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PURPOSE: Clival metastatic cancer is rare and has limited literature to guide management. We describe management of clival metastasis with Gamma Knife radiosurgery (GKRS). We augment our findings with a systematic review of all forms of radiation therapy for clival metastasis. METHODS: Records of 14 patients with clival metastasis who underwent GKRS at the University of Pittsburgh Medical Center from 2002 to 2023 were reviewed. Treatment parameters and clinical outcomes were assessed. A systematic review was conducted using evidence-based guidelines. RESULTS: The average age was 61 years with male predominance (n = 10) and average follow-up of 12.4 months. The most common primary cancers were prostate (n = 3) and lung (n = 3). The average time from cancer diagnosis to clival metastasis was 34 months. The most common presenting symptoms were headache (n = 9) and diplopia (n = 7). Five patients presented with abducens nerve palsies, and two presented with oculomotor nerve palsies. The median tumor volume was 9.3 cc, and the median margin dose was 15 Gy. Eleven patients achieved tumor control after one procedure, and three with progression obtained tumor control after repeat GKRS. One patient recovered abducens nerve function. The median survival from cancer diagnosis and GKRS were 49.7 and 15.3 months, respectively. The cause of death was progression of systemic cancer in six patients, clival metastasis in one, and unknown in four. The systematic review included 31 studies with heterogeneous descriptions of treatment and outcomes. CONCLUSION: Clival metastasis is rare and associated with poor prognosis. GKRS is a safe, effective treatment for clival metastasis.
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Fossa Craniana Posterior , Radiocirurgia , Neoplasias da Base do Crânio , Humanos , Pessoa de Meia-Idade , Masculino , Feminino , Fossa Craniana Posterior/patologia , Fossa Craniana Posterior/cirurgia , Idoso , Neoplasias da Base do Crânio/radioterapia , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/secundário , Neoplasias da Base do Crânio/cirurgia , AdultoRESUMO
Basilar invagination (BI) is a common deformity. This study aimed to quantitatively evaluate the height of clivus and atlanto-occipital lateral mass (LM) in patients with BI with or without atlas occipitalization (AOZ). We evaluated 166 images of patients with BI and of controls. Seventy-one participants were control subjects (group A), 68 had BI with AOZ (group B), and 27 had BI without AOZ (group C). Parameters were defined and measured for comparisons across the groups. Multiple linear regression analysis was used to test the relationship between Chamberlain's line violation (CLV) and the clivus height ratio or atlanto-occipital LM height. Based on the degree of AOZ, the lateral masses in group B were classified as follows: segmentation, incomplete AOZ, complete AOZ. From groups A to C, there was a decreasing trend in the clivus height and clivus height ratio. There was a linear negative correlation between the clivus height ratio and CLV in the three groups. Generally, the atlanto-occipital LM height followed the order of group B < group C < group A. The atlanto-occipital LM height was included only in the equations of groups B. There were no cases of atlantoaxial dislocation (AAD) in group C. There was a decreasing trend in LM height from the segmentation type to the complete AOZ type in group B. BI can be divided into three categories: AOZ causes LM height loss; Clivus height loss; Both clivus and LM height loss. The clivus height ratio was found to play a decisive role in both controls and BI group, while the atlanto-occipital LM height loss caused by AOZ could be a secondary factor in patients with BI and AOZ. AOZ may be a necessary factor for AAD in patients with congenital BI. The degree of AOZ is associated with LM height in group B.
