Pruritus in pediatric non-Hodgkin's lymphoma

A two-year-old girl presented with a 3-month history of generalized pruritus. One week before hospitalization she developed a superior vena cava syndrome and obstruction of the upper airways. Clinical and laboratory findings included generalized lymphadenopathy, a mediastinal mass compressing the tracheal lumen to the point of near closure, hepatomegaly and moderate eosinophilia. The diagnosis of an anaplastic large cell lymphoma (ALCL) was made by the histologic examination of a mediastinal lymph node. The history of generalized pruritus without diagnostic skin lesions was as uncommon as age at presentation. In conclusion, this case illustrates that generalized pruritus in a toddler can be an early sign of ALCL.     

The Eighth International Childhood Acute Lymphoblastic Leukemia Workshop ('Ponte di legno meeting') report: Vienna, Austria, April 27-28, 2005.

The International Acute Lymphoblastic Leukemia Working Group, the so-called 'Ponte di Legno Workshop' has led to substantial progress in international collaboration in leukemia research. On April 27-28, 2005, the 8th Meeting was held in Vienna, Austria, to continue the discussions about special common treatment elements in randomized clinical trials, ethical and clinical aspects of therapy. Furthermore, collaborative projects of clinical relevance with special emphasis on rare genetic subtypes of Childhood ALL were established. The following report summarizes the achievements and aspects of possible future cooperation.     

Adrenocortical carcinoma with Ectopic LH production

This report concerns a 3 1/4-year-old boy, in whom a left-sided adrenocortical adenocarcinoma of predominantly androgenic potency caused isosexual precocious development with enlargement of the testicles and, probably, predominantly testicular testosterone secretion. It was possible to identify an increase in LH production as the cause of this phenomenon; the seat of origin was the tumor. Ectopic LH production was proved upon extraction of the tumor and by the decline of gonadotropic activity after its removal.     

Allogeneic and autologous stem-cell transplantation in advanced Ewing tumors

Background:An update of results from the High Risk Protocol ofthe Meta-EICESS Study, conducted at the Pediatric Stem-Cell Transplant Centersof Düsseldorf and Vienna. In order to evaluate a possible therapeuticbenefit after allogeneic SCT in patients with advanced Ewing tumors (AET), wecompared outcome after autologous and allogeneic stem-cell transplantation(SCT). Patients and methods:We analyzed 36 patients treated with themyeloablative Hyper-ME protocol (hyperfractionated total body irradiation,melphalan, etoposide ± carboplatin) between November 1986 and December1994. Minimal follow-up for all patients was five years. All patientsunderwent remission induction chemotherapy and local treatment beforemyeloablative therapy. Seventeen of thirty-six patients had multifocal primaryEwing's tumor, eighteen of thirty-six had early, multiple or multifocalrelapse, one of thirty-six patients had unifocal late relapse. Twenty-six ofthirty-six were treated with autologous and ten of thirty-six with allogeneichematopoetic stem cells. We analyzed the following risk factors, that couldpossibly influence the event-free survival (EFS): number of involved bones,degree of remission at time of SCT, type of graft, indication for SCT, bonemarrow infiltration, bone with concomitant lung disease, age at time ofdiagnosis, pelvic involvement, involved compartment radiation,histopathological diagnosis. Results:EFS for the 36 patients was 0.24 (0.21) ± 0.07.Eighteen of thirty-six patients suffered relapse or died of disease, nine ofthirty-six died of treatment related toxicity (DOC). Nine of thirty-sixpatients are alive in CR. Age 17 years at initial diagnosis (P< 0.005) significantly deteriorated outcome. According to the type ofgraft, EFS was 0.25 ± 0.08 after autologous and 0.20 ± 0.13after allogeneic SCT. Incidence of DOC was more than twice as high afterallogeneic (40%) compared to autologous (19%) SCT, even thoughthe difference did not reach significance (P = 0.08, Fisher's exacttest). Conclusions:Because of the rather short observation period,secondary malignant neoplasm (SMN) may complicate the future clinical courseof some of our patients who are currently viewed as event-free survivors. EFSin AET is not improved by allogeneic SCT due to a higher complication rate.The patient group was to small to analyze for a possiblegraft-versus-tumor effect.     

Statement by members of the Ponte di Legno group on the right of children with leukemia to have full access to essential treatment for acute lymphoblastic leukemia.

During the panel discussion in the Sixth International ChildhoodAcute Lymphoblastic Leukemia (ALL) meeting of the Ponte di LegnoWorking Group, held in San Diego in December 2003, it was apparentthat recent improvements in treatment have further paradoxicallywidened the gap of inequality between children living in resource-richcountries and those in low-income countries. The representativesfrom 15 leading study groups and institutions decided to issuea statement [1] to lobby international and national agenciesto provide the necessary drugs at affordable cost throughoutthe world for children with ALL or other highly curable cancer.We wish to draw a wider readership and support     

Neuroblastoma mass screening in late infancy: insights into the biology of neuroblastic tumors.

PURPOSE: Neuroblastoma screening in early infancy has detected predominantly "favorable" tumors. We postponed screening to an age between 7 and 12 months to test whether this shift of screening age might influence the detection rate of genetically/clinically unfavorable tumors. PATIENTS AND METHODS: In a 10-year period, 313,860 infants were screened by analysis of urine catecholamines. When a neuroblastoma was diagnosed, at least two different areas from every tumor were analyzed for genetic features (MYCN amplification, 1p status, ploidy). Furthermore, neuroblastoma incidence and mortality of the screened group and the cohort of 572,483 children not participating in the screening program were compared. RESULTS: Forty-six neuroblastomas were detected by mass screening. In 17 tumors (37%) at least one of the biologic features was "unfavorable." In 10 of 17 patients, one or more of these alterations were only focally present (tumor heterogeneity). In the screened cohort, neuroblastoma incidence was significantly higher when compared with unscreened children (18.2 v 11.2/100,000 births), while there was a trend towards lower incidence of stage 4 over 1 year (2.2 v 3.8). Mortality was not significantly different (0.96 v 1.57). CONCLUSION: In contrast to other neuroblastoma screening programs, more than one-third of patients were found with unfavorable genetic markers in our study. The high proportion of focal alterations suggests that biologically young neuroblastomas may consist of genetically favorable and unfavorable parts/areas/clones. We conclude that at least one-third of neuroblastomas detected by screening in late infancy are anticipated cases. This, however, does not result in significantly reduced mortality.     

Akute Pankreatitis Seltene Komplikation bei primärem Hyperparathyroidismus (pHPT)

Background. Primary hyperparathyroidism (pHPT) in childhood and adolescence is a rare disease. Therefore diagnosis often is delayed. Methods. We report on a 13 year old boy, who sufferd from nausea, abdominal pain and weight loss for 9 months. We diagnosed an adenoma of the parathyroid gland and an acute pancreatitis in the course of a hypercalcemic crisis. Results. After stabilizing the patient, the adenoma was removed surgically. No problems occured after the surgical treatment. Within 3 months the boy regained his original weight. Conclusion. Symptoms like nausea, abdominal pain, cephalea, hypertonia, anorexia and renal stones could be a hint for pHPT. The coincidence of pHPT and pancreatitis is extremely rare. According to references in the literatur this association is most likely related to elevated calcium levels due to advanced pHPT.