Intracranial Involvement in Takayasu’s Arteritis

Takayasu’s arteritis (TAK) is a large-vessel vasculitis that targets the aorta and its major branches. Although extracranial vascular involvement is uniformly present in this disease, the frequency of intracranial involvement in TAK has not been well studied. We retrospectively reviewed the clinical and imaging records of patients diagnosed with TAK at a single Canadian university medical centre to determine the prevalence of intracranial vascular involvement. Intracranial vascular and non-vascular findings were described, and a review of the literature was performed. Of 20 patients with TAK, 12 had vascular neuroimaging completed. Intracranial vascular lesions were identified in 4 patients (33.3% of those with imaging available, 20% of all patients). The frequency of intracranial vessel involvement in TAK may be more common than appreciated. Imaging of both the intra- and extra-cranial vessels should be considered in these young patients.

Ameloblastoma and Intracranial Involvement: The Current Challenge of the Radical Surgical Treatment. Comprehensive Review of the Literature and Institution experience

Background Ameloblastoma (AMBL) is an odontogenic tumor, considered to be benign, but aggressive, whose principal risk is a recurrence. The growth can be enormous, and it can extend into the intracranial compartment with serious consequences. Purpose The intracranial involvement of AMBL is rare, and it may require an extensive surgery. Although it is a rare condition for the neurosurgeon to treat, knowing this condition can lead to a significant increase in survival for these patients. Methods A case of a 56-year-old woman presented with a history of recurrent left maxilla AMBL with intracranial extension and dural involvement of the anterior and medial cranial fossa is reported, followed by a systematic review of the literature with the aim to identify the best surgical treatment. Results A total of 32 cases were included in the qualitative analysis. Management is varied and often not described, resulting in an almost complete lack of information and indications for treatment. Radical surgery tends to yield the best outcomes, and it is recommended to have adequate surgical margins when possible. Conclusions Intracranial involvement from AMBL compartment is an uncommon manifestation of this rare pathology, but which deserves to be treated in a multidisciplinary way in order to ensure maximum surgical radicality. Recurrence reflects failure of the primary surgical resection. If recurrence is the major consideration, surgeons are encouraged to select radical surgery. Whenever a follicular-type maxillary AMBL is diagnosed, it is advisable to check for intracranial spreading and distant metastases during follow-up.

Are Facial Gunshot Wounds More Fatal When They Are Self-Inflicted or Other-Inflicted?

Study Design: This was a retrospective cohort study of the 2014 Nationwide Emergency Department Sample (NEDS). Objective: Intraoral and submental projectile entry points may be less fatal than other facial entry points due to the indirect access to the intracranial structures and the protection offered by the intervening maxillofacial complex. Because intraoral and submental trajectories are almost always present in the setting of attempted suicide, this study sought to determine if intent (self-harm versus other-harm) influenced mortality in facial gunshot wound (GSW) patients. Methods: All patients with a diagnosis of a facial fracture secondary to firearm injury were included in the study sample. The primary predictor was self-harm. Secondary predictors were derived from patient, injury, and hospitalization characteristics. The study outcome was death. Univariate time to event analyses were conducted for all study predictors. A multivariate regression model for mortality was created using all relevant predictors. Results: The final sample included 668 facial GSW injuries, of which 19.3% were attributed to self-harm. Self-inflicted GSWs were more likely to involve the mandible (58.9 vs 46.0%, P < 0.01), ZMC/maxilla (47.3 vs 32.5%, P < 0.01), and intracranial cavity (48.1 vs 22.6%, P < 0.01). The overall mortality rate was 7.3%, and the mean time to death was 2.2 days. After controlling for pertinent covariates, the risk of mortality was independently decreased with mandibular injury (HR = 0.36, P = 0.03). However, mortality was increased by self-harm intent (HR = 3.94, P < 0.01) and intracranial involvement (HR = 11.24, P < 0.01). Conclusions: Consistent with a pattern of intraoral and submental entry points, self-inflicted facial GSWs demonstrated higher rates of mandibular injury. Despite this finding, self-harm injuries still carried a higher incidence of intracranial injury and a greater independent risk of mortality. Our results refute any notion that the mechanism and trajectory of self-inflicted GSWs is less fatal.

Osteosarcoma of the skull base presenting as a petrocavernous pseudoaneurysm and masquerading as an intracranial abscess: illustrative case

BACKGROUND Telangiectatic osteosarcoma (TOS) is a rare and aggressive high-grade malignant neoplasm composed of blood-filled or empty cystic spaces resembling aneurysmal bone cysts. Uncommonly, TOSs can occur in the skull base. OBSERVATIONS The authors present a case of a TOS that presented as a petrocavernous carotid pseudoaneurysm and then masqueraded as an intracranial abscess. The prognosis for TOSs with intracranial involvement is typically unfavorable and inversely related to the degree of intracranial involvement. LESSONS Skull-based malignancies should be part of the differential diagnosis for a rapidly progressing lesion. Recovery of polymicrobial organisms during endoscopic sinus surgery should prompt reconsideration of the differential diagnosis. Postinflammatory changes from endovascular coiling have been described and can confound imaging and clinical findings.

