Biomedical subjects
Publications and source records attributed to Dominique Couanet.
Some radiological cases are presented in this article to train the reader to the BIRADS classification in mammography. Each case is described according to the fourth American version of the BIRADS lexicon. Some classifications difficulties will also be presented, in order to show the complexity and the observer variability, commonly encountered in BIRADS 3 and 4 categories.
We report a case of a 16-yr-old girl with a liver tumor revealed by thrombophlebitis of the left leg. On physical examination the patient was found to have painless hepatomegaly. Ultrasound and CAT scan showed a large tumor of the left portion of the liver, measuring 14 cm in diameter. Cytological preparations were touch imprints of the biopsy fragments obtained under ultrasound guidance. Cytological examination using May-Grünwald Giemsa stain revealed highly cellular smears containing large tumor cells with a round nucleus, prominent nucleoli, and abundant granular basophilic cytoplasm. Cytological features were those of fibrolamellar hepatocellular carcinoma, confirmed by histological examination of the biopsy sample as well as the surgical specimen obtained after wide excision of the lesion following ineffective neoadjuvant chemotherapy.
We report a case of a 16-year-old girl with a left renal tumor discovered by her family practitioner. On physical examination the patient had a painless abdominal mass, located in the upper medium portion of the abdomen on the left side with a voussure of the abdominal wall. Ultrasound and abdominal pelvic CAT scan revealed a large heterogeneous mass with calcifications in the inferior portion of the left kidney. We made touch-imprint cytological preparations of the biopsy fragments, obtained under ultrasound guidance. Cytological analysis revealed highly cellular smears with malignant cells arranged in large clusters or rarely isolated, sometimes surrounding hyaline nodules with numerous psammoma bodies. After May-Grünwald-Giemsa staining, cells displayed moderately irregular nuclei with an abundant and pale basophilic cytoplasm with well-defined borders and a finely granular texture. The diagnosis of a special type of renal cell carcinoma was suspected, and was then confirmed after examination of the biopsy sample and the corresponding surgical specimen. The histomorphologic features were those of a renal cell carcinoma associated with an Xp11.2 translocation. Immunohistochemistry revealed this translocation by showing nuclear positivity in tumor cells for an antibody raised against the TFE3 protein. The clinical outcome was marked several months later by metastases in lymph nodes, bone, lung, and adrenal gland as well as a local recurrence.
BACKGROUND: Morbidity and mortality are high in young children with medulloblastoma who receive craniospinal radiotherapy. We aimed to assess whether adjuvant treatment with protracted chemotherapy alone could replace radiotherapy. METHODS: We enrolled 79 children aged younger than 5 years who had had surgical resection of medulloblastoma onto a multicentre trial. Patients were treated with combination chemotherapy, which did not include methotrexate, for more than 16 months irrespective of the extent of disease. Early postoperative imaging defined three groups: R0M0 (no residual disease, no metastasis), R1M0 (radiological residual disease alone), and RXM+ (presence of metastases). Patients who did not relapse did not receive radiotherapy. Patients who relapsed or had disease progression received salvage treatment, which consisted of high-dose chemotherapy and stem-cell transplantation followed by local or craniospinal radiotherapy. For children classified as R0M0, the primary endpoint was 5-year overall survival and the secondary endpoint was 5-year progression-free survival. For children classified as R1M0 or RXM+, the primary endpoint was best radiological response and the secondary endpoints were 5-year overall survival and 5-year progression-free survival. Analyses were done by intention to treat. FINDINGS: Two of 15 patients classified as RXM+ and four of 17 patients classified as R1M0 had a complete radiological response. 5-year progression-free survival was 29% (95% CI 18-44) in the R0M0 group, 6% (1-27) in the R1M0 group, and 13% (4-38) in the RXM+ group. 5-year overall survival was 73% (59-84) in the R0M0 group, 41% (22-64) in the R1M0 group, and 13% (4-38) in the RXM+ group. In the R0M0 group, 5-year progression-free survival was 41% (26-58) for the 34 patients who underwent gross total resection compared with 0% for the 13 patients who had subtotal resection (relative risk 2.7 [1.3-5.6], p=0.0065). INTERPRETATION: Conventional chemotherapy alone can be used to cure children with non-metastatic medulloblastoma who have gross total resection confirmed by early radiological assessment, but is not sufficient for treatment of those with metastatic or incompletely resected medulloblastoma. Salvage treatment followed by posterior-fossa radiotherapy can effectively treat local relapses or progression.
