Intracranial Extension Research Articles

Clinical and Anthropological Analyses of a Case From 19th Century South Africa With a Confluence of Uncommon Cranial Pathological Conditions.

This study combines clinical and anthropological analyses to investigate the complex cranial pathology of a South African individual from the 19th century. The cranium was examined macroscopically and radiographically. Conducting a standard differential diagnosis was challenging given the complexity and uncommon nature of the pathology and required drawing on relatively sparse paleopathological and clinical case reports. Multiple conditions were identified including biparietal thinning, basilar invagination, platybasia, and complicated chronic frontal sinusitis, where the intracranial extension of sinus infection may likely have contributed to the individual's death. The authors urge for awareness of these uncommon conditions, as their presence can easily be overlooked or confound skeletal assessments. This clinical study contributes to the authors' understanding of uncommon and poorly described paleopathological diseases and will help to better facilitate their diagnosis in future research. It represents one of the first studies describing such an unusual cooccurrence of uncommon pathologies in an archeological individual.

Nasosinusal Teratocarcinosarcoma: Case Report and Literature Review

Sinonasal cancers are the most common malignant tumors of the anterior skull base. Teratocarcinosarcomas are extremely unusual malignat tumors involving nasosinusal area, they are characterized by their poor prognosis and high likelihood of recurrence and potentiel intracranial extension. Because of its histological heterogeneity, the diagnosis is very difficult and therefore requires a sufficient sample to avoid erroneous diagnoses. The most commonly used treatment option is radical surgery resection followed by radiotherapy +/- chemotherapy. We report herein the case of 44-year-old woman, presented with sinonasal teratocarcinosarcoma revealed by nasal obstruction and intermittent epistaxis. The diagnosis was confirmed by a histopathological study of surgical specimen. Pre-treatment workup was negative for metastasis disease. She has received adjuvant radiotherapy and chemotherapy after radical endoscopic sinonasal surgery. A follow-up examination at 2 years showed no evidence of recurrence.

A Clinico-pathological Study and Management of Fungal Rhinosinusitis

Background: Fungi are ubiquitous in the environment; hence we are constantly exposed to them. Objectives were to know the clinical presentation, radiological and pathological characteristics and management aspects of fungal rhinosinusitis.Methods: 30 cases of fungal rhinosinusitis reported during study period were evaluated and data was collected through history taking, clinical examination, haematological, histopathological and radiological investigations. Management by surgical & medical modalities were evaluated.Results: Fungal rhinosinusitis is more common in 3rd decade of life with male preponderance and more in low socio-economic status (73.3%). Nasal discharge (100%), nasal obstruction (96.7%), chronic headache (90%) and visual disturbances (23.3%) were important clinical features. Sinu-nasal polyp (66.7%) and allergic mucin (40%) were important associated factors. Orbital extension was seen in 33.3%, intracranial extension (20%) and extension to hard palate (6.7%). Computed Tomography scan had a sensitivity of 56.7% while histopathology and potassium hydroxide mount had a sensitivity of 93.33% in detecting cases. Observed mortality rate was 16.6%.Conclusion: Fungal rhinosinusitis is an important clinical entity which must be considered in all patientspresenting with unresolving rhinosinusitis

An interesting case of orbital tuberculosis with intracranial extension

Tuberculosis (TB) is a chronic airborne infectious disease caused by Mycobacterium tuberculosis and is one of the causes of morbidity and mortality in the underdeveloped nations. TB most commonly involves the lungs, but it can affect nearly any tissue in the body, including the orbit. Orbital TB is a rare manifestation even in endemic countries and mostly occurs as part of systemic involvement secondary to pulmonary disease. Exclusive extrapulmonary involvement of the orbit with intracranial extension is very rare; diagnostic dilemma, unique management, and the outcome make our case interesting and worthy of reporting.

