Giant fronto-spheno-orbitary juvenile psammomatoid ossifying fibroma: Case report and literature review.

Juvenile psammomatoid ossifying fibroma (JPOF) is an osteofibrous neoplasm that originates in the craniofacial skeleton typically during the first three decades of life. JPOFs usually involve the orbit, paranasal sinuses or the jaws. Extensive involvement of the anterior cranial base with compromised visual function is a rare phenomenon. In such clinical context, a definite diagnosis can only be made on the basis of histopathological findings, given the absence of pathognomonic radiological features. Despite being considered a benign entity, JPOFs present a locally aggressive behavior. Therefore, these neoplasms must be included in the differential diagnosis in every patient harboring a skull base osteofibrous lesion, and, once diagnosed, gross total surgical removal should be attempted. In this study, we present our experience in the diagnosis and treatment of a patient diagnosed with a giant JPOF involving the cranial base.

Syndrome of the third frontal convolution: Léon Ectors´ legacy on paradoxical ipsilateral hemiparesis.

Since the crossed control of sensitive-motor body functions by the contralateral cerebral hemispheres was recognized in the early 18th century, clinicians have been baffled by patients developing a motor deficit involving the extremities on the same side as an intracranial lesion. In the first third of the 20th century, three main hypotheses were proposed to explain this so-called ipsilateral or paradoxical hemiparesis: (1) the absence of decussation of the corticospinal tracts; (2) diaschisis, or blocking of the normal input to a brain region anatomically distant from the injured site; and (3) compression of the contralateral cerebral peduncle against the tentorial border, also known as the Kernohan-Woltman notch phenomenon. Here, we deal with the less widely known contributions of the Belgian neurosurgeon Léon Ectors, who included this paradoxical deficit within a neurological syndrome he considered highly specific for an early diagnosis of those meningiomas growing over the third frontal convolution. The present manuscript includes a systematic review of the cases of ipsilateral hemiparesis secondary to intracranial masses reported in ancient and modern scientific medical literature. We also address in-depth the physiopathological theories accounting for this syndrome and contrast them with Léon Ectors' observations.

Randomized Phase III Trial of Prophylactic Cranial Irradiation With or Without Hippocampal Avoidance for Small-Cell Lung Cancer (PREMER): A GICOR-GOECP-SEOR Study.

PURPOSE: Radiation dose received by the neural stem cells of the hippocampus during whole-brain radiotherapy has been associated with neurocognitive decline. The key concern using hippocampal avoidance-prophylactic cranial irradiation (HA-PCI) in patients with small-cell lung cancer (SCLC) is the incidence of brain metastasis within the hippocampal avoidance zone. METHODS: This phase III trial enrolled 150 patients with SCLC (71.3% with limited disease) to standard prophylactic cranial irradiation (PCI; 25 Gy in 10 fractions) or HA-PCI. The primary objective was the delayed free recall (DFR) on the Free and Cued Selective Reminding Test (FCSRT) at 3 months; a decrease of 3 points or greater from baseline was considered a decline. Secondary end points included other FCSRT scores, quality of life (QoL), evaluation of the incidence and location of brain metastases, and overall survival (OS). Data were recorded at baseline, and 3, 6, 12, and 24 months after PCI. RESULTS: Participants' baseline characteristics were well balanced between the two groups. The median follow-up time for living patients was 40.4 months. Decline on DFR from baseline to 3 months was lower in the HA-PCI arm (5.8%) compared with the PCI arm (23.5%; odds ratio, 5; 95% CI, 1.57 to 15.86; P = .003). Analysis of all FCSRT scores showed a decline on the total recall (TR; 8.7% v 20.6%) at 3 months; DFR (11.1% v 33.3%), TR (20.3% v 38.9%), and total free recall (14.8% v 31.5%) at 6 months, and TR (14.2% v 47.6%) at 24 months. The incidence of brain metastases, OS, and QoL were not significantly different. CONCLUSION: Sparing the hippocampus during PCI better preserves cognitive function in patients with SCLC. No differences were observed with regard to brain failure, OS, and QoL compared with standard PCI.

Clinical relevance of tumor consistency in pituitary adenoma.

