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Background: Sinonasal neuroendocrine carcinoma is a rare head and neck tumor that represents only 5% of sinonasal neoplasms. This lesion has a high risk of invasion to adjacent structures such as the orbit, skull base, and soft tissues, with symptoms usually being nonspecific. Most cases are diagnosed in late stages, decreasing overall survival without treatment. To date, there is no consensus on management given its low prevalence; however, it has been shown that multimodal therapy, with the correct surgical approach as the mainstay, offers a better disease-free prognosis. Case Description: A 46-year-old woman presented with a 1 year history of nasal symptoms, characterized by obstruction and epistaxis. Imaging studies showed an extra-axial mass causing skull base erosion and displacement of the right fronto-orbital region, without invasion of brain parenchyma or meninges. A biopsy was performed and an unresectable poorly differentiated sinonasal neuroendocrine carcinoma was diagnosed. Treatment with radio and chemotherapy was initiated and, as the tumoral volume decreased, she was referred for neurosurgical intervention; an endonasal endoscopic approach was performed. Gross total resection was achieved and the patient was discharged without postoperative complications and no residual lesion on imaging. Conclusion: We describe the evolution of a rare advanced-stage neoplasm. It highlights that despite receiving an initial diagnosis of an unresectable mass, multimodal therapy, and an adequate surgical approach deemed the entire lesion to be resected. Despite the favorable clinical evolution, the follow-up of neuroendocrine carcinoma is prioritized as a neoplasm with a high rate of recurrence and metastasis.

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Objective: The perfusion profile of vestibular schwannomas (VSs) and the factors that influence it have yet to be determined. Materials and Methods: Twenty patients with sporadic VS were analyzed by calculating parameters related to the extravascular extracellular space (EES)-the volume transfer constant between a vessel and the EES (Ktrans); the EES volume per unit of tissue volume (Ve); and the rate transfer constant between EES and blood plasma (Kep)-as well as the relative cerebral blood volume (rCBV), and by correlating those parameters with the size of the tumor and its structure (solid, cystic, or heterogeneous). Results: Although Ktrans, Ve, and Kep were measurable in all tumors, rCBV was measurable only in large tumors. We detected a positive correlation between Ktrans and rCBV (r = 0.62, p = 0.031), a negative correlation between Ve and Kep (r = -0.51, p = 0.021), and a positive correlation between Ktrans and Ve only in solid VSs (r = 0.64, p = 0.048). Comparing the means for small and large VSs, we found that the former showed lower Ktrans (0.13 vs. 0.029, p < 0.001), higher Kep (0.68 vs. 0.46, p = 0.037), and lower Ve (0.45 vs. 0.83, p < 0.001). The mean Ktrans was lower in the cystic portions of cystic VSs than in their solid portions (0.14 vs. 0.32, p < 0.001), as was the mean Ve (0.37 vs. 0.78, p < 0.001). There were positive correlations between the solid and cystic portions for Ktrans (r = 0.71, p = 0.048) and Kep (r = 0.74, p = 0.037). Conclusion: In VS, tumor size appears to be consistently associated with perfusion values. In cystic VS, the cystic portions seem to have lower Ktrans and Ve than do the solid portions.

Objetivo: O perfil de perfusão do schwannoma vestibular (SV) não tem sido estudado, nem os fatores que o influenciam. Materiais e Métodos: Vinte pacientes com SV esporádico foram analisados usando Ktrans, Ve, Kep e rCBV e correlacionados com tamanho e estádio cístico. Resultados: Ktrans, Ve e Kep foram medidos em todos os casos. rCBV só foi possível em tumores grandes. Ktrans e rCBV estavam correlacionados positivamente (r = 0,62, p = 0, 0 31 ) . Ve e Kep estavam negativamente correlacionados (r = ­0,51, p = 0,021). Ktrans estava correlacionado positivamente com Ve em SVs sólidos (r = 0,64, p = 0,048). Em SVs pequenos, Ktrans foi menor (0,13 vs 0,029, p < 0,001), Kep foi maior (0,68 vs 0,46, p = 0,037) e Ve foi menor (0,45 vs 0,83, p < 0,001) que nos SVs grandes. Ktrans e Ve foram menores dentro dos cistos que nas porções solidas dos SVs císticos (0,14 vs 0,32, p < 0,001; 0,37 vs 0,78, p < 0.001, respectivamente). Foi encontrada correlação positiva em Ktrans (r = 0,71, p = 0,048) e Kep (r = 0,74, p = 0,037) entre as áreas sólidas e císticas. Conclusão: Nos SVs, o tamanho está consistentemente associado com os valores da perfusão. Nos SVs císticos, as porções císticas parecem ter valores menores de Ktrans e Ve do que nas porções sólidas.