Long-Term Outcomes Following Definitive or Adjuvant Proton Radiotherapy for Adenoid Cystic Carcinoma

PurposeAdenoid cystic carcinoma (ACC) is rare malignancy accounting for 1% of all head and neck cancers. Treatment for ACC has its challenges and risks, yet few outcomes studies exist. We present long-term outcomes of patients with ACC of the head and neck treated with proton therapy (PT). Materials and MethodsUnder an IRB-approved, single-institutional prospective outcomes registry, we reviewed the records of 56 patients with de novo, nonmetastatic ACC of the head and neck treated with PT with definitive (n = 9) or adjuvant PT (n = 47) from June 2007 – December 2021. The median dose to the primary site was 72.6 GyRBE (range, 64 – 74.4) delivered as either once (n = 19) or twice (n = 37) daily treatments. Thirty patients received concurrent chemotherapy. Thirty-one patients received nodal radiation, 30 electively and 1 for nodal involvement. ResultsWith a median follow-up of 6.2 years (range, 0.9–14.7), the 5-year local-regional control (LRC), disease-free survival, cause-specific survival, and overall survival rates were 88%, 85%, 89%, and 89%, respectively. Intracranial extension (P = 0.003) and gross residual tumor (P = 0.0388) were factors associated with LRC rates. While the LRC rate for those with a gross total resection was 96%, those with subtotal resection (STR) or biopsy alone were 81% and 76%, respectively. The 5-year cumulative incidence of clinically significant grade ≥3 toxicity was 15%, and the crude incidence at the most recent follow-up was 23% (n = 13). ConclusionsThis is the largest sample size with the longest median follow-up to date of patients with ACC treated with PT. PT can provide excellent disease control for ACC of the head and neck with acceptable toxicity. T4 disease, intracranial involvement, and gross residual disease at the time of PT following either biopsy or STR were significant prognostic features for worse outcomes. Data Sharing StatementThe authors agree to share anonymized data upon reasonable request by researchers.

Gross-total resection in optic nerve sheath meningiomas: minimally invasive and cosmetic pleasing.

The optic nerve sheath meningioma (ONSM) is one of the most challenging tumors in orbital surgery. From the perspective of mental health and patient needs, we analyzed the necessity and importance of the endoscopic transnasal approach (ETA) combined with optic nerve transection (ONT) in gross-total resection (GTR) in ONSM patients with residual vision and aim to broaden the use of ONT for specific people. The authors included patients with ONSMs who were treated between 2014 and 2022. We divided those cases into two groups named ETA group and lateral orbitotomy approach (LOA) group. We present the application of ETA and analyze the preoperative indication of the ONT and compared the advantages and disadvantages between ETA and LOA. The degree of tumor resection was based on imaging and surgical evaluation. A total of 23 patients with ONSM were included. Sixteen patients underwent ETA, and seven underwent LOA. Among ETA cases, GTR was achieved in 14 patients with ONT and most patients maintained normal eye movement function (75%) and morphology (93.75%). In the ETA group, 14 patients experienced vision loss, while two other patients saw improvements in vision. And proptosis was alleviated (5.20 ± 2.34 vs 0.27 ± 0.46, p < 0.0001). Six patients with blindness and proptosis of the LOA group resulted in GTR with ONT and ophthalmectomy. Although intracranial extension and recurrence included no cases in the two groups, a significant psychological gap was presented due to cosmetic problems. Under the premise of reducing damage and improving aesthetics, the selection of ETA combined with ONT to gross-total resect ONSMs successfully provides a minimally invasive access with acceptable complications. As an important adjunct to GTR in the surgical treatment of ONSM, the scope of ONT application should be expanded to relieve the patient's psychological burden.

A Rare Presentation of Occipital Dermoid Cyst with Intracranial Extension and Secondary Infection: Case Report and Follow-Up

AbstractCraniofacial dermoid cysts are congenital anomalies that rarely have intracranial extension and can be associated with other anomalies. Common sites of these lesions are the lateral brow and parietal scalp. Presentation of the dermoid cyst in the occipital region with intracranial extension is extremely rare. We report a 2-year-old female with a presentation of an occipital dermoid cyst with intracranial extension and secondary cerebellar abscess. This case highlights the rarity of the presentation of an occipital dermoid cyst with intracranial extension and secondary infection and the importance of early imaging for suspected dermoid cysts in the occipital region for identification of intracranial extension.

