易误诊为神经肿瘤的一种疾病——肿块型中枢神经系统原发性血管炎

背景与目的：由于缺乏特异性临床和影像表现，肿块型中枢神经系统原发性血管炎（primary angiitris of central nervous system，PACNS）常被误诊为胶质瘤．本文旨在探讨肿块型PACNS的诊断与鉴别诊断。方法：回顾性分析手术治疗的37例肿块型PACNS患者的临床及影像表现。结果：85．3％的患者出现局灶性神经功能减退，55．9％患者有不同程度皮层高级功能减退症状。70．6％的颅内病变累及脑皮层：73．5％患者的正常区域脑血管周围间隙增宽，其中76％出现血管强化；病变出现特征性的“C”形强化。结论：局灶性神经功能和高级功能减退、病变累及皮层和“C”形强化等特征．有助于诊断肿块型PACNS。     

M.R.I. Spectrum of Intrinsic Spinal Cord Lesions

We report our experience of 42 patients undergoing magnetic resonance imaging (MRI) studies demonstrating intrinsic lesions of the spinal cord, excluding those secondary to spondylosis, trauma or congenital malformations. Histological confirmation of the radiological diagnosis was obtained in 15 (36%). MRI is more sensitive than either myelography or CT. MRI identified a previously non-visualised lesion in 17 of 30 cases. In a further 13 cases, MRI contributed by identifying associated features such as cysts and haemorrhage which have aided in the diagnosis and management of the patients. The last 12 cases, which were patients with multiple sclerosis, had MRI as the only neuroradiological examination. These illustrate a range of appearances. The overlap of imaging appearances of the various pathologies implies that a useful diagnosis and differential can only be offered by considering the MRI appearances of a lesion, with its associated features, with the clinical presentation, and with other imaging modalities where appropriate.     

Delayed Reversible Posterior Encephalopathy Syndrome Following Chemotherapy with Oxaliplatin

Reversible posterior leukoencephalopathy (RPLS), also known as posterior reversible encephalopathy syndrome, is characterized by magnetic resonance imaging (MRI) findings of reversible vasogenic subcortical edema without infarction. The clinical presentation is usually nonspecific and typically involves global encephalopathy, seizures, headache, or visual symptoms. MRI of the brain is essential to the diagnosis of RPLS. Typical findings of RPLS include high-intensity signal on T2-weighted images predominantly in the posterior lobes of the brain that is caused by subcortical white matter vasogenic edema. Fluid-attenuated inversion recovery (FLAIR) sequences on MRI improve sensitivity and detect subtle peripheral lesions. This clinical radiographic syndrome has been described in a number of medical conditions, with hypertensive encephalopathy, eclampsia, and the use of immunosuppressant drugs (most notably calcineurin inhibitors) being the most common. It has occasionally been reported with cisplatin and rarely with carboplatin. Its occurrence with oxaliplatin is very unusual. An extensive literature search including PUBMED and direct contact with the drug manufacturer yielded only 2 known case reports. Herein, we describe a case that had classic clinical and radiologic features of RPLS. We also briefly describe 2 other patients who have been described to have RPLS with oxaliplatin in the literature.     

9例脑原发淋巴瘤的MRI影像特征

背景与目的:脑原发淋巴瘤少见,术前常被误诊为恶性胶质瘤或转移瘤.由于脑原发淋巴瘤对化学治疗及放射治疗敏感,因此术前明确诊断具有重要意义.本研究回顾性分析9例经病理证实的免疫状态正常人脑原发淋巴瘤的MRI表现,以提高对本病的诊断及鉴别诊断能力.方法:分析9例脑原发淋巴瘤MRI表现特征(包括病灶的数目、位置、信号强度、瘤周水肿、占位效应程度及瘤体强化特点).结果:9例病变均为单发,病灶位于幕上者8例,同时累及幕上幕下者1例.平扫T1WI上呈低信号8例,等信号1例;平扫T2WI上等信号6例,低信号1例,稍高信号2例.2例病灶中见坏死改变.瘤周水肿轻度5例,中度3例,重度1例.增强后肿瘤呈团块状强化4例,分叶状强化3例,环状强化2例.结论:免疫状态正常人脑原发淋巴瘤具有较典型的MRI征象,结合患者影像学及临床资料,术前可作出明确诊断.     

