Primitive intracranial ependymomas. Salah-Aza?z institute experience]

PURPOSE: - To assess the outcome and prognostic factors of patients with primitive intracranial ependymomas treated at the department of radiotherapy of Salah-Aza?z Institute. METHODS AND MATERIAL: - Between 1972 and 1997, 31 patients aged one to 53 years received postoperative radiotherapy. There were 16 males and 15 females. Location of tumor was infratentorial in 24 cases and supratentoriel in seven cases. Surgical treatment consisted of complete resection in 14, incomplete resection in 13 and unknown type in four patients. All patients were treated with radiotherapy to the craniospinal axis in 26 cases, whole brain in five cases. RESULTS: - Five years survival rate was 63%. For infratentorial tumors, two patients failed locally, two patients failed locally and at distance and four patients failed only at distance, while one patient with supratentorial tumor relapsed locally. Age, performances status, tumor site, gender and extent of surgery had no impact on survival. The treatment field extent was the only variable predictive of outcome. Patients treated with craniospinal irradiation had a survival rate of 86% compared with 37,5% for patients treated with whole brain irradiation. CONCLUSION:- Infratentorial ependymomas seem to have a worse prognosis than supratentorial ones. Irradiation field extent should be correlated to prognostic factors.     

Primary intramedullary ependymomas: retrospective study of 16 cases]

PURPOSE: To review the radiotherapy department experience in treating primary spinal cord ependymomas (PSCE), analyse prognostic factors and provide treatment recommendations regarding literature review. MATERIALS AND METHODS: Sixteen patients with PSCE received postoperative radiotherapy between 1972 and 1997. There were 10 male and 6 female patients with a mean age of 34 years (range 2-63). Surgery was gross total resection in 2 cases, subtotal resection in 9, biopsy in 4 and of unknown type in one patient. All patients were treated with radiotherapy to the craniospinal axis (4 cases), spinal cord (1 case) and to the site of primary tumour in 11 cases. RESULTS: Five year-survival rate was 73%. Two patients had recurrent tumours within the primary site. Gender, extent of surgery and treatment field extent were not prognostic factors. Histologic type was the only variable predictive of outcome. Patients with myxopapillary type had a 5-year survival rate of 100% compared with 47% for those with other histology types. CONCLUSION: We conclude that aggressive surgery is not necessary in the management of PSCE, localised field radiotherapy is associated with favourable outcome, and tumour grade is an important prognostic factor.     

Antinuclear antibodies in children: clinical signification and diagnosis utility

Background: Antinuclear antibodies (ANA) test is used to screen adults as well as children for connective tissue diseases (CTD) and autoimmune hepatitis. However, interpretation of ANA positivity can be delicate. Aim: to determine clinical significance and diagnosis utility of ANA positivity in children. Methods: Patients from a general pediatric department with ANA positive results were included (follow-up period of 2 years). ANA screening was performed by indirect immunofluorescence (IIF) on HEp-2 cells substrate (BioSystems^®). In case of ANA positivity (cut-off: 1:80), the specificity was determined by IIF on Crithidia luciliea substrate (BioSystems^®) and immunodot (Euroimmun^®). Results: Among 102 ANA tests, 55 (53,9%) were positive. We recorded the data of 38 patients (age average: 9,5 years - sex ratio: 0.72). The most frequent signs were join pain (55,3%). ANA titer varied between 1:80 (39,5% of cases) and 1:1280 (2,6% of cases). Typing was negative in 89,5% of cases. The majority (42,1%) of children with positive ANA test had musculoskeletal diseases. The others (57,9%) had systemic lupus erythematosus(n=2), overlap syndrome(n=1), rheumatoid purpura(n=2), idiopathic thrombocytopenic purpura(n=1), coeliac disease(n=1) or non-autoimmune diseases/no confirmed diagnosis(n=15). Conclusions: ANA prevalence in children was relatively high. When the pretest probability is low, the positive predictive value for CTD or autoimmune hepatitis is low. However, depending on the clinical context, ANA detection can represent a supplement diagnostic tool for these diseases and/or can lead to a clinico-biological monitoring.     

