Congenital arteriovenous malformation associated with progressive hydrocephalus in a newborn.

Intracranial arteriovenous malformation is rarely presented in newborns or infants. We describe an unusual case of congenital arteriovenous malformation of the brain with multiple sequestered grape-like venous sacs presented with congenital hydrocephalus. This 4-month-old girl born with a large head, presented with progressive hydrocephalus over a period of 4 months. The brain CT showed multiloculated cysts with a high-density mural nodule and thin cerebral mantle. The right lateral ventricle was collapsed by the cystic lesion, and the contralateral ventricle was markedly dilated, which was thought to be due to aqueductal obstruction by the conglomerated nidus of the arteriovenous malformation. Surgical removal of both hemispheric masses, including the overlying thin mantle. The lesion was pathologically confirmed as the arteriovenous malformation which was composed of markedly dilated veins and multiple sequestered aneurysmal sacs. The overlying cerebral tissue was dysplastic and partly infarcted. This case shows that intracerebral arteriovenous malformation is indeed a congenital anomaly and suggests that intrauterine vascular compromise can result in focal or wide maldevelopment of the brain.     

Sick sinus syndrome associated with hypopituitarism：a case report and literature review

Though an association between autoimmune diseases and sick sinus syndrome has been reported,there has been no report on the association of hypopituitarism and sick sinus syndrome.Herein,we provide the first case report of hypopituitarism accompanying sick sinus syndrome in a 51-year-old woman presented to our hospital with syncope due to cardiac arrest.The patient was successfully managed by pacemaker installation and hormone replacement therapy.     

上腔静脉伴冠状窦异常1例

在解剖一具成人男性尸体标本时，观察其心脏，见上腔静脉位置与走行及冠状窭异常,报道如下。     

Xanthomatous meningioma: a case report with review of the literature

Xanthomatous meningioma is an extremely rare variant of meningioma that is characterized histopathologically by the presence of tumor cells with lipid-filled vacuolated cytoplasm. In this report, we describe the fifth documented case of xanthomatous meningioma and review its clinicopathological features. A 76-year-old Japanese male presented with dizziness. Magnetic resonance imaging demonstrated a well-circumscribed tumor in the left parasagittal to frontal region with attachment of the dura mater. Histopathological examination of the resected specimen revealed proliferation of polygonal to spindle cells with eosinophilic cytoplasm and bland round to oval nuclei. Whorl formation and psammomas were scattered, and mitotic figures were rarely seen. A peculiar finding was the presence of extensive xanthomatous change continuing to the above-mentioned typical meningothelial meningioma. These tumor cells had clear vacuolated cytoplasm and bland round to oval nuclei. Immunohistochemically, xanthomatous cells were positive for epithelial membrane antigen. Accordingly, an ultimate diagnosis of xanthomatous meningioma was made. Our clinicopathological analysis revealed that xanthomatous meningioma affects children to young persons or the elderly, and four of five cases were located in the supratentorial region. Although the detailed mechanism underlying the xanthomatous change has not been clarified, this change is thought to result from a metabolic abnormality of the neoplastic meningothelial cells. Further, xanthomatous change has also been reported in atypical and anaplastic meningiomas. Therefore, it is important to recognize that xanthomatous change can occur in meningiomas, and to avoid misidentifying these cells as macrophages.     

The multiple brain abscesses associated with congenital pulmonary arteriovenous malformations: a case report

In this report, we describe a case of multiple brain abscesses associated with diffuse congenital pulmonary arteriovenous malformations (PAVM). Although the cases of brain abscesses associated with congenital PAVM are very rare, the brain abscess could be an initial clinical manifestation in asymptomatic PAVM as in the case presented in this report. PAVM may contribute to the development of a brain abscess by allowing easy bacterial access to systemic circulation through the right-to-left pulmonary vascular shunt, bypassing the filtering effect of the pulmonary capillaries. Hence, this association should be considered in cases with brain abscesses of undetermined etiologic factors.     

