[Pseudotumor phenotype of sarcoidosis: about two cases]. / Phénotype pseudotumoral de la sarcoïdose: à propos de deux cas.

INTRODUCTION: Sarcoidosis is a multisystem benign granulomatous disease of unknown etiology. It can sometimes cause diagnostic confusion by presenting in the form of a pseudotumor, thus constituting a trap for the unwary. COMMENTS: The authors report two cases of pseudotumor sarcoidosis. In the first, the pseudotumor occurred in the context of multisystem disease in a 48-year-old man. The response to treatment with systemic corticosteroids was complicated by the development of disseminated tuberculosis, which was rapidly fatal. The second case, by contrast, was about a 58-year-old woman and the disease was self-limiting, resolving spontaneously in less than 3 months. CONCLUSION: Through these two cases, the authors focus on the particularities of the pseudotumor manifestation of pulmonary sarcoidosis. The diagnosis is often difficult. The mismatch between the clinical picture and the extent of radiological lesions should, however, suggest the diagnosis. Histological evidence is needed to eliminate other etiologies including malignant tumors. The condition usually resolves either spontaneously or after treatment with systemic corticosteroids. However, relapses are possible, including on discontinuation of corticosteroid therapy.

[Mediastinal mature teratoma with spontaneous malignant transformation]. / Tératome médiastinal mature avec transformation maligne spontanée.

INTRODUCTION: Malignant teratomas can be either immature teratomas or they can develop after treatment of other malignant germ cell tumors with chemotherapy or radiotherapy. Spontaneous malignant transformation of mature teratomas is very rare. OBSERVATION: We report the case of a patient complaining of atypical chest pain. Chest plain radiography showed a right anterior mediastinal mass. Computed tomography revealed a heterogeneous mediastinal mass with fat areas and calcification. There were hilar and mediastinal lymph nodes as well as lung parenchymal nodules. CT-guided and then surgical biopsies confirmed the diagnosis of a mature teratoma, which had degenerated into a digestive adenocarcinoma. CONCLUSION: Spontaneous malignant transformation of mature mediastinal teratoma is very rare. The diagnosis of malignant transformation is based on radiological examinations, laboratory tests and histological studies.

[Increased cardiovascular risk and premature atherosclerosis in rheumatoid arthritis]. / Augmentation du risque cardiovasculaire et accélération de l'athérosclérose au cours de la polyarthrite rhumatoïde.

UNLABELLED: Patients with rheumatoid arthritis (RA) are at increased risk of mortality compared with the general population. Evidence suggests that this increased mortality can largely be attributed to increased cardiovascular (CV) death. In a prospective study, 34 patients with RA were compared with age- and sex-matched controls. RESULTS: We found a lower C-HDL, apolipoprotein A1 and B in patients with RA. However, CT/C-HDL and C-LDL/C-HDL were significantly higher than control patients. The intima-media thickness was significantly higher in patients with RA (0.759 mm vs 0.558 mm; P<0.001). CONCLUSION: Increased attention to cardiovascular risk in RA will be necessary to reduce the excess CV mortality and morbidity in RA patients. It appears that the excess risk that is observed in the RA population can be explained, in part, by promotion of CV disease through increased systemic inflammation associated with RA.

[MR imaging features of tuberculosis of the sellar region]. / Aspects IRM de la tuberculose de la région sellaire.

PURPOSE: Tuberculosis of the sellar region, especially the pituitary gland, is rare. The purpose of this article is to demonstrate through a review of five clinical cases the value of imaging, especially MR imaging, in the evaluation of this pathology. PATIENTS AND METHODS: CT and MRI of the brain were obtained in all cases along with a chest radiograph. RESULTS: Four patterns were detected on MRI: pituitary tuberculoma mimicking adenoma; pituitary abscess, extending to the cavernous sinus in one case and associated with infundibulum thickening in another; hypophysitis with suprasellar extension in association with tuberculous meningoencephalitis; and infundibular thickening associated with tuberculous meningoencephalitis. Diagnosis was based on biopsy in two cases and combination of imaging and clinical data in three cases. Outcome was favorable with anti-tuberculosis drugs. CONCLUSION: Irrespective of the imaging features, a history of travel to an endemic region combined to other findings such as infundibular thickening should raise concern for the possibility of tuberculosis even in the absence of signs of systemic infection.

[Central neurocytoma: three case reports]. / Neurocytome central: à propos de trois observations.

Central neurocytoma is classically recognized as an intraventricular benign brain tumour. Extraventricular localisation is rarer. We report two intraventricular neurocytoma and another bulbar extraventricular neurocytoma. Histologically, central neurocytoma presents remarkable likeness characteristics to oligodendroglioma, but immunohistochemical study distinguishes this tumour. Imaging appearances (CT, MRI) raise the diagnosis and immunohistochemical study confirm it. The purpose of our work is to assess the value of imaging (CT, MRI) in the diagnosis of central neurocytoma.

[Melanotic schwannoma of the temporozygomatic region]. / Schwannome mélanocytique de la région temporozygomatique.

INTRODUCTION: Melanotic schwannoma is a rare benign tumor, which arises from nerve sheath cells. Melanotic differentiation is its most histologic characteristic. CASE REPORT: We report a case of melanotic schwannoma arising in the temporozygomatic region for a man of 38-year-old. Outcome was favourable after tumor resection. DISCUSSION: This benign neoplasm occurs pronominally in spinal nerve roots. Craniofacial locations are rare. Most melanotic schwannomas are slow growing tumors, but prognosis can be poor because of local recurrence or malign behavior, especially when multiple lesions are present and/or involve the Carney complex.

