Relapsed ALCL are highly chemosensitive but over 40% of the patients experience several relapses. Prolonged conventional chemotherapy based on vinblastine might, in some cases, be as efficient as short intensive treatment with ABMT.

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.

BACKGROUNDThe 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.METHODSThe 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.RESULTSFNH 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.CONCLUSIONSFNH appears to be a late complication of an iatrogenic vascular disease in children with a history of malignancy. Cancer 2003;97:3107–13. © 2003 American Cancer Society.DOI 10.1002/cncr.11452

Transplantation of PBSCs is associated with more rapid hematologic recovery than is bone marrow transplantation after high-dose chemotherapy for solid tumors or lymphomas. Furthermore, global costs are lower and cost-effectiveness ratios are better with PBSC transplantation.

Semiquantitative MIBG score early in therapy provides valuable prognostic information for overall response and EFS, which may be useful in tailoring treatment.

The pharmacokinetics of high-dose busulfan given orally at 1 mg/kg every 6 h over 4 days (total dose, 16 mg/kg) in combined chemotherapy followed by autologous bone marrow transplantation was studied in 12 children with a mean age of 7 years (range, 4-14 years). Busulfan levels in biological fluids were measured by a gas chromatographic assay with mass fragmentographic detection, using a deuterated analogue as the internal standard. In a high-dose regimen, busulfan followed one-compartment model kinetics with zero-order absorption. A mean maximal concentration of 803 +/- 228 ng/ml was achieved at 92-255 min after dosing. The mean elimination half-life was 2.33 h, and the mean total clearance was 119 +/- 54 ml/min per m2, with an apparent distribution volume of 27.10 +/- 11.50 l/m2. A mean trough level of 370 ng/ml was found throughout the 4 days of the chemotherapy course. There were no significant variations in pharmacokinetic parameters measured after the first and last doses. Busulfan was monitored in the CSF of nine children at 3.25-7 h after the last dose and was detected in all patients, with a mean CSF-to-plasma concentration ratio of 0.95 (range, 0.5-1.4).

Two hundred ninety-one courses of high-dose chemotherapy (HDC) with bone marrow transplantation (BMT) in children with malignancies were reviewed in order to assess the incidence, clinical course, outcome, and predisposing factors of hemorrhagic cystitis. Hemorrhagic cystitis occurred in 19 HDC courses (6.5%). Three patients had grade I hematuria linked to thrombopenia, nine had grade II hematuria despite platelet levels greater than 50 x 10(9)/L, and seven had grade III hematuria with clots and bladder obstruction. Severe complications occurred in grade III patients, but no deaths were directly linked to the cystitis. Fourteen patients recovered within two to 120 days of onset. The other patients died before the cystitis resolved, either of a relapse of the malignancy or of infection. Predisposing factors were age (increased incidence in older children), conditioning regimen containing cyclophosphamide, previous vesical irradiation, association with prolonged aplasia, and hepatic complications. The role of busulfan was also probable. No viral agent was found.

A healthy 37-year-old woman with a familial history of cutaneous melanoma and a recent history (6 months) of atypical nevus was seen in consultation by a dermatologist. Complete skin mapping revealed only an oval, 6 ϫ 4-mm pigmented lesion on her upper left arm that had a history of rapid growth and a variegated chromatic pattern ( Fig 1A). In vivo digital epiluminescence microscopy showed digitated radial streaming, grayish-blue reticular patterns, opaque gypseous alabaster lacunas, brown-black dots on a blue-gray background, pseudopodia at the periphery, blue-in-pink areas, and a blue-gray velum (Fig 1B). Surprisingly, histologic examination of the excised lesion (Fig 1C and 1D) displayed all the typical pathologic features of completely regressed melanocytic lesion and led to a diagnosis of atypical pigmented lesion in regression and a suspicion of melanoma (uninvolved flattened epidermis, fibrosis parallel to the epidermis together with many melanophages, and chronic inflammatory cell infiltration consisting mainly of lymphocytes). Although partial regression in malignant cutaneous melanoma occurred in 10% to 58% in several large series, spontaneous complete regression of primary melanomas are rarely described in the literature.1,2 The present case did not meet all the stringent criteria for the diagnosis of spontaneous complete regression of primary melanoma because of the absence of clinically tumor-involved lymph nodes draining the area where the regressed pigmented lesion was localized. Because of the diagnostic dilemma, 3 months after surgical excision we performed a reverse transcriptase-polymerase chain reaction (RT-PCR)-based assessment of malignant melanoma cells in three sequential peripheral-blood samplings.3-5 Tyrosinase-positive circulating cells ( Fig 1E) were detected in all the samples (lanes 1, 2, and 3: peripheral blood from the patient obtained in three sequential samplings at different time points; lane 4: peripheral blood from a healthy donor; lane 5: A375M melanoma cell line).A skin and systemic search for an occult primary melanoma and systemic staging with axillary ultrasonography, total-body computed tomography, and total-bone scintigraphy were negative. On the basis of these data, a diagnosis of complete spontaneous regression of cutaneous malignant melanoma was suggested and a local surgical resection was performed.Results of an RT-PCR assay for tyrosinase from peripheral blood continued to be positive 10 months after the diagnosis in the absence of any other sign of systemic disease or atypical skin lesions. To the best of our knowledge, this is the first report of a diagnosis of a completely regressed primary cutaneous melanoma formulated with the help of RT-PCR tyrosinase detection.Daniele Santini, Giuseppe Tonini, Bruno Vincenzi, Raffaele Murace, Giulio Ferranti, and Alfonso Baldi Università Campus Bio-Medico, Centro Diagnostico, and Istituto Dermopatico dell'Immacolata, Rome, and Seconda Università di Napoli, Naples, Italy A 39-year-old woman presented in December 1999 with...