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Abstract Objective: The perfusion profile of vestibular schwannomas (VSs) and the factors that influence it have yet to be determined. Materials and Methods: Twenty patients with sporadic VS were analyzed by calculating parameters related to the extravascular extracellular space (EES)—the volume transfer constant between a vessel and the EES (Ktrans); the EES volume per unit of tissue volume (Ve); and the rate transfer constant between EES and blood plasma (Kep)—as well as the relative cerebral blood volume (rCBV), and by correlating those parameters with the size of the tumor and its structure (solid, cystic, or heterogeneous). Results: Although Ktrans, Ve, and Kep were measurable in all tumors, rCBV was measurable only in large tumors. We detected a positive correlation between Ktrans and rCBV (r = 0.62, p = 0.031), a negative correlation between Ve and Kep (r = -0.51, p = 0.021), and a positive correlation between Ktrans and Ve only in solid VSs (r = 0.64, p = 0.048). Comparing the means for small and large VSs, we found that the former showed lower Ktrans (0.13 vs. 0.029, p < 0.001), higher Kep (0.68 vs. 0.46, p = 0.037), and lower Ve (0.45 vs. 0.83, p < 0.001). The mean Ktrans was lower in the cystic portions of cystic VSs than in their solid portions (0.14 vs. 0.32, p < 0.001), as was the mean Ve (0.37 vs. 0.78, p < 0.001). There were positive correlations between the solid and cystic portions for Ktrans (r = 0.71, p = 0.048) and Kep (r = 0.74, p = 0.037). Conclusion: In VS, tumor size appears to be consistently associated with perfusion values. In cystic VS, the cystic portions seem to have lower Ktrans and Ve than do the solid portions.

Resumo Objetivo: O perfil de perfusão do schwannoma vestibular (SV) não tem sido estudado, nem os fatores que o influenciam. Materiais e Métodos: Vinte pacientes com SV esporádico foram analisados usando Ktrans, Ve, Kep e rCBV e correlacionados com tamanho e estádio cístico. Resultados: Ktrans, Ve e Kep foram medidos em todos os casos. rCBV só foi possível em tumores grandes. Ktrans e rCBV estavam correlacionados positivamente (r = 0,62, p = 0, 0 31 ) . Ve e Kep estavam negativamente correlacionados (r = -0,51, p = 0,021). Ktrans estava correlacionado positivamente com Ve em SVs sólidos (r = 0,64, p = 0,048). Em SVs pequenos, Ktrans foi menor (0,13 vs 0,029, p < 0,001), Kep foi maior (0,68 vs 0,46, p = 0,037) e Ve foi menor (0,45 vs 0,83, p < 0,001) que nos SVs grandes. Ktrans e Ve foram menores dentro dos cistos que nas porções solidas dos SVs císticos (0,14 vs 0,32, p < 0,001; 0,37 vs 0,78, p < 0.001, respectivamente). Foi encontrada correlação positiva em Ktrans (r = 0,71, p = 0,048) e Kep (r = 0,74, p = 0,037) entre as áreas sólidas e císticas. Conclusão: Nos SVs, o tamanho está consistentemente associado com os valores da perfusão. Nos SVs císticos, as porções císticas parecem ter valores menores de Ktrans e Ve do que nas porções sólidas.