Ventricular empyema in a dog diagnosed by magnetic resonance imaging and immediate postmortem cerebrospinal fluid culture

AbstractA 1‐year 5‐month‐old, female, neutered springer spaniel presented collapsed in extensor rigidity. The dog had undergone ovariectomy 6 weeks previously, and a tooth root abscess was documented 2 months before this. Neurological signs were first reported 24 days after surgery, starting with lethargy and tremors, which progressed to generalised tonic‐clonic cluster seizures with inter‐ictal neurological deficits by 33 days after surgery. Magnetic resonance imaging of the brain revealed a conspicuous fluid–fluid level in the lateral ventricles consistent with ventricular empyema. Treatment was declined in favour of euthanasia, but postmortem cerebrospinal fluid sample showed marked pleocytosis with elevated protein and cultured a heavy growth of Staphylococcus pseudintermedius. The magnetic resonance imaging findings and positive cerebrospinal fluid culture were consistent with bacterial meningoencephalitis. Aetiology in this case was unknown, but may have been secondary to haematogenous spread, which is a rare but potential sequel to abdominal surgery, or intracranial extension of an oral infection.

Primary Extracranial Meningiomas of the Sinonasal Tract: A Systematic Review

Abstract Background Primary extracranial meningiomas (PEMs) of the sinonasal tract with no intracranial extension are rare. Our study presents the largest systematic review to date, providing a comprehensive overview and comparison of the characteristics, treatment, and prognosis of PEMs, with comparison to primary intracranial meningiomas (PIMs). Methods A systematic review was conducted according to the PRISMA guidelines on PubMed, Embase, and Google Scholar up to November 1, 2022. A total of 70 documented cases from 64 articles were included. Data analysis was performed to compare low-grade (World Health Organization [WHO] Grade I) and high-grade (Grade II, III) PEMs. Results Majority of patients were symptomatic with nasal symptoms (72.1%), followed by eye symptoms (41.2%) and facial swellings (41.2%). The nasal cavity was the most commonly affected site (70.0%). Most tumors were WHO Grade 1 (87.5%) with meningothelial histological subtype. PEMs have a predilection for involvement of surrounding structures, with more than half reporting erosion of sinonasal walls or orbital involvement (64.3%). Despite so, PEMs have a favorable prognosis, with high survival rates (95.3%) and low rate of recurrence (8.5%). Mainstay of treatment is surgical resection (98.6%). A total of 69/70 cases (98.6%) underwent surgical resection. Conclusions There are no clear defining clinical features of PEMs; hence, it is imperative for clinicians to prioritize histological diagnosis to guide further management. A conclusive diagnosis of PEMs, i.e., exclusion of PIMs, requires imaging and potential intraoperative assessment. Its prognosis is likely dependent on both WHO grading and the ability to achieve complete surgical extirpation. Endoscopic resection is recommended even in high-grade PEMs for localized and resectable disease. Radiotherapy may be reserved for unresectable or recurrent tumors as salvage therapy.

Toward Individual Treatment in Cervical Artery Dissection: Subgroup Analysis of the TREAT-CAD Randomized Trial.

Uncertainty remains regarding antithrombotic treatment in cervical artery dissection. This analysis aimed to explore whether certain patient profiles influence the effects of different types of antithrombotic treatment. This was a post hoc exploratory analysis based on the per-protocol dataset from TREAT-CAD (NCT02046460), a randomized controlled trial comparing aspirin to anticoagulation in patients with cervical artery dissection. We explored the potential effects of distinct patient profiles on outcomes in participants treated with either aspirin or anticoagulation. Profiles included (1) presenting with ischemia (no/yes), (2) occlusion of the dissected artery (no/yes), (3) early versus delayed treatment start (</>median), and (4) intracranial extension of the dissection (no/yes). Outcomes included clinical (stroke, major hemorrhage, death) and magnetic resonance imaging outcomes (new ischemic or hemorrhagic brain lesions) and were assessed for each subgroup in separate logistic models without adjustment for multiple testing. All 173 (100%) per-protocol participants were eligible for the analyses. Participants without occlusion had decreased odds of events when treated with anticoagulation (odds ratio [OR] = 0.28, 95% confidence interval [CI] = 0.07-0.86). This effect was more pronounced in participants presenting with cerebral ischemia (n = 118; OR = 0.16, 95% CI = 0.04-0.55). In the latter, those with early treatment (OR = 0.26, 95% CI = 0.07-0.85) or without intracranial extension of the dissection (OR = 0.34, 95% CI = 0.11-0.97) had decreased odds of events when treated with anticoagulation. Anticoagulation might be preferable in patients with cervical artery dissection presenting with ischemia and no occlusion or no intracranial extension of the dissection. These findings need confirmation. ANN NEUROL 2024.

