An orbital perivascular epithelioid cell tumor (PEComa) in a 9-year-old boy: Case report and review of the literature.

Perivascular epithelioid cell tumors (PEComas) are a family of benign neoplasms characterized by smooth muscle and melanocytic differentiation. Orbital cases are rare. A 9-year-old male presented with a slowly growing orbital mass. Magnetic resonance imaging (MRI) revealed a well-defined orbital mass without intracranial extension. The microscopic appearance of the complete resection specimen showed large nests of epithelioid cells with wide cytoplasm containing melanin pigment and round to oval nuclei with mild cytonuclear atypia and low mitotic activity. Immunohistochemistry was positive for HMB45 and negative for melanA, smooth muscle actin, desmin and S-100 protein. Pangenomic RNA-sequencing identified an in-frame NONO-TFE3 rearrangement, and clustering data showed that the tumor's gene expression profile was grouped with other previously studied PEComas. A diagnosis of orbital pigmented PEComa with uncertain malignant potential associated with a NONO-TFE3 rearrangement was made. There was no recurrence after 1 year of follow-up.

Massed versus distributed wordprocessor training.

Two groups of subjects were trained on 12 basic word processing (WP) functions. One group was trained in a 'massed' 60-minute session and the other on a 'distributed' schedule of 60 min in two sessions separated by a ten-minute break. Subjects were tested on speed and accuracy immediately after training, and one week later to measure retention. It was found that the distributed training group performed significantly faster and more accurately than the massed group. This result was obtained at the end of the training period and also on the retention test. The results strongly indicate that a session of 60 min is too long for efficient WP training.

[Transient painful lumbar blocks]. / Les à-coups douloureux bloquants lombaires.

Recurrent painful locking of the lower back is suggested as an appropriate term for designating sudden episodes of jabbing low back pain with locking of the spine occurring in the absence of unusual exertion, resolving within one or two minutes, and recurring many times over several weeks or months. This retrospective study included 20 outpatients classified as having recurrent painful locking of the lower back between 1978 and 1992. Inclusion criteria were availability of roentgenograms taken at the time of the acute symptoms and a follow-up of at least two years. Two typical cases are reported with diagrams to show the timing of recurrent painful locking of the lower back with respects to other low back symptoms. Mean age at onset was 56 years. Seventy per cent of patients were females. A 10-year to 30 year history of lumbago was found in every case. Recurrent painful locking of the lower back occurred substantially later than lumbago suggesting that it denoted more advanced damage to the lumbar spine. Pathophysiologic hypotheses are discussed: the causative lesion may be a mobile flap at the posterior aspect of the anulus. In most patients, the symptoms resolved within six months.

[Uterine sarcoma in Tunisia: retrospective study about 14 cases]. / Les sarcomes utérins en Tunisie : étude rétrospective à propos de 14 cas.

OBJECTIVE: To report epidemiologic, anatomoclinical treatment and results about a Tunisian retrospective serie of 14 patients with uterine sarcoma (US). PATIENTS AND METHODS: A retrospective study of 14 cases of uterine sarcoma treated in the Radiotherapy Unit of Farhat Hached Hospital of Sousse between 1995 and 2008. Epidemiologic and anatomoclinical features were assessed. A complete work-up including abdominal ultrasonography and abdominopelvic CT scan were perfomed in 7 and 10 cases, respectively. RESULTS: The median age was 48.5 years (15 to 78) without previous medical history of irradiation or prolonged drug exposition. There were 3 cases of leimyosarcoma, 7 cases of mixed Mullerian tumor and 4 cases of endometrial stromal sarcoma. The diagnosis was made postoperatively in 6 patients and after curettage in 8 cases. According to the classification of the International Federation of Gynecology and Obstetrics (FIGO), 8 patients were in FIGO stage I, 3 in stage II and 3 in stage III. Hysterectomy was associated with annexectomy in 12 cases. Bilateral pelvic lymphadenectomy was performed in one patient. Eight of our 14 patients underwent postoperative pelvic radiotherapy, associated with adjuvant chemotherapy in 3 cases. The evolution was marked by the occurrence of local recurrence in 4 patients who did not have adjuvant therapy with lung metastases in one case and peritoneal carcinomatosis in another case. With a medium follow-up from 54 months, 7 patients are free from disease, 5 died of their disease (after a mean of 24 months) and 2 patients were lost to follow-up. DISCUSSION AND CONCLUSION: Uterine sarcomas are rare tumors with poor prognosis. Adjuvant therapy (radiotherapy and/or chemotherapy should be discussed due to the high risk of recurrence or metastases.