Multifocal Intradural Extramedullary Anaplastic Ependymoma With Intracranial Involvement at Presentation: A Case Report

Background and Importance: Ependymomas are a rare malignant neoplasm. Multifocal intradural extramedullary anaplastic ependymomas are even more of a rare entity with much of the current knowledge derived from case reports. We presented a case of a multifocal intradural extramedullary anaplastic ependymoma with intracranial involvement at presentation. Case Presentation: A 53-year-old male presented with urinary symptoms. Magnetic resonance imaging revealed two lesions along the spinal cord and two lesions, intracranially. Histopathological examination was consistent with the World Health Organization grade III anaplastic ependymoma. The patient was treated with the gross total resections of spinal cord lesions, followed by radiation therapy to the resection cavities and intracranial lesions. At the 10-month follow-up visit, he reported almost complete resolution of symptoms, and magnetic resonance imaging revealed no recurrence. Conclusion: Despite their rarity, ependymomas should be considered as the differential diagnosis when evaluating spinal tumors. Gross total resection followed by targeted radiotherapy appears to be an effective treatment modality for high-grade lesions.

Rosai-Dorfman disease mimicking images of meningiomas: Two case reports and literature review

Background: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferative disorder classically as a massive cervical lymphadenopathy. However, over the years, extranodal locations were confirmed with the central nervous system involvement in less than 5% of cases, which is marked as a significant differential diagnosis of meningiomas, with which they are widely confused due to the similarity of their radiological images. Case Description: We report a 37-year-old man and 45-year-old man who were diagnosed with intracranial RDD but whose radiological images mimic meningiomas, requiring anatomopathological and tumor’s immunohistochemistry for definitive diagnosis. Moreover, a review of 184 publications with 285 cases of intracranial involvement of this disease was also performed, comparing these findings with those brought in the previous studies. Conclusion: Intracranial Rosai-Dorfman tumors should always be remembered as differential diagnosis of meningiomas since they are similar radiologically and macroscopically. Once remembered and diagnosed, the lesion must be treated following the same pattern of resection done in meningiomas and, treatment’s differences will not occur in the surgical excision technique, but in complementary chemotherapy implementation, radiotherapy, and even with radiosurgery aid, depending on the case. Thus, it is possible to obtain better results than with just the isolated surgical procedure.

Skull Base Primary Extracranial Meningioma with Hyperostosis in a Small Mixed-Breed Dog

ABSTRACT A 7 yr old female spayed Chihuahua-terrier mix was presented for a progressive dry, hacking cough over 9 mo, with dyspnea aggravated by eating and drinking. Computed tomography of the skull revealed a large mineral attenuating mass associated with the left skull base, without intracranial involvement. A modified ventral paramedian hypophysectomy approach along the medial aspect of the left ramus was used to approach the base of the skull. Ninety percent of the mass was debulked via high-speed pneumatic burr. Histopathology was consistent with hyperostosis originating from a primary extracranial meningioma (ECM), with the tissue staining positive for vimentin and negative for cytokeratin. The patient was symptom free for 9 mo before clinical signs returned because of tumor recurrence and was euthanized 11 mo postoperation because of diminished quality of life. ECM is uncommonly reported in the dog, and to the authors’ knowledge has not previously been reported with hyperostosis or located along the skull base at the level of the tympanic bulla. Additionally, although hyperostosis predominantly occurs as diffuse bone thickening adjacent to a meningioma, proliferative focal hyperostosis is uncommon. Given the findings in this patient, ECM should be considered as a differential diagnosis for osseous skull base masses.

A case of multiple synchronously diagnosed brain metastases from alveolar soft part sarcoma without concurrent lung involvement

Background: Alveolar soft part sarcoma (ASPS) is a rare soft-tissue sarcoma with a propensity for early hematogenous dissemination to the lungs and frequent brain metastasis. The development of lung metastasis almost invariably precedes intracranial involvement. There are no previously reported cases in which a patient was synchronously diagnosed with ASPS and multiple brain metastasis without lung involvement. Case Description: A 29-year-old gentleman was found to have three intracranial lesions following the onset of generalized seizures. Staging studies identified a soft-tissue mass in the left thigh and an adjacent femoral lesion. Biopsy of the soft-tissue mass was consistent with ASPS. The patient then underwent neoadjuvant stereotactic radiotherapy to all three brain lesions, followed by en bloc resection of the dominant lesion. The patient was then started on a programmed death-ligand 1 (PD-L1) inhibitor. Subsequent surgical resection of the primary lesion and femur metastasis demonstrates a histopathologic complete response of the bony metastasis and partial response of the primary lesion. At present, the patient has received 14 cycles of atezolizumab without recurrence of the primary or bony lesions and the irradiated intracranial disease has remained stable without recurrence of the resected dominant lesion. Conclusion: While intracranial involvement is relatively common in ASPS, a case with multiple, synchronously diagnosed brain metastasis without concurrent lung metastasis has not been described. The presented case discusses the safety and efficacy of aggressive management of intracranial disease in the setting of atezolizumab. Prospective evaluation of the efficacy of checkpoint inhibitors and the prognostic value of PD-L1 expression in ASPS with brain metastasis are necessary.