Granulomatous angiitis of the spinal cord associated with Hodgkin's disease

A 28-year-old man had a 5-month history of focal and generalized neurologic symptoms culminating in a thoracic myelopathy. Evaluation revealed granulomatous angiitis of the spinal cord in association with occult nodular sclerosing Hodgkin's disease. In previous reports, manifestations indicative of intracranial involvement have dominated the clinical presentation of granulomatous angiitis associated with Hodgkin's disease. Successful therapy for Hodgkin's disease may result in marked improvement of associated granulomatous angiitis, whereas the lack or failure of therapy results in a uniformly fatal outcome. Definitive antemortem diagnosis of granulomatous angiitis requires a biopsy of involved tissue. The cause of granulomatous angiitis, as well as the nature of its association with Hodgkin's disease, remains unexplained.     

Solitary hemispheric demyelination in acute disseminated encephalomyelitis: Clinicoradiological correlation

We retrospectively studied 13 patients with solitary hemispheric demyelination in acute disseminated encephalo­‐myelitis (solitary‐ADEM) to look for specific MRI features. Thirteen patients were subjected to routine MRI with varying initial clinical diagnosis, including demyelination, neoplasm, encephalitis and infarct. The provisional MRI diagnosis was based on lesion morphology, size, location, mass effect, effect on adjacent sulci, cisterns and gyral shape. On long repetition time (TR)/long echo time (TE) spin echo sequences, the lesions were heterogeneously hyperintense, and on short TR/short TE spin echo sequences, heterogeneously hypointense. Later, the provisional MRI diagnosis was corroborated with clinical parameters such as multimode‐evoked potentials, electro­‐physiological studies and analysis of cerebrospinal fluid (CSF) in order to establish a definitive diagnosis. The diagnosis was established in all except one patient with tumour‐like clinical and MRI features. The combination of MRI features and clinical parameters can establish a definitive diagnosis in the majority of cases, and this avoids a biopsy.     

Impact of Preoperative MRI in Invasive Ductal Carcinoma With Lobular Features on Core Biopsy

BackgroundInvasive breast cancer is comprised of a wide spectrum of histological types with different clinical presentations, imaging characteristics, and behaviors. Almost 10% of breast cancers with predominantly invasive ductal features have lobular components on core biopsy at primary diagnosis. Although the role of magnetic resonance imaging (MRI) in patients with purely lobular cancers is well-established, it is not clear if preoperative MRI is indicated in ductal cancer with lobular features. The aim of this study was to assess the role of preoperative MRI in patients with invasive ductal cancers with lobular features on core biopsy.Materials and MethodsData regarding patients with lobular features on core biopsy who underwent a preoperative MRI from January 2015 to December 2017 were retrospectively identified and analyzed. Imaging findings, additional investigations, and changes in treatment plans following the MRI scan were reviewed.ResultsThe study included 120 patients, of whom 42 (35%) patients required a second-look ultrasound. Following a repeat ultrasound scan, 25 breasts and 4 axillae were biopsied. Thirty-eight percent of the breast biopsies and 50% of the axillary biopsies were malignant. Based on MRI findings, treatment plans changed in 22.5% of patients. MRI size was concordant with the histological size in 58.3% of cases, and MRI was accurate in 90% of patients in detecting multifocal disease requiring mastectomy. The majority of patients with changes in the management plans had mixed ductal and lobular cancer on final histology.ConclusionThis study has demonstrated that MRI picks up additional malignancies and changes management plans in patients with lobular features on core biopsy and should be considered in the preoperative workup.     

Cerebellar metastases: diagnostic and management considerations

Prompted by several unsatisfactory outcomes, we reviewed the records of 59 patients with cerebellar metastases (26 solitary) with respect to clinical presentation, diagnosis, and natural history. Eighty-seven percent of patients initially complained of headache, gait disturbance, and/or dizziness. At time of diagnosis, 92% of patients with solitary cerebellar metastases and 74% of the overall series complained of headache and/or difficulty walking. In three of four cases, magnetic resonance imaging (MRI) was superior to x-ray computed tomography (CT) in detecting the cerebellar lesions. Several patients acutely deteriorated during evaluation or at the initiation of radiation therapy. We conclude that a cancer patient presenting with headache and gait difficulty with or without nausea/vomiting and dizziness should promptly undergo head CT scanning, and that MRI is useful even if CT is negative. In addition, we recommend that patients with documented cerebellar metastases receive high-dose glucocorticoid therapy for 48 to 72 hours before beginning radiation therapy. The presence of symptomatic hydrocephalus or failure to respond to glucocorticoids initially are particularly ominous features that may be best managed by early neurosurgical consultation before beginning radiation therapy.     

