Secondary hypophysitis associated with Rathke's cleft cyst resembling a pituitary abscess.

Background: Although rare, cases of hypophysitis resembling a pituitary abscess (PA) have been reported. Differential diagnosis between hypophysitis and PA is crucial as the two diseases require different treatments. Case Description: A 38-year-old woman with headaches underwent head magnetic resonance imaging (MRI), which revealed an 11-mm mass lesion in the sella turcica. Due to breastfeeding, contrast-enhanced MRI was avoided. Pituitary adenomas and Rathke's cleft cyst (RCC) were suspected, and she was initially treated conservatively. Five months later, she acquired syndrome coronavirus two infections, and while the fever subsided with acetaminophen, the headache persisted. One month later, the headache worsened, followed by fever and diabetes insipidus. MRI revealed a pituitary cystic mass with ring-shaped contrast enhancement on T1-weighted MRI and increased signal intensity on diffusion-weighted imaging (DWI). PA was suspected, and emergency endoscopic transsphenoidal surgery was performed. The microbiological examination of the yellowish-brown content drained from the cystic mass was negative. Microscopically, the cystic lesion was covered with ciliated columnar epithelium and stratified squamous epithelium, with a dense inflammatory cell infiltrate consisting mainly of lymphocytes and plasma cells observed around the cyst. This supported the diagnosis of secondary hypophysitis associated with RCC without PA. Conclusion: We report a case of hypophysitis secondary to RCC resembling PA with ring-shaped contrast enhancement on MRI and increased signal intensity on DWI. This case emphasizes the need for cautious diagnosis of secondary hypophysitis due to RCC in individuals with MRIs and clinical manifestations resembling an abscess.

Radiological Analysis of Cerebrospinal Fluid Dynamics at the Craniovertebral Junction Using Time-Spatial Labeling Inversion Pulse Magnetic Resonance Imaging in Patients with Cervical Spinal Canal Stenosis.

OBJECTIVE: Spondylotic changes in the cervical spine cause degeneration, leading to cervical spinal canal stenosis. This stenotic change can affect cerebrospinal fluid (CSF) dynamics by compressing the dural sac and reducing space in the subarachnoid space. We examined CSF dynamics at the craniovertebral junction (CVJ) using time-spatial labeling inversion pulse magnetic resonance imaging (Time-SLIP MRI) in patients with cervical spinal canal stenosis. METHODS: The maximum longitudinal movement of the CSF at the CVJ was measured as length of motion (LOM) in the Time-SLIP MRI of 56 patients. The sum of ventral and dorsal LOM was defined as the total LOM. Patients were classified into 3 groups depending on their spinal sagittal magnetic resonance imaging findings: control (n = 27, Kang classification grades 0 and 1), stenosis (n = 14, Kang classification grade 2), and severe stenosis (n = 15, Kang classification grade 3). RESULTS: Time-SLIP MRI revealed pulsatile movement of the CSF at the CVJ. The mean total, ventral, and dorsal LOM was 14.2 ± 9, 8.1 ± 5.7, and 3.8 ± 2.9 mm, respectively. The ventral LOM was significantly larger than the dorsal LOM. The total LOM was significantly smaller in the severe stenosis group (6.1 ± 3.4 mm) than in the control (16.0 ± 8.4 mm) or stenosis (11 ± 5.4 mm) groups (P < 0.001, Kruskal-Wallis H-test). In 5 patients, postoperative total LOM was improved after adequate decompression surgery. CONCLUSIONS: This study demonstrates that CSF dynamics at the CVJ are influenced by cervical spinal canal stenosis. Time-SLIP MRI is useful for evaluating CSF dynamics at the CVJ in patients with spinal canal stenosis.

Indocyanine green endoscopic evaluation of pituitary stalk and gland blood flow in craniopharyngiomas.

