A Rare Tumor Causing Optic and Oculomotor Nerve Compression: Clivus Plasmacytoma Case Report.

Plasmacytomas rarely affect the skull base and may be found as an isolated lesion or as a part of multiple myeloma. The typical feature of plasmacytomas is aggressive bone destruction in the skull. It is often confused with the chordoma of the clivus. The most common location for skull-base plasmacytomas is the nasopharynx. The most commonly affected cranial nerve in clivus tumors is the abducens nerve. In our 64-year-old male case, a plasmacytoma was detected in the clivus. There was ptosis and decreased vision due to optic nerve and oculomotor nerve involvement due to the plasmacytoma. Radiotherapy was applied for the treatment.

Skull base surgery for malignant tumors: The 2nd international collaborative study (1995-2015).

BACKGROUND: The current study presents the effort of a global collaborative group to review the management and outcomes of malignant tumors of the skull base worldwide. PATIENTS AND METHODS: A total of 28 institutions contributed data on 3061 patients. Analysis evaluated clinical variables, survival outcomes, and multivariable factors associated with outcomes. RESULTS: The median age was 56 years (IQR 44-67). The open surgical approach was used in 55% (n = 1680) of cases, endoscopic resection was performed in 36% (n = 1087), and the combined approach in 9.6% (n = 294). With a median follow-up of 7.1 years, the 5-year OS DSS and RFS were 65%, 71.7% and 53%, respectively. On multivariable analysis, older age, comorbidities, histology, dural/intracranial involvement, positive margins, advanced stage, and primary site were independent prognostic factors for OS, DSS, and RFS. Adjuvant RT was a protective prognostic factor. CONCLUSION: The progress across various disciplines may have contributed to improved OS and DSS in this study compared to previous reports.

Surgical Proposition of a Slow-Growing Calvarial Exostosis in a Female Patient With a Congenital Iris Cyst of the Anterior Chamber and Mandibular Tori.

We present an unusual case of a woman in her early 50s with a slow-growing calvarial exostosis. Exostoses are bony spurs or osteomas extending outward beyond a bone's surface and may be benign or malignant. Calvarial exostoses are a less common bone tumor that can occur in the population. We present a case of a rare, slow-growing calvarial exostosis with a combination of mandibular tori and a congenital iris cyst. We discuss differentials of this exostosis and different syndromes that may cause it such as hereditary multiple exostoses and Gardner syndrome. The current article aims to spread awareness of this atypical presentation of exostoses and present our institution's surgical proposition for removing a calvarial exostosis to obtain a further histological analysis of its composition. As these masses may commonly be benign, a definitive diagnosis cannot be made through imaging alone to rule out more threatening conditions. We have addressed radiological findings and diagnostic and treatment options offered to the patient. The patient decided not to move forward with removing the mass and would continue to monitor and return should she notice any unusual or acute changes.

Relevance of the Foramen of Vesalius for Preoperative Tumor Embolization in Skull Base Meningioma.

OBJECTIVE: The objective of this study was to investigate the role of the foramen of Vesalius (FV) in the pathogenesis of skull base meningioma by analyzing data from various multi-image modalities. METHODS: For this single-center retrospective study, 39 consecutive patients with skull base meningioma who underwent tumor resection between January 2020 and March 2023 were enrolled. The anatomical and pathological characteristics of the FV were evaluated using computed tomography and 3-dimensional digital subtraction angiography. The clinical significance of the FV in tumor hemodynamics and treatment, such as preoperative tumor embolization, was investigated using the 3-dimensional digital subtraction angiography/computed tomography fusion images. RESULTS: We identified FV in 52% (17/27) of the finally included patients. In 10 (30%) patients, the FV was found bilaterally with no significant variation in appearance between the healthy and tumor-affected sides (P = 0.786). The mean FV diameter was significantly larger on the tumor-affected side (P = 0.010). No significant anatomical differences, like duplication and partial assimilation with the foramen ovale, were observed between the 2 sides. The FV was involved in venous skull base perfusion around the tumor in 9 cases. In 4 cases where it was the pathway for tumor feeders, preoperative tumor embolization via the FV resulted in disappearance of the tumor stain. No complications associated with endovascular treatment were observed. CONCLUSIONS: This study elucidated the anatomical asymmetry of the FV and its role in the hemodynamics of skull base meningioma. Our findings highlight the significance of performing anatomical and pathological evaluations of the FV in determining treatment strategies, including preoperative embolization, for skull base lesions.

Free temporalis muscle fascia graft in dural reconstruction following surgical resection of intermediate and malignant skull base tumors: A 10-year experience from a single center.

