Aggressive papillary tumor of endolymphatic sac: case report of a rare neoplasia / Tumor papilar agressivo do saco endolinfático: relato de caso de uma rara neoplasia

ABSTRACT Aggressive papillary endolymphatic sac tumor (ELST) is a rare neoplasm, occasionally related to von Hippel-Lindau disease, characterized by locally aggressive growth with temporal bone destruction. The authors report a case of ELST in a female patient exhibiting fifth through eighth cranial nerve paralysis. Computed tomography (CT) revealed a large lytic process involving the right temporal bone. The patient underwent surgical resection. At microscopy, a neoplastic process was identified exhibiting monomorphic columnar cells with mild atypias, arranged in a papillary pattern. The lesion exhibited positivity for A31/AE3, epithelial membrane antigen (EMA), and vimentin; and negativity for synaptophysin, glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE), thyroglobulin, transthyretin, chromogranin, thyroid transcription factor 1 (TTF-1), trans-acting T-cell specific transcription factor GATA-3, and intestinal transcription factor CDX-2. The diagnosis of ELST was then established. Six years after surgical resection, lesion recurrence was observed.

RESUMO O tumor papilar agressivo do saco endolinfático (TPASE) é uma neoplasia rara, ocasionalmente relacionada com a doença de von Hippel-Lindau, que se caracteriza pelo crescimento agressivo local com destruição do osso temporal. Os autores relatam um caso de TPASE em paciente do sexo feminino, exibindo paralisia do quinto ao oitavo par craniano. A tomografia computadorizada (TC) revelou grande processo lítico comprometendo o osso temporal direito. A paciente foi submetida a ressecção cirúrgica. À microscopia, identificou-se processo neoplásico que exibiu células cilíndricas monomórficas com atipias leves, dispostas em padrão papilar. A lesão apresentou positividade para AE1/ AE3, antígeno da membrana epitelial (EMA) e vimentina; e negatividade para sinaptofisina, proteína ácida fibrilar glial (GFAP), enolase específica do neurônio (NSE), tireoglobulina, transtirretina, cromogranina, fator de transcrição da tireoide 1 (TTF-1), fator de transcrição de ação trans específico de células GATA-3 e fator de transcrição intestinal CDX-2. O diagnóstico de TPASE foi então estabelecido. Após seis anos da ressecção cirúrgica, foi identificada recorrência da lesão.

Reproducibility of quantitative fiber tracking measurements in diffusion tensor imaging of frontal lobe tracts: A protocol based on the fiber dissection technique.

BACKGROUND: Diffusion tensor imaging (DTI)-based tractography is a noninvasive in vivo method for tracing white matter bundles. This raises possibilities for qualitative and quantitative assessment of the structural organization of tracts. Nevertheless, questions remain about neuroanatomical accuracy, reproducibility for clinical purposes, and accessibility of the best method for broader application. The aim of this study was to combine the fiber dissection technique and tractography to provide more pertinent insight into brain anatomy and, as a result, to test a protocol for reconstruction of six major frontal lobe tracts. METHODS: A combination of fiber dissection of formalin-fixed brain tissue after freezing (Klingler's technique) and virtual dissection (tractography) was used to develop a protocol to reconstruct major frontal tracts. Apparent diffusion coefficient (ADC), fractional anisotropy (FA), number of voxels (NVO), volume (VOL), number (NTR), and length (LEN) of tracts were evaluated to assess intra- and interobserver reproducibility. Statistical reliability was evaluated using intraclass correlation coefficients (ICCs) and the Pearson association coefficient (r). RESULTS: The virtual dissection obtained by tractography seemed to reproduce the anatomic knowledge of the white matter tracts obtained through the classic method. In reliability study, most ICC and r values corresponded at least to large correlation. The magnitude of correlation was very high (ICC 0.7-0.9) or almost perfect (ICC 0.9-1.0) for the FA and ADC measures of every tract studied. CONCLUSION: The DTI protocol proposed herein provided a reliable method for analysis of reconstructed frontal lobe tracts, especially for the FA and ADC variables.