Early MRI findings in High Grade Glioma

Magnetic resonance imaging (MRI) is more sensitive than computerized tomography in the detection of many intracerebral lesions; however, the significance of some MRI findings may be unclear. Over four years, nine patients, aged 40–79 years, have been encountered whose initial MRI scans were negative or had minimal abnormalities and soon thereafter had high grade glioma. Initial MRI was performed in eight patients for new-onset seizures and one patient for a focal deficit. MRI was negative in four of the patients and mildly abnormal in five of the patients (small areas of increased T2 and/or minimal enhancement). The initial diagnoses usually included inconclusive differentials of stroke and infection with neoplasm less frequently considered. Radiographic progression leading to the diagnosis of high grade glioma became evident on repeat MRI in 1–8 months with six patients showing progression within three months. All patients underwent surgery and had histologic diagnosis of glioma. Although MRI is quite sensitive, four of the initial scans were negative with reasonable quality studies. Conversely, in five of the initial scans, the tumors were detected when so small that the radiographic findings were not typically diagnostic. Glioma must be considered as a possible cause of initial seizures or new neurologic deficits in adults with normal or minimally abnormal MRI. In this group, seizures were the overwhelming hallmark of presentation. In such a clinical situation, close follow-up with short interval repeat MRI should be performed.     

Clinical features and diagnosis of primary central nervous system lymphoma

This article reviews the clinical features of primary central nervous system lymphoma (PCNSL) in immunocompetent and immunocompromised patients. Clinical presentation, differential diagnosis, diagnostic testing, and staging evaluation in both immunocompetent and AIDS patients who have PCNSL are discussed. The differing role of biopsy in these two populations also is addressed.     

Different clinical features of primary and secondary tumors in patients with multiple malignancies

Clinical features of the first and second primaries in patients with multiple malignancies have not been extensively studied. We compared patient and treatment characteristics of the primary malignancy in 48 consequent multiple primary cancer patients with those of the second primary in the same cohort. The second primaries comprised fewer breast cancers; 29.2% of primaries as opposed to 10.4% of second tumors were breast cancer (P = 0.049). In addition, primary tumors tended to be at a lower TNM stage than secondary tumors (P = 0.060). The median overall survival after the diagnosis of the first primary for the whole cohort was 22.3 years (95% CI, 2.0-42.5) and the median time to presentation of the second malignancy was 38 months after the diagnosis of the first primary (range, 0 to 384). Therefore, the prognosis of cancers in the multiple malignancy group appears to be good and they appear to have an indolent clinical behavior. Thus, we recommend a long screening time for secondary tumors after a curative treatment in patients with common cancers, taking into account the different occurrence patterns of second primaries with respect to first primaries.     

A study of patients with brain metastases as the initial manifestation of their systemic cancer in a Chinese population

To investigate the clinical characteristics of patients with brain metastases as the initial manifestation of their systemic cancer in a Chinese population, a retrospective study of 254 such patients admitted to Huashan Hospital, Fudan University, Shanghai, China between January 1, 2003 and December 30, 2008 was performed. Data were collected to determine the features of this group (i.e., manifesting signs and symptoms, imaging studies, extracerebral metastases, primary tumor sites, initial diagnosis, and survival data). Common symptoms included headache and motor impairment. The distribution of brain metastases paralleled blood flow, and the majority of brain metastases were located in the cerebral hemispheres. Magnetic resonance imaging (MRI) was more sensitive than computed tomography (CT) for confirming presence of brain lesions. This distinct clinical entity exhibited high rates of misdiagnosis at initial presentation. Pathology varied, and adenocarcinomas were most commonly observed. Underlying primary tumors were identified in 84.2% of patients, most often located in lung (71.7%), followed by digestive tract. Chest CT had high yield. Sixty-two patients presented with silent extracerebral metastases at initial presentation. Median survival time was 15 months (95% confidence interval, 12.2–17.8 months). Survival rates for 1, 2, and 5 years were 59.2%, 23.2%, and 15.1%, respectively. Contrast-enhanced MRI had high yield for detection of brain metastases. Adenocarcinoma was the most common histologic type. Given the high frequency of primary lung tumors and the sensitivity of chest CT, chest CT should be a part of the initial screen of primary site with brain metastases as the initial manifestation. Metastatic dissemination of malignancy to the brain as the initial manifestation is generally associated with dismal prognosis, with the exception of a minority who experience long survival.     

