Retinoblastoma is the most common malignant intraocular tumor in children under 5 years old. Survival rates, ocular and visual preservation are major challenges in this disease. This study aims to determine the epidemio-clinical, therapeutic and prognostic profile of patients with retinoblastoma who have been treated in the pediatric hemato-oncology department of the university Hospital Hassan II of Fez. It is a retrospective and descriptive study of all children followed for retinoblastoma at the pediatric hemato-oncology department, over a period of 11 years and 10 months from January 1st, 2012 to October 31, 2023. During this period, we collected 65 cases. The mean age at diagnosis is 25 months and the ratio is 0.9. The mean time to diagnosis is 5 months. Symptoms are dominated by leukocoria (84.2% of cases), followed by exophthalmia (15.8% of cases) and sarbismus (13,2% of cases). The retinoblastoma is unilateral in 70.7% of cases and bilateral in 29,3% of cases. Enucleation was indicated in 97% of cases. The majority of patients were treated by the combination of chemotherapy and surgery (69.2% of cases). Surgery only was indicated in 27,7% of cases and conservative treatment in 26,1% of cases. Radiotherapy was indicated in 9,5% of cases. The overall survival rate at the end of treatment is 75.4% and the five-year survival rate is 60%. The prognosis of retinoblastoma is generally good. In our context, most cases are still diagnosed at advanced stages of the disease, which considerably reduce the overall survival rate and the preservation of the eye and the vision.
Glanzmann thrombasthenia is a rare hereditary thrombopathy, mainly responsible for spontaneous mucocutaneous hemorrhages. It is due to a qualitative or quantitative deficiency of the platelet receptor GPIIbIIIa, involved in platelet aggregation. This disease is mostly observed in populations with high consanguinity. The aim of our work is to study the epidemiological, clinical, biological, therapeutic and evolutionary particularities of Glanzmann thrombasthenia in children. A total of 11 patients were diagnosed and followed up in the pediatric hematology-oncology department of the Hassan II University Hospital of Fez, Morocco. The average age at diagnosis was 2 years and 6 months, 81% of the cases were from consanguineous parents, mucocutaneous hemorrhages were the most reported, and the diagnosis of Glanzmann thrombasthenia was confirmed in all cases by aggregometry. Treatment is based mainly on local symptomatic measures and platelet transfusion, activated factor VII is reserved in case of anti-platelet immunization. The evolution is good in all cases, only one death is reported.
Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare idiopathic disorder with unknown etiology. The présentation of RDD is painless bilateral cervical lymphadenopathy. Extranodale RDD without nodale involvement is very rare. Isolated form to the bone occurs in only 2% of patients. We report a rare case of RDD revealed by left radius involvement in an infant aged 21 months with lung involvement in the assessment of extension, treated with chemotherapy with good outcome. Bone involvement is rare, hence the interest in knowing the different etiological diagnoses in order to make the rapid diagnosis and start the appropriate treatment.