Recurrent Orbital Subperiosteal Abscess in Patient with Nasopharyngeal Carcinoma

Introduction : Subperiosteal abscess (SPA) is a rare finding in cancer patients with orbital cellulitis. Severe complications associated with this condition are permanent visual loss, intracranial extension, and death.&#x0D; Case Illustration : A 38-year-old male with history of nasopharyngeal carcinoma (NPC) came with progressive right eyelid swelling, non-axial proptosis, and visual acuity was NLP. CT scan revealed right SPA associated with frontal sinusitis. NPC staging was T3N2M0. Despite the treatment of intravenous antibiotics and drainage incision of SPA, there was recurrence of right SPA two weeks after procedure and re-staging revealed T4N3M0. A joint operation with otolaryngology department to do debulking of the mass, frontal sinus surgery, and drainage incision was held. Culture resulted in Talaromyces marneffei. Patient was assessed with talaromycosis and given itraconazole for ten weeks. Moreover, patient came again with proptosis seven weeks postoperatively. Re-drainage incision was performed and clinical presentation was improved.&#x0D; Discussion : This case describes rare biological nature of NPC where extensive blockage of paranasal sinuses allows microorganism overgrowth and infection. The anatomical changes and cancer-related immunosuppression contribute to recurrent SPA. Chemotherapy and radiotherapy are ideal treatments for our patient, however, infection must be treated adequately first. Surgical interventionis needed to manage the infection by SPA drainage where antimicrobial has poor penetration into the abscess and debulking of tumor to clear the blockage of drainage pathway.&#x0D; Conclusion : Recurrent orbital SPA could happen in patients with NPC, therefore appropriate medical treatment and timely surgical intervention are essential in managing orbital SPA. Multidisciplinary management is required to optimize patient outcomes.

Role of magnetic resonance imaging in sinonasal pathology: a review

The last several years have seen a considerable advancement in the treatment of sinonasal diseases. The advent of minimally invasive functional endoscopic sinus surgery has led to an increased understanding of the anatomy and function of the sinonasal region, making previously inaccessible areas safe to operate in. The advancement in imaging of the sinonasal tract has been associated with the evolution of endoscopic sinus surgery. The imaging of sinonasal pathology has progressed from conventional radiographs (plain films) to computed tomography (CT) and magnetic resonance imaging (MRI). Technological advancements in CT and MRI have given a precise differential diagnosis and greater detail about the anatomical extent of sinonasal pathologies. Because the high water content of the inflammatory state causes a markedly enhanced signal on T2-weighted scans, MRI can currently distinguish between retained secretions and inflammatory responses from the mass of the tumor. The invasive lesions affecting the soft tissues outside of the sinonasal region are more easily distinguished by MRI. MRI largely took the position of CT scan for malignant sinonasal lesions due to its superior capacity to distinguish between inflammation and tumor and its increased sensitivity for intracranial extension. MRI can be more useful than a CT scan in demonstrating the degree of soft tissue abnormalities and in identifying lesions that have returned after surgery.

Inflammatory myofibroblastic disease of right petrous apex: A rare case with review of literature.

