[Nephroblastoma and xeroderma pigmentosum: A rare association]. / Xeroderma pigmentosum et néphroblastome : une association rare.

INTRODUCTION: Xeroderma pigmentosum (XP) is a rare, genetically heterogeneous, autosomal recessive disorder, more common in cases of consanguinity. The basic defect underlying the clinical manifestations is a nucleotide excision repair defect leading to the defective repair of DNA damaged by ultraviolet (UV) radiation. XP is characterized by a high incidence of skin cancer on exposed regions. CASE REPORT: We report the case of a 5-year-old boy, followed for xeroderma pigmentosum since the age of 4 years. His sister also has the same anomaly. He presented an abdominal mass revealed by abdominal pain and vomiting. Radiological examinations revealed a nephroblastoma with lung metastases. He received primary chemotherapy for six cycles (vincristine, and actinomycin-d adriamycin), then surgery with ureteronephrectomy. Pathological examination of the nephrectomy specimen confirmed the diagnosis of Wilms tumor with a diffuse anaplastic component reaching 50%. The patient was treated according to the GFAOP stage III protocol, with high histological risk. The outcome was favorable but complicated by renal failure due to the toxicity of the treatment. He is currently in complete remission at 1 year from the end of treatment. CONCLUSION: The association of xeroderma pigmentosum and nephroblastoma is a rare combination. This case illustrates the problem of management of both severe and difficult conditions.

The prioritisation of paediatrics and palliative care in cancer control plans in Africa.

BACKGROUND: Given the burden of childhood cancer and palliative care need in Africa, this paper investigated the paediatric and palliative care elements in cancer control plans. METHODS: We conducted a comparative content analysis of accessible national cancer control plans in Africa, using a health systems perspective attentive to context, development, scope, and monitoring/evaluation. Burden estimates were derived from World Bank, World Health Organisation, and Worldwide Palliative Care Alliance. RESULTS: Eighteen national plans and one Africa-wide plan (10 English, 9 French) were accessible, representing 9 low-, 4 lower-middle-, and 5 upper-middle-income settings. Ten plans discussed cancer control in the context of noncommunicable diseases. Paediatric cancer was mentioned in 7 national plans, representing 5127 children, or 13% of the estimated continental burden for children aged 0-14 years. Palliative care needs were recognised in 11 national plans, representing 157 490 children, or 24% of the estimated Africa-wide burden for children aged 0-14 years; four plans specified paediatric palliative needs. Palliative care was itemised in four budgets. Sample indicators and equity measures were identified, including those highlighting contextual needs for treatment access and completion. CONCLUSIONS: Recognising explicit strategies and funding for paediatric and palliative services may guide prioritised cancer control efforts in resource-limited settings.

[Retinoblastoma: preliminary results of national treatment protocol at Casablanca university medical center]. / Rétinoblastome : résultats préliminaires du protocole national de prise en charge au CHU de Casablanca.

Retinoblastoma is the most frequent intraocular cancer, affecting almost exclusively children. We report prospective study results assessing the national protocol for retinoblastoma treatment in Morocco. Treatment included, depending on stage and laterality, primary chemotherapy either to facilitate enucleation or to make conservative treatment possible, postoperative chemotherapy, enucleation and conservative treatments such as transpupillary thermotherapy, thermochemotherapy and cryotherapy. Radiation was used in a few cases. Close supervision was performed until the age of 5. The incidence of retinoblastoma within the study period was 18 new cases per year in our department. Observations of 32 children were included in the study: 18 unilateral retinoblastomas (56%) and 14 bilateral retinoblastomas (44%), for a total of 46 eyes. Leucocoria was the most frequent presenting symptom (69%). Buphthalmia or proptosis were present in 47% of cases. The stage of retinoblastoma was V/D or E (Reese-Elsworth/ABC) in 69.5% of cases. Enucleation was necessary for 28 eyes. Transpupillary thermotherapy or thermochemotherapy were used for 13 eyes (11 children) and cryotherapy for 13 eyes (10 children). After an average follow-up period of 52 months, among 32 children, 4 died and 2 abandoned treatment. Ocular salvage rate was 85.7% (12 eyes out of 14, among which 11 without radiation). Retinoblastoma is a genetic tumor, which occurs in two forms: sporadic, always unilateral, and hereditary, often bilateral. The latter is the most challenging case. Current treatment protocols rely primarily on chemotherapy and local treatments. The future is oriented toward purely local treatments such as intra-arterial chemotherapy and intraocular chemotherapy.