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Context Tumors of the jugular foramen present a challenge to skull base surgeons. Their rarity, coupled with the complex anatomy of the region require exquisite knowledge and surgical technique. We present the history of the craniocervical approach to the jugular foramen, as well as surgical advice on how to manage the different extensions these tumors may present. The surgical nuances come from the experience of our skull base team managing over 150 tumors of the jugular foramen over the past 30 years. The history of the craniocervical approach was obtained by reviewing articles on surgery of the jugular foramen published on PUBMED over the past 100 years. History The first craniocervical approach may be attributed to Gardner et al. in 1891, with posterior contributions from Shapiro and Neues, Gejrot, Kempe et al, Hilding and Greenberg, and Glasscock. Nuances Cervical dissection with identification of the jugular vein, carotid bifurcation, and IX to XII cranial nerves was performed. Mastoidectomy with exposition of the fallopian canal, labyrinth, middle ear, sigmoid sinus, followed by a trans-sigmoid craniotomy with transposition of the vertebral artery gave access to the temporal and intracranial region. Conclusion Approaching the jugular foramen is the epitome of skull base surgery. Several modifications of the standard approach may be necessary depending on the extension of the tumor.

Contexto Os tumores do forame jugular apresentam um desafio para os cirurgiões da base do crânio. A sua raridade e a anatomia complexa da região requerem conhecimento específico da técnica cirúrgica. O presente manuscrito apresenta a história do acesso crâniocervical ao forame jugular, assim como dicas cirúrgicas para manejar as diferentes extensões que os tumores podem apresentar. As dicas são derivadas da experiência da nossa equipe em mais de 150 tumores do forame jugular nos últimos 30 anos. A história do acesso crâniocervical foi obtida da revisão de artigos em PUBMED dos últimos 100 anos. História A primeira abordagem craniocervical pode ser atribuída a Gardner et al. em 1891, com contribuições posteriores de Shapiro e Neues, Gejrot, Kempe et al, Hilding e Greenberg e Glasscock. Descrição A dissecção cervical necessita identificação da veia jugular, bifurcação carotídea, e dos nervos cranianos de IX a XII. A mastoidectomia com exposição do canal de falópio, labirinto, ouvido médio e seio sigmoide foi seguida por uma craniotomia transsigmoide com transposição da artéria vertebral, permitindo abordar a região temporal e intracraniana. Conclusão Acessar o forame jugular é o epítome da cirurgia da base do crânio. Múltiplas modificações do acesso tradicional podem ser necessárias dependendo da extensão do tumor.

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Introducción: los tumores en la cavidad nasal y los senos paranasales son un problema serio en la población pediátrica, principalmente por la inespecificidad de los síntomas que lleva a que pasen de meses a años antes de la sospecha de una patología neoplásica, con un impacto en la calidad de vida del paciente y su entorno. Al sospechar de la presencia de un tumor nasosinusal se requiere la realización de imágenes diagnósticas como la resonancia magnética y la tomografía computarizada de los senos paranasales. Cuando se tiene un diagnóstico etiológico, siempre se debe realizar un abordaje multidisciplinario. Materiales y métodos: realizamos un estudio retrospectivo de corte transversal de la cohorte de pacientes con tumores de nariz y senos paranasales atendidos en un hospital pediátrico de cuarto nivel en Bogotá, Colombia, entre 2013-2018. Resultados: se incluyeron un total de 54 pacientes con tumores malignos y benignos de nariz y senos paranasales, la mayoría fueron hombres con un promedio de edad de ocho años. Generalmente se presentaron con síntomas nasosinusales, el principal fue obstrucción nasal en el 80 % de los pacientes. El diagnóstico mas común fue craneofaringioma en un tercio de los pacientes, seguido por angiofibroma nasofaríngeo y linfoma de Burkitt. Conclusión: es importante conocer los síntomas y características clínicas de los pacientes pediátricos con tumores nasofaríngeos. Por esta razón, se considera importante presentar la casuística y características de los tumores de nariz y senos paranasales recogida durante 5 años, en un hospital pediátrico de cuarto nivel en la ciudad de Bogotá, Colombia