PURPOSE: To evaluate a strategy aimed at avoiding radiotherapy during first-line treatment of children with progressive optic pathway tumors (OPT), by exclusively administering multiagent chemotherapy during 16 months. PATIENTS AND METHODS: Between 1990 and 1998, 85 children with progressive OPT were enrolled onto this multicenter nationwide trial. Chemotherapy alternating procarbazine plus carboplatin, etoposide plus cisplatin, and vincristine plus cyclophosphamide was given every 3 weeks. At the time of relapse or progression, second-line chemotherapy was authorized before recourse to radiotherapy. RESULTS: Objective response rate (partial response [PR] + complete response [CR]) to chemotherapy was 42%. Five-year progression-free survival (PFS) and overall survival rates were 34% and 89%, respectively. The 5-year radiotherapy-free survival rate was 61%. In the multivariate analysis of the 85 patients that entered onto the study, factors associated with the risk of disease progression were age younger than 1 year at diagnosis (P =.047) and absence of neurofibromatosis type 1 (P =.035). In the multivariate analysis of the 74 patients that remained on study after the first cycle of chemotherapy, factors associated with the risk of disease progression were age younger than 1 year at diagnosis (P =.0053) and no objective response to chemotherapy (P =.0029). Three-year PFS was 44% in infants < or = 1 year versus 66% in children older than 1 year. Three-year PFS was 53% in the absence of an objective response to chemotherapy versus 68% after a PR or CR. CONCLUSION: A significant proportion of children with OPT can avoid radiotherapy after prolonged chemotherapy. Deferring irradiation with chemotherapy protocols did not compromise overall survival of the entire population or visual function.
BACKGROUND: Central nervous system (CNS) metastases rarely occur in patients with neuroblastoma, although recent reports suggest an increase in the rate. CNS recurrence may represent a different mechanism of spread than bone and bone marrow metastases and may be associated with unique genetic determinants. Further definition of the radiologic, clinical, and biologic features may provide clues to the predisposing factors and mechanisms of CNS dissemination. METHODS: A retrospective analysis of all children ages 0-21 years with Stage IV neuroblastoma who were diagnosed at the Institut Curie and the Institut Gustave-Roussy between 1985 and 2000 was performed with direct review of medical records and magnetic resonance images, computed tomography scans, and iodine-123 or iodine-131 metaiodobenzylguanidine scintiscans (MIBG scans). When tumor tissue was available, genetic analysis was performed using comparative genomic hybridization (CGH). RESULTS: Of 434 patients with Stage 4 disease, 23 children had the CNS as their site of first recurrence. The estimated risk of CNS recurrence was 8.0% at 3 years, with no significant change in risk over the 15-year period. Eleven patients had isolated CNS recurrences, and the remaining patients developed recurrences concomitantly in other sites. The sites of recurrences were parenchymal (n = 8 patients), parenchymal with meningeal (n = 7 patients), and meningeal alone (n = 8 patients). MIBG scans detected CNS lesions in only 43% of patients. Significant risk factors for CNS recurrence included lumbar puncture at diagnosis, ages 2-3 years, and MYCN gene amplification. Abnormalities that were identified using CGH, in addition to 2p24 amplification in 5/7, included gains of 17q and 18q and losses of 1p, 3p, 10q25-26, and 11q. CONCLUSIONS: The risk of CNS recurrence in patients with neuroblastoma is 8% at 3 years after diagnosis and has not increased in the last 15 years. Because the CNS often is the sole site of recurrence, either it may be a sanctuary site, or the biologic determinants of CNS metastasis may be unique. Elucidation of risk factors and pathogenesis may allow prevention of this fatal event. Cancer 2003;98:155-65.
BACKGROUND: The detection of hepatic nodules, particularly in patients treated for a previous malignancy, raises a diagnostic dilemma. Focal nodular hyperplasia (FNH) of the liver is an uncommon, benign tumor in children and must be differentiated from malignant hepatic lesions. The etiology of FNH is obscure, and its pathogenesis is poorly understood. FNH may be a reaction to localized vascular abnormalities and circulatory disturbances. The goal of the current study was to identify risk factors for the occurrence of FNH in children who had received prior treatment for a malignant tumor. METHODS: The current retrospective study examined 14 cases of FNH in pediatric patients who previously had been treated for a malignancy. Diagnosis was based on clinical and radiologic findings and was proven histologically in four cases. RESULTS: FNH lesions were discovered by chance during routine examination in 78% of patients. The incidence of FNH was particularly high in the current series (0.45%) compared with the incidence in the general pediatric population. High doses of alkylating agents (e.g., busulfan or melphalan), venoocclusive disease, and liver radiotherapy may be responsible for injury to the vascular endothelium and subsequent localized circulatory disturbances. FNH is characterized by the absence of complications after its detection; therefore, only close follow-up is recommended. CONCLUSIONS: FNH appears to be a late complication of an iatrogenic vascular disease in children with a history of malignancy.
In patients with pineal tumors, clinical symptoms are due to direct compression of adjacent structures. The most common signs include increased intracranial pressure (80%) caused by obstruction of the sylvian aqueduct, and Parinaud syndrome (50%) caused by direct compression of the superior colliculi. Hearing loss is rare in patients with tumors in this location. The authors report on the case of a 12-year-old boy in whom a malignant pineal germ cell tumor was found together with the unusual occurrence of severe hearing loss due to direct bilateral compression of the inferior colliculi. This condition resolved completely after tumor regression.