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Primary Intracranial Extraskeletal Mesenchymal Chondrosarcoma: Clinical Mimicry as Glomus Jugulare

Background: Extraskeletal mesenchymal chondrosarcoma (ESMCS) is an unusual pathologic variant of chondrosarcoma. There are no specific clinicoradiographic parameters to distinguish it from other intracranial pathologies. The diagnosis can be established only on the basis of histopathology, which may pose significant challenges in certain unusual locations. Purpose: In this case, we discuss the pitfalls in diagnosis, management, and major characteristics of ESMCS with a review of current literature. Methods and Results: A 47-year-old female patient presented with a jugular foramen tumor which was misdiagnosed as glomus jugulare tumor for which she received primary gamma knife radiosurgery at 2 instances. But unfortunately, the patient died because of the increase in size of the lesion associated with necrosis. Autopsy analysis revealed a highly cellular tumor, rich in sarcomatous cells, and well-differentiated cartilages consistent with ESMCS. Conclusion: A definite preoperative diagnosis of ESMCS is crucial though difficult especially when located at complex sites like jugular foramen and clinicoradiological mimicry. The most crucial step in the management of ESMCS is accurate diagnosis with critical evaluation of clinical, radiological, and histopathological parameters and realization of highly variable clinical course of the disease.