Inflammatory myofibroblastic tumor (IMFT) is a rare tumor of unknown etiology. It can involve any part of the body. The IMFT involving the base of skull is rare with only 36 cases reported in the literature. We report a rare case of IMFT of temporal bone with review of literature. A 42 year old male presented with complaints of headache and double vision and MRI brain showed lesion in the right petrous apex region suggestive of a neurogenic mass. He had excision of lesion and histopathology was suggestive of IMFT with IgG4 and ALK positive. He had complete clinical response but a month later he presented with right eyelid ptosis and decreased rotation of eye medially with recurrent lesion on MRI. Patient received radiation by SRT technique and then started on Ceretinib with partial response. The IMFT is rare tumor of unknown etiology and tumors of temporal bone are more aggressive. It is benign but locally invasive tumor. Treatment of IMFT is controversial. Extensive surgery with complete excision has about 80% response rates and with intracranial extension, adjuvant radiation is need. In head and neck IMFT response rates are lower (30 to 40%). Monoclonal antibodies and steroids are used in IMFT at recurrence. In advanced or metastatic ALK positive tumors, Crizotinib is used with a response rate of 50%. Radiotherapy (25 to 30 Gy) induces remission and helps to taper the steroids. Temporal bone IMFT is a rare tumor with multimodality approach and variable response to treatment.

A large cirsoid aneurysm of the scalp with multiple arterial supply: illustrative case.

Cirsoid aneurysm of the scalp is a rare arteriovenous fistula having a traumatic, congenital, iatrogenic, or idiopathic etiology. Its presentation can range from a small swelling to a large pulsatile mass with tinnitus, headache, and scalp necrosis. A 67-year-old female presented with a gradually increasing swelling on her forehead and head since childhood and no history of trauma. Examination revealed 12 × 5 cm tortuous midline swelling. Computed tomography angiography revealed a mass of tortuous vessels in the right frontoparietal region of the scalp with no bony defect or intracranial extension. Contrast-enhanced computed tomography of the head showed no intracranial pathology. The diagnosis of cirsoid aneurysm was made, and surgery was planned. A bicoronal incision was made. The feeding arteries were dissected and ligated. The nidus was carefully separated, cauterized, and excised in toto. Inadvertently, a buttonhole in the skin was created while dissecting the nidus, which was sutured. The patient developed a small area of scalp necrosis on the 10th postoperative day, which was debrided and sutured. At the 6-month follow-up, no signs of recurrence were present. A large cirsoid aneurysm of the scalp with multiple arterial supplies can be treated successfully with surgery. Meticulous dissection and hemostasis are warranted to avoid perioperative complications.

SMARCB1-Deficient Skull Base Chondrosarcoma with 12p Duplication Presenting as Somatic-Type Malignancy Arising from Metastatic Seminoma

Somatic-type malignancy (STM) can occur infrequently within a primary or metastatic testicular germ cell tumor (TGCT) and is associated with dismal prognosis and survival. STM with chondrosarcomatous features is exceedingly rare and head and neck involvement has not been previously documented. A 39-year-old white man presented with nasal obstruction and epistaxis. Imaging disclosed a 6.9-cm expansile tumor involving the nasal cavity and skull base with intraorbital and intracranial extension. The histopathologic properties of the tumor were compatible with chondrosarcoma, grade II-III. Immunohistochemically, malignant cells were strongly and diffusely positive for S100 and epithelial markers, and showed loss of SMARCB1 expression. IDH1/2 mutations were not detected. Following whole-body PET scan, a 7.0-cm left testicular mass was discovered and diagnosed as seminoma with syncytiotrophoblastic cells, stage pT3NXM1b. Extensive retroperitoneal, mediastinal, and supraclavicular lymphadenopathy was also noticed. Histopathologic examination of the left supraclavicular lymph node revealed metastatic seminoma. By FISH, most metastatic nodal seminoma cells harbored 1 to 4 copies of isochromosome 12p, while the chondrosarcoma featured duplication of 12p. Presence of a malignant TGCT with disseminated supradiaphragmatic lymphadenopathy, the unique immunophenotypic properties of the skull-based chondrosarcoma and lack of IDH1/2 aberrations with gain of 12p strongly support the diagnosis of STM chondrosarcoma arising from metastatic TGCT. The patient did not respond to chemotherapy and succumbed three months after diagnosis. Although exceedingly uncommon, metastasis to the head and neck may occur in patients with TGCT. This case of STM chondrosarcoma demonstrated divergent immunophenotypic and molecular characteristics compared to “typical” examples of head and neck chondrosarcoma. High index of suspicion is advised regarding the diagnosis of lesions that present with otherwise typical histomorphology but unexpected immunohistochemical or molecular features.

