Rosai-Dorfman sphenoorbital histiocytosis with intraparenchymal invasion: Do we have to consider this skull base pathology as a malignant disease?

Background: Rosai-Dorfman disease (RDD) is a rare type of histiocytosis that can manifest with diverse symptoms. It usually presents with systemic involvement, and only a few cases have been reported at the level of the skull base. RDD typically follows a benign course during the progression of the disease. In this particular case reported, after the skull base invasion, the disease started to infiltrate the brain parenchyma. Our objective for this case report was to present this particular progression pattern and the nuances of its surgical treatment. In addition, a revision of the current literature was performed about skull base RDD with intracranial invasion and brain parenchyma infiltration not previously described. Case Description: We are presenting the case study of a 57-year-old male patient who was experiencing severe headaches and an increase in volume in the right fronto-orbital region. On clinical examination, no neurologic clinical symptoms were observed. Contrast computed tomography and magnetic resonance imaging showed a tumor mass that affected the right orbit, frontal paranasal sinus, greater sphenoid wing, and right frontal lobe with moderate adjacent brain edema. The patient underwent surgery using an extended pterional approach with intracranial, orbital decompression, and frontal sinus cranialization, accompanied by frontal lobe tumor resection. Neuropathologic diagnosis revealed a Rosai-Dorfman histiocytosis disease. Conclusion: The etiopathogenesis of RDD is still not completely understood. The current literature considers this disease to have a predominantly benign course. Nevertheless, as we have shown in this case, it may, in some cases, present direct parenchymal invasion. We consider that prompt surgical treatment should be ideal to avoid the local and systemic progression of the disease.

Intraparenchymal low-grade B-cell lymphomas of the central nervous system: Clinicopathologic and molecular analysis of three cases and a review of the literature.

Primary central nervous system (CNS) lymphomas represent 1 % of all non-Hodgkin lymphomas, with diffuse large B-cell lymphomas as the prevailing subtype. Low-grade B-cell lymphomas are exceptional with only 24 marginal zone B-cell lymphomas (EMZL) and 1 follicular lymphoma (FL) previously reported so far. While their molecular profiles are studied elsewhere, data on primary intraparenchymal CNS cases remain limited. The objective of the present study is to contribute new cases of primary intraprenchymal low-grade B-cell lymphomas in the CNS and characterize their mutational profile. We conducted a comprehensive review of cases and a literature review to identify similar instances. Clinical, imaging, histological, immunohistochemical, and molecular characteristics were analyzed. Diagnoses were established according to established criteria. We present three novel cases of intraparenchymal CNS low-grade B-cell lymphomas. One case of intraparenchymal EMZL exhibited plasmacytic differentiation, while another lacked a plasma cell component. The third case was diagnosed as FL. The L265P mutation of MYD88 was absent in all cases. Next generation sequencing revealed pathogenic mutations in SPEN (Glu1970ValfsTer64) and ARID1A (Pro1355LeufsTer118) genes in one EMZL case. In conclusion, intraparenchymal CNS low-grade B-cell lymphomas are rare, with few reported cases. Our findings expand knowledge on their clinical and molecular features. We present the first molecular profile of primary CNS intraparenchymal EMZL, underscoring the need for further research to understand their biology and optimize treatment strategies.

Malignant transformation of an intraparenchymal hemangioma in the cervical spinal cord of a German shepherd dog.

An 8-year-old female spayed German shepherd dog was presented for evaluation of a 1-week history of right thoracic limb monoparesis. Magnetic resonance imaging (MRI) identified an intraparenchymal, T2 hypointense and T1 isointense, strongly heterogeneously contrast-enhancing mass with moderate internal susceptibility artifact on T2* images at the level of the cranial extent of the C5 vertebral body. Euthanasia was elected after a rapid neurologic decline in the 24 hours after MRI. Necropsy and histopathology identified an intraparenchymal hemangiosarcoma arising from a hemangioma in the cervical spinal cord, with no evidence of neoplastic disease in any other examined organs. The spectrum of vasoproliferative disorders in the central nervous system in veterinary species has been codified recently, but hemangiosarcoma is considered metastatic to the central nervous system. Herein we describe the clinical, imaging, and histologic findings in a dog with a novel primary location of hemangiosarcoma in the cervical spinal cord.

