Pulmonary pleomorphic carcinoma arising in mixed squamous and glandular papilloma: A case report.

Solitary pulmonary papillomas (SPPs) are rare lung neoplasms. Histologically, SPP is classified into three subtypes, and mixed squamous and glandular papilloma (MP) is the rarest subtype. Although SPPs are considered benign tumors, there have been several reports on the synchronous malignant transformation in SPPs. An 82-year-old asymptomatic man was referred to our hospital for further examination of a 2.2 cm-sized left lung tumor. Pathology of bronchoscopic specimens showed the possibility of pulmonary papilloma but did not reveal any malignancy. The patient complained of bloody sputum during the eighth month after the initial visit. The size of the lesion had increased to 4.3 cm. These data suggested the existence of malignancy, and the patient underwent an operation. Histologically, the tumor was composed of fibrovascular cores and papillomatous fronds lined by pseudostratified columnar cells and mucin-filled goblet cells. Keratinizing squamous epithelium was also observed. Overall, the diagnosis of MP was obtained by fundamental histology. In addition, a solid part beneath mild atypical squamous epithelia, which was composed of malignant-appearing squamous cells and spindle-shaped atypical cells, was observed. The spindle portion was positive for cytokeratin AE1/AE3 and vimentin, and focally positive for alpha-smooth muscle actin (αSMA). The final diagnosis was pulmonary pleomorphic carcinoma (PPC) arising in the MP. Only two cases have been reported for atypical spindle tumor cells that are found in MP or bronchiolar adenoma/ciliated muconodular papillary tumor (BA/CMPT), which has histologically similar features to MP. This is the second case report of PPC arising in MP.

Acute subdural hematoma caused by hemorrhagic falx meningioma: A case report and review of the literature.

We herein report a case of acute subdural hematoma caused by hemorrhagic falx meningioma. The patient was a 64-year-old woman with no significant medical history or prior history of trauma. She experienced a sudden onset of headache and weakness in her extremities. Computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a mass lesion with intratumoral hemorrhage or faint calcification along the left side of the fronto-parietal cerebral falx. There was also a linear lesion at the left side of the falx, suggesting acute subdural hematoma. MRI was performed again on the eleventh day. On precontrast T1-weighted images, intratumoral hemorrhage and widespread left subdural hematoma were shown as high intensity. On postcontrast T1-weighted images, the tumor showed heterogeneous enhancement with a dural tail sign on the falx, indicative of a falx meningioma. She underwent surgical resection, and the histological subtype was transitional meningioma. Nine cases of hemorrhagic falx meningioma associated with acute subdural hematoma have been reported. If not limited to the site of occurrence, there have been 59 reported cases overall. In our investigation, the incidence of hemorrhage is higher in the convexity and lower in the skull base. It is higher for fibrous, angiomatous, and metaplastic subtypes and lower for meningothelial subtype. The location and histological subtype might be risk factors for meningioma associated with subdural hematoma. Further accumulation of cases will be necessary to establish the cause of bleeding.

Rapidly Progressing Intramedullary Spinal Cord Abscess: A Case Report.

Intramedullary spinal cord abscess is a rare and severe infectious disease characterized by devastating neurological deficits. We report a case of cervical intramedullary spinal cord abscess in a 74-year-old diabetic male with a 3-day history of neck pain and weakness in the right lower extremity. Magnetic resonance imaging revealed a ring-shaped contrast lesion in C3-C6 of the cervical spinal cord with extensive edema. Further, 1 day after admission, he became comatose (Glasgow Coma Scale E1VtM1), and a computed tomography head scan revealed hydrocephalus. Despite emergency ventricular drainage, the patient's level of consciousness remained unchanged. Magnetic resonance imaging performed 1 day after surgery revealed bilateral intracranial extension of the abscess into the thalamus and caudate nucleus. The patient died 19 days after admission. Our report is the first case of extensive brain abscess development over a short period. Based on our experience, prompt administration of antibiotics and emergency abscess drainage of the cervical cord (and ventricular drainage, if necessary) are recommended in cases of neurological deterioration in patients with cervical intramedullary spinal cord abscess.

The predominance of "astrocytic" intranuclear inclusions in neuronal intranuclear inclusion disease manifesting encephalopathy-like symptoms: A case series with brain biopsy.

Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder represented by eosinophilic intranuclear inclusions (EIIs) and GGC/CGG repeat expansion in the NOTCH2NLC gene. We report here two adult cases of NIID, genetically confirmed, with manifestation of encephalopathy-like symptoms and address the histopathologic findings obtained by brain biopsies, with a focus on "astrocytic" intranuclear inclusions (AIIs). Case 1 presented with paroxysmal restlessness, vertigo, or fever and was later involved in severe dementia and tetraparesis. Case 2 presented with forgetfulness and then with paroxysmal fever and headache. In both cases, delimited areas with gadolinium enhancement on magnetic resonance imaging and corresponding hyperperfusion were detected, leading to brain biopsies of the cortex. On histology, Case 1 showed an abnormal lamination, where the thickness of layers was different from usual. Both neurons and astrocytes showed some dysmorphologic features. Notably, astrocytes rather than neurons harbored EIIs. Case 2 showed a cortex, where neurons tended to be arrayed in a columnar fashion. Astrocytes showed some dysmorphologic features. Notably, much more astrocytes than neurons harbored EIIs. By a double-labeling immunofluorescence study for p62/NeuN and p62/glial fibrillary acidic protein, the predominance of AIIs was confirmed in both cases. Considering the physiological functions of astrocytes for the development and maintenance of the cortex, the encephalopathy-like symptoms, dynamic change of cerebral blood flow, and cortical dysmorphology can reasonably be explained by the dysfunction of EII-bearing astrocytes rather than EII-bearing neurons. This study suggests the presence of a subtype of NIID where AIIs rather than "neuronal" intranuclear inclusions are likely a key player in the pathogenesis of NIID, particularly in cases with encephalopathy-like symptoms. The importance of AIIs ("gliopathy") should be more appreciated in future studies of NIID.

Differential immunophenotypes of neuronal cytoplasmic inclusions in the dentate gyrus of multiple system atrophy and their association with clinicopathological features.

Although hippocampal pathologies of multiple system atrophy (MSA) and their association with dementia have been reported, no studies have reported clinicopathological differences among MSA patients with and without neuronal cytoplasmic inclusions (NCIs) in the dentate gyrus (dntNCIs). We investigated hippocampal NCI pathology in 18 MSA patient autopsies, focusing on phosphorylated α-synuclein (pAS)- and phosphorylated tau (pT)-positive dntNCIs. There were 8 MSA patients without and 10 with dntNCIs. The latter group was subclassified by immunophenotype: those with pAS-positive dntNCIs (pAS-dntNCI subtype), those with pT-positive dntNCIs (pT-dntNCI subtype), and those with both types of dntNCIs. MSA patients with dntNCIs survived longer with prolonged tracheostomy and had dementia more frequently than those without dntNCIs. The brain weights of patients with dntNCIs were lower than those without dntNCIs. The presence of dementia was similar among the dntNCI subtypes. The pAS-dntNCI subtype was associated with longer survival and smaller brain weights; the pT-dntNCI subtype exhibited more frequent tau pathologies than the pAS-dntNCI subtype. Thus, MSA with dntNCIs is a possible pathological subtype of longer survivors that correlates with longer disease duration, prolonged tracheostomy, and high frequency of dementia. Understanding clinicopathological differences in MSA patients with and without dntNCIs may lead to improved personalized management strategies.

Neuropathological features of adult-onset neuronal intranuclear inclusion disease with fluid-attenuated inversion recovery high-intensity signals in the cerebellar paravermal area from an early stage: A case report.

Neuronal intranuclear inclusion disease (NIID) is a neurological disorder characterized by eosinophilic intranuclear inclusions (INI) in systemic organs and various cell types. High-intensity signals along the corticomedullary junction on diffusion-weighted imaging and presence of cellular p62-INI in skin biopsy are known indicators for NIID. Furthermore, GGC repeat expansion in NOTCH2NLC is a characteristic genetic alteration in patients with NIID. This report presents the clinical and detailed pathological features of a male older adult with NIID. We also confirmed the presence of fluid-attenuated inversion recovery high-intensity signals in the cerebellar paravermal area, showing similar pathological changes in high-intensity signals along the corticomedullary junction on diffusion-weighted imaging.

Primary intramedullary spinal cord pilocytic astrocytoma with anaplasia in an adult: illustrative case.

