[Regression of primary melanoma with metastases associated with amyopathic dermatomyositis]. / Dermatomyosite amyopathique associée à un mélanome régressif métastasé.

BACKGROUND: Dermatomyositis is a rare and serious inflammatory connective tissue disease characterized by a typical cutaneous rash and myopathy. Amyopathic dermatomyositis is a particular form of dermatomyositis involving only cutaneous signs and without myopathy present for over 2 years. PATIENTS AND METHODS: A 48 year-old woman presented with a 3-year history of cutaneous rash without myopathy characteristic of amyopathic dermatomyositis. Clinical examination revealed extensive axillary adenopathy, histological examination of which suggested secondary melanoma. The patient reported a black nevus in the axillary area that had disappeared 1 year earlier. Curettage of the lymph node was negative and the patient was treated with interferon (3M 3 times a week). Regression of the cutaneous signs was noted. DISCUSSION: The data, there have been no other reports of paraneoplastic amyopathic dermatomyositis associated with regression of primary melanoma. The literature contains few reports of dermatomyositis associated with melanoma. Amyopathic dermatomyositis may be associated with malignancy.

[Management of thymic cysts]. / Prise en charge des kystes thymiques.

OBJECTIVE: The thymic cysts are rare tumors of the neck and anterior mediastinum. The management of these patients in our institution is reported. Minimally invasive procedures are discussed. PATIENTS AND METHODS: Six patients operated in our institution within ten years, with a follow-up of 7.1 +/-3.7 years are studied retrospectively. RESULTS: There were four women and two men with an average of 39.8 +/-16.5 years. The tumor was found on chest radiograph in four asymptomatic patients, one took medical advice for laryngeal discomfort and another for dysphagia and dyspnea. The tumor was localized in the anterior mediastinum in three cases, in the cervicomediastinal site in two cases and in the cervical site in one case. CT scan was practiced in three patients with a mediastinal tumor and MR imaging in one of them. In patients with cervical or cervicomediastinal tumor, a cervical echography was practiced. All patients were operated on: three by cervicotomy, one by sternotomy, one by partial upper mini-sternotomy and one by right lateral video-assisted mini-thoracotomy. Histology confirmed benign epithelial thymic cyst. CONCLUSION: There is no specific marker of thymic cysts. Only the surgical management, leads to precise the diagnosis and to treat these tumors. No mortality, no complications or recurrences are reported. The minimally invasive surgery takes an interesting place for thoracic location, to explore and treat these benign mediastinal lesions.

[Bilateral papilledema as the manifestation of Schwannoma of the cauda equina]. / Oedème papillaire bilatéral révélant un schwannome de la queue-de-cheval. A propos d'un cas.

PURPOSE: A case of schwannoma of the cauda equina in which the initial manifestation was visual loss, due to papilledema, is described. OBSERVATION: A 56-year-old man presented a history of 1 month of progressively blurred vision. On examination, visual acuity was 20/1,000 due to bilateral papilledema without spinal symptoms or neurological signs. Brain computed tomography demonstrated a nonobstructive hydrocephalus. He underwent a ventriculoperitoneal shunt without improvement. One month later, he complained of disturbance while walking associated with lower back pain. A lumbar puncture revealed an increased opening pressure. Analysis of the cerebrospinal fluid demonstrated an elevated protein level (6.8 g/l). Spinal magnetic resonance imaging showed a neurinoma of the cauda equina. We performed complete surgical resection and the histopathology diagnosis was schwannoma. Neurological dysfunctions resolved except visual acuity due to bilateral optic atrophy. DISCUSSION: The association of papilledema and spinal tumor is an unusual but well-known phenomenon. Several theories have been proposed to explain the development of increased intracranial pressure in such cases but the exact physiopathology remains unknown. CONCLUSION: Spinal cord tumor should be considered when acute papilledema associated with normal brain computed tomography and high cerebrospinal fluid protein levels are encountered. Spinal magnetic resonance imaging should then be performed.

Protective role of selectin ligand inhibition in a large animal model of kidney ischemia-reperfusion injury.

Experiments in rodents have demonstrated an important role for selectins in kidney ischemia-reperfusion injury (IRI). However, the relevance of this in larger mammals, as well as the impact on long-term structure and function is unknown. We tested the hypothesis that small molecule selectin ligand inhibition attenuates IRI, cellular inflammation, and long-term effects on renal interstitial fibrosis. We used a porcine model of kidney IRI and used Texas Biotechnology Corporation (TBC)-1269, a selectin ligand inhibitor. Renal function, tissue inflammation, and tubulointerstitial fibrosis development were evaluated up to 16 weeks. Both warm and cold ischemia models were studied for relevance to native and transplant kidney injury. Pigs treated with TBC-1269 during 45 min of warm ischemia (WI) showed significantly increased glomerular filtration rate compared to control animals. In pigs with severe IRI (WI for 60 min), TBC-1269 treatment during IRI significantly increased renal recovery. Cellular inflammation was strongly reduced, particularly influx of CD4 cells. Quantitative measurement of fibrosis by picrosirius red staining showed strong reduction in TBC-1269-treated groups. TBC-1269 also reduced cold IRI, inflammation, and fibrosis in kidneys preserved for 24 h at 4 degrees C and autotransplanted. The selectin ligand inhibitor TBC-1269 provides a novel and effective approach to attenuate IRI in both warm and cold ischemia in large mammals, in both short and long terms. Selectin ligand inhibition is an attractive strategy for evaluation in human kidney IRI.

Supratentorial primitive neuroectodermal tumours of the brain: multidirectional differentiation does not influence prognosis. A clinicopathological report of 18 patients.

AIMS: To examine the clinical and pathological characteristics of supratentorial primitive neuroectodermal tumours (PNETs) in a retrospective series of 18 patients, according to the strict definition of the World Health Organization classification of tumours that excludes other types of malignant embryonal tumours of the brain. METHODS AND RESULTS: Eleven children and seven adults with supratentorial PNETs were diagnosed between 1993 and 2002 and their medical records were reviewed. An immunohistochemical study was performed on formalin-fixed paraffin-embedded tissue of 18 primary tumours and five recurrences with antibodies for neuronal (neuron specific enolase, synaptophysin, neurofilament, chromogranin A), epithelial [epithelial membrane antigen (EMA), cytokeratin], glial [glial fibrillary acidic protein (GFAP)], muscle (desmin, h-caldesmon, alpha-smooth muscle actin, myogenin) differentiation and with two anti-CD99 antibodies. All tumours showed at least one neuronal marker except chromogranin A; a variable number of cells were GFAP+ or EMA+ in 18/23 tumours. Six primary tumours and one recurrence were positive for cytokeratin and/or one muscle antigen except myogenin. CD99 was observed in 33% of the cases. The mean duration of overall survival was 20 months. The estimated overall survival rates were 61% at 1 year, 29% at 2 years, and 18% at 3 years. Two factors of poor prognosis were identified by univariate analysis: a positive cerebrospinal fluid cytology at diagnosis and the absence of complete resection. No distinct immunophenotype was statistically related to survival. CONCLUSIONS: A multidirectional differentiation is a frequent event in supratentorial PNETs but has no apparent influence on the outcome of this aggressive neoplasm.