Simultaneous diagnosis of acute lymphoblastic leukemia and peripheral neuroblastic tumor in a child.

We report the case of a 3-year-old girl with a mediastinal mass, severe anemia, leukocytosis and neutropenia, in whom, after initial suspicion of metastatic neuroblastoma, a final diagnosis of concurrent ganglioneuroblastoma and acute lymphoblastic leukemia was made. The mediastinal tumor was surgically excised and the child subsequently underwent chemotherapy for acute lymphoblastic leukemia. The patient remains in complete remission from both diseases 4 years after the diagnosis and 24 months after completion of all treatment. The simultaneous occurrence of 2 different neoplasms in a child is very infrequent, and no comparable cases are reported in the literature.

Hepatopathy-thrombocytopenia syndrome (HTS) after actinomycin-D therapy: report of three cases and review of the literature.

Hepatopathy-thrombocytopenia syndrome (HTS) is a severe complication very similar to vein occlusive disease (VOD), also known as hepatic sinusoidal obstructive syndrome (SOS), characterized by fever, hepatopathy (hepatomegaly with abnormal liver function tests), ascites, weight gain, jaundice, and thrombocytopenia (platelet count less than 25 × 10(3)/µL). It has been generally observed in patients with Wilms tumor, and is commonly associated to administration of actinomycin D. We report three children with Wilms tumor, with severe HTS/SOS, but had a different outcome, in spite of vigorous supportive therapy.

Hodgkin lymphoma and nephrotic syndrome in childhood.

An association between nephrotic syndrome and extrarenal neoplasia was described for the first time in 1922. Since then a large number of cases have been published, few of them describing the link between Hodgkin disease (HD) and nephrotic syndrome (NS). It shows that the incidence of nephrotic syndrome in Hodgkin lymphoma is less than 1%. Till date, to the best of author's knowledge, there are about 50 pediatric cases published, no one among Italian children. In the present paper, the authors report 2 cases observed in their department in the 7 yrs period.

Breast metastases in children and adolescents with rhabdomyosarcoma: Experience of the Italian Soft Tissue Sarcoma Committee.

BACKGROUND: Breast metastasis from rhabdomyosarcoma (RMS) is an uncommon event but may be problematic in treatment decision-making. Aim of the study was to evaluate clinical characteristics, treatment, and subsequent outcome, of patients with RMS metastasis in the breast, enrolled in four consecutive Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) Soft Tissue Sarcoma Committee protocols during the last 20 years, in order to obtain information to establish a more adequate diagnostic and therapeutic approach. PROCEDURES: Data were derived from the AIEOP STSC database and reviewed for the purpose of this study. RESULTS: From 1988 to 2008, among 189 patients with metastatic RMS, we identified 7 (3.7%) patients with RMS with breast involvement at diagnosis. All patients were females, aged 13-17 years with alveolar histology and multiple metastasis sites (2-5). The primary tumor was located in the extremities in 3/7 patients. In spite of intensive treatment no patient survived. The cause of treatment failure was distant relapse in six patients, including two on the mammary region. Treatment data analysis revealed that local measures to control breast lesions were used in only two patients. CONCLUSIONS: Our data suggest that investigations of the mammary region should be part of the usual diagnostic workup in adolescent girls with alveolar RMS, especially if the primary tumor arises in the extremities. New and more effective strategies are needed to improve the outcome of these patients including aggressive local measures to control breast disease.