Osteosarcoma associated with acromegaly: a case report.

A case of acromegaly who developed an osteosarcoma is recorded, with persistently raised levels of growth hormone. It is possible that there was some aetiological relationship between the increased rate of bone formation and the development of the osteosarcoma.     

OEIS complex with major cardiac malformation: a case report

OEIS Complex is a rare congenital multisystem defect that consists of omphalocele, exstrophy, imperforate anus and spinal defects. We report a case of such complex with additional major cardiac and other multisystem anomalies which are rarely described in literature. The authors give a review of literature on this infrequent complex along with a discussion on its pathogenesis, differential diagnosis and prenatal diagnosis.     

Oncocytic meningioma: report of a case with progression after radiosurgery

Oncocytic meningioma is an uncommon variant of meningioma, characterized histologically by cells rich in mitochondria. This subtype of meningioma needs to be distinguished from other types of meningioma because of its more aggressive behavior. A case of oncocytic meningioma showing rapid clinical progression with diffuse intracranial dissemination after radiosurgery is described. Resistance to radiotherapy has been previously reported in oncocytic tumors of various body sites. This new case is a further example of the greater aggressiveness of oncocytic meningioma, suggesting that radiation therapy might worsen the course of disease.     

Uterine hemorrhage in a menopausal female associated with an arteriovenous malformation and myometritis

A case is described of vaginal bleeding in a 67-year-old female as the first manifestation of a uterine arteriovenous malformation (AVM). Although bleeding is the commonest presentation of an AVM, this is the first case known to have been precipitated by florid myometritis associated with the long-standing presence of an intra-uterine contraceptive device (IUCD). Uterine imaging studies utilising ultrasound scan with color Doppler were instrumental in suggesting the source of bleeding in this case. Subsequently, the patient suffered a torrential hemorrhage which necessitated hysterectomy.     

Recurrent lower gastrointestinal bleeding from congenital arteriovenous malformation in the terminal ileum mimicking intestinal varicosis: a case report

We report on an exceptional vascular cause of gastrointestinal hemorrhage. A 30-yr-old man was admitted because of recurrent hematochezia. Colonoscopy showed circumferential, erythematous, and nodular vascular distensions with hematocystic spots in the terminal ileum resembling varicosis and subsequent computed tomography with 3-dimensional angiographic reconstruction revealed a vascular architecture around the terminal ileum. No other potential source of bleeding was identified. The patient was treated by ileocecectomy and the final diagnosis was of an arteriovenous malformation confined to the terminal ileum. He has been followed up without a further hemorrhagic episode.     

双侧重复肾并双侧融合畸形1例

患者,男,15岁。因右侧腰痛不适3月余,于2006年7月入院。排尿正常,无尿频、尿痛、尿急、血尿、尿失禁及漏尿,无腹胀、腹痛、恶心、呕吐,无发热、咳嗽及外伤史。体检:T37℃,R19     

肺原发性脑膜瘤1例并文献复习

目的：探讨肺原发性脑膜瘤的临床病理特征、诊断和鉴别诊断、治疗及预后。方法：回顾性分析1例肺原发性脑膜瘤患者的临床资料、组织病理形态、免疫组织化学染色、治疗及随访结果,并回顾相关文献。结果：光镜下肿瘤细胞呈短梭形,细胞呈漩涡状排列,细胞异型性不明显,未见明确核分裂象。免疫组织化学染色结果显示肿瘤细胞胞浆AE1/AE3、CK8/18灶状(+)、EMA(+), S100、SMA、TTF-1、CD34、CD31、PR、ALK、Syn均(–),Ki-67阳性率约1%。组织学和免疫组织化学染色结果均支持良性脑膜瘤的诊断。结论：肺原发性脑膜瘤十分少见,掌握其临床病理特征对该病的诊断、鉴别诊断、治疗及预后具有重要意义。