Tim-3 mediates T cell trogocytosis to limit antitumor immunity

T cell immunoglobulin mucin domain-containing protein 3 (Tim-3) negatively regulates innate and adaptive immunity in cancer. To identify the mechanisms of Tim-3 in cancer immunity, we evaluated the effects of Tim-3 blockade in human and mouse melanoma. Here, we show that human programmed cell death 1–positive (PD-1⁺) Tim-3⁺CD8⁺ tumor-infiltrating lymphocytes (TILs) upregulate phosphatidylserine (PS), a receptor for Tim-3, and acquire cell surface myeloid markers from antigen-presenting cells (APCs) through transfer of membrane fragments called trogocytosis. Tim-3 blockade acted on Tim-3⁺ APCs in a PS-dependent fashion to disrupt the trogocytosis of activated tumor antigen–specific CD8⁺ T cells and PD-1⁺Tim-3⁺ CD8⁺ TILs isolated from patients with melanoma. Tim-3 and PD-1 blockades cooperated to disrupt trogocytosis of CD8⁺ TILs in 2 melanoma mouse models, decreasing tumor burden and prolonging survival. Deleting Tim-3 in dendritic cells but not in CD8⁺ T cells impeded the trogocytosis of CD8⁺ TILs in vivo. Trogocytosed CD8⁺ T cells presented tumor peptide–major histocompatibility complexes and became the target of fratricide T cell killing, which was reversed by Tim-3 blockade. Our findings have uncovered a mechanism Tim-3 uses to limit antitumor immunity.     

Crohn's disease lesions in the upper gastrointestinal tract: correlation between clinical, radiological, endoscopic, and histological features in adolescents and children

The incidence of Crohn's disease (CD) lesions in the upper gastrointestinal (GI) tract of both adults and children is frequently underestimated. In this prospective study, a total of 31 children suspected of having Crohn's disease were systematically examined to identify upper digestive tract lesions. They all underwent barium transit endoscopy with multiple-level biopsies. Typical clinical symptoms suggestive of upper GI tract involvement were found in 5 children (16%), radiological signs in only one child (3%), endoscopic lesions in 13 children (42%), and specific granulomas in 12 children (39%). In eight of these 12 children, the biopsies were taken from macroscopically normal areas of the esophagogastroduodenal mucosa. One of the 31 children had no abnormal radiological and endoscopic features suggestive of CD on the distal small bowel and the colon. There was no correlation between the clinical, radiological, and histological data. Endoscopy plus biopsy provided a positive diagnosis in 39% of cases and a confirmation of the diagnosis in 87% of cases. Endoscopic and histological evidence of CD of the upper GI tract is often present despite an absence of clinical symptoms or radiological changes. Upper GI tract endoscopy with multiple biopsies may be important in the evaluation of this condition and even in some cases for the establishment of the diagnosis.     

Congenital diaphragmatic hernias in the adult: report of six cases

The anterior hernia or the fissure of Larrey and Bochdalek hernia are exceptional in adult. We present 6 cases of congenital diaphragmatic hernia revealed in adult. Three hernia of the fissure of Larrey and three Bochdalek hernia. We discuss the different clinical, radiological, and therapeutic aspects of these hernia. We insist on the gravity of strangulated hernia and their surgical treatment.     

Cholangitis revealing an intrahepatic Osler's disease

Osler Weber Rendu Disease is an hereditary haemorrhagic télangectasia habitually revealed by reccurent bleeding (epistaxis). Hepatic involvement in Osler disease is found in 8 to 31%, manifested by cholestasis. We report an original observation of a cholangitis revealing Osler disease.     

Sarcomas of the small intestine

Sarcomas of the small intestine are rare, clearly differentiated, malignant, mesenchymatous tumours that can be of smooth muscle, Schwann cell or fibroblastic origin. From a clinical point of view, the pain and abdominal mass are the 2 types of symptoms that frequently reveal the disease. In rare cases, sarcomas of the small intestine are manifested by an acute complication. No imaging method can clearly confirm the diagnosis. Before immunohistochemistry, differential diagnosis was made on undifferentiated mesenchymatous "stromal" tumours, which are also rare. Exeresis must be complete and without perforation of the tumour because of the risk of locoregional relapse. The benefits provided by chemotherapy and radiotherapy are limited because of the low mitotic activity of the tumour cells and its weak vascularisation. Long-term survival is limited by poor prognosis criteria: high grade malignancy, size greater than 5 cm, tumour extension, perforation of the tumour, quality of surgical resection and histological type.