[Complementary medicine in cancer patients under treatment in Marrakech, Morocco: a prospective study]. / Les médecines alternatives et complémentaires chez les patients cancéreux en cours de traitement à Marrakech, Maroc : étude prospective.

Use of complementary and alternative medicine (CAM) is very frequent in cancer patients. The aims of this study were to analyze the frequency, the reasons of use of CAM in patients with a cancer treated in a Moroccan oncology department. We included in this study 400 patients. An anonymous questionnaire was proposed to patients during treatment. Over 384 analyzable questionnaires, 71% of patients were using CAM. The most frequent method was religious therapy (60%). The second one was herbal medicine (36%). The main reason for using CAM was reducing psychic pain in 53%, and boosting the immune system in 32%. Adverse effects were reported in 2% of cases. Only 5% of patients discussed CAM with their doctors. The cost of CAM was less than 100 Euros in 88% of cases. To optimize the patient-physician relationship and to avoid a propensity to unproved treatments, accurate and adequate communication is necessary.

[Managing pain in children with cancer in Africa: a survey from the French-African Pediatric Oncology group]. / Prise en charge de la douleur de l'enfant atteint de cancer en Afrique : état des lieux au sein du Groupe franco-africain d'oncologie pédiatrique.

INTRODUCTION: The French-African Pediatric Oncology group (GFAOP) has contributed to a significant improvement in the prognosis of pediatric cancers in Africa through the development of therapeutic protocols adapted to local conditions. In parallel, the development of supportive care such as pain management, is now a major priority of our group. OBJECTIVE: The main objective of this study was to evaluate the management of pain in children with cancer in sub-Saharan Africa. METHODOLOGY: A questionnaire was sent to both the doctor and nurse responsible for each of the 8 pilot units (PUs). RESULTS: All physicians and 2 nurses answered the questionnaire. Human resources (3 doctors and 4 nurses per PU) were modest compared to the size of the pediatric population (71 million) and the mean number of 102 cases of cancer/year per PU. Only six physicians had received specific training in pediatric oncology. Morphine was available in only 5 of the 8 PUs; major limitations for its prescription were mainly inconsistent availability (62.5%), fear of side effects (50%), and regulations (37.5%). During the curative phase, more than half of the parents had to buy analgesics when leaving the PU. Pain assessment relied mainly on clinical evaluation (8/8), but was generally concordant with parents' assessment. The majority of patients experienced pain at diagnosis and at a higher degree at relapse, cancer being the predominant etiology. Pain related to other causes such as medical procedures, mucositis, and neuropathic pain was detected; however, its management remained inadequate. Only 2 of 8 PUs had a written protocol for pain management. These deficiencies can be explained by limited resources, both human and financial, but also by cultural factors such as endurance against pain still advocated by many African traditions. CONCLUSION: In recent years, pain prevention and relief, whatever its origin, have become a priority for all medical teams, especially for children with cancer. With its profound sociocultural changes, Africa is no exception. All teams in our group are aware of the situation and hope for major improvement in pain management in the near future through increased resources and training.

[Retinoblastoma: inventory in Mali and program to develop early diagnosis, treatments and rehabilitation]. / Le rétinoblastome: état des lieux au Mali et programme d'aide au diagnostic précoce, aux traitements et à la réhabilitation.