BACKGROUND: Primary intramedullary spinal cord (IMSC) pilocytic astrocytoma (PA) with anaplasia is extremely rare. OBSERVATIONS: A 50-year-old man presented to our hospital with numbness of the left posterior rib region, back, and bilateral lower limbs. Contrast-enhanced T1-weighted magnetic resonance imaging (MRI) revealed an intramedullary lesion at T2-T3 with no contrast enhancement. The patient opted for conservative treatment. Eighteen months after the first consultation, the patient presented with slowly progressive numbness of the bilateral upper limbs, paraparesis, and dysuria, with rapid deterioration over the following 3 months. T1- and T2-weighted MRI revealed expansion of the intramedullary lesion, which extended from C7 to T5, and syringomyelia at C5-C6. Contrast-enhanced T1-weighted MRI revealed an enhancing intramedullary lesion at C7-T5. Open biopsy and C5-T5 laminectomy were performed for diagnosis and decompression. PA with anaplasia was diagnosed based on pathological and immunohistochemical findings. The patient received postoperative radiotherapy and chemotherapy. LESSONS: Rapidly progressive IMSC PA with a change in contrast enhancement is extremely rare in adults. PA may undergo a spontaneous malignant transformation during its natural clinical course. In this case, the change in contrast enhancement may have been associated with the malignant transformation of the PA.

True anaplastic oligoastrocytoma with dual genotype: illustrative case.

BACKGROUND: The revised fourth edition of the World Health Organization classification of central nervous system tumors was published in 2016. Based on this classification, one of the infiltrating glioma entities named "oligoastrocytoma/anaplastic oligoastrocytoma" is discouraged. It is proposed that these mixed gliomas should be classified as diffuse astrocytoma/anaplastic astrocytoma or oligodendroglioma/anaplastic oligodendroglioma when analyzing their genetic alteration. OBSERVATIONS: A 78-year-old female underwent brain computed tomography (CT) because of a traffic accident. Cranial CT revealed a brain tumor in the left temporoparietal lobe; therefore, she was hospitalized. She underwent awake craniotomy. After the operation, she was treated with only local radiotherapy; the authors could not prescribe temozolomide, because she had had levetiracetam-induced pancytopenia. The remaining tumor neuroradiologically disappeared, and she was alive 40 months after the operation without tumor recurrence. LESSONS: Histopathologically, this tumor was diagnosed as an anaplastic oligoastrocytoma with a distinct dual phenotype of astrocytoma and oligodendroglioma components. Genetically, these two components revealed astrocytoma and oligodendroglioma genotypes, respectively. Therefore, the authors considered the integrated diagnosis of the temporal tumor as a true anaplastic oligoastrocytoma with a dual genotype. Interestingly, this case also included an area composed of spindle to oval neoplastic cells that revealed intermediate genetic alterations between astrocytomas and oligodendrogliomas.

Cytopathological and histopathological features of cerebral granular cell astrocytoma: A case report.

This report describes the cytological features of granular cell astrocytoma (GCA), to aid in the diagnosis of intraoperative frozen samples of brain lesions, and discuss cytological similarities and differences between GCA, two significant non-neoplastic central nervous system lesions (brain infarction and demyelinating disorder), and three central nervous system tumours (gemistocytic astrocytoma, pleomorphic xanthoastrocytoma, and subependymal giant cell astrocytoma).

Tufted astrocyte-like glia in two autopsy cases of multiple system atrophy: Is it a concomitant neurodegenerative disorder with multiple system atrophy and progressive supranuclear palsy?

Tufted astrocytes are one of the core histopathological features of progressive supranuclear palsy (PSP). To our knowledge, only three cases of multiple system atrophy (MSA) with PSP pathology have been reported. Here, we report two autopsy cases of MSA associated with the appearance of tufted astrocyte-like glia (TuALG). Clinically, the patients' symptoms were atypical of MSA; one showed vertical gaze palsy, and the other was a long-term survivor who progressed to a bedridden state shortly after the onset of the disease. These neuropathological observations were characterized by the copresence of MSA-specific changes and TuALG in some of the cerebral cortices but few or none of the other PSP tau pathologies. These cases might emphasize the significance of TuALG in non-PSP neurodegenerative disorders.

Digital mapping of Lewy bodies and neurites in alpha-synuclein stained large cerebral hemispheric sections from three patients with dementia with Lewy bodies showing psychotic manifestations: A pilot study.