The Clinical Course of Paranasal Sinus Mucocoeles-A Retrospective Case Series.

Paranasal sinus mucocoeles can be secondary to chronic rhinosinusitis and can result in intra-orbital and intra-cranial complications requiring surgical management. The natural history of conservatively managed mucocoeles is not well established. We aimed to quantify the proportion of radiologically identified paranasal sinus mucocoeles resulting in complications over 10 years. We retrospectively reviewed anonymised data on radiologically diagnosed mucocoeles between 2011 and 2021 at two UK hospitals. We collected data on age at presentation, extent of sinus involvement, management and complications. We identified 60 patients with mucocoeles, of which 35 (58%) were incidental findings from radiological investigations. The mean age was 58 years. Fifteen patients (25%) were managed surgically and one presented with recurrence following surgery. Overall, six patients (10%) had an intra-orbital extension of their mucocoele and three (5%) had an intra-cranial extension. There was no difference in the rates of intra-cranial extension between conservative and surgical cases but surgical cases included a higher rate of intra-orbital extensions (27% vs. 4%, p = 0.01). The proportion of patients requiring surgical intervention in this study is low. Incidental and asymptomatic mucocoeles have a relatively benign disease course and selected uncomplicated cases can be considered for conservative management with serial scanning at 12 months.

Surgical Treatment of Malignant Peripheral Nerve Sheath Tumor of the Scalp With Intracranial and Extracranial Extension: A Rare Case Report.

Malignant peripheral nerve sheath tumors of the scalp are rare neoplasms of the peripheral nervous system. Here, we describe an unusual malignant peripheral nerve sheath tumor of the scalp in an 84-year-old Asian man. The tumor was associated with bony destruction, intracranial, and extracranial extension. Trans-arterial embolization was done twice preoperatively. En block excision was performed and the dura and soft tissue defect were reconstructed by anterolateral thigh free fasciocutaneous flap. There is no recurrence and the wound healed well during follow-up.

Diagnosis and surgical management of a type IVb dermoid sinus in a Cavalier King Charles Spaniel

AbstractA 6‐year‐old, male, neutered Cavalier King Charles Spaniel dog was referred with a 4‐year history of an intermittently draining fistula on the midline of the frontal bones of the skull. Magnetic resonance imaging and computed tomography scan of the head demonstrated an extra‐axial mass in the midline between the frontal lobes, contacting the meninges and extending rostrally through a defect in the frontal bones. The mass was surgically excised via a frontal craniotomy. Histopathology of the mass demonstrated stratified squamous and keratinising epithelium with adnexal structures, consistent with the diagnosis of a dermoid sinus. Previous reports have documented dermoid sinuses in the nasal region in dogs and cats with intracranial extension. This case report documents a dermoid sinus in the frontal region with intracranial extension in a dog, confirmed by advanced diagnostic imaging, histopathology, and successfully treated surgically.

Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension.

Primary Ewing's sarcoma of the skull is a very rare malignant neoplasm, predominantly occurring in children and adolescents. We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the standards of therapy for this type of tumor. This 18-year-old male patient presented with a primary Ewing's sarcoma of the skull, involving the dura of the frontal and parietal lobes of the left cerebral hemisphere. He was treated with gross total surgical excision of tumor, skull reconstruction, chemotherapy, and irradiation. Twelve years after the surgery, the patient has no evidence of local recurrence or distant metastases. Radical surgical excision of the primary tumor with safety margins is thought to play a role in the favorable clinical course. The presented case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull bone. This rare type of tumor may allow better survival rates under adequate management than sarcoma elsewhere in the body.

Atretic cephalocele and differential considerations: A small case series.

Any posterior midline cystic or soft tissue scalp mass in an infant needs evaluation with MRI brain and MRV brain to look for intracranial extension of the lesion. One of the differential considerations is an atretic cephalocele, which can be a difficult diagnosis, especially when the cystic lesions are small. The presence of a small calvarial defect adjacent to the cystic mass and a persistent primitive falcine vein, which points towards the cystic mass, are imaging clues to this diagnosis. Correct diagnosis is needed prior to surgical resection to avoid potentially serious postoperative complications.