Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity

Paragangliomas are rare, benign and slow-growing neuroendocrine tumors that can arise from the adrenal medulla (85­90%) or from the extra-adrenal paraganglia. In the central nervous system (CNS), they can be found at several sites, but more often at the cauda equina and filum terminale region, where they account for between 2.5 and 3.8% of total tumor cases of that region. There are only 8 cases described in the literature that mention the presence of the gangliocytic variant of this entity at the filum terminale. We present the case of a 41-year-old man with chronic lumbar pain refractory to medical treatment, without any associated neurological deficits. Magnetic resonance imaging (MRI) revealed an intradural, extramedullar oval lesion with regular contours and homogeneous caption of contrast at L1 level. He was submitted to surgical treatment, with complete resection of the lesion. The histological analysis revealed a gangliocytic paraganglioma of the filum terminale. At 5 years of follow-up, he remains asymptomatic and without any signs of relapse. These are lesions with an overall good prognosis with gross total resection. Although the recurrence rate is extremely low, prolonged observation is recommended due to the slow-growing nature of the tumor, being estimated that between 1 and 4% can recur even after gross total removal.

Rehabilitation of unilateral peripheral facial paralysis in Guillain-Barré: a case study / Reabilitação de paralisia facial periférica unilateral em Guillain-Barré: estudo de caso

ABSTRACT Facial palsies have multiple etiologies, but have in common the negative impact not only on the functions of the stomatognathic system but also on the self-image and emotional expression. This article aimed to describe a case study of unilateral peripheral facial palsy caused by the Guillain-Barré Syndrome. Hence, it shares assessment and intervention experiences related to this type of change and presents its functional results. It also highlights the important role of the interdisciplinary team (which comprised a physiatrist, physical therapist, speech-language-hearing therapist, occupational therapist, and nurses) to catalyze the patient's evolution and the management of possible complications. Moreover, it is rather important to integrate the patients as members of the rehabilitation team, empowering them and giving them responsibility for the success of the intervention.

RESUMO As paralisias faciais podem ter uma multiplicidade de etiologias, contudo têm em comum o impacto negativo não só para as funções do sistema estomatognático, como também na autoimagem e expressão emocional. O presente artigo tem como objetivo a descrição de um estudo de caso relativo a uma paralisia facial periférica unilateral na sequência de Síndrome de Guillain-Barré. Pretende-se deste modo a partilha de experiência em termos de avaliação e intervenção neste tipo de alterações e a apresentação do respetivo resultado funcional. Ressalta-se, também, o importante papel da equipa interdisciplinar (composta por médico fisiatra, fisioterapeuta, fonoaudiólogo, terapeuta ocupacional e equipa de enfermagem) enquanto catalisador para a evolução e na gestão de eventuais intercorrências. Salienta-se, ainda, a importância da integração do paciente enquanto membro da equipa de reabilitação, empoderando-o e responsabilizando-o pelo sucesso da intervenção.

The Unknown Cause of Pott's Puffy Tumor ­ Importance of Early Diagnose

Pott's puffy tumor (PPT) is characterized by swelling of the glabelar region and osteomyelitis of the frontal bone, owing to a subperiosteal pseudoinflammatory tumor responsible for the detachment of the pericranium from the outer table of the skull. Nowadays, the incidence of PPT is very low, so this entity is frequently underdiagnosed. The late treatment and identification of PPT are strongly associated with intracranial complications, which could jeopardize the life of the patient. In the literature, PPT is described as a complication of frontal head trauma or of chronic sinusitis. There are a few cases reported in patients with frontal insect bites or in recreational nasal drug users, such as cocaine or methamphetamines. In the present case report, the authors describe the case of a 40-year-old male who was submitted to a frontal sebaceous cyst surgery. In the postoperative period, he developed an infectious process compatible with PPT. After an extensive review of the literature, no similar cases were identified. Therefore, in the opinion of the authors, sebaceous cyst surgery should be included in the short list of risk factors for the development of PPT.

Subcutaneous Dissemination in Highly Aggressive Intracranial Glioblastoma: Case Report and Literature Review

Glioblastoma (GBM) is a highly invasive and incurable primary central nervous system (CNS) tumor. Despite its aggressive behavior, extracranial metastases are rare, with an estimated incidence of less than 2%. In our literature review, we found only 21 reported cases of skin and soft tissue dissemination. We report a case of an early (two and a half months) postoperative skin and muscle flap-associated dissemination of a temporal glioblastoma. The particular aspect of this case, besides its rarity, is that the clinical presentation, the image reports and even the surgical findings were always in favor of a postoperative subdural empyema and epicranial abscesses. The diagnosis of soft tissue dissemination was only possible after negative microbiological cultures and histopathological confirmation of muscle and skin invasion by the tumor. This case illustrates the rare but potential risk of myocutaneous flap tumor dissemination through the durotomy/craniotomy site that can mimic a much more common, post-surgical infection.

O glioblastoma é um tumor do sistema nervoso central extremamente invasivo e incurável. Apesar do comportamento agressivo desses tumores, a metastização extracraniana é rara, apresentando uma incidência inferior a 2%. A nossa revisão da literatura revelou apenas 21 casos descritos de disseminação de glioblastoma para a pele e tecidos moles. Nós descrevemos um caso de disseminação precoce de um glioblastoma temporal para o retalho miocutâneo associado à cirurgia, cerca de 2 meses e meio após a intervenção. Além da raridade, este caso é peculiar, uma vez que tanto a forma de apresentação clínica quanto a descrição imagiológica e os achados intraoperatórios foram sempre muito sugestivos de um empiema subdural e de abcessos epicranianos. O diagnóstico definitivo de disseminação tumoral para os tecidos moles apenas foi possível após o resultado negativo das culturas microbiológicas e a confirmação histológica de invasão do músculo e da pele pelo tumor. Este caso ilustra o raro, mas potencial risco de disseminação tumoral de um glioblastoma através do retalho miocutâneo de acesso cirúrgico e do local de craniotomia/durotomia, que se pode facilmente confundir com uma situação mais frequente de infecção após cirurgia.