Retinoblastoma (RB) is a rare embryonic tumour that represents 1/16,000 births in France. In Mali, a study showed the characteristics of a hospital series of cases seen in Bamako in the Pediatric Oncology Unit of Gabriel Touré Teaching Hospital and in the Tropical Ophthalmology Institute of Africa (IOTA) between January 2005 and June 2007. Median age was 4 years versus 2 years in France for unilateral disease. Near two third of children with RB had extra-ocular extension at diagnosis, which is now exceptional in France. Only 11% were bilateral versus 35% in France. Cure rate was around 50%, but it is estimated only on the cases arriving in Bamako and with at least 20% lost of follow-up. Cure rate is over 95% in France within an exhaustive register. RB appears as an exemplary tumor and rapid improvements could be obtained in low-income countries with relatively limited means. This is why, the Alliance mondiale contre le cancer (AMCC), the Institut Curie in Paris, which is the reference center in France for RB, and teams in Bamako were proposing a program to help the development of early diagnosis, treatments, including eye preservation, and rehabilitation of children with RB in sub-Saharan Africa in collaboration with the Groupe franco-africain d'oncologie pédiatrique (GFAOP). The official launching of this program was held in Bamako November 4, 2011 for Mali and the surrounding regions. After this first experience, this program is now implemented in other countries.

Characterization of acute lymphoblastic leukemia subtypes in moroccan children.

We present the incidence and the immunologic characteristics of acute lymphoblastic leukemia (ALL) subsets in Moroccan children. We studied 279 unselected patients below the age of 18 years with newly diagnosed ALL. Cases were classified according to immunophenotype: 216 (77.42%) precursor B-cell phenotype (pB-cell), mature B-cell in 4 (1.43%), and T-cell in 59 (21.15%) cases. The subclassification using the CD10 antibody revealed 197 cases pB-ALL CD10+ (91.2%) and 9 cases T-ALL CD10+ (19.2%). The age distribution showed a peak in incidence between 3 and 5 years among the pB-cell ALLs subtype. There was a significantly higher frequency of males in the T-ALL subset (M/F ratio: 2.93 : 1) and more females in the T-ALL CD10+ subset when compared with the T-ALL CD10- subset. All tested pB-cell-lineage ALLs expressed CD19, CD79a, and surface CD22, terminal deoxynucleotidyl transferase (TdT) was detectable in 89.9% of cases, and cells in 74.1% of cases express CD34. All tested T-lineage ALL cells have surface CD7 and cytoplasmic CD3 (cCD3) antigens, CD5 was found in 98.2% cases, and 70.5% express TdT. CD1a, surface CD3 (sCD3), and CD4 are detected in more than 80% of cases; this frequency is higher than the 45% generally observed. Myeloid antigens occur more frequently and were expressed in 124 (57.4%) of pB-cell-ALL cases and 20 (33.9%) of T-cell ALL cases. Our results show that the distribution of ALLs in Moroccan children is similar with the general distribution pattern in developed countries except for the high frequency of T-ALL phenotype. The phenotypic profiles of our patients are close to those reported in literature for B-lineage ALLs; for the T-cell ALL subgroup, the blast cells express more CD1a, surface CD3, and CD4 while expressing less TdT. The high frequency of CD1a expression resulted in an excess of the common thymocyte subtype.

[Askin tumor: a case report with literature review]. / Tumeur d'Askin: à propos d'un cas avec revue de la littérature.

Askin tumor is a rare malignant tumor arising from soft tissues of the chest wall, rarely in the lung. It occurs predominantly in young adults. It still raises many questions about its individualisation and its links with Ewing's sarcoma. We report a case of Askin tumor in a 5-year-old child with reviewing the different data from the literature.

Present and future of hematology and stem cell transplantation in Morocco.

The first hematology department in Morocco was created in Casablanca in 1980 and it is still the only public facility where adult patients can be treated for hematological diseases. Two other units treat pediatric hematological disorders and cancer. Some patients are treated in private clinics located primarily in Casablanca and Rabat. The public hospitals have very limited resources and rely heavily on nongovernmental organizations (NGOs) for the care of these patients. The NGOs have also played an important role in developing successful cooperative programs with hospitals and groups in Europe and the United States. Till 2004, all patients who needed BMT and could afford the cost went to Europe. In 2004, SCT was initiated in Casablanca, with a four-bed ward set up and properly equipped. By May 2007, 27 patients have received autologous PBSCs. With 15 patients in continuous CR, the program is considered a success so far. The development of allogeneic transplantation in Morocco planned in the current year is also considered a real challenge.