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Articulação Atlantoccipital , Fossa Craniana Posterior , Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Adolescente , Atlas Cervical/diagnóstico por imagem , Imageamento por Ressonância Magnética , Idoso , Criança , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVE: The aim of this study was to provide a quantitative synthesis of the survival outcomes for patients with skull base chordomas, focusing on the role of 1) the extent of resection (gross-total [GTR] vs non-GTR), 2) the type of surgery (primary vs revision), 3) tumor histology, and 4) the different use of adjuvant therapies (proton beam radiotherapy [PBRT], photon radiotherapy [RT], or none). METHODS: A systematic review with a meta-analysis was conducted following the 2020 PRISMA guidelines. Observational studies describing adult and pediatric patient cohorts harboring skull base chordomas were included. The primary outcome measures were represented by the 5-year overall survival (OS) and progression-free survival (PFS) rates. The main intervention effects were represented by the extent of resection (GTR vs non-GTR), type of surgical excision (primary vs revision surgeries), tumor histology, and the different use of adjuvant therapies (PBRT, RT, or none). The pooled estimates were calculated using random forest models. The risk of bias was evaluated using the Joanna Briggs Institute checklist for case series. RESULTS: Six hundred forty-four studies were identified through a database and register search. After study selection, 51 studies and 3871 patients were included in the meta-analysis. The overall 5-year OS rate was 73%, which increased to 84% among patients undergoing GTR. The overall 5-year PFS rate was 52%, increasing to 74% for patients receiving GTR. The 5-year OS and PFS rates for patients undergoing PBRT were 86% and 71%, compared with 71% and 54% for patients receiving RT, and 55% and 25% when no adjuvant treatments were used. Patients undergoing their first surgery had 2.13-fold greater chances of being disease-free and 1.4-fold greater chances of being alive at 5 years follow-up compared with patients who received a revision surgery. Patients harboring chondroid chordomas had 1.13- and 1.9-fold greater chances of being alive at 5 years compared with patients with conventional and de-differentiated chordomas, respectively. The overall risk of bias was low in the included studies. CONCLUSIONS: The results of this comprehensive meta-analysis highlight the tremendous impact of GTR and adjuvant PBRT on improving OS and PFS of patients harboring skull base chordomas, with better survival rates demonstrated for patients with chondroid tumors. Even in experienced hands, the rate of surgical morbidity remains high. Proper management in high-volume centers is mandatory to reach the expected resection goal at the first surgical attempt and to reduce surgical morbidity. The introduction of the endoscopic endonasal approach was related to improved surgical and functional outcomes.
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Cordoma , Estudos Observacionais como Assunto , Neoplasias da Base do Crânio , Humanos , Neoplasias da Base do Crânio/cirurgia , Neoplasias da Base do Crânio/radioterapia , Cordoma/cirurgia , Estudos Observacionais como Assunto/métodos , Procedimentos Neurocirúrgicos/métodos , Intervalo Livre de ProgressãoRESUMO
OBJECTIVE: This study aimed to provide data on extended outcomes in primary clival chordomas, focusing on progression-free survival (PFS) and overall survival (OS). METHODS: A retrospective single-center analysis was conducted on patients with clival chordoma treated between 1987 and 2022 using surgery, stereotactic radiosurgery, or proton radiation therapy (PRT). RESULTS: The study included 100 patients (median age 44 years, 51% male). Surgery was performed using the endoscopic endonasal approach in 71 patients (71%). Gross-total resection (GTR) or near-total resection (NTR) was attained in 39 patients (39%). Postoperatively, new cranial nerve deficits occurred in 7%, CSF leak in 4%, and meningitis in none of the patients. Radiation therapy was performed in 79 patients (79%), with PRT in 50 patients (50%) as the primary treatment. During the median follow-up period of 73 (interquartile range [IQR] 38-132) months, 41 recurrences (41%) and 31 deaths (31%) were confirmed. Patients with GTR/NTR had a median PFS of 41 (IQR 24-70) months. Patients with subtotal resection or biopsy had a median PFS of 38 (IQR 16-97) months. The median PFS of patients who received radiation therapy was 43 (IQR 26-86) months, while that of patients who did not receive radiation therapy was 18 (IQR 5-62) months. The Kaplan-Meier method showed that patients with GTR/NTR (p = 0.007) and those who received radiation therapy (p < 0.001) had longer PFS than their counterparts. The PFS rates following primary treatment at 5, 10, 15, and 20 years were 51%, 25%, 17%, and 7%, respectively. The OS rates at the same intervals were 84%, 60%, 42%, and 34%, respectively. Multivariate Cox regression analysis showed that age < 44 years (p = 0.02), greater extent of resection (EOR; p = 0.03), and radiation therapy (p < 0.001) were associated with lower recurrence rates. Another multivariate analysis showed that age < 44 years (p = 0.01), greater EOR (p = 0.04), and freedom from recurrence (p = 0.02) were associated with lower mortality rates. Regarding pathology data, brachyury was positive in 98%, pan-cytokeratin in 93%, epithelial membrane antigen in 85%, and S100 in 74%. No immunohistochemical markers were associated with recurrence. CONCLUSIONS: In this study, younger age, maximal safe resection, and radiation therapy were important factors for longer PFS in patients with primary clival chordomas. Preventing recurrences played a crucial role in achieving longer OS.
