Myelopathy after intrathecal chemotherapy: a case report with unique magnetic resonance imaging changes.
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Biomedical subjects
Publications and source records attributed to A Verdeguer.
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Common origin of sympathoblasts and pheochromoblasts from the neural crest cells is generally accepted. Neuroblastoma and pheochromocytoma result from an abnormal proliferation of these committed cells. They are included in the group of neuroendocrine neoplasms, formerly named Apudomas. Previous reports of mixed tumours of neuroblastoma and pheochromocytoma, and ganglioneuroma and pheochromocytoma, support this hypothesis. A case of extra-adrenal pheochromocytoma in an adolescent who 15 years before was successfully treated for a stage IV-S neuroblastoma without evidence of primitive tumour is reported. Two hypothesis are formulated: (1) the second neoplasm may have arisen from the unlocated primitive tumour, probably silent evolution could be due to its maturation into ganglioneuroma-pheochromocytoma; (2) taken into account a common origin of both neoplasms, the patient may have a genetic predisposition to develop neuroendocrine tumours. Whatever the mechanism, second neoplasms, as the case reported, late relapses, and late toxic effects may justify long follow-up of neuroblastoma survivors.
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Some cases of conversion from acute lymphoblastic leukemia (ALL) to acute nonlymphoblastic leukemia (ANLL) at relapse have been reported recently. We report three cases initially diagnosed as having ALL and showing morphological, cytochemical, and immunophenotypic features of ANLL at relapse (lineage switch). Conversion was observed among 14 patients who developed bone marrow relapse while undergoing intensive treatment with our ALL protocol, which includes teniposide, and that had been administered to 62 patients. The three cases converted at first relapse, with a mean time of 20 months (13-29 months). Clinical and immunologic characteristics of T-cell leukemia were present in one patient. Changes documented in cytogenetic studies are discussed. The underlying mechanisms for the lineage switch remain unclear as does its relation with mixed lineage leukemias, but we believe that drugs employed in our therapy protocol could have had an influence on this conversion.
Three children with acute lymphoblastic leukemia developed disseminated fungal disease predominantly involving the liver and spleen. The three patients were undergoing induction chemotherapy and had neutropenia when they presented prolonged fever not responsive to antibiotics. Once neutropenia was recovered, hepatosplenomegaly leukocytosis, elevated serum alkaline phosphatase, and hypoechoic areas in the spleen and liver ultrasound were observed. All fungal blood cultures were negative, with the diagnosis being confirmed by histologic study. One of the patients died without achieving control of the candidiasis. The other two patients received prolonged antifungal treatment concurrently with chemotherapy and both are alive, one of them cured and in complete remission. The increasing frequency of this infection in recent years and the importance of a prompt and prolonged administration of antifungal therapy to obtain the cure are discussed.
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Results of 70 fine-needle aspiration biopsies (FNAB) were evaluated retrospectively in 61 pediatric patients. Over a period of 9 months all mass lesions suspected being malignant were aspirated. Twelve of the 70 aspirations were performed in children having known tumours, in order to exclude recurrence or metastasis. The others were carried out to obtain a diagnosis. Satisfactory specimens were obtained from 58 (83%). There were 21 benign diagnoses, 36 malignant diagnoses, and 1 with suspected malignancy. Correlation of histologic and cytologic diagnoses was possible in 45 cases. The diagnostic sensitivity and specificity were 95% and 80%, respectively. We have found FNAB more accurate in the diagnosis of malignancies than in benign lesions. The results suggest that this is a useful technique for obtaining a first diagnosis of malignancy, as well as for excluding recurrence or metastatic disease.
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Sixteen consecutive children diagnosed of "high risk" acute lymphoblastic leukemia were treated with a protocol including VM-26, ARC and intermediate-dose Mtx. Complete remission was obtained in 94 por 100 of patients. Actuarial haematologic remission rate at 36 months is 87% and continuous complete remission rate 58%. Twelve doses of Mtx (IV + IT) appear to be insufficient as SNC prophylaxis in these high risk children. Nevertheless hematologic relapse rate is very low. Mean follow-up time is 31 months and survival rate 87%.
Ten patients with neuroblastoma were scintigraphed with I131-meta-iodobenzylguanidine (MIBG). Lugol solution was previously administered orally to all patients in order to avoid uptake of radioactive iodide by the thyroid gland. The compound was injected intravenously, 0.5 mCi/1.73 m2 of body surface. Scintigraphy was performed at 24, 48 and 72 hours and 6-7 days after administration of the radioactive preparation. Positive radiotracer uptake was demonstrated in all primitive tumors and metastases, showing a positivity of 100%. MIBG has proven to be the most useful technique in defining the stage of the disease. Mechanism of the tumoral uptake in relationship to catecholamine metabolism is discussed. Differences observed in MIBG radioactivity seen in the urinary bladder, heart and liver in children with or without. Neuroblastoma are discussed. MIBG has also been useful in the follow-up of patients with neuroblastoma.
A case report of ganglioneuroma secreting catecholamines is presented. The 131-I-MIBG gammagraphy was positive. We conclude that an uptake of 131-I-MIBG is not synonimous of a malignant tumour.
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