[The Prize of the President of the Republic of Tunisia for Inter-Maghreb Medical Societies].
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Biomedical subjects
Publications and source records attributed to S Haouet.
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We report a case of minimal deviation adenocarcinoma of the uterine cervix in a 32 year old women who present a Peutz Jeghers syndrome. This patient also had fibroadenoma of the breast and dystrophic mastopathy. This case represent an example of predisposition to developing tumors of the Peutz Jeghers syndrome.
Paragangliomas are unusual neuroendocrine tumors. The most common anatomical site is within head and neck (90%). It has rarely been described in the region of the cauda equina. We report the case of a 49 years-old white woman who presented with a slow, progressive cauda equina syndrome over a 20-years period. Radiological examination showed erosion of the vertebral laminae of L4, L5 and S1 and disclosed an intra-dural mass lesion occupying the entire spinal canal between L4 and S1. The patient underwent sub-total excision and adjuvant radiation therapy. Histological examination concluded to a paraganglioma. This diagnosis was confirmed by immunohistochemical studies.
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BACKGROUND: Vulvar involvement in Crohn's disease is uncommon. The elementary lesion is usually an ulceration: 44 cases have been reported in the literature. In 20 p. 100 of the cases, vulvar involvement is the only manifestation of the disease. We report 3 new cases of vulvar lesions in Crohn's disease. CASE REPORTS: The first case had vulvar lesions which complicated ileocolic Crohn's disease, in the other two cases there were no digestive manifestations. One patient developed voluminous edema of the right labium. The diagnosis of Crohn's disease of the vulva was suggested by the characteristic lesions, presence of epithelioid and giant cell granuloma at histology examination of the vulva specimen and finally on rapidly favorable course after treatment with sulfasalazine. The ulcers regressed with medical treatment but remission was temporary with recurrence at treatment withdrawal. DISCUSSION: These cases underline the difficulties encountered in establishing the diagnosis of Crohn's disease in patients who develop vulvar ulcerations alone with no signs of digestive disease and emphasize the difficult problem of long-term control.
A 56-year-old patient with an uneventful history was hospitalized for investigation of recent dypnea. The physical examination was normal and chest X-ray showed localized interstitial lesions in the upper right lobe. Endoscopy showed evidence of diffuse infiltration of the bronchial mucosa and narrowing of the lumen. Transbronchial histology showed evidence of bronchopulmonary amyloidosis. There were no signs of extrapulmonary localizations. Search in other organs was negative demonstrating the primary nature of the lung disease. Primary bronchopulmonary amyloidosis is rarely encountered.
INTRODUCTION: Pemphigus frequently seen in Tunisia has specific characteristics distinguishing it from the European or American forms. Between 1974 and 1992, we observed 70 cases in the dermatology unit of the La Rabta University Hospital in Tunis. Most patients were young (mean age 39 yr), predominantly women (80%). Deep pemphigus (pemphigus vulgaris and vegetans) occurred in 56% and superficial pemphigus (seborrheic and foliaceus) in 44%. High-dose corticosteroids were given as initial treatment (1.4 mg/kg/day). Infectious complications were frequent. Mortality was 14%. Pemphigus in Europe and North America usually occurs in adults in the 50-60 year age range. Superficial forms are rare (2-10%). In Tunisia, pemphigus is similar to Brazilian pemphigus with high frequency and predominant superficial forms (pemphigus foliaceus).
The synovial lipoma are uncommon articular tumors of unknown origin. We report two cases of synovial lipoma arborescens arisen in a man and a woman respectively 41 and 27 years old. Both had already consulted for a tumefaction progressively increasing in volume. It was accompanied in the first case by a laxity in the knee joint. Radiography and arthroscopy showed an important hyperplasia of the articular synovia. A total synovectomy was performed in both cases. The synovia had a hairy aspect and was extremely thickened. It weighted 1.5 kg in the first case and its section had a fatty aspect. The histological examination confirmed the diagnosis of synovial lipoma arborescens. The observed aspect of the two tumors and particularly their volume, which was very important in the first case are arguments in favor of their tumoral nature.
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Tumors of ceruminous glands, called "ceruminoma" are rare. They arise from the ceruminous glands of the external auditory canal. They realise a group of glandular tumors diversified in histologic pattern and prognosis. Three patients with ceruminous gland tumors are reported: one case of ceruminous adenoma, one case of adenocarcinoma and adenoid cystic carcinoma in the third one.
Two cases of aggressive angiomyxoma of the pelvis and perineum are described; the first occurred in 32 year old woman who had a vulvar mass, the second in a 78 year old man who presented inguinal hernia. This recently described entity is characterized by its microscopic appearance (low cellularity, loose myxoid stroma and prominent vascular component), and propensity to local aggressivity, and common recurrence after surgery.
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INTRODUCTION: Keratoacanthoma centrifugum marginatum (KCM) is a rare keratoacanthoma. Treatment relies essentially on exeresis which is however difficult due to the widespread nature of the lesions. CASE REPORT: A patient with a 1 year history of KCM associated with psoriasis vulgaris was successfully treated with etretinate (1 mg/kg/day) for 5 months. This medical therapy made it possible to avoid the classical surgery and to blanch the associated psoriasis. COMMENTS: Several authors have shown the favourable effect of etretinate on multiple or large single-site keratoacanthomas. To our knowledge, this is the first case reported of successful treatment with etretinate in a case of keratoacanthoma associated with psoriasis. We suggest that this drug could be an effective alternative to mutilating surgery for this specific variety of keratoacanthoma.
Inflammatory fibroid polyp is a rare benign tumor of the digestive tract. The authors report three cases: two intestinal and one gastric. These cases are of particular interest because of the association ill one case of an intestinal submucosal lipoma with the inflammatory fibroid polyp, and the existence in the gastric case of an inflammatory reaction caused by food debris. This offers, further evidence in support of the theory implicating a chronic inflammatory reaction.
Collagenous enterocolitis is a recent entity of which only two cases have been published. The authors report the association of collagenous colitis and collagenous sprue in a 67-year-old woman. The diagnosis of collagenous enterocolitis is based on the presence of a duodenal collagen deposit with partial villous atrophy associated with collagenous colitis. The symptom appeared one month after non-steroidal antiinflammatory treatment. The authors discuss the role of drug toxicity in the pathogenesis of collagenous enterocolitis.
Extra-intestinal manifestations of Crohn's disease are well described. A very uncommon gynaecologic manifestation of this disease is vulvar involvement. Till now, sixteen cases have been reported in the literature. We report a case of a pregnant woman with isolated vulvar involvement, without bowel manifestations. The diagnosis have been clinically uncertain, but the histopathology goes with Crohn's disease. Discussion point out the difficulties in diagnosis and management of this vulvar condition, and of its association with pregnancy.
The authors report a new case of cerebral malignant rhabdoid tumour in an eight year old girl. The diagnosis was confirmed on immunohistochemical and ultrastructural examinations showing cytoplasmic perinuclear inclusions composed of whorled filaments of vimentin. Base on a review of six cases reported in the literature, they analyse the clinical, histological, immunohistochemical and ultrastructural features of this exceptional central nervous system neoplasm. Different histogenetic hypotheses are discussed.