To assess the use of indocyanine green (ICG) fluorescence endoscopy to evaluate pituitary blood flow in craniopharyngioma resection and its possible impact on intraoperative decisions regarding pituitary stalk processing. Patients with craniopharyngiomas who had undergone transsphenoidal surgery since March 2021, when an ICG endoscope was introduced at the Kagoshima University Hospital, were included in the study. When targeted tumor removal was approaching completion, 10 mg of ICG was administered intravenously to evaluate blood flow in the pituitary stalk and gland. ICG signals and endocrinological status before and after surgery were evaluated retrospectively. Pituitary stalk and gland blood flow were evaluated as positive (++), weakly positive (+), and no signal (-).Ten patients with craniopharyngiomas underwent transsphenoidal surgery using an ICG endoscope (mean age 56.6 ± 14.2 years; 40% male). Among the eight patients in whom the pituitary stalk was preserved, pituitary function with positive signal on the stalk was intact in two. Two other patients with weakly positive stalk and positive pituitary gland signals showed intact function or minimal pituitary dysfunction. Four patients had impairments in more than three axes with poor ICG signals in the stalk or pituitary gland. Two patients underwent pituitary amputation because of high tumor invasion and lack of ICG signal in the stalk after tumor removal, resulting in panhypopituitarism. A negative ICG signal in the pituitary stalk is likely to indicate postoperative pituitary function loss. Craniopharyngioma surgery using ICG endoscopy may be useful for predicting endocrine prognosis and improving tumor outcomes.

Alterations in EGFR and PDGFRA are associated with the localization of contrast-enhancing lesions in glioblastoma.

Background: Glioblastoma (GBM) is a malignant brain tumor, with radiological and genetic heterogeneity. We examined the association between radiological characteristics and driver gene alterations. Methods: We analyzed the driver genes of 124 patients with IDH wild-type GBM with contrast enhancement using magnetic resonance imaging. We used a next-generation sequencing panel to identify mutations in driver genes and matched them with radiological information. Contrast-enhancing lesion localization of GBMs was classified into 4 groups based on their relationship with the subventricular zone (SVZ) and cortex (Ctx). Results: The cohort included 69 men (55.6%) and 55 women (44.4%) with a mean age of 66.4 ±â€…13.3 years. EGFR and PDGFRA alterations were detected in 28.2% and 22.6% of the patients, respectively. Contrast-enhancing lesion touching both the SVZ and Ctx was excluded because it was difficult to determine whether it originated from the SVZ or Ctx. Contrast-enhancing lesions touching the SVZ but not the Ctx had significantly worse overall survival than non-SVZ lesions (441 days vs. 897 days, P = .002). GBM touching only the Ctx had a better prognosis (901 days vs. 473 days, P < .001) than non-Ctx lesions and was associated with EGFR alteration (39.4% vs. 13.2%, P = .015). Multiple contrast lesions were predominant in PDGFRA alteration and RB1-wild type (P = .036 and P = .031, respectively). Conclusions: EGFR alteration was associated with cortical lesions. And PDGFRA alteration correlated with multiple lesions. Our results suggest that clarifying the association between driver genes and tumor localization may be useful in clinical practice, including prognosis prediction.

Favorable prognostic impact of phosphatase and tensin homolog alterations in wild-type isocitrate dehydrogenase and telomerase reverse transcriptase promoter glioblastoma.

