Isolated diffuse hemangiomatosis of the spleen with Kasabach-Merritt-like syndrome

AIMS: Diffuse haemangiomatosis of the spleen is a rare benign vascular condition occurring as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen. It is sometimes accompanied by severe disturbance of blood coagulation. The goal of this study was to characterize an additional case of isolated diffuse haemangiomatosis of the spleen and to determine the histogenesis of this lesion which remains obscure. METHODS AND RESULTS: We describe a case of isolated diffuse haemangiomatosis of the spleen in which histological and immunohistological findings suggested the possibility of a malformative tumour-like lesion. The pathological cavernous vessels were distributed randomly through the red pulp, without continuity with sinuses. The endothelial cells expressed vimentin, factor VIII related antigen and CD34, but not CD8. Some cells lining the sinus lumen expressed CD68, lysozyme and myeloperoxidase. In addition, trabecular veins presented with intimal thickening. These results allow making a diagnosis between diffuse haemangiomatosis and other tumours/tumour-like lesions of the spleen, especially littoral cell angioma, splenoma and peliosis. CONCLUSION: If diffuse haemangiomatosis is usually classified as a benign proliferation of endothelial cells, we suggest that diffuse haemangiomatosis, when confined to the spleen, could be a tumour-like vascular lesion. In this hypothesis, the aetiology may be hamartomatous or malformative as is suspected in arterio-venous haemangioma of the lower extremities. The histogenesis is still questionable and no definitive proof in favour of one or the other hypothesis has been reported.     

Facial haemangioma,agenesis of the internal carotid artery and dysplasia of cerebral cortex: case report

We describe a girl with a facial haemangioma, associated with other vascular anomalies: agenesis of the internal carotid artery, cerebral cortical dysplasia and hypoplasia of the cerebral hemisphere on the same side of the angioma. We studied the patient by conventional arteriography, T1- and T2-weighted MRI and magnetic resonance angiography.     

Cavernous Haemangiomas of the Spinal Cord. A Review of 117 Cases

 The data of 48 studies (published between 1903 and 1996), presenting information of all together 107 patients (108 lesions) regarding pre-treatment clinical and radiological factors, treatment strategies, and the outcome, plus our own experience of nine patients were retrospectively re-analyzed. The prognostic influence of pre-treatment factors was estimated with the chi-square statistics. Clinical evaluation before/after treatment was performed using the Frankel scale. The average bleeding rate was obtained from the ratio of percentage of first bleeding events in the population to the mean age of the population.  There were 47 males and 69 females (aged from twelve to 88 years). Thirty nine percent of the lesions were found in the cervical, 54% in the thoracic (30% upper, 24% lower) and 7% in the lumbar cord. The peak age of presentation was in the fourth decade, the median duration of symptoms was 32 months. Clinical symptoms before treatment were progressive in all cases. Three patterns of clinical presentation could be identified: a) episodes of stepwise clinical deterioration (30%), b) slow progression of neurological decline (41%), c) acute onset with rapid or gradual decline over weeks or months (26%). 58% of the lesions showed clinical or radiological signs of haemorrhage. In 66% of surgical patients (91 efficiently documented cases), clinical improvement was achieved, 28% remained unchanged and 6% deteriorated. Whereas age, sex and lesion location had no influence on the results, duration of symptoms (<three years) correlated significantly to a better outcome (p<0.02).  Surgical management in symptomatic patients is recommended. Once clinical signs caused by the malformation have appeared, the patients tend to experience progressive neurological deterioration.     