PURPOSE: To review the clinical relevance of pituitary adenoma (PA) consistency and its relationship to clinical presentation, radiologic and histopathological characteristics, and surgical outcomes. BACKGROUND: PA consistency is a critical factor influencing operative planning, surgical outcomes, and patient counseling. There is no validated classification of PA consistency in the literature, and there are no current preoperative variables capable of predicting it. REVIEW: We conducted a thorough literature review of the Medline, Embase, Web of Science, and Cochrane Library databases. The inclusion criteria were all articles that described PA consistency and correlated it with preoperative aspects, radiological, pathological, and operative findings, or clinical outcomes. DISCUSSION: Although most authors differentiate easily aspirated (soft) tumors from those that are not (fibrous, might require prior fragmentation), there is no universally accepted PA consistency classification. Fibrous PA tends to be hypointense on T2WI and has lower apparent diffusion coefficient (ADC) values. Fibrous tumors seemed to present higher invasion into neighboring structures, including the cavernous sinus. Several articles suggest that dopamine agonists could increase PA consistency and that prior surgery and radiotherapy also make PA more fibrous. The anatomopathological studies identify collagen as being mainly responsible for fibrous consistency of adenomas. CONCLUSIONS: Preoperative knowledge of PA consistency affords the neurosurgeon substantial benefit, which clearly appears to be relevant to surgical planning, risks, and surgery outcomes. It could also encourage the centralization of these high complexity tumors in reference centers. Further studies may be enhanced by applying standard consistency classification of the PA and analyzing a more extensive and prospective series of fibrous PA.

Diagnosis and Therapeutic Management of Ventricular Gangliogliomas: An Illustrated Review.

BACKGROUND: Gangliogliomas (GGs) are extremely rare benign neoplasms frequently located within the temporal lobe that usually present with seizures. GGs growing predominantly within the ventricular system (VGGs) are even more infrequent, so definite conclusions concerning their diagnosis and therapeutic management are lacking. METHODS: A retrospective review of case reports of VGGs was performed from the introduction of modern imaging techniques, including 4 new illustrative cases treated in our department. RESULTS: Thirty-four cases were collected. Ages ranged from 10 to 71 years (mean, 26.62 years), and 55.9% were male. Most patients developed symptoms related to high intracranial pressure. The lateral ventricles were predominantly involved (58.8%). Obstructive hydrocephalus was observed in 54.5% of patients. Cystic degeneration and calcification were frequently observed. Surgical treatment was carried out in all cases. Morbidity and mortality were 17.6% and 2.9%, respectively. Gross total tumor resection was achieved in 64.5% of patients. Four patients experienced tumor dissemination along the neural axis. More than 90% of patients maintained a good functional status at last follow-up. CONCLUSIONS: Despite their low incidence, a diagnosis of VGGs should be considered in young male adults who progressively develop intracranial hypertension, caused by a ventricular mass showing signs of cystic degeneration and calcification. Maximal and safe surgical resection represents the gold standard for the treatment of symptomatic VGGs, although total removal is frequently precluded by difficulties in defining appropriate tumor boundaries. Adjuvant radiotherapy should be considered if an incomplete resection was carried out, especially in World Health Organization grade III neoplasms.

Nerve Sheath Myxoma Involving the Medial Rectus Muscle: A Case Report.

A 63-year-old man reported a 2-year history of painless OS proptosis. The first MRI, performed in the institution of origin, resulted in a diagnosis of hemangioma versus orbital pseudotumor. Examination revealed proptosis and mild chemosis. Ancillary tests were normal, and oral prednisone was initiated without remarkable changes. Later, an incisional biopsy identified the lesion as an intramuscular myxoma of the left rectus, and a new MRI was ordered. It described a fusiform thickening of the medial rectus with and exophytic growth below the inferior rectus and upward toward the superior oblique. Due to the localization of the lesion, a combined transcaruncular and swinging eyelid approach with a lateral orbitotomy was performed. Histopathological examination revealed a proliferation of stromal origin composed of myxoid matrix with fusocellular and stellate-shaped cells. Cells were S-100, CD34, and CD56 positive and negative for epithelial membrane antigen, CD68, CD10, actin, and desmin. Results were consistent with a left medial rectus nerve sheath myxoma.