Local Delivery of Irinotecan to Recurrent GBM Patients at Reoperation Offers a Safe Route of Administration.

Glioblastomas are impossible to completely resect and almost always recur at the borders of the resection margin. There is no established chemotherapy regimen available to patients who recur, while systemic treatment is hampered by the blood-brain barrier. Here, we report on the first evaluation in humans of the intraparenchymal injection of irinotecan into the resection cavity after surgical resection of recurrent glioblastoma patients. The cytotoxicity of irinotecan was compared to SN-38 in primary cells from recurrent glioblastoma patients. Irinotecan was injected at multiple (~30) sites of the resection cavity wall at a depth of 3 to 5 mm. SN-38 was more cytotoxic than irinotecan at concentrations below 1 µM due to enzyme kinetics. The intraparenchymal administration of irinotecan was safe, with good wound healing and an absence of swelling, inflammation, or pseudo-abscess formation. The median survival post irinotecan administration was 32.6 weeks. The median overall survival was 30.5 months, with a two-year survival rate of 56%. This study demonstrates that local delivery of irinotecan into the brain parenchyma offers a safe route of administration over systemic delivery in the treatment of recurrent glioblastoma.

Intraparenchymal Chordoma in the Brain Stem: A Review of Surgical Management and Case Highlight.

We present a rare case of an intraparenchymal chordoma in the brain stem of a 69-year-old male with a history of multiple chordoma recurrences. Chordomas are uncommon tumors that originate from notochordal remnants, with intraparenchymal presentations in the brain stem being particularly rare. A 69-year-old male with a history of clival chordoma three years after primary endoscopic resection and adjuvant proton-beam radiotherapy and a recurrence one year postoperatively for which he underwent a second surgery, presented with severe headaches, weakness, diaphoresis, and difficulty ambulating. Head CT in the ER revealed a 2.7 x 3.5 cm hyperdense lesion in the pons, indicating acute hemorrhage. Magnetic resonance imaging (MRI) suggested a hemorrhagic radiation-induced cavernoma. A right retrosigmoid craniotomy was performed, and the lesion was resected without major complications. Final pathology reported an intraparenchymal hemorrhagic chordoma. To our knowledge, this is the first case of intra-axial chordoma, particularly in the brain stem. It highlights the importance of considering intraparenchymal chordoma on the differential when evaluating for recurrence versus other treatment-induced pathologies and changes. This may prompt the neurosurgeon to reconsider treatment options and weigh the risks of watchful waiting versus biopsy or even aggressive surgical management.

A Case of Basal Ganglia Intraparenchymal Hemorrhage Following Lumbar Spinal Surgery.

We report on a rare case of basal ganglia intraparenchymal hemorrhage with intraventricular extension occurring after a lumbar spinal surgery. A 65-year-old female presented for an elective L4-L5 lumbar laminectomy and posterior spinal fixation. Her initial operation was complicated by a cerebrospinal fluid (CSF) leak repaired with a dural synthetic graft. Her immediate post-operative course was complicated by delayed emergence, eye-opening apraxia, and left-sided hemiplegia and subsequent computed tomography (CT) of the head demonstrated a right-sided basal ganglia intraparenchymal hemorrhage (IPH) with intraventricular extension. CT angiogram of the head was unremarkable. She was taken back to the operating room for right-sided decompressive hemicraniectomy and external ventricular drainage (EVD) for hydrocephalus. Her EVD was discontinued on post-bleed day 13 and she was discharged on post-bleed day 14 to a long-term care facility with a modified Rankin scale (mRS) score of 6. She returned for a cranioplasty six months later, and on her last follow-up at nine months, had a mRS of 4 with persistent confusion and severe left-sided hemiparesis but was able to form simple sentences. In summary, intracranial hemorrhage is a rare complication of spine surgery, occurring in a small percentage of the population. Lobar IPH following spinal surgery is a rare complication, and has been hypothesized to be a result of excessive CSF loss during durotomy.

