[Radiotherapy in the treatment of retinoblastoma: about 40 cases]. / Place de la radiothérapie dans le traitement du rétinoblastome : à propos de 40 cas.

PURPOSE: The aim of this study is to analyze the results and the complications of radiotherapy in the treatment of retinoblastoma. PATIENTS AND METHODS: Between 1994 and 2004, 40 children received radiotherapy for a retinoblastoma in Salah Azaiz Institute. The average age of the patients was 36 months (four to 132 months). There were 16 girls and 24 boys. Sixteen children presented a bilateral disease and 24 children a unilateral disease. Twenty eyes and thirty-six orbital cavities in 40 children with retinoblastoma were treated by radiotherapy. One child with a unilateral anterior retinoblastoma was treated with 106 ruthenium brachytherapy. External radiotherapy has been used to treat the 39 patients. In 20 cases the irradiation was conservative and in 36 cases postoperatively. The latter (n=36) presented at least one risk factor of relapses noted in the histological examination. The average dose was 44 Gy (1.8 to 2 Gy per fraction, five fractions weekly). This radiotherapy was associated with chemotherapy in 24 cases. RESULTS: Thirty-five children were followed with an average follow-up of 53 months (3-108 months). The average delay of relapses was of 10 months (two to 26 months). We found four orbital relapses and seven metastasis in nine children. The conservation of the eye with a useful visual field was noted in 18 cases among the 20 conservative irradiated eyes. The major therapeutic complication was the growth defect of the bones face. A femoral bone sarcoma was noted five years after the end of the irradiation and chemotherapy in one case. CONCLUSION: If the radiotherapy offers the advantage of the functional conservation and the improvement of the local control, its indications are more and more restricted in favor of the other therapeutic methods (chemotherapy, thermochemotherapy) and this considering the iatrogene risk. The development of new techniques of brachytherapy and the progresses of the conformational radiotherapy appear to reduce considerably this risk.

[Frontal relapse of medulloblastoma. Causes and consequences (a case report)]. / Rechute frontale du médulloblastome. Causes et conséquences (à propos d'un cas).

Medulloblastoma is a common paediatric brain tumor. Its prognosis is improved since the use of radiotherapy after surgery. This radiotherapy, so widely and complex, has one purpose: tumor control with less toxicity. Frontal relapse of medulloblastoma is not rare. Two hypothesis were proposed to explain this failure pattern: a geographic miss in cribriform plate due to targeting deviation and the migration of tumor cells to the cribriform plate caused by the "face down position" of the patient during surgery. We report the case of a 10-year-old boy who was treated in 1998 for a medulloblastoma with surgery and radiotherapy of the craniospinal axis (24 Gy) and the posterior fossa (54 Gy). Four years later, tumor relapsed only in the frontal area. A new surgery and chemotherapy were used with a complete response. A second frontal relapse associated with posterior fossa recurrence was detected after one year of the second treatment and treated by chemotherapy. Frontal relapse in medulloblastoma is an avoidable failure pattern when surgical and radiotherapeutic procedures are well controlled.

[Merkel cell carcinoma: outcome and role of radiotherapy]. / Carcinome à cellules de Merkel : prise en charge et place de la radiothérapie.

Merkel cell carcinoma (MCC) are rare neuroendocrine malignant tumor of the skin, occurring in elderly patients. It affects primarily the sun-exposed areas of the skin, with approximately 50% of all tumors occurring in the face and neck and 40% in the extremities. Immunohistochemical markers (CK20+, CK7- and TTF1-) are used to distinguish between MCC and other tumors. MCC have a tendency to rapid local progression, frequent spread to regional lymph nodes and distant metastases. Due to the rarity of the disease, the optimal treatment has not been fully defined. Localized stages (stages I and II) are treated by surgical excision of the primary tumor (with 2 to 3 cm margin) and lymphadenectomy in case of node-positive disease, followed by external beam radiotherapy (EBRT) to a total dose of 50 to 60Gy in the tumor bed. Adjuvant EBRT has been shown to decrease markedly locoregional recurrences and to increase survival in recent studies. Treatment of lymph nodes area is more controversial. Chemotherapy is recommended only for metastatic disease.

[Brain radionecrosis in patients irradiated for nasopharyngeal carcinoma: about nine cases]. / Radionécrose cérébrale chez les patients irradiés pour cancer du nasopharynx: à propos de neuf cas.

PURPOSE: To study the clinical, radiological, therapeutic and progressive aspects of brain radionecrosis after treatment for nasopharyngeal carcinoma. PATIENTS AND METHODS: Nine patients (seven men and two women) of mean age 47.7 years old (extremes: 18-57 years old) were treated for UCNT (undifferentiated carcinoma of the nasopharynx) between 1989 and 2003 and developed cerebral radionecrosis. All patients were treated with radical radiotherapy. The mean total dose was 73.5 Gy (70-75 Gy). Dose per fraction was 2 to 2.5 Gy, one fraction daily. One patient received adjuvant brachytherapy to the dose of 8 Gy and four patients also received chemotherapy. RESULTS: Brain radionecrosis was authenticated by brain imaging (CT scan+/-MRI): the imaging was ordered in seven cases to elucidate non-specific neurological signs and two cases were discovered fortuitously. The time to the appearance of neurological signs was 40.3 months (10 to 108 months). The localization was temporal in six cases, parieto-occipital (one case) and bulbomedullar (two cases). After a mean follow-up period of 30.6 months (12-84 months), clinical outcomes were favorable in all cases receiving medical treatment (corticoids), with a stabilization of the radiological lesions in eight cases and complete radiological regression in one patient. CONCLUSION: Brain radionecrosis is a late complication rarely occurring in patients irradiated for UCNT. Imaging techniques (CT scan but more so MRI) play a major role in the diagnosis. Corticotherapy resulted in a durable objective response in all patients and, in most cases, resulted in radiological stabilization.

[Primary non-Hodgkin lymphoma of the liver: case report and review of the literature]. / Lymphome non hodgkinien primitif du foie: à propos d'un cas et revue de la littérature.

Purpose was to describe the clinical, radiological and therapeutic features in primary liver lymphoma. We report the case of a 54-year-old patient, who is followed since the age of 20 years for neutropenia associated with mediastinal adenopathy. Systematical ultrasound find a mass of the left liver confirmed by Computed tomography (CT). Histological examination of laparoscopic liver biopsy specimens confirmed diffuse large-cell non-Hodgkin's lymphoma. The disease was confined to the liver without any evidence of extrahepatic involvement. The serology of Epstein Barr virus was highly positive. PET-scan show increased FDG uptake at the site of hepatic lesion and the mediastin. The patient received chemotherapy followed by radiation therapy of the left liver at the dose of 31 Gy. The patient was alive and free of disease 20 months after the diagnosis of primary liver lymphoma. The primary hepatic lymphoma is a rare malignancy, which classically affects 50-year-old patients with a male preponderance. The incidence is increased in immunosuppressed patients and some authors have suggested an association with hepatitis B or C infection, and with the Epstein Barr virus. The imaging studies including ultrasound, CT, magnetic resonance imaging (MRI) and now PET-scan help to establish the diagnosis and to the following. Treatment options are surgery, radiation, chemotherapy, or a combination.

[Ovarian irradiation in recurrent endometriosis]. / Irradiation ovarienne pour endométriose réfractaire inopérable.

We describe a case of a young woman with a history of an aplastic anaemia in which pelvic radiotherapy was used successfully in the management of a recurrent and inoperable endometriosis. The use of therapeutic pelvic or ovarian irradiation in endometriosis may be considered, when surgical and medical treatments have been exhausted and have failed.