BACKGROUND: Data from patients with post-ablative dural defects reconstructed using a free temporalis muscle fascia graft (FTFG) after resection of anterior or central skull base tumors were retrospectively analyzed. METHODS: The primary predictor and outcome variables were the reconstructive methods for dural repair and postoperative cerebrospinal fluid (CSF) leakage rate, respectively. RESULTS: Eighty patients were included, and 94 postoperative dural reconstructions were performed using FTFG. The postoperative CSF leakage rate was 3.19%. The postoperative CSF leakage rates did not significantly differ between open and endonasal endoscopic surgeries (1.92% vs. 4.88%; p > 0.05). In cases completed using the endonasal endoscopic approach, the postoperative CSF leakage rate was significantly associated with the intraoperative CSF leak flow (p < 0.05). CONCLUSIONS: Post-ablative dural defect reconstruction using FTFG resulted in low postoperative CSF leakage and complication rates comparable to those of free fascia lata graft from available literature.

Subtotal Petrosectomy: Pictorial Review of Clinical Indications and Surgical Approach.

Subtotal petrosectomy (STP) is characterized by obliteration of the middle ear and occlusion of the external auditory canal. The advent of the endoscope has allowed a reduction in morbidity for some conditions such as cholesteatoma and other middle ear disorders, but STP still plays an important role. A retrospective review of medical records and videos of patients who had undergone STP was performed. Perioperative data and images were collected from various clinical cases who had undergone subtotal petrosectomy at our tertiary referral university hospital in Verona. We confronted our experience with a review of the literature to present the main indications for this type of procedure. STP allows a variety of diseases to be managed effectively as it offers the possibility of a definitive healing with radical clearance of temporal bone. Moreover, it can be safely combined with other procedures with a very low complication rate. Although the endoscope represents a revolution in ear surgery, STP, when indicated, is nowadays a surgical option that should be included in the otosurgeon's portfolio.

Neuroendocrine and undifferentiated sinonasal and skull base tumors: An up-to-date narrative review.

Tumors located in the nasal cavity, paranasal sinuses and the skull base comprise a wide range of histologic subtypes. Among them, neuroendocrine and undifferentiated tumors are rare but noteworthy, because of their distinctive features, aggressive nature, and diagnostic complexities. A literature search was conducted in the PubMed/MEDLINE and the Scopus databases from 2019 until inception. The keywords "neuroendocrine", "undifferentiated", "nose", "sinonasal", "paranasal", "skull base" were used. Thirty-eight articles referring to neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base were finally included and analyzed. Neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base are infrequent malignancies, most commonly affecting middle-aged men. They usually present with non-specific symptoms, even though ocular or neurologic manifestations may occur. Prognosis is generally poor; however, novel targeted and immunological therapies have shown promising results. Sinonasal Neuroendocrine Carcinomas (SNECs) carry distinct histological and immunohistochemical features. Management consists of surgical resection coupled with systematic therapy. Sinonasal Undifferentiated Carcinomas (SNUCs) lack specific squamous or glandular features. They typically stain positive for pancytokeratin and INI1 antibody. Treatment includes induction chemotherapy, followed by a combination of chemotherapy and radiotherapy. Olfactory neuroblastomas (ONBs) have neuroepithelial or neuroblastic features. They show diffuse positivity for various markers, including synaptophysin, chromogranin, and neuron-specific enolase (NSE). Surgical resection plus radiotherapy is considered the treatment of choice. In conclusion, neuroendocrine and undifferentiated tumors arising from the nose, paranasal sinuses and the skull base represent a unique group of malignancies. A thorough understanding of their clinical features, molecular changes, diagnostic approaches, treatment modalities, and prognostic factors is critical for providing optimal patient care. Still, continued research efforts and multidisciplinary collaboration are warranted, in order to improve outcomes for patients diagnosed with these rare and aggressive tumors.

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.

Clinical experience of resection for nasal skull-base tumors by microsurgical-assisted technique / 临床耳鼻咽喉头颈外科杂志

OBJECTIVES@#To investigate the application of the microsurgical treatment in nasal skull-base tumors resection.@*METHODS@#In a retrospective study, totally 15 cases with tumors in the nasal skull-base received microsurgical-assisted treatment in our department from February 2012 to June 2017 were analysed. Lateral rhinotomy approach was carried out in 11 patients and posterior wall of the maxillary sinus approach in 4 patients.@*RESULTS@#Tumors of all cases were completely resected under the microscope. Postoperative bleeding, cerebrospinal fluid leakage, infection and meningo-encephalocele did not occur in this series. The postoperative follow-up time were 6 months to 5 years. One case lost follow-up, seven cases were survivor of tumor-free. Seven cases had recurrence or metastasis, with one case died and other six alive with tumor.@*CONCLUSIONS@#Microsurgical-assisted resection for nasal skull-base tumors can obtain clear vision, with high surgical precision and security.