[MRI pituitary stalk abnormalities: etiology aspects in 11 patients]. / Anomalies de la tige pituitaire à l'IRM: aspects étiologiques à propos de 11 cas.

Newer techniques of magnetic resonance imaging (MRI) describe more accurately pituitary stalk abnormalities such as infections, infiltrative lesions and tumors. In absence of all the above mentioned etiological factors, genetics defects are suspected, mainly when other malformations are equally present. We attempt to show through 11 observations the variability of pathologies involving the pituitary stalk with their respective clinical and radiological features and associated endocrine abnormalities. This is a retrospective study of 7 men (67%) and 4 women (33%), mean age of 28 year (range: 15 to 53) in whom pituitary MRI was performed for hypopituitarism, diabetes insipidus or hyperprolactinemia. Three patients had brain MRI for an extra-pituitary condition. The pituitary MRI showed a stalk section in 3 cases (27%), atrophy in 1 case and thickening in 7 cases (67%). The pituitary stalk anomaly was associated with hyperprolactinemia in 3 cases (27%), central diabetes insipidus in 4 cases (36%), growth hormone deficiency in 4 cases (36%), adrenal insufficiency in 5 cases (45%), hypogonadism in 5 cases (45%) and hypothyroidism in one case (9%). Established diagnoses were: sellar metastasis in 2 cases (18%), Langerhans' histocytosis, tuberculosis and autoimmune hypophysitis respectively in 3 cases (9%). In 6 cases (54%), no clear etiology was found. Given the multitude of pituitary stalk pathologies, a detailed etiologic inquiry must be performed in order to detect elements able to reclassify an initially idiopathic disorder.

[Massive hemoptysis: experience of a surgical unit]. / Hémoptysies massives: expérience d'une unité chirurgicale.

Massive hemoptysis is a clinical entity characterized by its unpredictable and potential lethal course. We studied retrospectively the observations of 25 patients hospitalized in our surgical unit. We collected all the demographic, clinical and surgical data. A male predominance with a sex-ratio of 2,1 was noted. The average age was 45 years, with extremes of 17 and 75. The dominant cause was bronchiectasis. Twenty patients were operated on. The surgery consisted of a pulmonary resection in 9 cases (45%), an atypical lung resection in 4 cases (20%), a resection of an aspergilloma in 2 cases (10%), a kystectomy of hydatic cyst in 4 cases (20%) and one arterial ligature (5%). Five patients (25%) had emergent surgery, and 15 (75%) delayed surgery. Hospital mortality was 20% in the first group and 6.66% in the second. No recurrence of bleeding was observed after an average follow-up of 7 months. Surgery remains a therapy of choice for massive hemopysis. It must as far as possible be avoided during active bleeding.

[Breast cancer in young women in the south of Tunisia]. / Le cancer du sein de la femme jeune dans le sud tunisien.

OBJECTIVE: The objective of this retrospective study was to discuss the epidemioclinical criteria, the therapeutic results and the prognostic factors of breast cancer in young women throughout a comparative study of 72 young patients aged less than 35 years and a second group of older premenopausal patients aged between 36 and 50 years. PATIENTS AND METHODS: We reviewed the epidemioclinical records of all the patients. Non-metastatic and operable patients were treated with surgery (conservative or radical) followed by an adjuvant treatment (chemotherapy, radiotherapy, endocrine therapy) indicated according to the prognostic factors. Locally advanced or metastatic tumors were treated with chemotherapy. Overall survival was calculated according to the Kaplan-Meier method. The comparison of survival curves was performed according to log-rank test. The multivariate analysis was performed according to the Cox model. RESULTS: The mean age was of 31.5 years. T2N1, node positive (N+), high grade (SBRII and III) and endocrine non-responsive tumors were the most frequent. There was no difference with the second group of older patients regarding the risk factors and the clinical criteria but mammography was more sensitive in the second group. The 5 years overall survival of young patients was of 57% and pejorative prognostic factors in univariate analysis were: tumor size, N+ and endocrine non-responsiveness. There were not any significant prognostic factors at the multivariate analysis. Young age less than 35 years was not a prognostic factor influencing overall survival in the totality of patients or in the different sub-groups according to the other prognostic factors. CONCLUSION: Clinical presentation and outcome of breast cancer in our young patients aged under 35 years seems not to be different from that in older patients. The conclusions of the different authors are controversial but the majority has reported more advanced tumors with worse prognostic than those of older patients.

[Clinical case of the month. Iliac artery endofibrosis in a soccer player]. / Le cas clinique du mois. Endofibrose artérielle iliaque chez un footballeur.

Arterial endofibrosis is a rare disease, usually affecting the external iliac artery in highly trained athletes. We report a case of external iliac endofibrosis in 42-year-old top level athlete. Clinical consequences were a high intensity exercise related sensation of swollen thigh, with normal clinical examination. Ultra sound investigation showed fibrotic thickening of the left external iliac artery. Angiography confirmed stenosis of the left external iliac artery. The patient was treated surgically, he underwent remodeling of his left external iliac artery with venous angioplasty. No complications occurred in the post operative course, and the patient return to training after three months.

[Perrault's syndrome: two cases]. / Syndrome de Perrault.

Perrault's syndrome is an autosomal recessive ovarian dysgenesis associated with sensorineural deafness. We report two cases in sisters issuing from consanguinous parents. Aged 16 and 21 years, both patients present the two cardinal symptoms of the syndrome. Magnetic resonance imaging in the second sister showed high intensity signals in the periventricular and subcortical white substance and in the central ovale, suggestive of cerebral leucodystrophy. This element may be one of a wide spectrum of neurological symptoms found in Perrault's syndrome. The discovery of the causal genes may allow better understanding of the biomolecular mechanisms involved in gonad and sensorineural differentiation.