Co-Occurrence of Pineal-Region and Pituitary-Stalk Hemangioblastomas in a Patient Presenting with Von Hippel-Lindau Disease ­ A Case Report

Introduction Hemangioblastomas of the pineal region or pituitary stalk are extremely rare. Only two cases of hemangioblastomas involving the pineal region have been reported, and four involving the pituitary stalk. The purpose of the present manuscript is to describe an unusual case of supposed hemangioblastoma found concomitantly in the pineal region and pituitary stalk of a patient diagnosed with Von Hippel-Lindau (VHL) disease. Case Report A 35-year-old female patient with a previous diagnosis of VHL complaining of occipital headaches and balance disturbances for three weeks, who previously had a cerebellar hemangioblastoma resected. The visual characteristics of the tumor suggested a friable vascular lesion with a reddish-brown surface, and an incisional biopsy was performed. The tumor consisted of a dense vascular network surrounded by fibrous stroma abundant in reticulin and composed by both fusiform and dispersed xanthomatous cells; the immunohistochemistry was immunopositive for neuronspecific enolase and immunonegative for epithelial membranous antigen. The patient has been monitored closely for 2 years, and the supratentorial masses have not presented any volume alteration. Conclusion This rare association must be taken into account in patients with VHL disease, or at least be suspected in patients who present a thickening of the pituitary stalk and a pineal-region mass. We believe a biopsy of our asymptomatic patient could have been dangerous due to inherent complications like intraoperative bleeding. We recommend close observation of asymptomatic lesions with MRIs every six months or until the lesions become symptomatic. If the pineal-region tumor does become symptomatic, gross resection via a transcallosal approach would be ideal.

Cerebellar hemorrhage as a complication of spine surgery.

BACKGROUND: The association between remote cerebellar hematoma (RCH) and spinal surgery is poorly understood and rarely reported. We present seven cases of RCH after spinal surgery. METHODS: Seven patients were diagnosed with RCH utilizing computed tomography and/or magnetic resonance, between 2012 and 2016. Their clinical presentations, imaging data, treatment modalities, and outcome were analyzed. There were five females and two males with an average age of 55.8 ± 8.4 years. The age of onset ranged from 43 to 67 years and the time to clinical presentation ranged from 3 h to 5 days. Patients presented with: diplopia/strabismus (one patient), dysphagia/urinary incontinence (one patient), respiratory arrest (one patient), meningismus (one patient), and dysarthria (two patients), along with other symptoms/signs. RESULTS: Three patients were successfully managed without surgery, two required external ventricular drainage, and two were treated with posterior fossa decompression plus ventriculostomy. Four patients recovered completely, two showed mild residual deficits at discharge, while one expired 7 days postoperatively. CONCLUSION: RCH is an uncommon and underdiagnosed complication of spine surgery. It should be suspected when intracranial symptoms occur after spinal procedures.

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.

Endoscopic anatomy of sellar region.

The transsphenoidal approach is the preferred access used in surgical treatment of most sellar region pathologies. The use of endoscopy is advantageous, and it is considered a good alternative to the traditional microsurgical technique. The purpose of this study is to recognize and describe anatomical variations of the sphenoid sinus and the sellar region, mainly describing the anatomy of the posterior wall of the sphenoid sinus and analyzing intercarotid distances in 3 regions. Thirty sphenoid blocks treated with formaldehyde were injected and dissected. Using endoscopy, anatomical variations were studied and the intercarotid distances were measured at the tuberculum sellae, sellar floor and clivus. The types of sphenoid sinus found were: conchal in 1 (4.76 %), pré-sellar in 2 (9.52 %) and sellar in 19 (85.7 %) specimens. The mean distance found from the sphenoid sinus ostium to the sella turcica was 19 mm (±6.5) mm. The mean intercarotid distances found at the tuberculum sellae, sellar floor and clivus were respectively 13.32, 18.00 and 18.90 mm. Endoscopy, with its magnification and lighting provide a panoramic view of deep fields. The anatomical variations described in this study support the need for a careful evaluation of preoperative images in each case.

