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[Shave therapy for surgical treatment of persistent venous ulcer with large superficial dermatoliposclerosis].

Ulcers in chronic venous insufficiency, especially in postthrombotic syndrome, are often resistant to compression therapy. They can be treated effectively by the dermatologic surgeon with a fairly simple operative procedure. The removal of the ulcer together with the extensive lipodermatosclerosis by Humby knife (shave-therapy) and immediate grafting with a meshed split skin graft-possibly in combination with ligation of insufficient perforator veins-yields very good success rates. The results in 13 patients (18 legs) with an average follow up period of 11 months (up to 20 months) are reported.

Aged↗

Radiological-pathological correlation in lipoblastoma and lipoblastomatosis.

BACKGROUND: Lipoblastoma and lipoblastomatosis are uncommon benign mesenchymal lesions that predominantly occur in infancy and early childhood. OBJECTIVE: To evaluate the imaging and histological features of lipoblastoma and lipoblastomatosis. MATERIALS AND METHODS: Retrospective review of the radiological and pathological findings in children with lipoblastoma and lipoblastomatosis treated at a single centre between 1997 and 2004. RESULTS: Eight children (median age 18 months) had undergone imaging and surgery at our institution. An infiltrative growth pattern was identified at imaging in two children with lipoblastomatosis, and a well-defined mass in six children with lipoblastoma. In all patients, imaging showed a lesion composed mostly, but not entirely, of fat. There were no recurrences at follow-up of between 1 and 91 months. CONCLUSION: In infancy and early childhood, the identification of a tumour composed mostly of fat should suggest the diagnosis of lipoblastoma or lipoblastomatosis.

Arm↗

Lipoma arborescens affecting multiple joints.

Lipoma arborescens is a rare benign intra-articular lesion of unknown etiology that usually involves the suprapatellar pouch of the knee joint. Clinically, the most common finding is a slow-growing painless swelling, accompanied by intermittent effusion of the joint. We report a case of a multifocal lipoma arborescens localized in the knees and the hips in a 24-year-old man, initially mimicking an inflammatory arthropathy. The diagnosis of lipoma arborescens was made by magnetic resonance imaging of the hips and the knees. Under arthroscopic guidance, the synovial biopsy of the right knee disclosed the specific histological signs of lipoma arborescens. As far as we know, this is the third case of multifocal lipoma arborescens reported in the English literature.

Adult↗

Pneumatosis coli: a proposed pathogenesis based on study of 25 cases and review of the literature.

Pneumatosis coli is a rare condition characterised by multiple gas-filled cysts within the bowel wall. We present 25 cases treated over the past 30 years. The patients' mean age was 59 years: 15 were female. Presenting symptoms included diarrhoea (n = 17), mucus discharge (n = 17), rectal bleeding (n = 15) and constipation (n = 12). Pneumatosis usually affected the left colon (96%), and diagnosis was by sigmoidoscopy and biopsy in 18 cases (72%). Pneumatosis coli was associated with psychiatric disorders (36%), chronic lung disease (20%) and colitis (12%). A redundant sigmoid colon was observed in 80% of cases on contrast radiology. Five patients had mucosal pseudolipomatosis on histological examination. Histological and immunohistochemical review indicated that the cysts were lined by cells of macrophage/monocyte phenotype and that many similar mononuclear cells were present in the adjacent submucosa and overlying mucosa. Treatment with antidiarrhoeals and anti-inflammatory drugs in 14 patients resulted in improvement in 9 cases (64%). Oxygen therapy (n = 9) always alleviated symptoms. There was a high recurrence rate (50-78%), but with further courses of therapy lasting remission was achieved in five patients. Two patients underwent colectomy. The associations of pneumatosis coli with psychiatric disorders and mucosal pseudolipomatosis are new and of possible pathogenetic significance. A novel pathogenetic mechanism is suggested to explain the association of pneumatosis coli and chronic respiratory disorders. A model of pneumatosis coli linking some of the apparently disparate pathogenetic theories is proposed.

Anxiety↗

Lipomatous primitive neuroectodermal tumor with a glioblastoma component: a case report.

A case of extracerebellar lipomatous primitive neuroectodermal tumor (PNET) with glioblastoma multiforme (GBM) areas is reported. A 44-year-old woman who had been on antipsychotic agents for schizophrenia complained of hemiparesis and drowsiness. She deteriorated progressively and died 3 months later. The autopsy revealed a huge, ill-defined tumor located from right basal ganglia to brain stem. Microscopically, the tumor consisted of three distinct components: clusters of small primitive cells consistent with PNET, mature lipoma-like islands, and a GBM-like component. Neuronal differentiation in PNET areas was confirmed by the presence of Homer Wright rosette, synaptophysin-positive fibrillary background, and ultrastructural demonstration of neuritic processes. Lipoma-like areas composed of lipidized cells containing large lipid droplets were intimately intermingled and closely related with PNET areas. Furthermore, GBM areas were, although predominantly located in the brain stem, often blended with the previous two components. This component was characterized by glial fibrillary acid protein immunoreactivity of atypical tumor cells and the presence of necrosis and endothelial proliferation. PNET areas with lipomatous differentiation in the present tumor may suggest the morphological and histogenetic similarity to liponeurocytoma, although the neuronal element in the former was anaplastic. The association with a GBM component makes the present tumor a unique, and, to our knowledge, previously unrecognized lesion.

Adult↗

Lipoastrocytoma: a rare low-grade astrocytoma variant of pediatric age.