Primary intracranial pediatric ganglioneuroblastoma-report of two cases and review of an unusual masquerader.

Ganglioneuroblastoma (GNB) is a rare neurogenic tumor with a predilection for extracranial sites. Its primary intracranial occurrence is anecdotal, with less than ten cases reported in pediatric literature. We report two cases of this unusual entity. The first was a 1-year-old boy who presented with a progressive scalp swelling that radiologically appeared to be a mesenchymal tumor. The tumor was resected completely, and its histopathological examination was suggestive of a pigmented GNB. The clinico-radiological presentation and melanin pigmentation of the tumor were unique features of the case. The second case was a 7-year-old girl who presented with left hemiparesis and raised intracranial pressure due to a large right parietal intraparenchymal tumor that histologically proved to be a GNB. The child succumbed to disease progression a month after undergoing near-total resection of the tumor. Analysis of our cases in the light of a literature review reveals that pediatric intracranial GNBs have diverse clinico-radiological features and can easily be confused for commoner pathologies in both intra- and extra-axial locations.

New concept in selecting blue dye injection site effect on clinical outcome of early-stage breast cancer patients: a retrospective cohort.

BACKGROUND: Clinico-anatomical review and pilot studies demonstrated that intraparenchymal injection at any site, even those not containing the index lesion, or periareolar injections should provide concordant outcomes to peritumoral injections. METHOD: This was a single-center retrospective cohort at King Chulalongkorn Memorial Hospital. The electronic medical records of patients were characterized into conventional and new injection concept groups. The inclusion criteria were patients who had either a mastectomy or BCS along with SLNB. We excluded patients who underwent ALND, received neoadjuvant therapy, or had non-invasive breast cancer. The primary outcome was the 5-year rate of breast cancer regional recurrence. Additionally, we reported on the re-operation rate, disease-free period, distant disease-free period, mortality rate, and recurrence rates both locoregional and systemic. Recurrences were identified through clinical assessments and imaging. SURGICAL TECHNIQUE: 3 ml of 1%isosulfan blue dye was injected, with the injection site varying according to the specific concept being applied. In cases of SSM and NSM following the new concept, the blue dye was injected at non-periareolar and non-peritumoral sites. After the injection, a 10-minute interval was observed without massaging the injection site. Following this interval, an incision was made to access the SLNs, which were subsequently identified, excised, and sent for either frozen section analysis or permanent section examination. RESULT: There were no significant differences in DFS, DDFS or BCSS between the two groups (p = 0.832, 0.712, 0.157). Although the re-operation rate in the NI group was approximately half that of the CI group, this difference was not statistically significant (p = 0.355). CONCLUSION: Our study suggests that tailoring isosulfan blue dye injection site based on operation type rather than tumor location is safe and effective approach for SLN localization in early-stage breast cancer. However, this study has limitations, including being a single-center study with low recurrence and death cases. Future studies should aim to increase the sample size and follow-up period.

Intraparenchymal Indocyanine Green Use Improves Nodal Yield During Minimally Invasive Tumor Nephrectomy in Children.

Introduction: Correctly identifying positive lymph nodes associated with pediatric renal tumors is key to guiding management. Recommended targets for lymph node sampling are commonly missed during tumor nephrectomy, particularly if minimally invasive surgery (MIS) is performed. Indocyanine green (ICG) is used for lymph node mapping in adult oncology with excellent efficacy and safety profile. Materials and Methods: A prospective study was undertaken at a single-quaternary-level pediatric surgery center. All patients undergoing MIS radical or partial nephrectomy for renal tumors 2016-2023 were included. Patients managed from 2020 onwards received intra-parenchymal ICG prior to lymph node sampling. Main Results: Twenty-five patients underwent MIS nephrectomy at mean age 2 years 10 months. Eighteen patients were pre-ICG and 7 received ICG. ICG administration successfully demonstrated fluorescent nodes in all patients. Median number of nodes sampled was three pre-ICG and seven with ICG (P = 0.009). Forty-six nodes were sampled across 7 ICG patients-33 fluorescent, 10 non-fluorescent, and 3 identified histologically. Three nodes overall contained active disease, two pre-ICG and one fluorescent node with ICG. Neither operative time (180 pre-ICG versus 161 minutes ICG, P = 0.7) nor length of stay (72 versus 84 hours, P = 0.3) were significantly affected by ICG administration. There were no adverse events associated with ICG use. Conclusions: ICG is safe and effective at identifying nodes in MIS resection of pediatric renal tumors with the potential to increase the number of nodes sampled. Further research is needed, specifically a randomized control trial with extended follow-up.