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Cordoma , Fossa Craniana Posterior , Recidiva Local de Neoplasia , Radiocirurgia , Neoplasias da Base do Crânio , Humanos , Cordoma/cirurgia , Cordoma/radioterapia , Cordoma/mortalidade , Masculino , Feminino , Estudos Retrospectivos , Adulto , Pessoa de Meia-Idade , Neoplasias da Base do Crânio/cirurgia , Neoplasias da Base do Crânio/radioterapia , Fossa Craniana Posterior/cirurgia , Resultado do Tratamento , Radiocirurgia/métodos , Idoso , Intervalo Livre de Progressão , Adulto Jovem , Seguimentos , Procedimentos Neurocirúrgicos/métodos , AdolescenteRESUMO
OBJECTIVE: To assess the impact of tumor extension into the occipital condyle (OC) in lower clival chordoma management and the need for occipito-cervical fusion (OCF). METHODS: A retrospective analysis was conducted on 35 patients with lower clival chordoma. The preoperative area of the intact OCs, Hounsfield units, and the integrity of the apical ligament and the tectorial membrane were assessed using preoperative imaging. RESULTS: Seven (20%) patients were in the OCF group. The OCF group exhibited a higher prevalence of preoperative pain in the neck or head (P = 0.006), ligament absence (P = 0.022), and increased propensity for postoperative wound issues (P = 0.022) than the non-OCF group. The OCF group had less intact OCs (P < 0.001) and higher spinal instability neoplastic score (P = 0.002) than the non-OCF group. All patients with intact OCs < 60% underwent OCF, and those with OCs ≥ 70% were treated without OCF. Those with OCs between 60% and 69% underwent OCF if the ligaments were eroded, and did not undergo OCF if the ligaments were intact. Treatment strategies varied, with endoscopic endonasal approach alone being common. Radiation therapy was administered to 89% of patients. All 3 patients treated with OCF after tumor resection had wound issues; none treated with OCF before resection had wound issues. None developed atlanto-occipital instability. Survival rates did not significantly differ between groups. CONCLUSIONS: In the absence of mobility-related neck pain, patients with lower clival chordoma and intact OC ≥ 60%, intact apical ligament, and intact tectorial membrane, may not require OCF.
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Vértebras Cervicais , Cordoma , Osso Occipital , Neoplasias da Base do Crânio , Fusão Vertebral , Humanos , Cordoma/cirurgia , Cordoma/diagnóstico por imagem , Feminino , Masculino , Estudos Retrospectivos , Pessoa de Meia-Idade , Fusão Vertebral/métodos , Adulto , Neoplasias da Base do Crânio/cirurgia , Neoplasias da Base do Crânio/diagnóstico por imagem , Osso Occipital/cirurgia , Osso Occipital/diagnóstico por imagem , Idoso , Vértebras Cervicais/cirurgia , Fossa Craniana Posterior/cirurgia , Fossa Craniana Posterior/diagnóstico por imagem , Resultado do Tratamento , Adulto JovemRESUMO
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.
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Craniocervical tumors lead to cervical pain, instability, and neurological symptoms, reducing the quality of life. Effective surgical intervention at the craniocervical junction (CCJ) is critical and complex, involving comprehensive approaches and advanced reconstructive techniques. This study, conducted at Mexico City's National Institute of Cancerology, focused on three surgical cases that occurred in 2023 involving tumors at the CCJ: two chordomas and one prostate adenocarcinoma. We utilized a specialized technique: clivus-cervical stabilization reinforced with a polymethylmethacrylate (PMMA)-filled cervical mesh. Postoperatively, patients showed marked neurological recovery and reduced cervical pain, with enhanced Karnofsky and Eastern Cooperative Oncology Group (ECOG) scores indicating improved life quality. The surgical technique provided excellent exposure and effective tumor resection, utilizing PMMA-filled cervical mesh for stability. Tumoral lesions at the CCJ causing instability can be surgically treated through a transoral approach. This type of approach should be performed with precise indications to avoid complications associated with the procedure.
