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Objective One case of intracranial phosphaturic mesenchymal tumor (PMT) was reported to study the clinicopathologic characteristics, immunohistochemical features, differential diagnosis and prognosis of PMT. Methods and Results A 53⁃year⁃old man presented with 7 years history of bone pain with hypophosphatemia, aggravated for one month. A tumor was discovered in left occipital bone by 99Tcm-octreotide scanning. MRI showed left occipital bone and duramater masses. Tumor⁃induced osteomalacia (TIO) was diagnosed and the lesion was completely removed. The lesions meningeal tissue, periosteum and left occipital bone were examined pathologically. The microscopiy showed that the tumor growed diffusely, involving meningges and bones, and mainly composed of oval and short spindle cells. It contains malformed thick⁃walled vessels, dilated sinusoidal vessels, mature adipose tissue, interstitial mucinous degeneration and mitosis without typical mitotic activity and necrosis. Immunohistochemically, the tumor cells were positive for fibroblast growth factor⁃23 (FGF⁃23), Vimentin (Vim), somatostatin receptor 2 (SSTR2), CD56 and neuron⁃specific enolase (NSE), and focal expression of Bcl⁃2, D2⁃40; the Ki⁃67 labeling index was 5%. Pathological diagnosis was PMT. The patient's serum phosphate level returned to normal after operation. The case had followed⁃up for 22 months, and survived with normal phosphatemia and without local recurrence and distant metastasis at the last followed ⁃ up. Conclusions Intracranial PMT is very rare, and it is very difficult to detect the lesion early. The differential diagnosis should be considered with meningioma and hemangiopericytoma. Accurate localization and completely excision of tumor are effective therapeutic methods. Clinical features, blood biochemical characteristics, image localization and immunohistochemical phenotype are helpful for the correct diagnosis and identification of the spindle cell tumors such as meningoma. DOI：10.3969/j.issn.1672⁃6731.2019.11.012

A 50-year-old female was admitted to our department, complaining of progressive numbness of distal limbs for two years and unsteady gait for two months. “Peripheral neuropathy” was the presumed diagnosis. She has suffered dry mouth for months. Neurological examination revealed proximal upper muscle strength was normal and distal was 5-/5 while muscle strength in lower limbs was normal. Tendon reflexes in all limbs were reduced, and superficial sensation as well as deep sensation in all limbs was also diminished. Deep sensation below T8-10 was diminished. Romberg’s test was positive with negative pathological reflex. Several sensory nerves action potentials (SNAPs) were diminished or absent with normal compound muscle action potentials (CMAPs). Cervical MRI showed hyperintensities in the dorsal column. Serum anti-Ro/SSA antibody was positive. Tear break-up time was abnormal in either eye (5s, normal range>10s); the rate of saliva production declined 0.02 ml/min (> 1.50 ml/15 min); parotid gland contrast sialography was abnormal; lip biopsy was positive with focal lymphocytic sialadenitis with focus score ≥1. The patient was diagnosed as primary Sjogren's syndrome and sensory neuronopathy. She received oral prednisone in dose of 1mg/(kg·d) for four weeks, then reduce the dosage with 5mg/w to 0.50mg/ (kg·d). Later she reduced the dosage with 2.5mg/per week. At the same time, she got cyclophosphamide (100mg every other day) and hydroxychloroquine (0.20g twice a day). Numbness of limbs and unsteady gait were improved when the patient was discharged. Two month later, during the follow-up, the patient’ gait was slightly improved, but the numbness still existed. DOI: 10.3969/j.issn.1672-6731.2016.11.016

Objective To explore the clinicopathological features of rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle. Methods The clinical manifestations, neuroimaging, histopathological and immunohistochemical features were analysed in one case of RGNT of the fourth ventricle, and related literatures were reviewed. Results A 24-year-old female presented with progressive dizziness under no obvious predisposing causes and dyskinesia such as stumbling. MRI revealed expansion of the fourth ventricle, and a mass with long T1WI and T2WI signal and clear boundary could be seen within the fourth ventricle. The border of tumor showed slight enhancement. At surgery, it was observed that the solitary tumor arised from the fourth ventricle and appeared well demarcated with rhomboid fossa. The tumor was blocking the aqueduct of sylvius before it was removed. Microscopically, the tumor exhibited both neuronic and astrocytic components. In the neuronic components, neurocytes formed neurocytic rosettes and perivascular pseudorosettes. At the center of the neurocytic rosettes, there was an eosinophilic core and some region consisted of microcysts. While the astrocytic components of the tumor revealed typical pilocytic astrocytoma structure. The center of neuronic rosettes and perivascular pseudorosettes displayed strong positive staining with synaptophysin (Syn) and oligodendrocytes transcription factor-2 (Olig-2). The astrocytic components showed positive immunostaining of glial fibrillary acidic protein (GFAP). There were focal and partial positive immunostaining of neuron-specific enolase (NSE) in both components of the tumor. The Ki-67 labeling index was 1.50%-2.00% in two components. Conclusions Rosette-forming glioneuronal tumor of the fourth ventricle is an unusual neuronal and mixed neuronal-glial tumors. The imaging examination showed solid or mixed solid-cystic mass at the fourth ventricle with well demarcated border. The lesion has two characteristic components and the distinctive immunostaining of GFAP and Syn expression will help the differential diagnosis. DOI: 10.3969/j.issn.1672-6731.2015.11.013

Cardiac amyloidosis (CA) describes a group of heterogeneous diseases that are characterized by the extracellular fibril deposition of amyloid protein in the myocardium. The abnormal protein is usually derived from light-chain amyloidosis, mutant transthyretin amyloidosis and wild-type transthyretin. Patients with ischemic strokes and amyloidosis have been sporadically reported, however, they are not well summarized. In the present study, a case of cerebral ischemic stroke, secondary to CA was described. This patient presented with dyspnea on exertion, without any evidence of atrial fibrillation. A biopsy revealed deposition of amyloid in the myocardium and Congo Red staining was positive. He suffered from acute infarction of left basal ganglia, resulting from occlusion of the left middle cerebral arterial 6 months prior to admission. However, re-examination of cerebral magnetic resonance imaging in the present hospital revealed an old infarction in the region of the left basal ganglia with a normal appearance of the left middle cerebral artery. Transesophageal echocardiography (TEE) and cardiac magnetic resonance (CMR) both discovered intra-cardiac thrombi, confirming the diagnosis of cardiogenic cerebral embolism. The present study indicates that patients with CA may additionally present with cardiogenic cerebral embolism, and TEE and CMR imaging may help to avoid missing the presence of intra-cardiac thrombi. [ABSTRACT FROM AUTHOR]