[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.

[Thymic hyperplasia following treatment for nephroblastoma]. / Hypertrophie thymique après une chimiothérapie pour un néphroblastome.

UNLABELLED: Thymic hyperplasia in response to stress is a well known phenomenon. Thymic hyperplasia has also been described after chemotherapeutic treatment for malignancies in children. CASE REPORT: A three-year-old girl was followed up from the age of 18 months for a left kidney nephroblastoma treated by combination of chemotherapy (vincristin, actinomycin and adriamycin) and surgery. Assessment at the end of treatment was normal. Four months after the end of treatment, pulmonary radiography showed mediastinal enlargement, which was shown to originate in the thymus at thoracic CT scan. A recurrence of the disease was suspected. Biopsy showed thymic hyperplasia without evidence of tumor cells. Mediastinal enlargement then disappeared spontaneously 2 months later. CONCLUSION: Thymic hyperplasia occurring during remission of a cancer treated by chemotherapy is a diagnostic dilemma as it suggests mediastinal reccurence of the disease. Needle aspiration cytology is an appropriate investigation in thymic hyperplasia. No steroid therapy should be used before histologic diagnosis of thymic hyperplasia.

[Ophthalmic manifestations of acute leukemia]. / Manifestations ophtalmologiques des leucémies aiguës.

Orbital and ocular lesions are the third most frequent extramedullar locations of acute leukemia after the meninges and testicles. These lesions are treated as a central nervous system lesion; therefore diagnosis is essential to therapeutic adjustment. We present a retrospective study on charts of children treated for acute leukemia between 1996 and 1998. Ophthalmic examination was carried out when there were ocular symptoms. One hundred ninety-six children were treated for acute leukemia. Twelve children (6.1%) had an ocular and orbital lesion: 7 boys and 5 girls, with an average age of 6 years. Six had acute lymphoblastic leukemia, 6 had acute myeloid leukemia. Visual acuity was 1/10 in 9 children. Four children had an initial orbital lesion with a rapidly progressing exophthalmos. The hemogram and myelogram showed a granulocytic sarcoma. Two children who had been previously treated for acute leukemia presented a bilateral anterior uveitis with hypopyon; anterior chamber paracentesis showed blast cells and confirmed the ocular relapse. A corneal lesion was found in 2 children and in 2 cases, the optic nerve had edema; a decrease in visual acuity was the sign of the optic nerve lesion. Two patients had retinal infiltration with hemorrhages in the posterior pole. Prophylaxis consists of intrathecal injection of methotrexate and reinforcement of chemotherapy. Central nervous system irradiation is seldom used in children. Orbital and ocular lesions carry a poor prognosis according to the majority of authors. Two years after diagnosis of the ocular lesions, only 1 of the children studied remains alive.

[Clear cell sarcoma of the kidney. A study of 13 cases]. / Sarcome rénal à cellules claires. A propos d'une série de 13 cas.

UNLABELLED: Clear cell sarcoma of the kidney (CCSK) also called a "bone-metastasizing renal tumor of childhood" is the second common pediatric renal neoplasm. This tumor is associated with a higher rate of relapse and a wider distribution of metastases than Wilms' tumor. PATIENTS AND METHODS: We have reviewed records of 13 cases of CCSK among 277 renal tumors (5%) diagnosed at the children's hospital of Rabat between 1990 and 2002. RESULTS: The median age at diagnosis was 14 months (5 months-9 years). The male to female ratio was 5.5:1.00. Abdominal mass, usually the first physical finding, was associated with hematuria in four cases. No congenital malformation syndrome or familial Wilms' tumor were observed. Imaging studies found out seven right and six left intrarenal processes. Preoperative chemotherapy was given according to the SIOP9, SIOP93-01 and GFAOP 98 protocols. Twelve of 13 children underwent nephrectomy. Tumor measurements varied through 450-3450 g and 7-26 cm. The classic morphologic pattern was seen in nine cases (69%). The distribution local stage was I: three cases; II: three cases; III: six cases; IV: one case. Postoperative chemotherapy and radiotherapy (21 600-30 600 cGy) was done in 10 cases. With a median follow up of 44 months, four patients showed bone metastases (31%), four are alive in CR, four are lost for follow up and five died. CONCLUSION: CCSK remains the pediatric renal tumor most frequently misdiagnosed. Its aggressiveness and its ability to give bone metastases need to recognize early this diagnosis for an adapted treatment.

[Pleuro-pulmonary blastoma. Report of 4 cases]. / Le blastome pleuro-pulmonaire. A propos de quatre observations.

Pleuropulmonary blastoma is an uncommon malignant lung tumor observed in children. Outcome is often unfavorable. Two boys and two girls, mean age 4.5 years, were admitted for nonspecific respiratory signs. Oriented by radiology findings, the diagnosis of pleuropulmonary blastoma was confirmed at pathology examination of a pneumonectomy specimen. Three of the children were given postoperative adjuvant chemotherapy. There were three deaths and one child was lost to follow-up. We discuss the clinical features of pleuropulmonary blastoma. No optimal treatment has been defined for this often fatal tumor.

[Pseudotumorous focal xanthogranulomatous pyelonephritis in children. Apropos of a case]. / Pyélonéphrite xanthogranulomateuse focale pseudotumorale chez l'enfant. A propos d'un cas.

Xanthogranulomatous pyelonephritis is a particular form of chronic pyelonephritis. It is observed less frequently in children and can sometimes present with a focal pseudoneoplastic appearance. An 11-year-old child was admitted with abdominal pain, alteration of the general state and weight loss without fever or palpable mass. Medical imaging was unable to distinguish between malignant renal tumour or tuberculosis. The diagnosis was confirmed by the inflammatory and purulent appearance of the mass and histopathological examination of the biopsy fragment. Percutaneous drainage of the abscess, combined with antibiotics provided marked improvement. Despite its rarity and its nonspecific clinical features, focal xanthogranulomatous pyelonephritis must be considered in order to propose conservative treatment.

[Langerhans-cell histiocytosis of the orbit. A case study]. / Histiocytose langerhansienne à localisation orbitaire. A propos d'un cas.

PURPOSE: Langerhans' cell histiocytosis is a rare disease representing less than 1% of orbital tumors. METHODS: We report a case of Langerhans cell histiocytosis with orbital involvement in a 9-year-old boy. He presented with an inflammatory swelling if the left lateral orbital wall. The computed tomographic scan revealed an orbital cellular mass with lytic bone lesion within the orbital roof and intracranial enlargement. RESULTS: The cytological study after a biopsy showed infiltrates of histiocytes derived from Langerhans cells. Diagnosis was confirmed by immunohistochemistry, which identified positive staining with anti-S100 and anti-CD1a antibodies. The rapidly expanding orbital tumor, posing a threat of ocular compression as well as intracranial spreading, was treated by chemotherapy (Vinblastine) combined with a steroid. CONCLUSION: A 2-year follow-up showed no evidence of recurrence or systemic involvement. According to this observation, the authors describe the clinicopathological and histological features of orbital involvement in Langerhans cell histiocytosis.