BACKGROUND: Dementia with Lewy bodies (DLB) is one of the major neurodegenerative diseases and a clinical diagnosis is made based on the fourth consensus report on DLB. However, clinicopathological features of DLB are variable among cases. METHODS: We analyzed three autopsy-proven cases of DLB (patients 1-3). Their clinical features were variable in spite of their pathological commonality. The entire hemispheric sections were stained for phosphorylated alpha-synuclein (aS), digitized, and each aS positive lesion was mapped using a virtual slide system. RESULTS: The three patients were clinically diagnosed as having DLB. However, patient 1 exhibited amnesia and misrecognition, while patient 3 exhibited abnormal behavior in addition to dementia. Therefore, both patients 1 and 3 did not fulfill the clinical criteria of DLB, in contrast to patient 2. In spite of the clinical heterogeneity, Lewy pathology was similar in patients 1, 2, and 3. Additionally, patient 1 exhibited less frequent Lewy neurites of the amygdala and entorhinal cortex, and less frequent neurofibrillary tangles and senile plaque as compared to patient 2. On the other hand, the Lewy pathology of patient 3 extended far beyond those of patients 1 and 2, wherein the superior to middle frontal cortices, insular cortex, and lentiform nucleus were severely affected by Lewy pathology. CONCLUSIONS: Clinical features could be variable among autopsy-proven cases of DLB. Furthermore, we show that the accent of Lewy pathology differs according to the variability of the clinical symptoms. This method will provide a comprehensive strategy to analyze wide-spread aS lesions scattered throughout the entire cerebral hemisphere and help determine the corresponding pathological lesions responsible for the clinical variability of neurodegenerative disorders, including DLB.

Intracytoplasmic dot-like inclusions as cytopathologically useful findings of ependymoma: Case report of adolescent supratentorial anaplastic ependymoma with clear cell morphology.

Pediatric supratentorial ependymomas often have a clear cell morphology and reveal a RELA fusion. When a clear cell neoplasm is intraoperatively diagnosed, intracytoplasmic dot-like inclusions by cytology are a useful cytopathological feature of ependymoma.

A rare case of corneal keloid occurred 30 years after pterygium surgery and 3 years after cataract surgery.

PURPOSE: We report a case of corneal keloid occurring 30 years after pterygium surgery and 3 years after cataract surgery. OBSERVATIONS: The case of a 72-year-old man was referred because of blurred vision and corneal opacity in the right eye. Pterygium surgery had been performed on the right eye 30 years earlier, and bilateral cataract surgery had been done uneventfully via a temporal corneal incision 3 years ago. Deterioration of vision occurred in the right eye from 2 years ago. At the initial visit, his best corrected visual acuity (BCVA) was 20/2000 on the right. A white nodule that was well demarcated from the underlying stroma was seen on the right cornea. The nodule was excised by superficial keratectomy, with BCVA being 180/200 at 1 week after surgery. Pathological examination of the resected specimen revealed proliferation of fibroblasts and haphazard arrangement of collagen bundles, leading to a diagnosis of corneal keloid. Keloid-like lesion was also later noted in temporal corneal incision site of cataract surgery. CONCLUSIONS AND IMPORTANCE: This rare case of corneal keloid occurred as a late complication of pterygium surgery.

Cerebral white matter tau-positive granular glial pathology as a characteristic pathological feature in long survivors of multiple system atrophy.

INTRODUCTION: It is unclear whether tau-positive granular glial pathology is a characteristic feature of MSA. We aimed to analyse the prevalence and significance of tau-positive granular glial pathology in MSA. METHODS: Fourteen MSA cases were clinicopathologically investigated, focusing on tau-positive granular glial pathology in the frontal and temporal white matter and putamen. RESULTS: In five MSA cases, the temporal white matter showed AT8-positive granular glial pathology; this pathology was detected in the frontal white matter in three cases. AT8-positive granular glia in the white matter were associated with long disease duration with long-term tube feeding and/or long-term tracheotomy. Alpha-synuclein-positive glial cytoplasmic inclusion intensity was not associated with AT8-positive granular glial pathology. The tau isoform of AT8-positive granular glia in the cerebral white matter exhibited three-repeat, not four-repeat, tau. Ten MSA patients showed tau-positive granular glial pathology in the putamen; the tau isoform was predominantly three-repeat tau and four-repeat tau in cases with disease duration ≥13 years and < 13 years, respectively. CONCLUSIONS: Tau-positive granular glia in the putamen is a characteristic pathological feature of MSA. Tau-positive granular glia appear in the cerebral white matter in MSA patients and are associated with long disease duration with long-term tube feeding and/or long-term tracheotomy.

