Craneofaringiomas: experiencia y resultados / Craniopharyngiomas: experience and results

Introducción Los craneofaringiomas son un gran desafío en el campo neuroquirúrgico. Debido a que estas lesiones involucran estructuras importantes, los cirujanos deben sopesar los riesgos de una resección agresiva frente al riesgo de recurrencia a largo plazo. Presentamos los resultados de nuestra serie de pacientes en función de los resultados clínicos, el grado de resección, la recurrencia y la supervivencia libre de enfermedad. Materiales y métodos Se revisaron las historias clínicas de todos los pacientes que se sometieron a una resección quirúrgica por craneofaringioma en el (Hospital Italiano de Buenos Aires) entre 2007 y 2019. Se consideraron exámenes oftalmológicos, estudios de imágenes, estudios endocrinológicos y complicaciones quirúrgicas. En todos los pacientes se planificaron resecciones totales. Para la correcta elección del abordaje quirúrgico, los craneofaringiomas se clasificaron según su ubicación. Resultados Se analizaron treinta pacientes con diagnóstico de craneofaringioma. El 12,5% se clasificó como intraselar, el 12,5% como prequiasmático, el 43,75% como retroquiasmático y el 31,25% como intraventricular. En total, 38 casos incluyeron una cirugía transcraneal (15 abordaje orbitocigomático; 19 abordaje pterional y 4 abordaje transcalloso), siete incluyeron abordaje transeptoesfenoidal, dos abordajes transnasales microscópicos y una endoscopia ventricular para vaciar el quiste del craneofraingioma. La resección total se logró en el 43,7% de los pacientes y la resección casi total (mayor al 90%) en el 25%. El período de seguimiento promedio, después de la resección, fue de 4,7 años. La recurrencia tumoral ocurrió en el 48%, con un promedio de meses libres de enfermedad de 42,7. Conclusión La resección tumoral total es el mejor tratamiento para los craneofaringiomas. Debido a sus altas morbilidad y mortalidad, se necesita un equipo multidisciplinario para el manejo de estos tumores (AU)

Introduction Craniopharyngiomas are a big challenge in the neurosurgical field. Because these lesions involve important systems, surgeons must weigh the risks of aggressive resection against the long-term challenges of recurrence. We present the outcomes of our patients based on clinical results, degree of resection, recurrence and disease-free survival. Materials and methods We reviewed medical records in all patients who had undergone surgical resection for craniopharyngioma at (Hospital Italiano de Buenos Aires) between 2007 and 2019. We considered ophthalmological examinations, imaging studies, endocrinological studies and surgical complications. Radical resections were planned in all of the patients. To help choose the correct surgical approach, craniopharyngiomas were classified based on tumor location. Results Thirty cases of craniopharyngioma were analysed. 12.5% were classified as intrasellar, 12.5% as prechiasmatic, 43.75% as retrochiasmatic, and 31.25% as intraventricular. Overall, 38 cases involved a transcranial surgery (15 orbitozygomatic approach; 19 pterional approach and 4 transcallosal approach), seven involved a transsphenoidal approach, two microscopic transnasal approach and one ventricular endoscopy for emptying the craniopharyngioma cyst. Gross-total resection was achieved in 43.7% and near-total resection (more than 90%) in 25%. The mean follow-up period after resection was 4.7 years. Tumor recurrence occurred in 48%, with an average of 42.7 disease-free months. Conclusion Total tumor resection is the best treatment for craniopharyngioma. Due to its high morbidity and mortality, a multidisciplinary team is necessary for the management of these tumors (AU)

Craniopharyngiomas: experience and results. / Craneofaringiomas: experiencia y resultados.

INTRODUCTION: Craniopharyngiomas are a big challenge in the neurosurgical field. Because these lesions involve important systems, surgeons must weigh the risks of aggressive resection against the long-term challenges of recurrence. We present the outcomes of our patients based on clinical results, degree of resection, recurrence and disease-free survival. MATERIALS AND METHODS: We reviewed medical records in all patients who had undergone surgical resection for craniopharyngioma at (Hospital Italiano de Buenos Aires) between 2007 and 2019. We considered ophthalmological examinations, imaging studies, endocrinological studies and surgical complications. Radical resections were planned in all of the patients. To help choose the correct surgical approach, craniopharyngiomas were classified based on tumor location. RESULTS: Thirty cases of craniopharyngioma were analysed. 12.5% were classified as intrasellar, 12.5% as prechiasmatic, 43.75% as retrochiasmatic, and 31.25% as intraventricular. Overall, 38 cases involved a transcranial surgery (15 orbitozygomatic approach; 19 pterional approach and 4 transcallosal approach), seven involved a transsphenoidal approach, two microscopic transnasal approach and one ventricular endoscopy for emptying the craniopharyngioma cyst. Gross-total resection was achieved in 43.7% and near-total resection (more than 90%) in 25%. The mean follow-up period after resection was 4.7 years. Tumor recurrence occurred in 48%, with an average of 42.7 disease-free months. CONCLUSION: Total tumor resection is the best treatment for craniopharyngioma. Due to its high morbidity and mortality, a multidisciplinary team is necessary for the management of these tumors.