Multidisciplinary protocol of preoperative and surgical management of patients with pituitary tumors candidates to pituitary surgery.

The optimal planning of preoperative diagnosis, management and treatment of pituitary tumors (PT) candidates to pituitary surgery (PS) requires a multidisciplinary approach involving a team of endocrinologists, neurosurgeons, ENT, neuro-ophthalmologists and neuroradiologists with experience in pituitary diseases. Such teams improve surgical results, minimize complications and facilitate their correct treatment if occurring, and optimize the hormonal, ophthalmological and radiological preoperative and follow-up evaluation. We have developed a clinical practice protocol for patients with PT who are candidates to PS based on the most recent national and international guidelines and the relevant literature regarding PT published in the last years. The protocol has been elaborated by a multidisciplinary team of a Spanish Pituitary Tumor Center of Excellence (PTCE) that includes at least one neurosurgeon, ENT, neuroradiologist, neuro-ophthalmologist, endocrine pathologist and endocrinologist specialized in pituitary diseases. We elaborated this guideline with the aim of sharing our experience with other centers involved in the perioperative and surgical management of PT thereby facilitating the management of patients undergoing PS.

Bone Infarct as an Indicator of Acute Spinal Ischaemia.

Acute spinal cord infarct in childhood is extremely rare, generally secondary to spinal/cardiovascular surgery or severe vertebral injuries. However, spontaneous spinal cord infarct cases have been described. We present a clinical case of a teenager who developed an acute weakness and paraesthesia in lower limbs after playing piggyback. Laboratory tests and MRI (magnetic resonance imaging) were normal. During her hospital admission, her motor strength improved. After 10 days, MRI was repeated, and a bone infarct was observed. She was medicated with acetylsalicylic acid, and she completed a rehabilitation program.

Ipsilateral hemiparesis: the forgotten history of this paradoxical neurological sign.

OBJECTIVE: Establishing the neurological localization doctrine for the contralateral hemispheric control of motor functions in the second half of the 19th century, researchers faced the challenge of recognizing false localizing signs, in particular paradoxical or ipsilateral hemiparesis (IH). Despite tremendous progress in current methods of neuroradiological and electrophysiological exploration, a complete understanding of this phenomenon has yet to be attained. METHODS: The authors researched the well-described cases of hemiparesis/hemiplegia ipsilateral to an intracranial lesion published in the scientific literature in the pre-MRI era (before 1980). A comprehensive review of the physiopathological mechanisms proposed for paradoxical hemiparesis throughout this period, as well as the pathological evidence substantiating them, is provided. RESULTS: A collection of 75 patients with hemiparesis/hemiplegia ipsilateral to the primary intracranial lesion reported between 1858 and 1979 were eligible for analysis. Most cases occurred in adults with supratentorial, slowly developing, extraparenchymatous mass lesions, such as neoplasms (38%) or chronic subdural hematomas (36%). Physiopathological theories proposed by the neurologists who investigated IH can be grouped into 4 major concepts: 1) lack of anatomical decussation of the corticospinal tract; 2) impaired functional activation of the contralateral hemisphere by the lesioned dominant hemisphere through the callosal connections; 3) Kernohan's notch phenomenon, or mechanical injury of the contralateral cerebral peduncle against the free edge of the tentorium; and 4) cerebrovascular dysfunction involving the contralateral hemisphere owing to kinking and mechanical flattening of the carotid artery contralateral to the primary intracranial lesion. CONCLUSIONS: IH represents a still underdiagnosed paradoxical neurological phenomenon. With the aid of modern neuroradiological and neurophysiological methods, Kernohan's peduncle notch mechanism has been confirmed to cause IH in many of the cases reported in recent decades. Nevertheless, alternative functional and/or vascular mechanisms must be investigated further for unexplained IH cases, in particular for transitory IH without evidence of peduncle injury. The historical theories reviewed in this paper represent a conceptual framework that may be helpful for this purpose.