We report on two children with cerebral gliomas showing extensive lipomatous change of tumor cells. One tumor was a large mass occupying the temporal and occipital lobes of the left hemisphere; the other was a cystic lesion with a mural nodule in the left frontal lobe. Histologically, both tumors were composed of glial cells that contained fat droplets coalescing into a single large droplet, thus resulting in an appearance similar to adipocytes. Immunohistochemistry showed GFAP positivity of tumor cells, which was maintained in the cytoplasmic rim of lipidized cells. Synaptophysin and neurofilaments were negative. Ki-67/Mib1 labeling index was low. Electron microscopy showed intracytoplasmic lipid vacuoles, abundant intermediate filaments and a basal lamina surrounding the cell bodies. Molecular genetic analysis of one tumor revealed no TP53 mutation (exons 4-10), no loss of CDKN2A, and no amplification of EGFR, CDK4 or MDM2. Both patients are alive and well after 3 and 7 years, respectively. However, one of them had to be re-operated on circumscribed local recurrences. Our cases represent a rare variant of low-grade astrocytoma that may be designated as "lipoastrocytoma".

Age Factors↗

Cerebrotendinous xanthomatosis: evidence of lipomatous hypertrophy of the atrial septum.

Premature atherosclerosis and cardiac complications have been reported among the systemic manifestations of cerebrotendinous xanthomatosis (CTX), a rare bile acid disorder with predominantly neurological features. In some cases, myocardial infarction has been the cause of sudden death. We examined nine CTX patients to determine whether they also had clinical or subclinical signs of cardiovascular disorders. In four of them, transthoracic echocardiography showed thickening of the interatrial septum compatible with lipomatous hypertrophy. The unexpected association of the two abnormalities is unlikely to be coincidental and suggests that careful cardiac examination should be considered, even in the absence of clinical manifestations.

Adult↗

Intraosseous lipomata of adjacent vertebral bodies.

Osseous lipomata of vertebral bodies are rare. We present a very unusual case where adjacent vertebrae are involved and the plain radiographic and scintigraphic appearances gave cause for some concern. The findings on plain films, scintigraphy, computed tomography and magnetic resonance imaging are discussed.

Adult↗

Pathology of lipomatous lesions in Proteus syndrome.

Proteus syndrome is an extremely rare, complex hamartomatous disorder with markedly variable clinical expression. We present a case of Proteus syndrome with multiple disfiguring soft tissue masses that were present since early childhood. The lesions involved predominantly the right side of the body and included scoliosis, macrodactyly, and limited hyperostosis in the right foot. There was no evidence of cranial or skin lesions. The patient underwent multiple resections of soft tissue masses, including an amputation of the right foot because of severe gait disturbance. All specimens exhibited lipomatous lesions that were probably hamartomatous rather than neoplastic. She carried a diagnosis of neurofibromatosis for more than a decade, but a re-evaluation of clinical features and pathological findings prompted the diagnosis of Proteus syndrome. We believe that a more informed evaluation of the pathology material may help to identify this rare entity.

Adult↗

Head and neck cancers associated with Madelung's disease.

BACKGROUND: Madelung's disease is a rare lipodystrophy that presents with multiple fatty masses in the neck, trunk, and upper extremities. The fatty accumulation is considered a benign disease, but compression of the aerodigestive tract may occur in long-standing disease. METHODS: Eight Chinese patients with Madelung's disease were reviewed. All were male, aged 48 to 67 years, with a history of disease ranging from 4 to 20 years. Two of the eight patients developed aerodigestive symptoms and were subsequently found to have head and neck cancers. These two patients are described. RESULTS: The possible mechanism that may account for an increase in malignant tumors of the airway in this group of patients is the synergistic effect of smoking and alcohol abuse as risk factors for both Madelung's disease and malignant tumors of the airway. Currently it is recommended that these patients should have their fatty lesions removed surgically. The removal of fat facilitates examination of the neck for signs of cervical lymphadenopathy in malignant disease. CONCLUSIONS: Patients with Madelung's disease should be followed regularly. The development of aerodigestive symptoms should be fully investigated with endoscopy and imaging. The cause of symptoms should not be attributed to fatty compression until a carcinoma of the upper airway has been excluded.

Alcoholism↗

Lipomatous hypertrophy of the interatrial septum.

Lipomatous hypertrophy of the interatrial septum is a benign disorder that is characterized by the fat deposition in the interatrial septum. It typically occurs in elderly, obese patients and may cause arrhythmia. We report a case in which lipomatous hypertrophy of the interatrial septum was found incidentally.

Aged↗

Myocardial calcification and subendocardial fatty replacement of the left ventricle following myocardial infarction.

We report a 70 years old man with old myocardial infarction of the anteroseptal wall. He had an acute anterior myocardial infarction ten years ago and coronary angiography showed a total occlusion of the left middle anterior descending coronary artery. He underwent a multidetector cardiac computed tomography for rule out another coronary stenosis before the surgery of a postrenal aortic aneurysm. The unenhanced computed tomography image realized for the quantification of the coronary calcium reveals a low-attenuation curvilinear stripe consistent with subendocardial fat at the anterior ventricular wall and a curvilinear focal apical left ventricular calcification. In this case without a precontrast CT the subendocardial fat could have been misinterpreted , leading us to conclude that a hypoenhancement area (perfusion defect) secondary from the reduced delivery of contrast in the subendocardium was present. The presence of calcium in the myocardium can make not trustworthy the estimation of the size of the infarct and the presence or absence of viability in the same one especially when it is realized by means of magnetic resonance.

Aged↗

Bilateral lipomas of the tongue.

Multiple lipomas of the tongue are quite rare and are seldom reported in the literature. Presented herein is the case of a 76-year-old man with four symmetrical asymptomatic bilateral double lipomas of long standing on the tongue.

Aged↗