, Kim S, Cheon JE, Yeon M. Meningioangiomatosis: MR imaging and pathological correlation in two cases. Pediatr Radiol. 2002 Feb;32(2):96-8. Epub 2001 Dec 11.
4. Takeshima Y, Amatya VJ, Nakayori F, Nakano T, Sugiyama K, Inai K. Meningioangiomatosis occurring in a young male without neurofibromatosis: with special reference to its histogenesis and loss of heterozygosity in the NF2 gene region. Am J Surg Pathol. 2002 Jan;26(1):125-9.
5. Park MS, Suh DC, Choi WS, Lee SY, Kang GH. Multifocal meningioangiomatosis: a report of two cases. AJNR Am J Neuroradiol. 1999 Apr;20(4):677-80.
6. Goates JJ, Dickson DW, Horoupian DS. Meningioangiomatosis: an immunocytochemical study. ActaNeuropathol. 1991;82(6):527-32.
7. Suárez-Gauthier A, Gómez de la Bárcena MR, García-García E, Hinojosa J, Ricoy JR. Meningioangiomatosis: report of two cases and literature review. Neurocirugia (Astur). 2006 Jun;17(3):250-4. Spanish.
8. Aizpuru RN, Quencer RM, Norenberg M, Altman N, Smirniotopoulos J. Meningioangiomatosis: clinical, radiologic, and histopathologic correlation. Radiology. 1991 Jun;179(3):819-21.
9. Fujimoto K, Nikaidoh Y, Yuasa T, Nagata K, Ida Y, Fujioka M, Ohnishi H, Kurokawa S. Meningioangiomatosis not associated with von Recklinghausen's disease--case report. Neurol Med Chir(Tokyo). 1993 Sep;33(9):651-5. Review.
10. Wiebe S, Munoz DG, Smith S, Lee DH. Meningioangiomatosis. A comprehensive analysis of clinical and laboratory features. Brain. 1999 Apr;122 ( Pt 4):709-26. Review.
11. Plesec TP, Prayson RA. Frozen section discrepancy in the evaluation of nonneoplastic central nervous system samples. Ann Diagn Pathol. 2009 Dec;13(6):359-66. Epub 2009 Sep 8.
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