Introduction: Tumors in the nasal cavity and paranasal sinuses in children is a serious problem in the pediatric population, mainly due to the non-specificity of the symptoms that leads to years or months passing before the suspicion of a neoplastic pathology. With an important impact in quality of life not only in the patient but also in its family environment. When suspecting a sinonasal tumor, diagnostic images such as magnetic resonance and computed tomography of the paranasal sinuses are required. When you have an etiological diagnosis always do a multidisciplinary approach. Methods: We conducted a cross-sectional study of the cohort of patients that had been diagnose with tumors of the nasal cavity or paranasal sinuses in a fourth level pediatric hospital in Bogota, Colombia between 2013 - 2018. Results: 54 patients were included, the majority of them were men, with an average age of eight years. They mainly presented with nasal symptoms, the main one being nasal obstruction in 80% of patients. The most common diagnosis was cranipharyngioma in one third of the patients, followed by nasopharyngeal angiofibroma and Burkitt lymphoma. Conclusion: We present this article with the objective of presenting the tumors of nose and paranasal sinuses casuistry collected during 5 years in a fourth level pediatric hospital in the city of Bogotá and the imaging characteristics for the diagnosis of these are reviewed with some clinical cases as examples.

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BACKGROUND: Transpetrosal approaches have been used for treatment of tumors in the petroclival region for many years. Injury to the temporal lobe, however, has been a potential drawback of the techniques described to date. OBJECTIVE: To describe modifications of the transpetrosal surgical technique, which allows extradural manipulation of the temporal lobe during the focused combined transpetrosal approach. This extra layer of protection avoids mechanical brain retraction, direct trauma to the temporal lobe and disruption of the local venous structures. METHODS: The present manuscript describes an innovative technical nuance based on the combination of the focused combined transpetrosal approach, the peeling of the dural layers of the tentorium, and the reverse peeling of the middle fossa dura mater. Ample illustrative material is provided and illustrative cases are presented. CONCLUSION: Peeling of the dural layers of the tentorium is a promising modification of the transpetrosal approach to increase the safety of the temporal lobe manipulation.

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BACKGROUND: Patients undergoing endoscopic resection of neoplasms with both sinonasal and skull base involvement can develop chronic rhinosinusitis (CRS) after treatment and may occasionally benefit from additional endoscopic sinus surgery (ESS). We investigate risk factors and outcomes associated with revision ESS (rESS) after endoscopic skull-base surgery (SBS) for neoplasms with combined sinonasal and skull base involvement. METHODS: A retrospective review of patients with neoplasms with both sinonasal and skull base involvement who underwent endoscopic resection at a single tertiary care academic institution from 2004 through 2017 was performed. Eighty-three patients were included. Main outcome measures included incidence and timing of revision surgery, Lund-Mackay (LM) scores, and 22-item Sino-Nasal Outcome Test (SNOT-22) scores. RESULTS: rESS was performed in 21 (25%) cases, 15 (18%) of which were due to CRS. Time between initial resection and rESS was an average of 42 months (range, 6 to 142 months). Pre-SBS and post-SBS LM scores were not significantly different (5.0 vs 4.7, p = 0.640), although pre-SBS and post-SBS SNOT-22 scores showed significant improvement (32.6 vs 24.5, p = 0.030). Malignant pathology correlated with need for rESS (odds ratio [OR] 5.07, p = 0.04), as well as treatment including chemotherapy (OR 5.10, p = 0.003) and radiation (OR 4.15, p = 0.013). CONCLUSION: A significant proportion of patients develop clinically significant sinusitis after endoscopic SBS for neoplasms with combined sinonasal and skull base involvement and may benefit from rESS. Intervention occurred, on average, 3.5 years after initial tumor resection. Malignant pathology, radiation therapy, and chemotherapy correlate with need for rESS.

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Los recientes avances en el campo de cirugía endoscópica endonasal (CEE) han permitido disminuir la morbilidad de los abordajes abiertos tradicionales, mejorando o al menos igualando los resultados oncológicos y funcionales que hemos visto en estos procedimientos en el manejo de tumores malignos de senos paranasales y base del cráneo. El manejo oncológico de estas patologías complejas se beneficia de un abordaje multidisciplinario, en donde la decisión quirúrgica se basa en el estadio del tumor, localización, histología y relación con estructuras vecinas. La adecuada y cuidadosa selección de pacientes para este tipo de técnicas es esencial para lograr resultados exitosos. Este artículo pretende hacer una revisión narrativa y, exponer las indicaciones que utilizamos en nuestra institución para elegir cada uno de estos abordajes.