Adeno-associated virus vector delivery to the brain: Technology advancements and clinical applications.

Adeno-associated virus (AAV) vectors have emerged as a promising tool in the development of gene therapies for various neurological diseases, including Alzheimer's disease and Parkinson's disease. However, the blood-brain barrier (BBB) poses a significant challenge to successfully delivering AAV vectors to the brain. Strategies that can overcome the BBB to improve the AAV delivery efficiency to the brain are essential to successful brain-targeted gene therapy. This review provides an overview of existing strategies employed for AAV delivery to the brain, including direct intraparenchymal injection, intra-cerebral spinal fluid injection, intranasal delivery, and intravenous injection of BBB-permeable AAVs. Focused ultrasound has emerged as a promising technology for the noninvasive and spatially targeted delivery of AAV administered by intravenous injection. This review also summarizes each strategy's current preclinical and clinical applications in treating neurological diseases. Moreover, this review includes a detailed discussion of the recent advances in the emerging focused ultrasound-mediated AAV delivery. Understanding the state-of-the-art of these gene delivery approaches is critical for future technology development to fulfill the great promise of AAV in neurological disease treatment.

Remote supratentorial intraparenchymal bleed after posterior fossa surgery: A rare occurrence.

Background: Intraparenchymal hemorrhage at the operative site is one of the major complications of brain surgery. It is unusual to occur at a site remote from the operative site, but when it happens, it may cause significant morbidity and mortality. Case Description: We report the case of a 27-year-old male who presented with complaints of paresthesias over the left side of his face and decreased hearing from the left ear for two years. His radiology was suggestive of a large left cerebellopontine angle epidermoid cyst. The patient underwent left retro mastoid suboccipital craniotomy and near-total excision of the epidermoid cyst. The immediate postoperative non-contrast computed tomography scan of the brain was suggestive of no hematoma at the operated site but a remote left parafalcine frontoparietal intraparenchymal bleed, which was managed conservatively. At two months follow-up, he had no neuro deficits, and magnetic resonance imaging of the brain was suggestive of near-total excision of the epidermoid cyst with resolving left parafalcine frontoparietal bleed. Conclusion: We report this case due to the unique case observation of an intracranial bleed at a remote site rather than at the operated site.

Neurocysticercosis: A Challenging Case in New Mexico.

Neurocysticercosis, a parasitic infection caused by the larvae of Taenia solium, presents a diagnostic challenge, particularly in non-endemic regions. This case report evaluates the intricacies of diagnosing neurocysticercosis in a 62-year-old male presenting with an intractable headache and altered mental status, initially confounded by the absence of an exposure history. Despite lacking typical risk factors, including immunocompromise or HIV infection, his travel history to an endemic area prompted a rigorous clinical work-up. Imaging studies revealed characteristic ring-enhancing lesions indicative of neurocysticercosis, while further serological tests yielded mostly equivocal results. Infectious disease consultation and workup helped confirm a probable diagnosis. Prompt initiation of anti-helminthic therapy led to marked clinical improvement. This case underscores the importance of considering neurocysticercosis in the differential diagnosis of intracranial lesions, especially in regions with Hispanic populations, and advocates for increased awareness and research to enhance timely identification and management, thereby improving patient outcomes.

Successful coiling embolization of an intraparenchymal renal pseudoaneurysm: Case reports.