Cisto epidermoide da fossa posterior: relato de caso / Epidermoid cyst of the posterior fossa: a case report

Epidermoid cysts of the central nervous system are uncommon conditions, which are frequently located in the cerebellopontine angle and around the pons. They are covered with keratinized squamous epithelium and keratin lamella, which give its contents a soft, white-pearly appearance. Epidermoid cysts are mostly originated from malformations, presumably associated with surface elements of the nervous system ectoderm during the closure of the neural groove or formation of secondary cerebral vesicles. The authors describe a case of epidermoid cyst in the posterior fossa causing hydrocephalus and review morphologic and diagnostic criteria of this lesion.

Os cistos epidermoides do sistema nervoso central (SNC) são condições incomuns, estando localizados mais frequentemente no ângulo pontocerebelar e ao redor da ponte. Eles são revestidos por epitélio escamoso queratinizado e lamelas de queratina, tornando seu conteúdo branco-perolado e pastoso. Os cistos epidermoides são, na maioria das vezes, originados de malformações, possivelmente associados ao entremeio de elementos superficiais do ectoderma do SNC durante o fechamento da placa neural, ou formação das vesículas cerebrais secundárias. Os autores descrevem um caso de cisto epidermoide da fossa posterior determinando hidrocefalia e revisam critérios morfológicos e diagnósticos dessa lesão.

Anatomia microcirúrgica e abordagens ao seio cavernoso: um estudo tridimensional estereoscópico com correlação clínico-radiológica / Microsurgical anatomy and approaches to the cavernous sinus: an stereoscopic 3-D study with clinical-radiological correlations

O seio cavernoso é uma das regiões mais complexas do corpo humano e pode ser acometido por diversas patologias. Atualmente,diversos tipos de tratamento podem ser usados para tratar doenças nesta região, porém o conhecimento anatômico ainda é fundamental para estabelecer o melhor manejo. Nosso objetivo é apresentar a anatomia microcirúrgica do seio cavernoso,as abordagens cirúrgicas (incluindo a abordagem endonasalendoscópica), a correlação radiológica desta anatomia,bem como uma série inicial de casos. As fotos são apresentadas pelo método convencional e anaglífico estereoscópico. São descritas as estruturas neuro vasculares do seio cavernoso bem como suas relações ósseas e durais, as paredes e os triângulos da base do crânio relacionados ao seio cavernoso. As abordagens crânio-órbito zigomática, transpetrosa, zigomática e endonasal endoscópica são apresentadas, sendo as estruturas anatômicas identificadas também nos exames de imagem. Casos ilustrativos ilustram esta anatomia.

The cavernous sinus is one of the most complex regions of thehuman body and can be affected by several diseases. Currently,several types of treatment can be used in the management ofdiseases in this region. The anatomical knowledge is essentialto establish the best management. Our goal is to present themicrosurgical anatomy of the cavernous sinus, its surgical approaches(including the endoscopic endonasal approach), thecorrelation of radiological anatomy, including an initial seriesof cases. The anatomic features are presented by the conventionaland stereoscopic anaglyphic method. The neurovascularstructures of the cavernous sinus and its dural and bony relationships,triangles and the walls of the skull base related toit are presented with. We discuss the cranio-orbital zygomatic,transpetrosal, zygomatic and endonasal endoscopic approaches;MRI studies also show the anatomical structures, includingillustrative cases.

Migraçäo dos sistemas de derivaçäo ventrículoperitoneal para o ventrículo lateral. Relato de caso / Migration of netriculoperitoneal shunt system to left ventricular cavity: a case report

É relatado o caso de uma criança submetida à derivaçäo ventriculoperitoneal em que houve migraçäo de todo o sistema de derivaçäo para a cavidade ventricular. A permanência do corpo estranho colonizado dentro do ventrículo lateral impedia a cura da ventriculite

Saída do cateter de derivaçäo ventrículoperitoneal pelo ânus: relato de caso / Extrusion of ventriculoperitoneal shunt through the anus: a case report

É estudado o caso de um lactente que foi sumetido a tratamento cirúrgico de meningomielocele e derivaçäo ventriculoperitoneal (DVP) para tratamento de hidrocefalia descompensada no período neo-natal. Cinco meses após, ocorreu extrusäo do cateter peritoneal através do ânus. Após sua desconexäo ao nível do pescoço, mediante traçöes suaves e repetidas de sua porçäo extrusa, foi realizada remoçäo do cateter. Essa manobra simples evita a necessidade de cirurgia abdominal para a remoçäo do cateter contaminado na porçäo distal