Idiopathic myenteric ganglionitis as a cause of death in a young male patient with sudden abdominal pain: an autopsy case report.

BACKGROUND: Myenteric ganglionitis is a disorder that causes intestinal motor dysfunction. It may be caused due to neoplastic, central nervous system, or systemic infectious disorders. However, some cases are considered to be idiopathic in origin. CASE PRESENTATION: A 33-year-old man was admitted to the hospital with sudden severe abdominal pain accompanied by watery diarrhea. Computed tomography imaging revealed edema of the entire small intestinal wall without ischemic changes. Detailed examination could not be performed for identifying the cause of abdominal pain because of the patient's worsened general condition, and he died 7 days after onset. The autopsy results confirmed the cause of the patient's severe abdominal pain as an idiopathic myenteric ganglionitis. CONCLUSION: Some patients with idiopathic myenteric ganglionitis might die without a definitive diagnosis during their lifetime because of the rarity of this disease. When encountering severe intestinal motility abnormalities of unknown cause, physicians should consider idiopathic myenteric ganglionitis when choosing therapy.

A symptomatic large subependymoma with neuroradiological features mimicking a high-grade glioma: A case report / Gran subependimoma sintomático de características neurorradiológicas que imita a un glioma de alto grado: presentación de un caso clínico

A subependymoma is a benign primary brain tumor classified as a World Health Organization grade I tumor; it is asymptomatic in most cases. We present the case of a 66-year-old Japanese man with a complaint of recurrent vomiting that led to the discovery of a large mass with hemorrhage, peritumoral edema, and a midline shift in the posterior horn of the right lateral ventricle. The patient was pathologically diagnosed with subependymoma after undergoing total tumor resection; a year after the surgery, he was free from tumor recurrence. Although symptomatic subependymomas are rare, they tend to show hemorrhage with peritumoral edema on neuroradiological tests and tend to be confused with high-grade brain tumors. In the present case, we highlight the importance of the appropriate diagnosis for subependymomas showing neuroradiological features that mimic high-grade gliomas. This diagnosis will help in providing suitable treatment for subependymomas

Un subependimoma es un tumor cerebral primario benigno, clasificado por la Organización Mundial de la Salud como un tumor grado I. En la mayoría de los casos es asintomático. Presentamos el caso de un varón japonés de 66 años de edad que reportaba vómitos recurrentes, los que lo llevaron al descubrimiento de una gran masa con hemorragia, edema peritumoral y una desviación de la línea media del cuerno posterior del ventrículo lateral derecho. Se le realizó un diagnóstico patológico de subependimoma luego de que se le sometiera a una resección total del tumor. Un año después de la cirugía no presentaba recurrencia del tumor. Aunque los subependimomas sintomáticos son raros, en las pruebas neurorradiológicas tienden a presentar hemorragia con edema peritumoral y se tiende a confundirlos con tumores cerebrales de alto grado. En el presente caso, destacamos la importancia de un diagnóstico adecuado de los subependimomas que presentan características neurorradiológicas que imitan a gliomas de alto grado. Este diagnóstico ayudará a brindar el tratamiento adecuado para dichos subependimomas

Case of metastatic glioblastoma with primitive neuronal component to the lung.

Glioblastoma (GBM) with primitive neuronal component (GBM-PNC) is a rare GBM subtype recently categorized by the World Health Organization in the revised classification system of 2016. Extracranial metastases originating from GBM-PNC are rare and metastasis to solid organs has never been reported. Herein, we present the first case of metastasis of GBM-PNC to the lung. A 49-year-old man presenting with headache was diagnosed with multiple tumors adhering to the dura matter in the right temporal lobe. Despite surgery and chemoradiotherapy, 2 months after the initial therapy, the patient presented with CSF dissemination and lung metastases. The patient succumbed to the disease 12 months after the first surgery. We discuss the possibility that GBM-PNC may constitute a subtype of glioma with particularly poor prognosis, tending to dissemination and metastasis. Our results suggest that a complementary regular inspection of the whole body via CT may be recommended for the follow-up of patients with GBM- PNC.