[Ewing sarcoma osseous and extra osseous: a clinicopathological study of 29 cases]

Ewing's sarcoma [ES] is a rare tumour accounting for 10% of primary malignant bone tumours in children and 3% of all childhood malignancies. ES belongs to a group of small round cell tumours. In this review, we will describe the main clinicopathological features of this rare tumour and discuss its prognosis. We report a retrospective study of 29 cases of ES, of which 4 were extraosseous, diagnosed over a period 11 years [January 1989 - December 1999]. Clinicopathological data were described. Hematoxylin-eosin staining and immunohistochemical study were reviewed. 12 patients were male and 17 were female [ratio: 0.8] with a median age of 16 years. 62.5% of tumours were located in flat bone and 33.3% in long bone. The medium size of the tumor was 10.6 cm [range:3-25cm]. 27.5% of patients presented with metastatic disease at time of diagnosis. Microscopically, tumour tissue was composed of round, small, blue cells with fine granular chromatin. Tumour cells strongly coexpressed CD99 and vimentin [100%]. Systemic treatment consisted of adjuvant chemotherapy [84.2%]. Local control was based on and surgery [57. 9%] or radiation therapy [36.8%]. A good response to chemotherapy was obtained in 37.5%; 13.7% of patients were alive without disease [medium follow up: 169 mois]; 34.5% of patients developed metastases [medium follow up: 23 months] and 10.3% developed recurrences [medium follow up :13 months]. Our study emphasizes two points: the great size of the tumor and the frequent location in flat bone which may explain the poor prognosis of Ewing sarcoma in our series despite the multidisciplinary treatment

[Nephrotic syndrome in diabetis: evolution and prognosis about 35 cases]

The objective of our study was to evaluate the prognostic factors in the nephritic diabetic syndrome and its evolution. We realized a retrospective study of 35 cases of diabetic patients hospitalized between 1985 and 1999 who presented with a nephrotic syndrome. They included 17 females and 18 males, their mean age was 43 years [extremes: 18-77 years] and the mean duration of their diabetes was 10 years [extreme: 0-25 years]. The diabetes was insulin-dependent [type1] in 19 cases and non insulin-dependent [type 2] in 16 cases. Retinopathy and neuropathy were presents in 88% and 83% of the cases respectively. Nephrotic syndrome was secondary to a diabetic nephropathy in 26 cases [74%] and to a non-diabetic nephropathy in 9 cases [26%]. The mean follow-up was 4 years for 22 patients. Evolution was marked by a rapid decline of the renal function in 14 cases [63.6%]. Factors of bad prognosis seem to be the following: male sex [11 cases out of 14; 78,5%], type 1 diabetes [10 cases out of 14; 71,4%], poorly controlled hypertension [10 cases on 12; 83,3%], smoking [5 cases out of 14; 35,7%], hyperlipidemia [11 cases out of 14; 78,5%] and nephropathy caused by the diabetic condition [11 cases out of 14; 78,5%]. Control of these risk factors is thus important to prevent the progression of nephrotic syndrome

[Central neurocytoma: a study of five cases]

Central neurocytoma is a very rare brain tumour recently described. The diagnosis rests on the data provided by anatomopathological examination and immunohistochemestry. We report a retrospective study of five cases of neurocytoma diagnosed in the Pathological laboratories of Anatomy and Cytology of Habib Bourguiba and Fattouma Bourguiba Hospitals over a period of two years [1999/2000]; this study was based on clinical, radiological and anatomopathological data. It was about three women and two men with an average age of 29 years [17 to 49 years] The tumor was located in the side ventricles in three cases. Two differential diagnoses were often discussed: oligodendroglioma and ependymoma. Immunohistochemistry showed an expression of the synaptophysine in every case. The treatment was exclusively surgical and the course was favorable with an average follow up of 48 months; only one case of relapse was noted of 36 months after the diagnosis

[Peripheral hypothyroidism and Cushing disease]

The presence of thyroid disorders in Gushing's syndrome is very common. These abnormalities demonstrate the effect of the cortisol on the different levels of hypothalamo-hypophyso-thyroid axis'. However, the association between primary hypothyroidism and Cushing s' syndrome is more rare. We report the case of a 55 year-old woman with a Gushing's disease because of pituitary macroadenoma. A primal]] hypothyroidism was diagnosed 8 years before in this patient because of obvious clinical features and was confirmed by decreased thyroid hormones levels and moderate TSH increase. Hypercortisolism induces a decrease in thyroid hormones, and changes in TSH wih less reactivity to TRH Suppression of hypercortisolism is associated with a normalisation of these functionnal thyroid disorders but also leads to an increase in autoimmune thyroiditis