Intraneural Growth of a Capillary Haemangioma of the Cauda Equina

Solitary intraneural haemangiomas are very rare. A case of intraneural capillary haemangioma involving two nerve roots of the cauda equina is reported. The patient was a 63-year-old woman with a three years history of intermittent lumbalgia and numbness of the ventral surface of the left thigh. Magnetic resonance imaging detected an intradural extramedullary nodular space occupying mass at the level of the conus medullaris. Laminectomy of T12 and complete removal of the tumour were performed. Histopathological analysis demonstrated a capillary haemangioma. The tumour was located within the sheaths of a spinal nerve root. The lesion consisted of a myriad of small and very small vessels, reticularly arranged with normal nerve fascicles dispersed within the nodules of clustered capillaries. The present case of an intraneural capillary haemangioma of the cauda equina appears to be one of the first reported examples of this entity in the world's literature. The clinical presentation, diagnostic procedures and therapeutic options of intraneural haemangiomas of the conus medullaris and cauda equina are discussed. The current literature is reviewed.     

肺硬化性血管瘤整个病变的克隆性

目的:探讨肺硬化性血管瘤(PSH)整个病变的克隆性并阐明其本质.方法:利用激光显微切割技术获取22例女性肺PSH组织中多角形细胞和表面立方细胞构成的整个病变及病变周围正常肺组织,分别提取基因组DNA,通过基于女性体细胞构成组织内X染色体失活嵌合性和磷酸甘油酸激酶(PGK)基因位点的克隆性分析、观察PSH整个病变的克隆性.结果:22例肺PSH光镜下均表现为典型的组织病理学特征,即呈实性、乳头状、硬化性和出血性组织结构.病变主要由间质圆形或多角形细胞及被覆在乳头状结构表面的立方细胞所构成.克隆性检测结果表明有7例31.8%(7/22)显示PGK基因位点多态性.经HpaⅡ酶切后,所有具有多态性的标本2个条带中有1个条带的密度明显减弱或消失,而另1个条带保留,证明其均为肿瘤性增生.结论:PSH是一种真性肿瘤,构成PSH的2种主要细胞起源于同一类型细胞.     

Endoscopic ultrasonography allowing expectant management of pancreatic haemangioma

Pancreatic haemangiomas are rare benign tumours that can affect both adults and children. They have an unknown incidence and only 15 adult cases have been reported, all from histological examination. Patients present with vague symptoms relating to tumour mass or they are detected incidentally. Cross-sectional imaging is the mainstay of investigation and may reveal arterially enhancing cystic lesions but in the case presented here, it was non-diagnostic. The use of endoscopic ultrasonography confirmed the nature of the benign lesion, allowing a conservative approach as opposed to operative resection.     

Cauda Equina Intradural Extramedullary Cavernous Haemangioma: Case Report and Review of the Literature

Cavernous haemangioma (cavernoma) is a benign vascular lesion, exceptionally located in cauda equina. We report a case, diagnosed and operated in the Department of Neurosurgery from Pitesti County Emergency Hospital, of a 60-year-old woman with history of lumbar region distress, who presented with low back pain, paravertebral muscle contracture, and bilateral lumbar radiculopathy, with sudden onset after lifting effort. The preoperative diagnosis was done using computed tomography (CT) and magnetic resonance imaging (MRI), and the patient underwent surgery—two level laminectomy, dural incision, and tumor dissection from the cauda equina nerve roots under operatory microscope. Histopathological examination confirmed the positive diagnosis of cavernoma of cauda equina. The patient''s outcome was favorable, without postoperative neurological deficits.     

The use of calcium phosphate cement in vertebroplasty of the base of odontoid process

The authors describe the use of bone cement containing calcium phosphate for vertebroplasty of the cavity in the base of odontoid process. A 23-year-old female patient was operated on by incision in lateral cervical area (anterior open access). After a blunt dissection, the working cannula (Kyphon) was introduced under fluoroscopic guidance through the C2 vertebral body to the cavity in the base of the odontoid process. Intraoperatively biopsy of the lesion was taken and histopathological examination excluded the presence of neoplasm. The cavity, presumably haemangioma, was successfully filled with calcium phosphate bone cement KyphOs? FS (Kyphon). The proper filling without paravertebral cement leak was confirmed by postoperative computed tomography (CT). The CT and magnetic resonance imaging performed 9 months after the procedure showed that cement was still present in the cavity. This is the first use of calcium phosphate cement to conduct the vertebroplasty of C2 vertebra.