[Breast cancer in woman younger than 35 years in Tunisia: retrospective study about 124 cases]. / Cancer du sein chez la femme jeune de moins de 35 ans en Tunisie: étude rétrospective à propos de 124 cas.

OBJECTIVES: Our aim was to report the epidemiological and clinical characteristics of breast cancer in young women and to evaluate the therapeutic results in the central part of Tunisia. PATIENTS AND METHODS: We report the results of a retrospective study including 124 patients under 35years old treated for breast cancer between 1995 and 2007 in the Radiotherapy Department of CHU Farhat Hached, Sousse. RESULTS: The mean age of our patients was 31.3years. T2N0, node positive (N+), high grade (SBRII and III) and endocrine responsive tumors were the most frequent. Fourteen patients had metastatic disease. One hundred and fifteen patients underwent a surgical treatment, which was conservative in 35 cases and radical for the others followed by radiotherapy on 114 patients. Chemotherapy, especially based on the FEC protocol, was administrated to 89% of all patients. Forty-five patients received also hormonotherapy. After a median follow-up of 48.5months, 51 patients remained free of disease. The 5years overall survival was 67.7%, the 5years free disease survival was 58.2%. DISCUSSION AND CONCLUSION: The 5years overall survival of breast cancer in young women varies between 55 and 75%, it was 67.7% on our study. These results can be improved by early diagnosis, adapted treatment and nowadays with targeted therapies.

La radiothérapie postopératoire du cancer du larynx: Étude clinique et résultats thérapeutiques à propos de 164 cas

But:Étudier les caractéristiques épidémiologiques, cliniques et anatomopathologiques, ainsi que les résultats thérapeutiques des cancers laryngés traités par radiothérapie postopératoire.Méthodes: Une étude rétrospective a été menée entre Janvier 1995 et Décembre 2010 colligeant 164 patients atteints d'un cancer laryngé traités à Sousse (Tunisie).Résultats :L'âge médian était de 60 ans. La prédominance masculine était marquée avec un sex- ratio de 26,4. Il s'agissait d'un carcinome épidermoïde dans 98,2%. Tous les patients ont eu une laryngectomie totale ou partielle associée à un curage cervical uni ou bilatéral. Tous les patients ont eu une radiothérapie postopératoire. La dose totale délivrée variait de 50 à 74 Gy avec une fraction de 1,8- 2 Gy/ séance. Avec un recul moyen de 55 mois, 49,4 % des patients étaient en vie en situation de rémission complète. Les probabilités de survie globale à trois, cinq et dix ans étaient respectivement de 77 % et 73 %, 69%. Les facteurs pronostiques de survie globale et de survies sans récidive locorégionale retenus, en analyse uni-variée, étaient : La radicalité du curage ganglionnaire, l'atteinte ganglionnaire histologique et l'effraction capsulaire.Conclusion :Le cancer du larynx est un cancer fréquent occupant la première place des cancers des voies aéro-digestives chez l'homme en Tunisie. Notre étude présume que l'absence d'atteinte ganglionnaire, la sélectivité de l'évidement ganglionnaire et l'absence d'effraction capsulaire sont des facteurs pronostic favorables de survie globale et de contrôle local