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Spontaneous cerebrospinal fluid (CSF) leaks are an uncommon clinical entity, often challenging to diagnose accurately. We present a unique case of a patient with symptoms suggestive of an ethmoidal CSF leak, initially supported by radiological findings, but ultimately revealed to be a mid-clival CSF leak (from the posterior wall of the sphenoid sinus). This case underscores the complexities of diagnosing CSF leaks and highlights the importance of surgical exploration in cases where radiological evidence appears contradictory. The patient's Computed Tomography scan indicated a CSF leak in the cribriform plate, prompting a surgical approach to address this region. However, intraoperative findings surprisingly revealed no evidence of leak in the cribriform plate but instead a posterior wall of the sphenoid defect as the culprit. This report emphasizes the critical role of interdisciplinary collaboration, meticulous preoperative and intraoperative assessment, and adaptability in managing challenging cases of CSF leaks, ultimately leading to successful surgical repair and improved patient outcomes. It serves as a valuable reminder for clinicians to consider the possibility of a masquerading CSF leak when clinical and radiological findings do not align, thereby facilitating more precise diagnosis and targeted treatment.
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INTRODUCTION: Breast cancer, comprising 25 % of all diagnosed cancers, predominantly affects women globally. While bone metastasis is common, occurrences at the clivus or skull base are rarely documented. Treatment varies from surgery in early stages to a multifaceted approach for advanced cases, incorporating chemotherapy, radiotherapy, and surgery based on staging and histology. CLINICAL PRESENTATION: A 40-year-old female presented with a rapidly enlarging lump in her left breast and diplopia on right gaze. Histology was positive for invasive breast cancer (no special type) and Contrast-Enhanced Computed Tomography demonstrated metastatic deposits in multiple vertebrae and bilateral ilium. Further, magnetic resonance imaging of the brain and orbits revealed metastatic deposits in the clivus which extended up-to medial wall of the cavernous sinus, causing compression of the right abducens nerve. Multidisciplinary Tumor Board review recommended chemoradiotherapy. DISCUSSION: Metastases from breast cancer can occur locally or to distant sites like lymph nodes, bones, lungs, liver, and brain. Unusual symptoms, such as new-onset diplopia, trigger further investigation. Although bone metastases occur in 50-65 % of cases, clivus metastasis is rare. Treatment aims at extending survival, symptom management, and enhancing quality of life through chemotherapy and radiotherapy. Outcomes hinge on metastatic burden and regional therapy responsiveness. Distant metastases substantially reduce the 5-year survival rate from 80 % to approximately 25 %, but cases often improve with regional interventions. CONCLUSION: Although an extremely rare occurrence, clivus metastasis might be considered in patients with a history of malignancy, in particular, breast malignancy, presenting with new-onset sixth nerve palsy.
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The past decade has witnessed rapid advancements in various surgical techniques for the clivus and surrounding structures. However, effective treatment of this region still poses significant challenges for neurosurgeons. The advent of endoscopic surgical procedure has reshaped skull base surgery in recent years. We present the rare case of a 67-year-old male gunshot victim who underwent endoscopic endonasal removal of a bullet lodged in the anterior lower clivus. The patient did not receive prophylactic antibiotic therapy. Based on this case, we propose that the endonasal endoscopic approach is highly advantageous for removing foreign bodies from the clivus. Furthermore, intraoperative navigation facilitates rapid lesion identification and reduces the surgical duration.