A primary CNS lymphoma in spontaneous remission for 3.5 years after initial detection of the lesions by MRI

We describe the clinicopathological features of a patient with an autopsy-proven primary CNS lymphoma, who had a relatively long remission period after onset. A 61-year-old man experienced disorientation and gait disturbance. A ventriculoperitoneal shunt operation was performed, based on the diagnosis of hydrocephalus due to aqueductal stenosis, after which his symptoms subsided. Three months later, T₂-weighted magnetic resonance imaging (MRI) revealed high-intensity lesions in the corpus callosum and tectum. However, he remained asymptomatic for the next three and a half years, during which periodic MRI studies constantly detected the lesions. At the age of 65 years, he suffered respiratory and consciousness disturbances, and his general condition gradually deteriorated. MRI studies disclosed that the callosal lesion had spontaneously disappeared, whereas the tectal lesion had developed to become an enhanced linear lesion with a tendency to spread on the dorsal side of the brain stem. Examination of the cerebrospinal fluid disclosed a markedly elevated β₂-microglobulin content, and a tentative diagnosis of malignant lymphoma was made. Steroid pulse therapy had little effect, and the patient died four years after presentation. Examiaation at autopsy disclosed a malignant, large B-cell lymphoma that had diffusely infiltrated the cerebrum and brain stem. In the corpus callosum, a small number of residual lymphoma cells were seen around the vessels. Therefore, the initially detected lesions in the corpus callosum and tectum might have been attributable to lymphoma, and the unusual clinical and radiological features of this case provide further information that might aid in diagnosis and help to ensure prompt treatment.     

Cancers primitifs de l’intestin grêle: aspects épidémiologiques et diagnostiques au CHUYO: à propos de dix cas et revue de la littérature

Objective

Specify the epidemiological, clinical and paraclinical peculiarities of the small bowel primary cancers at the Yalgado Ouedraogo Teaching Hospital of Ouagadougou (CHUYO), in order to improve the prognosis.

Methodology

A retrospective study of 10 cases of small bowel primary cancer was conducted at the CHUYO, over a period of six years and five months. All the patients for whom the histological diagnosis of the biopsic or operation specimen revealed a cancer of the small bowel, were included in the study. Patients suffering from secondary cancers, benign tumors, and those who didn’t benefit from a histological diagnosis were excluded. In each case, the civil status, the time taken for consultation, the history, the clinical and the paraclinical signs were taken into account.

Results

The ten cases of small bowel primary cancers represented 4% of digestive cancers. There were six women and four men of an average age of 34 with the extreme ages of 16 and 52. Six patients were from the urban area and four from the rural area. The average time for consultation was eight months. Abdominal pains were the main reason for consultation. Six of the patients had history of Koenig’s syndrom. Eight patients were in a spoilt general state. At the time of consultation eight patients presenting abdominal emergencies (7 occlusions and 1 peritonitis). An echography and the scanner enabled a diagnosis in two cases, before the operation. Adenocarcinoma was the most recurrent histological type.

Conclusion

Cancers of the small bowel are rare. The diagnosis is late and difficult because of the lack of specific signs.     

Primary lymphoma of the brain--a report of 5 cases and review of the literature

Five cases of primary lymphoma of the brain are reported and the literature reviewed. The diverse clinical spectrum at presentation and variable response to therapy are few of its unique features. The literature is reviewed briefly and the importance of diagnosis at an early stage and prompt institution of therapy in form of radiation therapy and chemotherapy when indicated is stressed.     

Prognostic validation and clinical implications of the EANO ESMO classification of leptomeningeal metastasis from solid tumors

Background The EANO ESMO guidelines have proposed a classification of leptomeningeal metastases (LM) from solid cancers based on clinical, magnetic resonance imaging (MRI), and cerebrospinal fluid (CSF) cytology presentation. MRI patterns are classified as linear, nodular, both, or neither. Type I LM is defined by positive CSF cytology (confirmed LM) whereas type II LM is defined by typical clinical and MRI signs (probable or possible LM). Here we explored the clinical utility of these LM subtypes.Patients and methodsWe retrospectively assembled data from 254 patients with newly diagnosed LM from solid tumors. Survival curves were derived using the Kaplan–Meier method and compared by Log-rank test.ResultsMedian age at LM diagnosis was 56 years. Typical clinical LM features were noted in 225 patients (89%); 13 patients (5%) were clinically asymptomatic. Tumor cells in the CSF were observed in 186 patients (73%) whereas the CSF was equivocal in 24 patients (9.5%) and negative in 44 patients (17.5%). Patients with confirmed LM had inferior outcome compared with patients with probable or possible LM (P = 0.006). Type I patients had inferior outcome than type II patients (P = 0.002). Nodular disease on MRI was a negative prognostic factor in type II LM (P = 0.014), but not in type I LM. On multivariate analysis, administration of either intrathecal pharmacotherapy (P = 0.012) or systemic pharmacotherapy (P = 0.0003) was associated with improved outcome in type I LM, but not in type II LM.ConclusionThe EANO ESMO LM subtypes are highly prognostic and should be considered for stratification and overall design of clinical trials.     

