Radiocirugía cerebral estereotáctica en el tratamiento de tumores de base de cráneo / Stereotactic brain radiosurgery in the treatment of skull base tumors

La radiocirugía estereotáctica ha demostrado efectividad en el tratamiento multimodal de los tumores de base de cráneo, de todas maneras se sugiere que en áreas radiobioló;gicamente sensibles (ej. nervios ó;pticos y tronco cerebral) la dosis radiante debería reducirse por los efectos radiotó;xicos. Para evitar la ineficacia terapó;©utica, una alternativa recientemente implementada es la radiocirugía multisesió;n (RCH). Se analizó; en forma retrospectiva una serie de tumores de base de cráneo, con el fin de evaluar el tratamiento con RCH a travó;©s del control tumoral en las imágenes post-tratamiento. Se evaluó; tambín edad, sexo, histología tumoral, volumen tumoral, protocolo radiante, Karnofsky performance status (KPS) pre-tratamiento y neurocirugía y tratamiento radiante previos. Fueron tratados 84 pacientes entre enero 2009 y enero 2017. La mediana de edad fue 51.5 aó;±os; sexo femenino: 53.6%. Hubo un 92.7% de no-progresió;n luego del tratamiento, con una mediana de tiempo de seguimiento de 36 meses. Los tumores tratados fueron principalmente adenomas hipofisarios, neurinomas del acó;ºstico, y meningiomas de base de cráneo. La mayoría de los pacientes recibió; un esquema de tratamiento fraccionado de 5 días, con una dosis total de 25 Gy. No se observó; radiotoxicidad tardía clínicamente manifiesta. En el análisis multivariado, un KPS alto pre-tratamiento fue significativamente asociado a la no-progresió;n tumoral. En esta serie, la alta incidencia de no-progresió;n tumoral indicaría que el tratamiento con RCH podría ser una opció;n terapó;©utica en algunos casos de tumores de base de cráneo, principalmente recurrencias o remanencias tumorales de adenomas hipofisarios, neurinomas y meningiomas.

Stereotactic radiosurgery has shown effectiveness in the multimodal treatment of skull base tumors, however it is suggested that in radiobiologically sensitive areas (eg. optic nerves and brainstem) the radiation dose should be reduced due to radiotoxic effects. To avoid the consequent therapeutic ineffectiveness, a recently implemented alternative is multisession radiosurgery (RCH). We retrospectively analyzed a series of patients with skull base tumors, in order to evaluate the treatment with RCH through tumor control in the post-treatment images. Age, sex, tumor histology, tumor volume, radiation protocol, pre-treatment Karnofsky performance status (KPS) previous neurosurgery and radiant treatment were also evaluated. Eighty-four patients were treated between January 2009 and January 2017. The median age was 51.5 years; females: 53.6%. There was a 92.7% non-progression after treatment, with a median follow-up time of 36 months. Treated tumors were mainly pituitary adenomas, acoustic schwannomas, and skull base meningiomas. Most of the patients received a 5-day fractionated treatment scheme, with a total dose of 25 Gy. No clinically manifest late radiotoxicity was observed. In the multivariate analysis, a high pre-treatment KPS was significantly associated with tumor non-progression. In our series, the high incidence of tumor non-progression would indicate that treatment with RCH could be a therapeutic option in some cases of skull-base tumors, mainly recurrences or tumor residuals of pituitary adenomas, neurinomas and meningiomas.

Olfactory Groove Schwannoma ­ A Case Report and Literature Review

Olfactory groove schwannomas (OGSs) are rare benign tumors of the anterior skull base region. Considering the lack of Schwann cells in the optic and olfactory nerves, their origin remains enigmatic. Despite the precursor cell, total resection of the lesion is curative, as long as the histopathological features of the tumor are compatible with schwannoma. We report the case of a 32-year-old woman, addicted to crack, who was brought to the hospital presenting with cognitive dysfunction after being physically assaulted, whose neuroimaging revealed a large extra-axial mass in the subfrontal sagittal region. The presentation, immunohistochemical markers and histogenesis are discussed in the present study, along with a literature review.

Biopsia endoscópica de osteocondroma de fosa infratemporal / Endoscopic biopsy of infratemporal fossa osteochondroma

Presentamos el caso de una mujer de 13 años con un gran tumor de características óseas en la fosa infratemporal derecha, el cual fue biopsiado mediante un abordaje endoscópico transeptal transpterigoídeo. La biospia mostró un osteocondroma. Describimos el caso y discutimos sus aspectos relevantes.

We report the case of a 13-year-old woman with a large tumor with osseous appearance in her right infratemporal fossa, which was biopsied through an endoscopic transpterygoid approach. The biopsy showed an osteocondroma. We described the case and discuss its relevant aspects.

Correlation between C-Reactive Protein in Peripheral Vein and Coronary Sinus in Stable and Unstable Angina / Correlação entre Proteína C-Reativa em Veia Periférica e Seio Coronário na Angina Estável e Instável

Background: High sensitivity C-reactive protein (hs-CRP) is commonly used in clinical practice to assess cardiovascular risk. However, a correlation has not yet been established between the absolute levels of peripheral and central hs-CRP. Objective: To assess the correlation between serum hs-CRP levels (mg/L) in a peripheral vein in the left forearm (LFPV) with those in the coronary sinus (CS) of patients with coronary artery disease (CAD) and a diagnosis of stable angina (SA) or unstable angina (UA). Methods: This observational, descriptive, and cross-sectional study was conducted at the Instituto do Coração, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, and at the Hospital Beneficência Portuguesa de Sao Paulo, where CAD patients referred to the hospital for coronary angiography were evaluated. Results: Forty patients with CAD (20 with SA and 20 with UA) were included in the study. Blood samples from LFPV and CS were collected before coronary angiography. Furthermore, analysis of the correlation between serum levels of hs-CRP in LFPV versus CS showed a strong linear correlation for both SA (r = 0.993, p < 0.001) and UA (r = 0.976, p < 0.001) and for the entire sample (r = 0.985, p < 0.001). Conclusion: Our data suggest a strong linear correlation between hs-CRP levels in LFPV versus CS in patients with SA and UA. .