Recent advances in the field of endoscopic endonasal surgery (EES) have allowed us to reduce morbidity and to improve the oncological and functional outcomes observed when the traditional open approaches for management of malign sinonasal and skull base tumors are carried out. Oncologic care of these complex pathologies benefits from the inclusion of a multidisciplinary approach where the surgical decision is based on the tumor stage, location, relation with neighboring structures and histology. A careful and adequate patient selection constitutes the key to obtain successful outcomes. The objective of this paper is to perform an narrative review of the current literature and to present the benefits, limitations and the indications of endoscopic approach in our institute.

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Osteocondromas são neoplasias relativamente raras que podem comprometer o espaço intracraniano. Geralmente estão localizadas na base do crânio em decorrência de sua origem proveniente das sincondroses da base do crânio. Deve-se suspeitar do diagnóstico em pacientes com sintomas de hipertensão intracraniana associados à lesão calcificada que apresente áreas de hipo e hiperdensidade à tomografia computadorizada de crânio.

Osteochondromas are relative rare tumors involving the intracranial space. When present, they are often localized at the skull base because their origin generally comes from the synchondrosis of the skull base. We must suspect the presence of an osteochondroma whenever a patient presents symptoms of high intracranial pressure associated with a calcified mass, which is seen as a high and low density image at the CT scan.

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OBJETIVO: Analisar as indicações cirúrgicas e o seguimento pós operatório, ressaltando as complicações e efetividade da abordagem multidisciplinar, para os tumores avançados da base do crânio. MÉTODO: Análise retrospectiva de 46 prontuários de pacientes submetidos à ressecção de tumores invadindo a fossa craniana anterior e reconstruídos com retalhos microcirúrgicos, operados entre março de 1990 e julho de 2002. Todos os pacientes foram operados pelo núcleo de cirurgia de base do crânio do INCA. RESULTADOS: As estruturas mais envolvidas na ressecção foram por ordem: a órbita (76,5 por cento), seio maxilar (76,5 por cento), seio esfenoidal (63,8 por cento), paredes da cavidade nasal (59,5 por cento) e palato (42,5 por cento). A dura-máter estava acometida em 32,6 por cento dos casos. A reconstrução microcirúrgica utilizando os retalhos do músculo reto abdominal foi empregada em 93,5 por cento dos casos. A taxa de sucesso dos transplantes livres foi de 97,8 por cento. As complicações ocorreram em 58,6 por cento dos pacientes e as mais freqüentes foram: infecções locais (21,7 por cento), fístulas liquóricas (15,2 por cento), meningite (6,5 por cento) e hematoma (6,5 por cento). CONCLUSÕES: A reconstrução com técnica microcirúrgica permite que se realizem ressecções alargadas destes tumores com limites seguros e índices de complicações aceitáveis, permitindo a estes pacientes uma melhoria da qualidade de vida e da sobrevida, com baixo índice de recidiva.

BACKGROUND: The analysis of the surgical indications and the follow-up, stressing the surgical complications and efficiency of a team approach for the advanced tumors involving the anterior skull base are the purpose of the present study. METHODS: The authors present a retrospective evaluation of 46 patients who underwent resections of advanced tumors involving the anterior skull base, which were reconstructed with free flaps from May, 1990 to July, 2002. Those patients have been treated by the skull base surgical team of INCA. RESULTS: The commonest resected structures were: the orbit (76.5 percent), maxilar sinus (76.5 percent), sphenoidal sinus (63.8 percent), nasal cavity walls (59.5 percent) and palate (42.5 percent). The dura-mater was involved in 32,6 percent of the patients. Free-flap reconstructions employing the rectus abdominis muscle flaps were the leading procedure in 93.5 percent of patients. The successful rate of the free-tissue transfer was 97.8 percent. Complications occurred in 58,6 percent of patients, the most frequent were: local infection (21.7 percent), cerebrospinal fluid leakage (15.3 percent), meningitis(6.5 percent) and hematoma (6.5 percent). CONCLUSIONS: The microvascular free-tissue transfer reconstruction allows more extensive resections of those tumors with safety borders, presenting acceptable complication rates, offering the patients a better quality of life, longer life expectance with low incidence of recurrent disease.