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Background/Objectives: Clivus metastases from distant neoplasms are uncommon occurrences both in clinical practice and the neurosurgical literature. Surgical management is debated, particularly about the role of surgery and the preferable approach. The aim of this study was to report our surgical experience and review the concerning literature. Methods: Our institutional registry was retrospectively reviewed, and patients who underwent surgical treatment for clival metastasis from 1998 to 2023 were included. A PRISMA systematic review of the literature was performed. Results: Four patients were enrolled, and all of them underwent an endoscopic endonasal approach (EEA). Three presented with cranial nerve (CN) VI palsy. The aim of surgery was biopsy in all cases. No complications were reported. Mean overall survival (OS) was 6 ± 1 months. The systematic review retrieved 27 papers reporting 39 patients who underwent the surgical treatment of clivus metastases. Most of them (79.5%) presented with CN palsies, and EEA was the preferred approach in 92.3% of the cases, to perform a biopsy in most patients (59%). Two hemorrhagic complications (5.1%) were reported, and the mean OS was 9.4 ± 5.6 months. Conclusions: Clival metastases are uncommonly observed, in most cases, during advanced stages of oncological disease. The aim of surgery should be the confirmation of diagnosis and symptomatic relief, balancing the risk-benefit ratio in a multidisciplinary context. EEA is the approach of choice, and it should be carried out in experienced tertiary skull base centers.
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Background: The radiographic presentation of the primary intraosseous cavernous hemangiomas (PICHs) is nonspecific. We report a case of clival PICH mimicking a chordoma with a literature review. Case Description: A 57-year-old woman presented with diplopia that started a few days before the presentation. She had transient diplopia at the right lateral gaze and upper gaze with normal eye movement. The symptoms disappeared spontaneously 1 week later. She had no other complaints or neurological deficits. Computed tomography revealed an intraosseous mass lesion and bone erosion of the middle and lower clivus, extending laterally to the right occipital condyle. Magnetic resonance imaging (MRI) showed hyperintense and hypointense components on T2- and T1-weighted images, respectively. The lesion was larger than on MRI performed 10 years earlier. Chordoma or chondroma was considered a possible preoperative diagnosis. An endoscopic transsphenoidal approach removed the tumor. In the operating view, the lesion appeared as "moth-eaten" bony interstices filled with vascular soft tissue. Histologically, an intraosseous cavernous hemangioma was diagnosed. Conclusion: Diagnosis before surgery is difficult without characteristic radiographic findings. When making a differential diagnosis of malignant skull lesions, PICH should be considered.
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OBJECTIVE: This study endeavors to assess the clinical and radiologic findings of Ecchordosis physaliphora (EP) in patients under long-term observation at our clinic, as well as in cases reviewed from the existing literature. METHODS: In our study, we evaluated EP lesions in a total of 16 patients, who underwent follow-up and treatment in the neurosurgical unit. We conducted a retrospective review using magnetic resonance imaging (MRI) and computed tomography (CT) studies to confirm the diagnoses as EP. We conducted a systematic literature review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, encompassing a database search from inception to January 2024. We included confirmed cases of EP from both surgically and conservatively reported studies. RESULTS: Our study included a total of 16 patients, consisting of 9 (56.25%) men and 7 (43.75%) women, with an average age of 45±17.3 years. Among them, 7 (43.75%) patients presented with headaches, while 3 (18.75%) reported hearing loss. Incidental EP was detected in 6 (37.5%) patients in the study during imaging performed for different indications. The key radiological features of EP comprised hypointensity on T1, hyperintensity on T2, and an absence of MRI gadolinium enhancement. In one out of the sixteen cases, we employed an endoscopic endonasal approach for resection, and there was no recurrence observed over an average postoperative follow-up period of 24 months. Among the 15 patients, who underwent conservative follow-up, 12 (80%) had the classical Type B EP, one (10%) patient exhibited BNCT in the C2 vertebra, and another (10%) patient presented with a variant type EP. CONCLUSIONS: Utilizing a combination of imaging modalities, ensuring a clear radiological distinction between EP and chordoma, can offer substantial advantages in this context. Given that EP might be incidentally discovered, and non-resistant symptoms may resolve on their own, considering conservative treatment before surgery may be a viable option in all cases.
