Esthesioneuroblastoma

1. Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro RJ, Brazil. 2. Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil. Mailing address: Dr. Bruno Niemeyer de Freitas Ribeiro. Instituto Estadual do Cérebro Paulo Niemeyer – Departamento de Radiologia. Rua do Rezende, 156, Centro. Rio de Janeiro, RJ, Brazil, 20231-092. E-mail: bruno.niemeyer@ hotmail.com. cades of life, with a slight predilection for males and preferential involvement of the sacrococcygeal region (50%), followed by the spheno-occipital region (35%), cervical spine, and lumbar spine, occurring only rarely in the dorsal spine and posterior mediastinum. Symptoms often appear only after the lesion has reached large proportions, with local invasion affecting neurovascular structures. Local recurrence is common when complete resection was not possible. The differential diagnoses of chordoma include metastases, chondrosarcoma, multiple myeloma, neurogenic tumors, among others. Although imaging methods help delineate the lesion, the diagnosis is made on the basis of the histopathological analysis. On MRI, most chordomas show isointense or hypointense signals in T1-weighted sequences, whereas they show hyperintense signals in T2-weighted and short-tau inversion-recovery sequences, reflecting their high water content, some lesions containing fibrous septa and therefore showing low signal intensity in T2-weighted sequences. Gadolinium contrast enhancement tends to be moderate and heterogeneous. Lesions are often accompanied by bone erosion, which was not observed in the case reported here. Recent studies have highlighted the use of diffusion-weighted imaging in the differentiation between chordomas and chondrosarcomas, reporting that the latter show higher apparent diffusion coefficients. In addition to an unusual site of involvement, our patient presented the peculiarity of a synchronous lesion. Although some authors have reported similar cases, there is no specific criterion for differentiating between a multicentric chordoma and metastatic dissemination. We believe that our case could represent dissemination to the cerebrospinal fluid, because there was involvement of the vertebral canal. The treatment of choice for chordoma is surgical resection with adjuvant radiotherapy, resulting in a disease-free period approximately 2.5 years longer than that achieved after surgical treatment alone. Because chordoma is resistant to conventional radiotherapy, other modalities, such as stereotactic radiosurgery, are used. Chordoma does not respond well to chemotherapy, antitumor activity having been observed, in small studies, only with the use of imatinib mesylate. Albeit rare, a diagnosis of chordoma should be considered in patients with lesions affecting the posterior mediastinum. In addition, the possibility of synchronous lesions should be investigated in such patients.