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Laboratory Investigation; a Journal of... Aug 2023Central neurocytoma (CN) is a low-grade neuronal tumor that mainly arises from the lateral ventricle (LV). This tumor remains poorly understood in the sense that no...
Central neurocytoma (CN) is a low-grade neuronal tumor that mainly arises from the lateral ventricle (LV). This tumor remains poorly understood in the sense that no driver gene aberrations have been identified thus far. We investigated immunomarkers in fetal and adult brains and 45 supratentorial periventricular tumors to characterize the biomarkers, cell of origin, and tumorigenesis of CN. All CNs occurred in the LV. A minority involved the third ventricle, but none involved the fourth ventricle. As expected, next-generation sequencing performed using a brain-tumor-targeted gene panel in 7 CNs and whole exome sequencing in 5 CNs showed no driver mutations. Immunohistochemically, CNs were robustly positive for FGFR3 (100%), SSTR2 (92%), TTF-1 (Nkx2.1) (88%), GLUT-1 (84%), and L1CAM (76%), in addition to the well-known markers of CN, synaptophysin (100%) and NeuN (96%). TTF-1 was also positive in subependymal giant cell astrocytomas (100%, 5/5) and the pituicyte tumor family, including pituicytoma and spindle cell oncocytoma (100%, 5/5). Interestingly, 1 case of LV subependymoma (20%, 1/5) was positive for TTF-1, but all LV ependymomas were negative (0/5 positive). Because TTF-1-positive cells were detected in the medial ganglionic eminence around the foramen of Monro of the fetal brain and in the subventricular zone of the LV of the adult brain, CN may arise from subventricular TTF-1-positive cells undergoing neuronal differentiation. H3K27me3 loss was observed in all CNs and one case (20%) of LV subependymoma, suggesting that chromatin remodeling complexes or epigenetic alterations may be involved in the tumorigenesis of all CNs and some ST-subependymomas. Further studies are required to determine the exact tumorigenic mechanism of CN.
Topics: Humans; Neurocytoma; Histones; Glioma, Subependymal; Epigenesis, Genetic; Carcinogenesis
PubMed: 37088465
DOI: 10.1016/j.labinv.2023.100159 -
Polish Archives of Internal Medicine Dec 2023
Topics: Humans; Neuroendocrine Tumors; Pituitary Neoplasms; Magnetic Resonance Imaging; Growth Hormone
PubMed: 37943187
DOI: 10.20452/pamw.16599 -
Neuro-oncology Aug 2023"Primary papillary epithelial tumor of the sella (PPETS)" is a recently described rare tumor entity of the central nervous system (CNS) with stereotypic location in the...
BACKGROUND
"Primary papillary epithelial tumor of the sella (PPETS)" is a recently described rare tumor entity of the central nervous system (CNS) with stereotypic location in the sella. Comprehensive molecular investigations and epigenetic profiles of PPETS have not been performed to date.
METHODS
We report a comprehensive clinical, histopathologic, and molecular assessment of 5 PPETS cases in comparison with a cohort composed of 7 choroid plexus papilloma (CPP), 7 central neurocytoma (CN), 15 posterior pituitary tumor (PPT) including 4 pituicytoma, 6 granular cell tumors of the sellar region (GCT), and 5 spindle cell oncocytoma.
RESULTS
All PPETS had good outcomes. Immunohistochemically, PPETS tumors showed positive staining with TTF1, EMA, AE1/AE3, MAP2, and Vimentin, but were negatively stained with Syn, GFAP, CgA, and S100, and sporadically stained with Ki-67. In unsupervised hierarchical clustering and t-distributed stochastic neighbor embedding analyses of DNA-methylation data, PPETS and PPT tumors formed a distinct cluster irrespective of their histologic types. However, PPETS tumors did not cluster together with CPP and CN samples. Similar findings were obtained when our samples were projected into the reference cohort of the brain tumor classifier. Substantial fractions of the PPETS and PPT tumors shared broadly similar chromosomal copy number alterations. No mutations were detected using targeted next-generation sequencing.
CONCLUSIONS
Though more cases are needed to further elucidate the molecular pathogenesis of these tumors, our findings indicate that PPETS and PPT tumors may constitute a single neurooncological entity.
Topics: Humans; Pituitary Neoplasms; Neoplasms, Glandular and Epithelial; Adenoma, Oxyphilic; DNA Methylation; Central Nervous System
PubMed: 37058118
DOI: 10.1093/neuonc/noad067 -
International Journal of Surgery Case... Nov 2023Granular cell tumor (GCT) originating from the sellar and suprasellar regions, specifically from the neurohypophysis, is a rare neoplasm. Distinguishing GCT from other...