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Introduction: Clivus meningiomas are benign tumors that occur at the skull base in the posterior cranial fossa. Symptoms usually progress several months or years before diagnosis and may include: headache, vertigo, hearing impairment, ataxia with gait disturbances, sensory problems. In the neurological findings, paralysis of the lower cranial nerves is most often seen, which in the later course can be accompanied by cerebellar and pyramidal signs until the development of a consciousness impairment. Case presentation: We presented the case of a patient who at the time of diagnosis had only unilateral hypoglossal nerve paralysis with dysarthria and mild dysphagia. After the neurosurgical procedure, pathohistological analysis confirmed meningothelial meningioma. Conclusion: Early recognition of clivus tumors, which include meningiomas, is necessary in order to implement an adequate therapeutic procedure and prevent further deterioration of the patient's condition.
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Advances in technology, instrumentation, and reconstruction have paved the way for extended endoscopic approaches to skull base tumors. In the sagittal plane, the endonasal approach may safely access pathologies from the frontal sinus to the craniocervical junction in the sagittal plane, the petrous apex in the coronal plane, and extend posteriorly to the clivus and posterior cranial fossa. This review article describes these modular extended endoscopic approaches, along with crucial anatomic considerations, illustrative cases, and practical operative pearls.
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Intradural exposure in the extended middle fossa anterior transpetrosal approach is traditionally limited to the inferior petrosal sinus inferomedially. Expanding bone removal of the petrous apex around the petrous internal carotid artery (ICA), underneath the trigeminal ganglion/mandibular nerve, and into the lateral component of the clivus can significantly expand the limits of this approach beyond the inferior petrosal sinus and allows for exposure of the midline structures, aspects of the contralateral inferior clival region, and, when high riding, the vertebrobasilar junction. To date, no descriptive techniques for drilling into the lateral clivus in this approach have been published. The authors provide a detailed stepwise description of their complete anterior petrosectomy, in use at their institution, that involves skeletonization of the posteromedial petrous ICA, gentle elevation of the trigeminal ganglion/mandibular nerve, removal of the infratrigeminal petrous apex, and two techniques for drilling into the lateral clivus along the petroclival fissure. These techniques provide a direct and unobstructed corridor to the midpetroclival region and ventral brainstem with greater maneuverability and enhanced control of the midline structures, which is especially useful for resection of petroclival meningiomas, chondrosarcomas, and giant vascular lesions of the mid- and upper basilar artery and its proximal branches.
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Fossa Craniana Posterior , Procedimentos Neurocirúrgicos , Osso Petroso , Humanos , Osso Petroso/cirurgia , Fossa Craniana Posterior/cirurgia , Procedimentos Neurocirúrgicos/métodos , Neoplasias da Base do Crânio/cirurgia , Fossa Craniana Média/cirurgia , Artéria Carótida Interna/cirurgiaRESUMO
INTRODUCTION AND IMPORTANCE: Ectopic pituitary neuroendocrine tumor (EPNET) is a very rare entity, seldom with apoplexy evolution. Only three cases of intracranial ectropic pituitary neuroendocrine tumor apoplexy were reported in the literature. CASE PRESENTATION: We report the case of a 45-year-old woman with a history of amenorrhea, and headaches. Neuroimaging showed a very aggressive giant mass within the clivus with the invasion of the sphenoidal sinus and encasement of internal carotid arteries with an empty sella. Endocrinology work-up revealed an exceedingly high level of prolactin surprisingly without galactorrhea. Immunohistochemical analysis after an endonasal biopsy confirmed the diagnosis of prolactinoma. One month after Cabergoline initiation, an apoplexy of the ectopic pituitary neuroendocrine tumor occurred. Conservational management with a decrease in cabergoline dose was performed. DISCUSSION: This article highlights data from various cases reported in the literature in addition to our case to confirm the extreme rarity of apoplexy as a complication of EPNET. CONCLUSION: Pituitary apoplexy in ectopic pituitary neuroendocrine tumor is extremely rare. Therefore, in case of unusual localization of pituitary neuroendocrine tumor, a thorough follow-up is necessary to detect complications and ensure early management.