Clinical and radiological features of biliary papillomatosis

Biliary papillomatosis is a rare disease with strong potential for malignant degeneration. Diagnosis is often not easy and most are made intraoperatively. In the present study, five patients with biliary papillomatosis admitted between 1990 and 1997 were reviewed. Their clinical presentation, radiological and biochemical findings were analysed. The aim of the study was to discern a set of characteristic features that would enable an early diagnosis. All of the five patients presented with recurrent episodes of acute cholangitis and epigastric pain with raised serum alkaline phosphatase. Imaging modalities including ultrasound, CT, endoscopic retrograde cholangiopancreatogram, MRI and magnetic resonance cholangiopancreatogram were reviewed. Salient imaging features included a dilated biliary tree with multiple ill‐defined and fuzzy filling defects or endoluminal frond‐like mass lesions. In conclusion, biliary papillomatosis is a rare but important cause of biliary obstruction with relapsing cholangitis and obstructive jaundice. With a healthy index of suspicion, the diagnosis can be reached when the above features are available.     

Sarcome d’Ewing des parties molles. À propos d’un cas et revue de la littérature

A case of intraosseous Ewing sarcoma is reported. This pathology of the young adult is very rare, clinical or imaging (CT or MRI) findings are non-specific and diagnosis is based on histology. Nonethless, this diagnosis should be considered in all patients with primary soft tissue tumors.     

腮腺基底细胞腺瘤MRI特征与病理对照分析

背景与目的：腮腺基底细胞腺瘤(basal cell adenoma,BCA)发病率相对较低,临床误诊率高,目前为止,对该病的MRI表现报道较少。该研究结合动态增强磁共振成像(dynamic contrast enhanced magnetic resonance imaging,DCE-MRI)和扩散加权成像(diffusion weighed imaging,DWI)探讨BCA的MRI特征及其与病理学相关性。方法：回顾性分析经手术病理证实的26例腮腺BCA患者的临床表现、病理学改变及MRI影像学特征。分析患者临床表现、病灶数目、部位、大小、形态、信号与强化特征及其病理学表现。结果：26例患者中,女性18例;中位年龄51(32~72)岁;共27枚病灶(25例单发,1例双侧各1枚)。病灶平均大小22 mm(11~36 mm)。13枚病灶位于浅叶,5枚位于深叶,9枚同时累及深、浅叶;肿瘤呈圆形或椭圆形,均未见分叶,边界清楚,其中25枚病灶T2WI见周缘低信号环;对照颈部肌肉,病灶实性部分T1WI呈等或略高信号,T2WI呈高或略高信号,其中21枚病灶内见大小不一囊变区。增强后21枚病灶实性部分呈均匀强化,6枚呈不均匀强化。时间-信号强度曲线显示21枚病灶(77.8%)呈平台型,6枚(22.2%)呈流出型。9例患者(10枚病灶)行DWI检查,表观弥散系数图(apparent diffusion coefficient,ADC)均值为(1.22±0.20)×10^-3 mm²/s。镜下显示：肿瘤由单一基底细胞构成,并有明显的基底细胞层及基底膜样结构,缺乏黏液软骨样成分。结论：腮腺BCA的MRI表现有一定特征性,分析其MRI特征与其病理相关性,对于肿瘤的定性诊断及鉴别诊断有较大的价值。     

Magnetic resonance imaging of intramedullary spinal cord lesions: A pictorial review

Magnetic resonance imaging (MRI) is the modality of choice for the investigation of intramedullary lesions of the spinal cord. A wide variety of conditions may result in similar imaging findings on MRI, and it is essential that the reporting radiologist have a detailed understanding of spinal cord anatomy, the pertinent imaging features of specific intramedullary lesions and the typical clinical presentation of those conditions to aid clinicians to make a prompt diagnosis. This pictorial essay discusses the clinical features and MRI appearance of a number of intramedullary conditions, which can be broadly categorised as congenital, demyelinating, vascular, neoplastic or infectious, and highlights their differentiating features.