INTRODUCTION AND IMPORTANCE
Granular cell tumor (GCT) originating from the sellar and suprasellar regions, specifically from the neurohypophysis, is a rare neoplasm. Distinguishing GCT from other pituitary tumors, including pituitary adenoma, pituicytoma, and spindle cell oncocytoma, poses significant challenges. Here, we present a rare case of GCT originating from the posterior pituitary in the supra-sellar region.
CASE PRESENTATION
A 41-year-old woman, with no past medical history, presented to neurology department with decreased visual acuity and peripheral facial paralysis since 3 months. The MRI showed a well-defined supra-sellar, retrochiasmatic, oblong, hypothalamic expansive process. It was isointense T1-weighted, discretely hypotensive T2-weighted, measuring 19x17x16 mm, suggesting pituicytoma or craniopharyngioma. An endoscopic transsphenoidal surgical resection was performed. Microscopic examination showed a proliferation of diffuse architecture made up of rounded polyhedral cells with granular eosinophilic cytoplasm. On immunohistochemistry, tumor cells expressed diffusely TTF1, S-100 protein and SOX-10 confirming the diagnosis of supra-sellar GCT.
DISCUSSION AND CONCLUSION
GCTs are rare neoplasms that predominantly exhibit benign behavior, while the malignancy rate remains at 2 %. Histopathology serves as the definitive diagnostic approach for GCTs. These tumors are resistant to radiotherapy and chemotherapy, necessitating surgical resection as the primary treatment modality. Due to the potential absence of distinct tumor masses and local tissue infiltration by tumor cells, complete excision is crucial, with resection extent extending beyond areas of infiltration.
PubMed: 37883878
DOI: 10.1016/j.ijscr.2023.108977 -
Journal of Nuclear Medicine : Official... May 2024Small functional pituitary tumors can cause severely disabling symptoms and early death. The gold standard diagnostic approach includes laboratory tests and MRI, with or...
Small functional pituitary tumors can cause severely disabling symptoms and early death. The gold standard diagnostic approach includes laboratory tests and MRI, with or without inferior petrosal sinus sampling (IPSS). In up to 40% of patients, however, the source of excess hormone production remains unidentified or uncertain. This excludes patients from surgical, Gamma Knife, and CyberKnife therapy and adversely affects overall cure rates. We here assess the diagnostic yield of -(2-[F]fluoroethyl)-l-tyrosine ([F]FET) PET/MRI for detection of small functional pituitary tumors in these patients. This retrospective analysis included patients with Cushing disease (CD) but prior negative or inconclusive MRI results who underwent [F]FET PET/MRI between February 1, 2021, and December 1, 2022. PET/MR images and MR images alone were evaluated by experienced nuclear radiologists, neuroradiologists, or radiologists. Postoperative tissue analysis (when performed) was used as a reference standard to assess diagnostic metrics (i.e., sensitivity and positive predictive value). Results were also compared with previously obtained MR images, preceding IPSS, and clinical or biochemical follow-up. Twenty-two patients (68% female; mean age ± SD, 48 ± 15 y; range, 24-68 y) were scanned. All patients showed a clear metabolic focus on [F]FET PET, whereas reading of the MRI alone yielded a suspected lesion in only 50%. Fifteen patients underwent surgery directed at the [F]FET-positive focus. Tissue analysis confirmed a pituitary adenoma/pituitary neuroendocrine tumor of the corticotroph cell type (TPIT lineage) in 10 of 15 and a pituicytoma in 1 of 15, rendering a sensitivity of 100% and a positive predictive value of 73%. Lateralization was more accurate with [F]FET PET/MRI than with IPSS in 33%. Twelve of 16 (75%) patients who received surgical, Gamma Knife, or CyberKnife therapy after [F]FET PET/MRI reached short-term remission. [F]FET PET/MRI shows a high diagnostic yield for localizing small functional pituitary tumors. This multimodal imaging technique provides a welcome improvement for diagnosis, planning of surgery, and clinical outcome in patients with Cushing disease, particularly those with repeated negative or inconclusive MRI results with or without IPSS.
Topics: Humans; Female; Male; Middle Aged; Pituitary Neoplasms; Magnetic Resonance Imaging; Adult; Retrospective Studies; Positron-Emission Tomography; Tyrosine; Multimodal Imaging; Aged; Young Adult
PubMed: 38514085
DOI: 10.2967/jnumed.123.266853 -
Neuropathology : Official Journal of... Dec 2023Granular cell tumors of the neurohypophysis (GCT) are rare benign neoplasms belonging, along with pituicytoma and spindle cell oncocytoma, to the family of TTF1-positive...
Granular cell tumors of the neurohypophysis (GCT) are rare benign neoplasms belonging, along with pituicytoma and spindle cell oncocytoma, to the family of TTF1-positive low-grade neoplasms of the posterior pituitary gland. GCT usually present as a solid sellar mass, slowly growing and causing compressive symptoms over time, occasionally with suprasellar extension. They comprise polygonal monomorphous cells with abundant granular cytoplasm, which is ultrastructurally filled with lysosomes. Here we report the case of a GCT presenting as a third ventricle mass, radiologically mimicking chordoid glioma, with aberrant expression of GFAP and Annexin-A, which lends itself as an example of an integrated diagnostic approach to sellar/suprasellar and third ventricle masses.