Endoscopic Endonasal Transsphenoidal Pituitary Tumors Approach: Nuances of Neurosurgical Technique / Abordagem endoscópica endonasal transesfenoidal para tumores hipofisários: detalhes da técnica neurocirúrgica

Pituitary tumors are responsible for 7 to 17% of all intracranial lesions. Over the past decade, advances in endoscopic microsurgical techniques have resulted in an increasingly aggressive endonasal approach to tumors of the midline skull base. We present our series emphasizing technical nuances of endoscopic endonasal transsphenoidal approach to treat pituitary adenomas.

Tumores hipofisários são responsáveis por 7 a 17% de todas as lesões intracranianas. Durante a última década, avanços nas técnicas microcirúrgicas endoscópicas resultaram no aumento de acessos endonasais agressivos para tumores de base de crânio de linha média. Apresentamos nossa série de casos, com ênfase nos detalhes técnicos do acesso endoscópico endonasal transesfenoidal para o tratamento de tumores da hipófise.

The Jugular Foramen Schwannomas: Review of the Large Surgical Series

OBJECTIVE: Jugular foramen schwannomas are uncommon pathological conditions. This article is constituted for screening these tumors in a wide perspective. MATERIALS: One-hundred-and-ninty-nine patients published in 19 articles between 1984 to 2007 years was collected from Medline/Index Medicus. RESULTS: The series consist of 83 male and 98 female. The mean age of 199 operated patients was 40.4 years. The lesion located on the right side in 32 patients and on the left side in 60 patients. The most common presenting clinical symptoms were hearing loss, tinnitus, disphagia, ataxia, and hoarseness. Complete tumor removal was achieved in 159 patients. In fourteen patients tumor reappeared unexpectedly. The tumor was thought to originate from the glossopharyngeal nerve in forty seven cases; vagal nerve in twenty six cases; and cranial accessory nerve in eleven cases. The most common postoperative complications were lower cranial nerve palsy and facial nerve palsy. Cerebrospinal fluid leakage, meningitis, aspiration pneumonia and mastoiditis were seen as other complications. CONCLUSION: This review shows that jugular foramen schwannomas still have prominently high morbidity and those complications caused by postoperative lower cranial nerve injury are life threat.

Anterior Approaches to Midline Skull Base Tumors

Among various approaches to midline skull base tumors, anterior approaches can provide excellent visualization of the lesion. Since June 1989, 12 anterior procedures have been carried out on 9 consecutive patients presenting with midline skull base tumors(four transsphenodal approches, three Le Fort I osteotomies. Two lateral rhinotomies, two facial translocation approaches, and one craniofacial approach). These anterior procedures allowed good access to the lesions. Wound healing was rapid, with little discomfort to the patients. Cosmetic results were also excellent, and there were no significant problems related to malocclusion in the cases of Le Fort I osteotomy. Patients who underwent facial translocation approach developed nasolacrimal duct obstruction and small area hypesthesia on the cheek. Postoperatively, two patients died from tumor progression and meningitis secondary to CSF leakage, respectively. Although the number of cases and follow-up period are limited in our series, we think that anterior approaches may be useful in the surgical treatment to midline skull base tumors.

Improved Access to Skull Base Tumors by Resection of the Zygoma

Improved access to lesions at the medial end of the sphenoid ridge, in the cavernous sinus, or in the interpeduncular cistern after mobilization of the zygoma has been a subject of growing interest in recent years. This study described out experience with 23 patients who underwent the zygomatic osteotomy for skull base tumors in the past 6 years. The follow-up period ranged from 5 months to 49 months. The patient' age range was 11 to 75 years, with an average age of 45 years. This zygomatic osteotomy was used from eleven patients with medial sphenoid ridge lesions, six with lesions arising in or involving the cavernous sinus, three with clival lesions, two with sellar/parasellar lesions, and one with temporal lobe lesions. The histology of these patients showed fourteen meningiomas, two pituitary adenomas, two chordomas, one neurinoma, one chondrosarcoma, one osteochondroma, one malignant lymphoma, and one dermoid cyst. Surgical treatment consisted of total removal in ten patients, subtotal removal in twelve, and partial removal in one. Instances of morbidities associated with basic lesions included cranial nerve injury in five patients, hemorrhage/infarction in three, hemiparesis in two, and transient aphasia in one. There were no significant problems related to zygomatic osteotomy. One patient who underwent zygomatic osteotomy for medial sphenoid ridge meingioma developed a frontalis nerve injury. No patient experienced a detachment of zygomatic arch in our series. Postoperatively, one patient with parasellar malignant lymphoma died 14 months after surgery from tumor progression. Our cases treated via this zygomatic osteotomy are as yet insufficient to determine whether the method offers definite adventages, in terms of patient mortality and morbidity, over conventional operative approaches, but we suggest that this procedure has some advantages such as minimal brain retraction, exposure of lesion in shortest distance, multidirectional viewing of the lesions, and can serve as an alternative approach to a usual pterional approach when cranial base pathologies are large or complex.