Fundamento: A proteína C-reativa de alta sensibilidade (PCR-as) é comumente utilizada na prática clínica para avaliar o risco cardiovascular. Entretanto, a correlação entre os níveis séricos de PCR-as (valores absolutos) periférico versus central ainda não foi feita. Objetivo: Avaliar a correlação entre os níveis séricos de PCR-as (mg/L) em veia periférica do antebraço esquerdo (VPAE) versus seio coronário (SC), em pacientes portadores de doença arterial coronária (DAC) com diagnóstico de angina estável (AE) ou angina instável (AI). Métodos: Estudo observacional, descritivo, transversal, realizado no Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo e no Hospital Beneficência Portuguesa de São Paulo, onde foram avaliados os pacientes encaminhados ao hospital com DAC para angiografia coronária. Resultados: Quarenta pacientes com DAC (20 AE e 20 AI) foram incluídos no estudo. Amostras de sangue na VPAE e SC foram coletadas simultaneamente antes da angiografia coronária. A análise de correlação entre os níveis séricos de PCR-as em VPAE versus SC mostrou forte correlação linear tanto para AE (r = 0,993, p < 0,001) como para AI (r = 0,976, p < 0,001) e em toda a amostra (r = 0,985, p < 0,001). Conclusão: Nossos dados sugeriram forte correlação linear entre os níveis de PCR-as em VPAE versus SC na AE e AI. .

Head and neck paragangliomas: clinical and molecular genetic classification

Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I-III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.

The outcome of surgical treatment for tumors of the craniocervical junction.

OBJECTIVE: The authors report the clinical, radiological, and surgicalfindings ofpatients with craniocervical junction tumors surgically treated in the institution over the last 8 years. MATERIAL AND METHOD: A retrospective study was performed. Clinical, radiological, and operative data were evaluated, and follow-up information was obtained from outpatient examinations, and telephone interviews. RESULTS: There were 25 patients consisting of nine chordomas, eight meningiomas, three cysts, two schwannomas, one each of aneurysmal bone cyst, plasmacytoma, and metastasis. Twenty-nine operative procedures were performed, classified as 12 anterior nine posterior-lateral, and eight posterior approaches. Gross total removal was achieved in 17 cases, subtotal removal in six cases, and partial removal in two cases. Re-operation was performed in six cases. Median follow-up time was 31 months. The authors found significant improvement in Karnofsky Performance Scale scores. CONCLUSION: Appropriate surgical approaches provide successful tumor removal with less surgical morbidities, nevertheless recurrent tumors occasionally occur and so, long-term follow-up is mandatory.

Craniospinal dissemination of clival chondroid chordoma.

Chondroid chordoma commonly presents as clival osseous and extradural mass. A 15-year-old boy presented with progressive visual deficit, headaches and diplopia since three years. Computed tomography (CT scan) showed a skull base tumour, but was wrongly reported at the time as chronic sphenoidal sinusitis and nasal polyps. In the past three months, he developed dysphagia, urinary retention and constipation. Terminally, he had weakness of all limbs. Fundoscopy showed optic atrophy. Temporal and spatial variation in symptoms led to a clinical diagnosis of multiple sclerosis with optic neuritis. Partial brain autopsy revealed small gelatinous tumour nodules in the subarachnoid space of middle cranial fossa encasing base of brain like arachnoiditis. Tumour deposits extended down into the spinal cord along the subarachnoid space as far as vision allowed. Histopathology and immunohistochemistry confirmed a diagnosis of chondroid chordoma. Awareness of this rare mode of dissemination will avoid misdiagnosis and delay in treatment.

Giant pituitary adenoma invading the clivus.

A 26-year-old man was admitted with complaints of worsening of vision for one month. Investigations revealed a large sellar and parasellar tumor mass invading into both the cavernous sinuses. The tumor invaded the clivus and both petrous apices. It was radically but partially resected through a transsphenoidal approach. Following surgery, the patient showed symptomatic improvement. The residual tumor was treated by radiation treatment. The case is discussed and the literature on the subject is briefly reviewed.

Extranasal glial heterotopia: case report.

Glial heterotopia or the occurrence of isolated non-teratomatous extracranial glial tissue is rare. We report a neonate with extensive extranasal glial heterotopia involving the left buccopharyngeal region, palate and base of the skull and presenting with respiratory distress and a bleeding oral mass. A staged operative approach was adopted to excise the lesion. The literature on the subject is briefly reviewed.

Pure extradural approach for skull base lesions.

Lesions in the parasellar and paracavernous regions can be removed by various skull base approaches involving basal osteotomies. A major complication of intradural skull base approaches is CSF leak and associated meningitis. We have managed 5 patients with skull base lesions with a pure extradural approach using wide basal osteotomies. The operative techniques are described.

Osteocondroma da foice: relato de caso e revisäo da literatura / Falx osteochondroma: case report and review of the literature

Descrevemos o caso de um paciente com osteocondroma intracraniano originado da foice cerebral. O tumor foi parcialmente ressecado. Discutimos os condromas da foice, em particular quanto a seus aspectos cirúrgicos e etiopatogênicos, comparados com a literatura e ressaltando o valor diagnóstico da tomografia computadorizada e da ressonância magnética.