INTRODUCTION AND IMPORTANCE: Intraparenchymal renal artery aneurysms are a really rare condition that represents <10 % of all renal artery aneurysms. They are more often caused by trauma or iatrogenic injury and their rupture can lead to life-threatening hemorrhage. CASE PRESENTATION: We report the case of a 25-year-old male with history of back stab wound three months before being admitted to our unity. The patient presented a macroscopic hematuria three days after the injury. An abdominal CT angiography revealed an intraparenchymal renal artery aneurysm, and it was successfully treated with super selective endovascular coil embolization, achieving both pseudoaneurysm exclusion and renal preservation. CLINICAL DISCUSSION: Intraparenchymal renal artery aneurysms are rare. They are more often caused by trauma or iatrogenic injury. Renal artery pseudoaneurysms are mainly asymptomatic. Renal pseudoaneurysms can be diagnosed through contrast enhanced CT, renal Doppler ultrasound, or MRI. Super selective embolization of the feeding artery of the pseudoaneurysm using endovascular techniques is the treatment of choice. CONCLUSION: The management of patients with renal pseudo aneurysms should allow to achieve two main goals: Preserving Renal function, and excluding the pseudoaneurysm.

Intracranial parenchymal capillary hemangioma: A case report.

Background: Capillary hemangioma is a rare benign hemangioma that occurs in the soft tissues of the skin, orbit, head, and neck. Intracranial cases, especially intraparenchymal cases, are extremely rare. In this study, we report the course of an intracranial parenchymal capillary hemangioma with left mild motor paresis and involuntary movements of the left upper extremity and was successfully treated by surgical resection, including radiological and pathological examinations. Case Description: This is a case of a 60-year-old woman who presented with motor weakness and involuntary movement of the left upper extremity. Computed tomography and magnetic resonance imaging revealed the right frontal hemorrhagic mass lesion without enhancement of contrast medium. Cerebral digital subtraction angiography showed no vascular stain and abnormal arteriovenous shunt. Preoperatively, we diagnosed cavernous hemangioma with a hemorrhagic component located in the right motor cortex. Because this case was symptomatic, we performed a craniotomy and gross total resection of the right frontal lesion. The diagnosis of capillary hemangioma was made by histological examination, including immunohistological study. Conclusion: Because intraparenchymal capillary hemangiomas are difficult to diagnose with preoperative imaging, surgical treatment, and histopathological examination are important.

5-Aminolevulinic acid fluorescence-guided endoscopic surgery for deep-seated intraparenchymal tumors.

AIM: The usefulness of 5-aminolevulinic acid (5-ALA) fluorescence-assisted surgery for maximum resection of malignant gliomas has been established. However, its usefulness when combined with endoscopic surgery for deep-seated tumors has not been well established. In this study, whether 5-ALA photodynamic diagnosis (PDD) is feasible and useful for endoscopic surgery was investigated. METHODS: A specially designed endoscope for PDD that delivers white light or blue light (375-440 nm) as excitation light was used. The fluorescence emitted by the tumor was evaluated in the cavity during resection or at the tip of the sheath during biopsy. The intensity of fluorescence was classified into three categories: strong, vague, and negative. RESULTS: A total of 30 intraparenchymal tumors were observed with a neuroendoscope and 5-ALA PDD; 16 patients underwent resection, and 14 underwent biopsy. Overall, 67% (20/30) of tumors showed positive fluorescence of protoporphyrin IX. High-grade gliomas (HGGs) including glioblastoma (GBM) and anaplastic astrocytoma (AA) showed strong fluorescence in 47% (7/15), vague fluorescence in 33% (5/15), and negative fluorescence in 20% (3/15) of cases. Low-grade gliomas (LGGs) showed vague fluorescence in 33% (1/3) and negative fluorescence in 67% (2/3). Diffuse large B-cell lymphoma (DLBCL) showed vague fluorescence in 38% (3/8) and negative fluorescence in 63% (5/8). Metastatic tumors showed strong fluorescence in 25% (1/4) and vague fluorescence in 75% (3/4). In the comparison of fluorescence evaluation, a significant difference was observed only in the comparison between HGGs and DLBCL (p = 0.049). CONCLUSION: These results suggest that 5-ALA PDD-assisted endoscopic surgery is feasible and useful for deep-seated intraparenchymal tumors.