Primary Ewings sarcoma of the spine: case report.

Primary malignant sarcomas of the spine are extremely rare. Because of biological heterogeneity, these tumors have variable sensitivity to radiation and chemotherapy. Adequate local control through complete tumor removal is an important therapeutic goal. However, aggressive resection of tumors in the spinal column must be coupled with restoration of spinal column stability and minimization of neural deficits. The balance of these factors makes treatment of primary sarcomas of the spine challenging, and dictates an individual approach to treatment. We report on a 18 years old man with primary Ewing's sarcoma of the nonsacral spine. The clinical picture and imaging characteristics were analyzed as well as the management modalities and outcome.

Primary Ewing's sarcoma of the spine: case report / Sarcoma de Ewing primário da coluna vertebral: relato de caso

Primary malignant sarcomas of the spine are extremely rare. Because of biological heterogeneity, these tumors have variable sensitivity to radiation and chemotherapy. Adequate local control through complete tumor removal is an important therapeutic goal. However, aggressive resection of tumors in the spinal column must be coupled with restoration of spinal column stability and minimization of neural deficits. The balance of these factors makes treatment of primary sarcomas of the spine challenging, and dictates an individual approach to treatment. We report on a 18 years old man with primary Ewing's sarcoma of the nonsacral spine. The clinical picture and imaging characteristics were analyzed as well as the management modalities and outcome.

Sarcomas malignos primários da coluna são extremamente raros. Devido sua heterogeneidade biológica, estes tumores apresentam sensibilidade variada à radioterapia e à quimioterapia.O controle local adequado através da ressecção tumoral completa é um objetivo terapêutico importante. Contudo, a ressecção completa dos tumores da coluna vertebral deve ser realizada com a restauração da estabilidade espinhal e minimização do déficit neurológico. O equilíbrio entre estes fatores torna o tratamento dos sarcomas da coluna desafiador, exigindo-se uma abordagem individualizada para o tratamento de cada paciente. Relatamos um caso de sarcoma de Ewing da coluna não-sacral em um homem de 18 anos. O quadro clínico e características de imagem foram analisados, bem como a modalidades de manejo e o prognóstico.

Adamantinoma of the spine: case report.

OBJECTIVE: We report a patient with a cervicothoracic spinal and a mandibular adamantinoma. Adamantinoma is a rare malignant neoplasm of bone and, to our knowledge, there have been only five cases of spinal adamantinoma reported. The pathogenesis of the adamantinoma, as well as the management of this extremely rare spinal tumor, is reviewed. CLINICAL PRESENTATION: A 55-year-old man was admitted to our service with cervical pain and signs of C8 and T1 radiculopathy. On physical examination, cervical spine deformity, swelling in the left mandible region, and signs of C8 and T1 radiculopathy were observed. Neuroradiology examinations showed an osteolytic mass of the C6, C7, and T1 vertebral bodies, extending into the lateral masses and transverse processes. After surgical procedures, the patient had clinical improvement. INTERVENTION: Corpectomy of C6, C7, and T1 was performed through a cervicothoracic anterior approach. Anterior stabilization of the spine was obtained using an autologous iliac crest graft and osteosynthesis with an anterior plate. On a second procedure, posterior tumor resection and spinal stabilization were performed. After the 1-year follow-up examination, a new anterior procedure was performed because of tumor recidivity and spine instability. CONCLUSION: Adamantinoma, an extremely rare lesion, is a locally aggressive tumor with slow growth and the potential to metastasize. Although it is an extremely rare occurrence in the spine, adamantinoma should be considered on the diagnosis of tumors of the vertebrae. Neuroradiological examinations are not specific in the differentiation of this tumor from other conditions. This fact, coupled with the limited experience that most physicians in general have in dealing with this tumor, makes the diagnosis and treatment of adamantinoma challenging.

Bilateral isodense epidural hematoma: case report.

We present a case of a severe head injured 23 year-old male patient. The initial CT scan disclosed bilateral epidural hematoma, isodense with the brain, thus being a pitfall in diagnosis. Brief case report, image and literature review are presented.