Meningiomas quísticos: correlación radiológica y patológica con implicaciones quirúrgicas / Cystic meningiomas: Radiological and pathological correlation with surgical implications

Introducción: Los meningiomas que asocian cambios quísticos prominentes (MQ) han representado un reto diagnóstico y terapéutico desde los orígenes de la neurocirugía moderna. Presentamos la experiencia en el manejo clínico y quirúrgico de esta entidad en nuestro Servicio. Material y métodos: En este estudio descriptivo se incluyen los MQ intervenidos en nuestro Servicio en los últimos 15 años, evaluando las variables clínicas, diagnósticas y terapéuticas de este subgrupo de meningiomas, con especial énfasis en la correlación de los hallazgos radiológicos y patológicos. Los tumores se clasificaron siguiendo el esquema propuesto por Nauta y sus colaboradores. Resultados: Se identificaron 11 casos, lo que supone un 1,8% de los meningiomas intervenidos en ese periodo. Todos los pacientes eran adultos y 8 de ellos, mujeres. Entre los síntomas, destacó la alta frecuencia de crisis epilépticas, mientras que solo un caso desarrolló hipertensión intracraneal aguda. La morfología predominante fue el tipo 2, seguida de los tipos 1, 3 y 4. En 5 casos pudo establecerse un diagnóstico radiológico inequívoco de meningioma. Todos los pacientes fueron tratados mediante cirugía, sin evidenciarse casos de recurrencia durante un seguimiento medio de 52,8 meses. Aunque la mayoría de las neoplasias correspondieron al grado i de la OMS, incluyendo subtipos variados, 4 de ellas fueron diagnosticadas como meningiomas atípicos. Conclusiones: De forma excepcional, los meningiomas pueden desarrollar cambios quísticos, intra- o extratumorales, en número y tamaño variable. Estos constituyen hallazgos poco típicos que pueden dificultar seriamente su diagnóstico diferencial radiológico. Los meningiomas con quistes periféricos de cápsula fina (tipos 2 y 3) suponen el escenario quirúrgico más complejo, pues el realce capsular en las pruebas de imagen no traduce necesariamente infiltración tumoral, mientras que el quiste puede contener células neoplásicas. Por lo tanto, recomendamos realizar al menos biopsia de la cápsula o seudocápsula, y lavado minucioso de la cavidad quirúrgica especialmente en estos subtipos

Introduction: Meningiomas associating prominent cystic changes (CM) have challenged neurosurgeons since the beginning of this surgical discipline. We present the experience in the diagnostic and therapeutic management of this entity in our institution. Material and methods: A review of our patient database was carried out, searching for those CM that were operated on in the last 15 years. Relevant clinical data were recorded and analyzed for each case, with special emphasis in the correlation of radiological and pathological findings. Cystic changes were classified according to the scheme proposed by Nauta et al. Results: A total of 11 patients were gathered, which represents 1.8% of the meningiomas operated on in our department during the period studied. All were adults, predominantly female patients (8 cases). Among the clinical symptoms a high rate of epileptic seizures was observed while only one patient developed acute intracranial hypertension. Morphologically, most tumors corresponded to type 2 CMs, followed by types 1, 3 and 4. In only five patients an unequivocal radiological diagnosis of meningioma could be made. All neoplasms were surgically removed and there were no records of recurrence (mean follow-up: 52.8 months). Microscopic findings were consistent with the pathological diagnosis of atypical meningioma in 4 cases, while the remaining tumors corresponded to OMS grade I neoplasms with variable microscopic patterns. Conclusions: Meningiomas can exceptionally associate cystic changes, both intra and/or extratumoral, in variable number and size. When cystic changes become too prominent (a large number or big-sized cysts), a serious preoperative diagnostic dilemma may arise. The surgical management of those CMs displaying a peripheral, thin-walled cyst (types 2 and 3) is especially complex, as contrast enhancement of the tumor wall did not correlate strictly with neoplastic invasion; even in the absence of this feature free floating islands of meningothelial cells intermixed with cyst fluid can be found. Consequently both surgical biopsy of every suspicious tissue and copious irrigation of the surgical cavity are strongly recommended for these CM types

Central Hypothyroidism and Novel Clinical Phenotypes in Hemizygous Truncation of TBL1X.

