Multifocal Dura-Based Inflammatory Myofibroblastic Tumor with ALK Rearrangement: a Case Report and Review of Literature

Introdução: Tumor miofibroblástico inflamatório (TMI) é uma neoplasia distinta, descrita especialmente em pacientes jovens. Apesar de a cavidade abdominal ser o sítio de envolvimento mais frequente, localizações pouco usuais, como o sistema nervoso central (SNC), têm sido descritas na literatura. Desde as primeiras descrições de rearranjo de ALK em IMT, há sugestão de que casos previamente relatados como pseudotumores inflamatórios, na verdade, representam uma condição neoplásica, e que essa assinatura molecular pode estar relacionada a comportamento clínico mais agressivo. Relato de Caso: Apresentamos um caso de TMI com rearranjo do ALK em dura-máter, com descrição de suas características patológicas e moleculares. Discussão: O objetivo principal é descrever o TMI e apresentar uma revisão de literatura compreensível em relação a essa neoplasia em SNC, com suas implicações diagnósticas, prognósticas e terapêuticas. Conclusão: TMI com rearranjo de ALK é descrito na literatura e pode estar relacionado a comportamento mais agressivo e multifocalidade à apresentação.

Introduction: Inflammatory myofibroblastic tumor (IMT) is a distinctive neoplasm described especially in young patients. Although abdominal cavity is the most common site of involvement, unusual locations, such as the central nervous system (CNS), have been reported in the literature. Since the first descriptions of ALK rearrangements in IMT, it has been suggested that these cases, previously related as inflammatory pseudotumors, actually represent a neoplastic condition, and this molecular feature might be related to a more aggressive clinical course. Case Presentation: We present a case of multifocal dura-based ALK rearranged IMT with description of its pathological and molecular features. Discussion: We focused on describing IMT and presented a comprehensive review of the literature so far, regarding these tumors at the CNS and its diagnostic, prognostic and therapeutical implications. Conclusion: IMT with ALK rearrangement is also reported at the CNS and may be related to a more aggressive/multifocal presentation.

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