Background: Telomerase reverse transcriptase promoter (TERTp) mutations are a biological marker of glioblastoma; however, the prognostic significance of TERTp mutational status is controversial. We evaluated this impact by retrospectively analyzing the outcomes of patients with isocitrate dehydrogenase (IDH)- and TERTp-wild-type glioblastomas. Methods: Using custom next-generation sequencing, we analyzed 208 glioblastoma samples harboring wild-type IDH. Results: TERTp mutations were detected in 143 samples (68.8%). The remaining 65 (31.2%) were TERTp-wild-type. Among the TERTp-wild-type glioblastoma samples, we observed a significant difference in median progression-free survival (18.6 and 11.4 months, respectively) and overall survival (not reached and 15.7 months, respectively) in patients with and without phosphatase and tensin homolog (PTEN) loss and/or mutation. Patients with TERTp-wild-type glioblastomas with PTEN loss and/or mutation were younger and had higher Karnofsky Performance Status scores than those without PTEN loss and/or mutation. We divided the patients with TERTp-wild-type into 3 clusters using unsupervised hierarchical clustering: Good (PTEN and TP53 alterations; lack of CDKN2A/B homozygous deletion and platelet-derived growth factor receptor alpha (PDGFRA) alterations), intermediate (PTEN alterations, CDKN2A/B homozygous deletion, lack of PDGFRA, and TP53 alterations), and poor (PDGFRA and TP53 alterations, CDKN2A/B homozygous deletion, and lack of PTEN alterations) outcomes. Kaplan-Meier survival analysis indicated that these clusters significantly correlated with the overall survival of TERTp-wild-type glioblastoma patients. Conclusions: Here, we report that PTEN loss and/or mutation is the most useful marker for predicting favorable outcomes in patients with IDH- and TERTp-wild-type glioblastomas. The combination of 4 genes, PTEN, TP53, CDKN2A/B, and PDGFRA, is important for the molecular classification and individual prognosis of patients with IDH- and TERTp-wild-type glioblastomas.

Bilateral Internal Carotid Artery Hypoplasia with Craniofacial Anomalies: A Case of Suspected Treacher Collins Syndrome.

Internal carotid artery aplasia or hypoplasia above the cervical bifurcation is rare, occurring in less than 0.01% of the general population. Unilateral neurocristopathy complicated by unilateral internal carotid artery agenesis or hypogenesis has been reported, but bilateral internal carotid artery hypoplasia is rare and scarcely reported. Herein, we report a novel case of Treacher Collins syndrome complicated by bilateral internal carotid artery hypoplasia. A 94-year-old woman presented with complaints of headache and vomiting. Computed tomography revealed a subarachnoid hemorrhage and dysplasia of the bilateral zygoma, mandible, and external auditory meatus. The patient had severe hearing loss and visual impairment. Computed tomography angiography revealed bilateral internal carotid artery hypoplasia and multiple aneurysmal changes in the intracranial arteries. We diagnosed the patient with a ruptured anterior inferior cerebellar artery aneurysm and performed coil embolization. The patient's unique facial features were consistent with neurocristopathy, especially Treacher Collins syndrome. Developmental anomalies of neural crest cells can present as vascular abnormalities and craniofacial malformations. Special care is required for endovascular treatment and airway management in cases of neurocristopathy because of the specific craniofacial anomalies.

Distribution and favorable prognostic implication of genomic EGFR alterations in IDH-wildtype glioblastoma.

BACKGROUND: We aimed to evaluate the mutation profile, transcriptional variants, and prognostic impact of the epidermal growth factor receptor (EGFR) gene in isocitrate dehydrogenase (IDH)-wildtype glioblastomas (GBMs). METHODS: We sequenced EGFR, evaluated the EGFR splicing profile using a next-generation sequencing oncopanel, and analyzed the outcomes in 138 grade IV IDH-wildtype GBM cases. RESULTS: EGFR mutations were observed in 10% of GBMs. A total of 23.9% of the GBMs showed EGFR amplification. Moreover, 25% of the EGFR mutations occurred in the kinase domain. Notably, EGFR alterations were a predictor of good prognosis (p = 0.035). GBM with EGFR alterations was associated with higher Karnofsky Performance Scale scores (p = 0.014) and lower Ki-67 scores (p = 0.005) than GBM without EGFR alterations. EGFRvIII positivity was detected in 21% of EGFR-amplified GBMs. We identified two other EGFR variants in GBM cases with deletions of exons 6-7 (Δe 6-7) and exons 2-14 (Δe 2-14). In one case, the initial EGFRvIII mutation transformed into an EGFR Δe 2-14 mutation during recurrence. CONCLUSIONS: We found that the EGFR gene profiles of GBM differ among cohorts and that EGFR alterations are good prognostic markers of overall survival in patients with IDH-wildtype GBM. Additionally, we identified rare EGFR variants with longitudinal and temporal transformations of EGFRvIII.