[The fundal height measurements in single pregnancies and the detection of fetal growth retardation]. / La mesure de la hauteur utérine dans les grossesses uniques et le dépistage des retards de croissance intra-utérins.

Symphysis fundus measurements are a reliable mean for screening of fetal growth retardation. The techniques of the measurements, the curves of normality, and the efficiency of this method are discussed. A single formula is put forward to identify the patients witch can profit by extra ultrasound examinations and special clinical surveillance. In France, ultrasound examinations are scheduled at 22 and 32 weeks. Between 22 and 32 weeks, and after 32 weeks, symphysis fundus measurements are needed to suspect intra uterine fetal retardation, and, following suspicion, a new ultrasound examination is helpful.

[Treatment of childhood cancer in Africa. Action of the Franco-African childhood cancer group]. / Le traitement des cancers de l'enfant en Afrique. Travaux du groupe franco-africain d'oncologie pédiatrique.

The childhood cancer survival rate is currently 75% in industrialized countries. Rates in developing countries are much lower. The Franco-African Childhood Cancer Group (French acronym, GFAOP) was founded in 2000 with aim of reducing this unfavorable situation in Africa. The GFAOP has developed two forms of action. The main form consists of organizing two- to twelve-month training sessions for physicians and nurses in France and Morocco. The other form involves assessing the feasibility of modern treatment protocols for various cancers in Africa. The first feasibility trials were carried out on nephroblastoma and Burkitt's lymphoma in 12 pilot units in North Africa, West Africa, and Madagascar. In the first study from 2001 to 2005 we treated 306 cases of Burkitt's lymphoma using French LMB protocols adapted to the African setting and achieved a survival rate of 61%. A second study started in 2005 using Endoxan alone achieved a highly satisfactory survival rate of 73% for neuroblastoma in all stages except bilateral. Altogether from 2001 to 2007 more than 1000 cases of nephroblastoma and Burkitt's lymphoma were treated in African hospitals by African doctors and nurses. No patients were transferred to Europe. The GFAOP supplied drugs when necessary and took care of most travel expenses. African and French doctors worked together on protocol design, trial management, and data analysis. These promising results show that the latest therapeutic techniques can be used to treat childhood cancer in Africa by adapting the protocol to conditions in developing countries. Sanofi-Aventis Laboratories in association with the International Union against Cancer has launched a major campaign to improve Pediatric Oncology in developing countries. Projects in four GFAOP units are being financed through this campaign. In 2006 the GFAOP began assessment of two new treatment protocols, i.e., one for acute lymphoblastic leukemia and the other for Hodgkin's disease. Two other projects are being planned, i.e., one for treatment of retinoblastoma and the other for treatment of some types of brain tumors.

Treatment of Wilms tumor according to SIOP 9 protocol in Casablanca, Morocco.

BACKGROUND: Childhood Wilms tumor represents one of the challenge for pediatric oncologists in developing countries. We report the characteristics and treatment results of patients with Wilms tumor according to SIOP 9 protocol in Morocco. PROCEDURE: From January 1989 to December 2000, 86 children with Wilms tumor were admitted. The diagnosis was based on physical exam and abdominal ultrasound. The metastatic work-up was based on abdominal ultrasound and chest X-ray. RESULTS: The mean age was 36 months (3-120 months). The sex-ratio was 1. Abdominal mass was the main symptom at presentation (84 cases). There were 13 metastatic cases. Treatment applied was according to SIOP 9 Protocol without randomization. Local deases was present in 75 patients with stage I in 38 cases (50%), IIN0 in 4 cases (6%), IIN1 in 9 cases, and III in 24 cases (44%). The distribution of pathologic groups was: favorable in 4 cases, standard in 69 cases, and unfavorable anaplastic type in 2 cases. Sixty-nine patients were evaluable for therapeutic evaluation. Other patients were lost to follow-up. Three patients died of treatment related toxicity and 13 patients relapsed. With a median follow-up of 70 months, the 5-year EFS and 5 years overall survival for evaluable patients are 77.4% and 79%, respectively while the 5-year EFS for all patients was 56%. CONCLUSION: These results are encouraging for a developing country but special efforts should be done to reduce the rate of abandonment.