A rare case of intra-parenchymal meningioma in a female patient who presented with seizures: A case report.

Key Clinical Message: Meningiomas are slow-growing tumors that develop from the arachnoid cap cells' meningothelial cells. Males are more likely to develop intra-parenchymal meningiomas, which also manifest earlier than ordinary meningiomas and are uncommon. Abstract: Meningiomas are slow-growing neoplasms which arise from the meningothelial cells of the arachnoid cap cells. Unlike other meningiomas, intra-parenchymal meningiomas do not originate from dura. Intra-parenchymal meningiomas are more common in males and develop earlier than regular meningiomas. Because of the rare occurrence the intra-parenchymal meningiomas, they are commonly misdiagnosed.

Atypical Intraparenchymal Meningioma with YAP1-MAML2 Fusion in a Young Adult Male: A Case Report and Mini Literature Review.

Oncogenic Yes-associated protein (YAP) 1 fusions have been recently identified in several cases of meningioma mostly involving pediatric patients. The meningiomas harboring YAP1-MAML2, which is the most frequent fusion subtype, exhibit activated YAP1 signaling and share similarities with NF2 (neurofibromatosis type 2 gene) mutant meningiomas. We reported a rare case of atypical intraparenchymal meningioma with YAP1-MAML2 fusion in a 20-year-old male. The patient presented with an episode of seizure without a medical history. MRI revealed a lesion in the right temporal lobe without extra-axial involvement. The radiological and morphological findings, however, were indistinctive from other intracranial diseases, e.g., vascular malformation and glioma. Immunohistochemical results confirmed the presence of abundant meningothelial cells in the tumor and indicated brain invasion, supporting the diagnosis of atypical intraparenchymal meningioma. Targeted RNA fusion analysis further identified a YAP1-MAML2 rearrangement in the tumor. Non-dural-based intraparenchymal meningiomas are uncommon, and the careful selection of specific tumor markers is crucial for an accurate diagnosis. Additionally, the detection of the fusion gene provides valuable insights into the oncogenic mechanism of meningioma.

Ventriculostomy Associated with Reduced Mortality in Severe Traumatic Brain Injury Compared to Parenchymal ICP Monitoring: A Propensity Score-Adjusted Analysis.

BACKGROUND: There is a lack of data on whether intracranial pressure (ICP)-guided therapy with an intraparenchymal fiberoptic monitor (IPM) or an external ventricular drain (EVD) leads to superior outcomes. Our goal is to determine the relationship between ICP-guided therapy with an EVD or IPM and mortality. METHODS: Retrospective analysis of severe traumatic brain injury cases that required IPM or EVD placement for ICP-guided therapy from January 1, 2010 to December 31, 2020. The data were obtained from the Pennsylvania Trauma Systems Foundation registry. RESULTS: A total of 2305 patients met the inclusion criteria, with 1048 (45.5%) IPM and 1257 (54.5%) EVD placed. Inpatient mortality occurred in 337 (32.2%) and 334 (26.6%) patients in the IPM and EVD cohorts, respectively (P = 0.003). Even among those treated medically only, inpatient mortality occurred in 171 (30.8%) of those with an IPM and in 100 (23.4%) of those with an EVD (P = 0.010). Multivariable logistic regression analysis showed that older age (odds ratio [OR] 1.03, P < 0.001), lower Glasgow Coma Scale (GCS) score (OR 1.16, P < 0.001), requiring surgery (OR 1.22, P = 0.049), and an IPM (OR 1.40, P = 0.001) were significant predictors of mortality. Propensity score-adjusted analysis using inverse probability of treatment weighted method revealed a 28% decrease in mortality and a 14% decrease in length of hospital stay with EVD use when adjusting for age, sex, GCS, Injury Severity Score, surgery, and Hispanic ethnicity. CONCLUSIONS: A significant mortality benefit was associated with the use of EVD compared to IPM. This mortality benefit was observed regardless of whether patients required surgery or not.