Delayed postoperative hyponatremia in patients with acromegaly: incidence and predictive factors.

PURPOSE: Delayed postoperative hyponatremia (DPH) is a unique complication of transsphenoidal surgery (TSS) in pituitary tumors. Growth hormone (GH) enhances renal sodium reabsorption; however, the association between postoperative GH reduction and DPH in acromegaly is unclear. This study was performed to clarify the incidence of and the predictive factors for DPH in patients with acromegaly who underwent TSS. METHODS: Ninety-four patients with active acromegaly were examined retrospectively. During the postoperative course, patients with serum sodium levels ≤ 134 mEq/L were classified into the DPH group. We compared basic clinical characteristics, tumor characteristics, and preoperative and postoperative examination findings between the DPH and non-DPH groups. RESULTS: DPH occurred in 39 patients (41.5%), and the lowest serum sodium levels were generally observed during postoperative days (PODs) 7-9. They needed a 3-day longer hospital stay than those without DPH. The DPH group had lower preoperative body weight and body mass index. In addition, a transient increase in body weight during PODs 5-7 occurred with a transient decrease in urinary volume in the DPH group. Preoperative and postoperative GH and insulin-like growth factor-1 levels did not differ between the two groups. CONCLUSION: The findings suggested that lower preoperative weight and a postoperative transient gain in body weight are associated with an increased risk of DPH in acromegaly patients undergoing transsphenoidal surgery.

Spinal intradural solitary fibrous tumor/ hemangiopericytoma with intramedullary invasion mimicking a hemangioblastoma.

Background: Solitary fibrous tumor/hemangiopericytomas (SFT/HPCs) are rare mesenchymal tumors of nonmeningothelial origin that comprises <1% of all central nervous system tumors. Case Description: A 45-year-old male presented with sleep apnea (apnea-hypopnea index was 17.1 events/hour) and dysesthesias of the right upper and lower extremities. The magnetic resonance demonstrated a heterogeneous intradural extra-axial C1 mass with syringobulbia and syringomyelia. The right vertebral angiography revealed a hypervascular mass (i.e., intense tumor staining). With the preoperative diagnosis of a spinal hemangioblastoma, the patient underwent tumor removal. However, intraoperative findings demonstrated that the ventral component of the tumor was intramedullary without a dural attachment. Further, the histological diagnosis was consistent with SFT/HPC (HPC phenotype). The postoperative course was uneventful, and the patient's symptoms and the syrinxes spontaneously regressed. Conclusion: A 45-year-old male presented a rare spinal intradural lesion at C1 appeared to be a spinal hemangioblastoma, but proved to be SFT/HPC (HPC phenotype) with intramedullary invasion.

Diffuse Large B-Cell Lymphoma of the Central Nervous System Manifesting with Intratumoral Hemorrhage: A Case Report and Literature Review.

BACKGROUND: Cases of primary central nervous system lymphoma manifesting with hemorrhage are very rare, with only a few previous studies available. CASE DESCRIPTION: A 49-year-old man presented with occipital headache and visual disturbance for the past 4 months. Computed tomography showed a high-density area involving the left basal ganglia, with surrounding vasogenic edema. Head T2∗-weighted imaging showed a hypointense signaling area. Edematous changes and a midline shift were observed on fluid attenuated inversion recovery magnetic resonance imaging. Radiologic features were highly suggestive of intracerebral hemorrhage. Methylprednisolone pulse therapy improved his symptoms transiently and reduced the size of the lesion. Nonetheless, there was recurrence 1 month later. The patient was referred to our institution; a biopsy was performed, and a diffuse large B-cell lymphoma was diagnosed. After 3 cycles of high-dose methotrexate and whole-brain radiation therapy, his symptoms improved, and there were no signs of recurrence. CONCLUSIONS: We report a very rare case of diffuse large B-cell lymphoma manifested with intratumoral hemorrhage. This case indicates the importance of regular clinical and radiologic follow-up, histopathologic examination, and combined treatment with high-dose methotrexate and whole-brain radiation therapy.