Transducin ß-like 1 X-linked (TBL1X) gene encodes a subunit of the nuclear corepressor-silencing mediator for retinoid and thyroid hormone receptor complex (NCoR-SMRT) involved in repression of thyroid hormone action in the pituitary and hypothalamus. TBL1X defects were recently associated with central hypothyroidism and hearing loss. The current study aims to describe the clinical and genetic characterization of a male diagnosed with central hypothyroidism through thyroid hormone profiling, TRH test, brain MRI, audiometry, and psychological evaluation. Next-generation sequencing of known genes involved in thyroid disorders was implemented. The 6-year-old boy was diagnosed with central hypothyroidism [free T4: 10.42 pmol/L (normal: 12 to 22 pmol/L); TSH: 1.57 mIU/L (normal: 0.7 to 5.7 mIU/L)], with a mildly reduced TSH response to TRH. He was further diagnosed with attention-deficit/hyperactivity disorder (ADHD) at 7 years, alternating episodes of encopresis and constipation, and frequent headaches. MRI showed a normal pituitary but detected a Chiari malformation type I (CMI). At 10 years, audiometry identified poor hearing threshold at high frequencies. Sequencing revealed a nonsense hemizygous mutation in TBL1X [c.1015C>T; p.(Arg339Ter)] largely truncating its WD-40 repeat domain involved in nuclear protein-protein interactions. In conclusion, to our knowledge, we identified the first severely truncating TBL1X mutation in a patient with central hypothyroidism, hypoacusia, and novel clinical features like ADHD, gastrointestinal dysmotility, and CMI. Given the relevance of TBL1X and NCoR-SMRT for the regulation of transcriptional programs at different tissues (pituitary, cochlea, brain, fossa posterior, and cerebellum), severe mutations in TBL1X may lead to a distinct syndrome with a phenotypic spectrum wider than previously reported.

Cystic meningiomas: Radiological and pathological correlation with surgical implications. / Meningiomas quísticos: correlación radiológica y patológica con implicaciones quirúrgicas.

INTRODUCTION: Meningiomas associating prominent cystic changes (CM) have challenged neurosurgeons since the beginning of this surgical discipline. We present the experience in the diagnostic and therapeutic management of this entity in our institution. MATERIAL AND METHODS: A review of our patient database was carried out, searching for those CM that were operated on in the last 15 years. Relevant clinical data were recorded and analyzed for each case, with special emphasis in the correlation of radiological and pathological findings. Cystic changes were classified according to the scheme proposed by Nauta et al. RESULTS: A total of 11 patients were gathered, which represents 1.8% of the meningiomas operated on in our department during the period studied. All were adults, predominantly female patients (8 cases). Among the clinical symptoms a high rate of epileptic seizures was observed while only one patient developed acute intracranial hypertension. Morphologically, most tumors corresponded to type 2 CMs, followed by types 1, 3 and 4. In only five patients an unequivocal radiological diagnosis of meningioma could be made. All neoplasms were surgically removed and there were no records of recurrence (mean follow-up: 52.8 months). Microscopic findings were consistent with the pathological diagnosis of atypical meningioma in 4 cases, while the remaining tumors corresponded to OMS grade I neoplasms with variable microscopic patterns. CONCLUSIONS: Meningiomas can exceptionally associate cystic changes, both intra and/or extratumoral, in variable number and size. When cystic changes become too prominent (a large number or big-sized cysts), a serious preoperative diagnostic dilemma may arise. The surgical management of those CMs displaying a peripheral, thin-walled cyst (types 2 and 3) is especially complex, as contrast enhancement of the tumor wall did not correlate strictly with neoplastic invasion; even in the absence of this feature free floating islands of meningothelial cells intermixed with cyst fluid can be found. Consequently both surgical biopsy of every suspicious tissue and copious irrigation of the surgical cavity are strongly recommended for these CM types.

Fenómeno de vacío intervertebral: una causa de abombamiento de la pared faríngea / Intervertebral vacuum phenomenon: A cause of bulging of the pharyngeal wall

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Parálisis del nervio laríngeo recurrente en paciente con carcinoma broncogénico: una causa de hipermetabolismo de la cuerda vocal contralateral en PET/TC / Recurrent laryngeal nerve paralysis in a lung cancer patient: A cause of contralateral vocal cord hypermetabolism on PET/CT

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