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BACKGROUND: Plasmacytoma of the skull base is a rare manifestation of plasma cell neoplasm with only a few cases documented in literature involving young adults. Plasmacytoma can be an isolated solitary lesion or a secondary manifestation of multiple myeloma (MM). In this study, we report the clinical and radiological characteristics, management, and outcomes of patients under the age of 40 who presented with skull base plasmacytoma and associated neurological manifestations. Additionally, we share our experience in treating a rare case of skull base plasmacytoma diagnosed during pregnancy, in which the patient exhibited a favorable response to myeloma treatment initiated after delivery. CASE SERIES: Four patients were identified, comprising one pregnant female and three male patients, with a median age of 36 years (range 33-37 years). The main presenting symptoms were headache, dizziness, and cranial nerve palsy. All patients received underwent systemic myeloma therapy and radiotherapy with three patients also underwent autologous stem cell transplantation (ASCT). Notably, all patients achieved complete remission. CONCLUSION: Skull base plasmacytoma represents a rare manifestation of plasma cell neoplasms, underscoring the importance of considering it in the differential diagnosis of skull base lesions to ensure early intervention and avoid potential serious complications. Throughout our series, the cornerstone of therapy involved radiotherapy, systemic myeloma therapy, and ASCT, all of which elicited a favorable response in every case.
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Plasmocitoma , Neoplasias da Base do Crânio , Humanos , Masculino , Plasmocitoma/terapia , Plasmocitoma/patologia , Plasmocitoma/diagnóstico , Adulto , Feminino , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/terapia , Gravidez , Mieloma Múltiplo/terapia , Mieloma Múltiplo/patologia , Mieloma Múltiplo/diagnóstico , Transplante Autólogo , Resultado do Tratamento , Complicações Neoplásicas na Gravidez/patologia , Complicações Neoplásicas na Gravidez/terapia , Complicações Neoplásicas na Gravidez/diagnóstico , Imageamento por Ressonância MagnéticaRESUMO
Giant cell tumor (GCT) of the skull is an extremely rare condition, accounting for less than one percent of all bone GCTs. Clival GCT is even rarer, with only 25 cases documented to date. It generally follows a benign course; however, due to its location and vascularity, it can be locally aggressive. Complete resection of GCT in this location may be challenging, resulting in residual tumors. In this paper, we report a case of a 19-year-old male who presented with a chronic headache later accompanied by diplopia and was noted to have a mass spanning the sella and the clivus on cranial imaging. The histopathology report of the excised mass revealed findings compatible with GCT of the bone. Most GCTs remain stable in the first two years after initial treatment. However, four months after its partial excision, the clival GCT continued to progress. The patient underwent adjuvant radiation therapy, yet symptoms persisted. This profile highlights the crucial role of long-term surveillance and prompt adjuvant radiation therapy and chemotherapy.
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BACKGROUND AND OBJECTIVE: Nonfunctional pituitary neuroendocrine tumors (PitNETs) exhibit wide variability in growth pattern based on subtype. Silent corticotroph adenomas (SCAs) demonstrate aggressive growth compared to other nonfunctional adenomas (NFPAs), especially into the cavernous sinus. In this study, we sought to characterize other growth patterns of SCAs in comparison to NFPAs. METHODS: We performed a retrospective analysis of all patients with nonfunctional PitNETs treated with surgical resection via endoscopic endonasal approach (EEA) at a single institution from August 1, 2018, to May 11, 2024. Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) were reviewed to determine extension into the suprasellar space, sphenoid sinus, cavernous sinus, and clivus. RESULTS: Ninety-one patients were included, including 20 SCAs and 71 NFPAs. SCAs demonstrated significantly greater rates of growth into the sphenoid sinus (55.0% vs. 23.94%, p=0.013), clivus (65.0% vs. 16.9%, p<0.0001), and cavernous sinus (defined as Knosp grade 3 or 4; 55.0% vs. 23.35%, p=0.016). Other NFPAs were more likely to grow into the suprasellar space (92.96% vs. 75.0%, p=0.038). Tumor volume was similar between groups (11.93 cm3 vs. 9.06 cm3, p=0.2). CONCLUSIONS: Silent corticotroph PitNETs demonstrate predilection for invasion of bony structures, with higher rates of growing through the sellar floor into the sphenoid sinus, growing posteroinferiorly into the clivus, and laterally into the cavernous sinuses. Other nonfunctional PitNETs tended to follow the path of least resistance, growing superiorly into the suprasellar space. These differences in growth patterns may account for some of the clinical challenges of treating silent corticotroph PitNETs.