Protein stabilization by RSUME accounts for PTTG pituitary tumor abundance and oncogenicity.

Increased levels of the proto-oncogene pituitary tumor-transforming gene 1 (PTTG) have been repeatedly reported in several human solid tumors, especially in endocrine-related tumors such as pituitary adenomas. Securin PTTG has a critical role in pituitary tumorigenesis. However, the cause of upregulation has not been found yet, despite analyses made at the gene, promoter and mRNA level that show that no mutations, epigenetic modifications or other mechanisms that deregulate its expression may explain its overexpression and action as an oncogene. We describe that high PTTG protein levels are induced by the RWD-containing sumoylation enhancer (RWDD3 or RSUME), a protein originally identified in the same pituitary tumor cell line in which PTTG was also cloned. We demonstrate that PTTG and RSUME have a positive expression correlation in human pituitary adenomas. RSUME increases PTTG protein in pituitary tumor cell lines, prolongs the half-life of PTTG protein and regulates the PTTG induction by estradiol. As a consequence, RSUME enhances PTTG transcription factor and securin activities. PTTG hyperactivity on the cell cycle resulted in recurrent and unequal divisions without cytokinesis, and the consequential appearance of aneuploidies and multinucleated cells in the tumor. RSUME knockdown diminishes securin PTTG and reduces its tumorigenic potential in a xenograft mouse model. Taken together, our findings show that PTTG high protein steady state levels account for PTTG tumor abundance and demonstrate a critical role of RSUME in this process in pituitary tumor cells.

Quiste epidermoide del ventrículo lateral / Epidermoid cyst of the lateral ventricle

Los quistes epidermoides del ventrículo lateral son lesiones extremadamente infrecuentes originadas por la inclusión de elementos epiteliales al momento del cierre del tubo neural. Son tumores de lento crecimiento y se presentan clínicamente con síntomas inespecíficos. La Resonancia Magnética de encéfalo permite realizar un diagnóstico presuntivo, que se confirma por hallazgos característicos durante la cirugía. El diagnóstico de certeza es por la anatomía patológica en donde se observa un epitelio escamoso estratificado. Se presenta un caso de una mujer de 39 años operada por vía microquirúrgica con asistencia endoscópica.

Epidermoid cysts occurring within the lateral ventricles are uncommon lesions. These tumors are of developmental etiology, due to migration of epiblast inclusion at the time of neural tube occlussion of the neural tube. They are slow growing tumors and clinical presentation is nonspecific. Magnetic Resonance is suggestive of a cystic lesion, and is confirmed to be a epidermoid cyst at operation. Histopathology reveals typical stratified squamous epithelium. A case of a woman of 39 years old woman operated on with microsurgery and endoscopic assistance is presented..

Sellar hemangioblastoma mimicking a macroadenoma.

PURPOSE: Hemangioblastomas (HGB) are slow growing benign vascular tumors that arise almost always from the cerebellum and the spinal cord. Supratentorial location is extremely rare with approximately 130 cases published to date. We present a case of a sellar hemangioblastoma. CASE REPORT: An eleven year-old girl presented with a sellar and suprasellar tumor that seemed to be a macroadenoma. A transsphenoidal approach was attempted but excessive intraoperative bleeding made the resection not feasible. A second transcranial approach was successful in partially removing the lesion and decompressing the optic chiasm and the pituitary stalk. Pathological review revealed a seller hemangioblastoma. screening for Von Hippel Lindeau Syndrome was negative. CONCLUSION: The preoperative diagnosis of HGB is extremely difficult in this case but would have been essential in order to choose the right surgical approach.

Mielomeningocele cervical: Caso clínico / Cervical myelomrningocele: clinical case

El mielomeningocele cervical representa el 3,7 por ciento de los pacientes con espina bífida quística. El saco contiene elementos neurales en continuidad con la "médula espinal madre".Un tratamiento inicial inadecuado es causa de médula anclada, causante de deterioro neurológico y discapacidad, a menos que se realice el tratamiento definitivo en edad temprana.

Mielomeningocele cervical: Caso clínico / Cervical myelomrningocele: clinical case

El mielomeningocele cervical representa el 3,7 por ciento de los pacientes con espina bífida quística. El saco contiene elementos neurales en continuidad con la "médula espinal madre".Un tratamiento inicial inadecuado es causa de médula anclada, causante de deterioro neurológico y discapacidad, a menos que se realice el tratamiento definitivo en edad temprana.(AU)

Mielomeningocele cervical: Caso clínico / Cervical myelomrningocele: clinical case

El mielomeningocele cervical representa el 3,7 por ciento de los pacientes con espina bífida quística. El saco contiene elementos neurales en continuidad con la "médula espinal madre".Un tratamiento inicial inadecuado es causa de médula anclada, causante de deterioro neurológico y discapacidad, a menos que se realice el tratamiento definitivo en edad temprana.(AU)