Topics: Humans; Pituitary Gland, Posterior; Third Ventricle; Granular Cell Tumor; Cerebral Ventricle Neoplasms; Craniopharyngioma; Pituitary Neoplasms; Glioma
PubMed: 37147874
DOI: 10.1111/neup.12907 -
Neurosurgical Focus Dec 2023The aim of this study was to report the authors' experience developing a Lean Six Sigma clinical care pathway (CCP) for endoscopic endonasal transsphenoidal operations.
OBJECTIVE
The aim of this study was to report the authors' experience developing a Lean Six Sigma clinical care pathway (CCP) for endoscopic endonasal transsphenoidal operations.
METHODS
Using Lean Six Sigma quality improvement principles-including the define, measure, analyze, improve, and control framework-the authors developed a CCP for endoscopic endonasal transsphenoidal operations, incorporating preoperative, intraoperative, and inpatient and outpatient postoperative phases of care. Efficacy and quality metrics were defined as postoperative length of stay (LOS), presentation to the emergency department (ED) or readmission within 30 days of discharge, and hospital charges. The study included all adult patients who underwent elective endoscopic endonasal resection for pituitary adenoma, Rathke's cleft cyst, craniopharyngioma, pituicytoma, or arachnoid cyst during the sampling period (April 1, 2018, to December 31, 2022).
RESULTS
Two hundred twenty-eight patients met criteria and were included; 94 were treated before and 134 were treated after implementation of the CCP. Differences between groups in age, gender, race, BMI, American Society of Anesthesiologists classification, geographic distribution, preoperative serum sodium, tumor size, adenoma functional status, and prior surgery were not significant. The mean postoperative LOS significantly decreased from 4.5 to 1.7 days following CCP implementation (p < 0.0001); LOS variability also decreased, with the standard deviation declining from 3.1 to 1.5 days. The proportion of patients discharged on postoperative day (POD) 1 significantly increased from 0% to 61.9% (p < 0.0001). Fewer than one-quarter of the patients (23.4%) were discharged by POD 2 prior to the CCP, while 88.8% of were discharged by POD 2 after CCP implementation (p < 0.0001). Rates of 30-day ED presentations or readmissions were not significantly different (2.1% vs 6.0%, p = 0.20, and 7.5% vs 6.7%, p > 0.99, respectively). Mean per-patient hospital costs declined from $38,326 to $26,289 (p < 0.0001), with an associated change in cost variability from a standard deviation of $16,716 to $12,498.
CONCLUSIONS
CCP implementation significantly improved LOS and costs of endoscopic endonasal resection, without adversely impacting postoperative ED presentations or readmissions.
Topics: Adult; Humans; Quality Improvement; Pituitary Neoplasms; Pituitary Gland; Nose; Endoscopy; Adenoma; Craniopharyngioma; Retrospective Studies; Treatment Outcome; Postoperative Complications
PubMed: 38039538
DOI: 10.3171/2023.9.FOCUS23545 -
American Journal of Clinical Pathology Jun 2024The incidence of pituitary neuroendocrine tumors has been reported high at autopsy. This study aimed to detect many tumors in both anterior and posterior lobes to prove...
OBJECTIVES
The incidence of pituitary neuroendocrine tumors has been reported high at autopsy. This study aimed to detect many tumors in both anterior and posterior lobes to prove tumor histogenesis.
METHODS
In total, 150 pituitary glands were studied from the University of Kansas Medical Center from 1995 to 2000. The pituitary gland was sagittally sliced from anterior to posterior into 6 to 8 sections. When H&E-stained sections revealed tumors, the tumors were immunohistochemically stained for 6 pituitary hormones.
RESULTS
Among 150 autopsy cases, 38 (25.3%) harbored microadenomas, including 4 cases with double tumors. Twenty-three (54.7%) cases were negative to all pituitary hormones. Of the remaining 19 tumors, 13 (30.9%) were lactotrophs, with 4 cases being concomitantly somatotrophs and gonadotrophs, and 2 cases were corticotropes. More than 85% of pituitary neuroendocrine tumors were adjacent to the capsule. Thirteen (8.7%) granular cell pituicytomas were found in the posterior lobe. There were pituicytes transforming into granular cell tumors.
CONCLUSIONS
The incidence was 25.3% for pituitary neuroendocrine tumors and 8.7% for granular cell pituicytomas. Since most pituitary neuroendocrine tumors were adjacent to the pituitary capsule, the capsule appeared to be the germinal center. Both pituitary tumors belonged to the 2 different transcription factor lineages.
PubMed: 38913876
DOI: 10.1093/ajcp/aqae067