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Biomedical subjects

S Zoger

Publications and source records attributed to S Zoger.

17 recordsLinked to original sources
PubMed1998

Ewing's sarcoma masquerading as osteomyelitis.

Ewing's sarcoma in the infant and young child is rare, highly malignant, and can be difficult to identify. An erroneous diagnosis of osteomyelitis may be considered first because the presentation may be similar to that of Ewing's sarcoma, and routine laboratory evaluation may not distinguish between these entities. Two such cases are presented, one involving the tibia in a 16-month-old child and another a finger phalanx in a 7-month-old child. In both cases the correct diagnosis of Ewing's sarcoma was delayed because of initial misdiagnosis of osteomyelitis. This diagnostic dilemma is summarized, and the literature reviewed. Special attention is given to recent advances in histochemistry and cytogenetics that assist in tumor identification. The conclusion highlights areas of remaining controversies for which additional study may facilitate distinction between osteomyelitis and Ewing's sarcoma.

Bone Neoplasms↗
PubMed1997

Primary intracerebral osteosarcoma: a case report.

Primary intracerebral sarcomas are rare tumors. Malignant fibrous histiocytoma and fibrosarcoma are the predominant histologies. A 3 year old girl with a primary, intraparenchymal cerebral osteosarcoma, the second case reported in the literature, is described. Partial surgical resection, systemic chemotherapy and radio-surgery has provided tumor control at 18 months. Treatment issues are discussed.

Brain Neoplasms↗
PubMed1996

Disease course and late sequelae of Langerhans' cell histiocytosis: 25-year experience at the University of California, San Francisco.

PURPOSE: The purpose of our investigation was to correlate the extent and degree of organ involvement at presentation of Langerhans' cell histiocytosis (LCH) with subsequent disease course, survival, and late sequelae. MATERIALS AND METHODS: The medical records of 71 patients with a pathologic diagnosis of LCH, age 0 to 21 years, who presented between January 1, 1969 and June 30, 1994, were reviewed for organ involvement at diagnosis, treatment, disease course, and late sequelae. Supplementary data were obtained by mailed questionnaire. RESULTS: The median follow-up time from diagnosis for all patients was 8.1 years. Involvement at diagnosis included nine patients with skin-only disease, 22 with monostotic disease, 12 with polyostotic disease, and 28 with multisystem presentation. Treatment was surgery only in 17 and chemotherapy and/or radiotherapy in 54 patients. Recurrences were seen in 35 patients, with the highest rate in the polyostotic group. Ten patients died: seven with the multisystem presentation, two with monostotic disease, and one with skin-only disease. Causes included progressive LCH (n = 6) and late sequelae of either treatment (n = 3) or disease (n = 1). Late sequelae were seen in 64% of 51 patients with more than 3 years of follow-up data. The most common were skeletal defects in 42%, dental problems in 30%, diabetes insipidus in 25%, growth failure in 20%, sex hormone deficiency in 16%, hypothyroidism in 14%, hearing loss in 16%, and other CNS dysfunction in 14%. The overall estimated survival rates at 5, 15, and 20 years are 88%, 88%, and 77%, with an estimated event-free survival rate of only 30% at 15 years. CONCLUSION: Despite the favorable survival, more than half of LCH patients will have further dissemination of disease or late sequelae, including even some patients with single-system disease at diagnosis. Future treatment needs to be designed to prevent disease progression and late sequelae.

Adolescent↗
PubMed1992

Necrotizing fasciitis in two children with acute lymphoblastic leukemia.

Necrotizing fasciitis is a severe, soft tissue infection, and is an unusual condition in children. The cornerstone of therapy is prompt, aggressive surgical treatment. Despite vigorous treatment, mortality rates are high. We report the occurrence of necrotizing fasciitis in two children during the granulocytopenic phase of induction chemotherapy for acute lymphoblastic leukemia. The diagnosis and treatment of necrotizing fasciitis in these two children was made more difficult by their underlying disease and its chemotherapy. The successful treatment of their infections relied on a multimodality approach. Aggressive surgical debridement was the mainstay of therapy. Adjuvant therapy was vital to the successful outcomes and included meticulous wound care, intravenous hyperalimentation, appropriate antibiotics, and granulocyte transfusions.

Agranulocytosis↗
PubMed1988

Complete pathologic maturation and regression of stage IVS neuroblastoma without treatment.

Spontaneous maturation of Stage IVS neuroblastoma has been postulated as a mechanism for its favorable prognosis, but this has rarely been documented pathologically. We report on a patient with congenital Stage IVS neuroblastoma who had extensive subcutaneous and bone-marrow involvement. Serial photographs, biopsies, and vanillomandelic acid determinations documented the tumor's initial progression which was followed by spontaneous maturation and involution of the patient's disease over a 6-year period. No cytotoxic therapy was administered. Favorable biologic prognostic factors were documented, including tumor DNA and protein analyses for N-myc amplification or overexpression and analysis for serum neuron-specific enolase and ferritin. Implications for management and therapy of Stage IVS neuroblastoma are discussed with reference to this case and the recent literature.

Biomarkers, Tumor↗
PubMed1988

Patient, parent, and physician perspectives on pediatric oncology rounds.

To provide data to guide physicians regarding the extent to which pediatric patients and their families should be involved in decision-making discussions by their health care teams, we compared the standard rounding procedure in a pediatric oncology unit (rounds conducted out of earshot) with bedside rounds in which management was discussed in front of patients and parents. Type of rounds was alternated in 2-week blocks for 4 months. The impact of the two types of rounds of 35 parents and children was studied. Parents preferred bedside rounds to standard rounds, and perceived increased opportunities to obtain information and ask questions. Parents and children differed in their reports of how much bedside rounds upset children. Older children, compared with younger children, more strongly disliked standard rounds; this appeared to be related to feelings of exclusion. The two types of rounds differed in their effectiveness in providing specific types of education to residents. The findings suggest that bedside rounds have a positive impact on parents' attitudes toward physicians, that they do not dilute the child's sense of relationship with the primary attending physician, and that they contribute to certain aspects of resident education.

Adolescent↗
PubMed1987

Juvenile monosomy 7 syndrome: evidence that the disease originates in a pluripotent hemopoietic stem cell.

The present study was undertaken to investigate the hemopoietic cell from which malignant change evolves in juvenile dyshemopoiesis with monosomy 7. Two male patients, aged 18 and 5 months, were studied using progenitor assays combined with cytogenetics. Both had hepatosplenomegaly, cytopenias and a cellular marrow. The karyotype in direct marrow was 45,XY-7/47,XY,+8/46,XY in patient 1 and 45,XY,-7/46,XY in patient 2. Patient 1 received chemotherapy but developed acute nonlymphocytic leukemia after 17 months and died 20 months after diagnosis. During this time marrow metaphases with 45,XY,-7 increased to 100% (25/25). Patient 2 received an allogeneic marrow transplant 4 months after diagnosis which did not engraft. In both patients progenitors of both small (CFU-E) and large (BFU-E) erythroid colonies were present at normal frequencies. However, the colonies produced were small and poorly hemoglobinized with some erythropoietin-independent maturation. Progenitors of large granulocyte/macrophage colonies (CFU-GM) were present at an elevated frequency in the marrow of patient 1 and in the blood all progenitor classes were markedly increased. