Neuroblastoma;Variable Symptoms of A Neurogenic Tumor; A Report From Iran

ObjectiveAccording to current data available, neuroblastoma is the most frequent extracranial solid tumor in infants and children; because of its relationship to the primitive sympathetic ganglia, it may progress or regress spontaneously to more malignant or benign forms of tumors, respectively. It is also an important cause of the Opsoclonus Myoclonus Ataxia Syndrome (OMAS), or the "dancing eyes"syndrome. The risk factor of neuroblastoma includes patient's age at diagnosis; degree of tumor spread, and selected biologic variables such as serum LDH, urinary and serum catecholamines such as VMA and HVA, ploidy and MYCN copy numbers. So, detection of risk factors and risk directed therapy are the mainstay of patient management.Materials & MethodsFor this study the records of 43 out of 46 patients, aged less than 14 years, admitted over 8 years (1996-2004), with the confirmed diagnosis of neuroblastoma or ganglioneuroblastoma were evaluated for full course of therapy and follow up.ResultsOf the patient group, 60% were male and 40% female. The most frequent clinical stage was stage 3 (34.7%), followed with stage 4 (32.6%) and 2 (26%).Less than 2% of patients presented with pure neurologic symptoms and these responded well to treatment. Relapse was seen more frequently in stage 4 patients and less in stage 4S. The OPEC protocol, started in 81% of patients, achieved a 54% response; on the other hand, in 15%, N6 was used, with the response rate of 40%. Patient data was analyzed and interpreted using SPSS software to reveal which clinical and biologic factors improve neuroblastoma outcome.ConclusionStaging and patients' age at the time of diagnosis are the most important clinical factors to predict outcome, while primary tumor site and some biologic findings such as urinary VMA and serum LDH levels have a less important value

Hemophagocytic Lymphohistiocytosis Complicating Erythroleukemia in a Child with Monosomy 7: A Case Report and Review of the Literature

Herein, the first case of childhood erythrophagocytosis following chemotherapy for erythroleukemia in a child with monosomy 7 is reported. A 5-year-old boy presented with anemia, thrombocytopenia, and hepatosplenomegaly in whom erythroleukemia was diagnosed. Prolonged pancytopenia accompanied by persistent fever and huge splenomegaly and hepatomegaly became evident after 2 courses of chemotherapy. On bone marrow aspiration, macrophages phagocytosing erythroid precursors were observed and the diagnosis of HLH was established; additionally, monosomy 7 was detected on bone marrow cytogenetic examination. In conclusion, monosomy 7 can lead to erythrophagocytosis associated with erythroid leukemia and should be considered among the chromosomal abnormalities contributing to the association

Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Replacing an Absent Kidney in a Child

Malignant peripheral nerve sheath tumors (MPNSTs) are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. He underwent surgical resection of the tumor with a primary impression of Wilms tumor. To the authors’ knowledge, this is the first case of retroperitoneal malignant peripheral nerve sheath tumor and absent kidney. This case suggests the very rare probability of association of MPNSTs in children with genitourinary tract anomalies such as renal agenesis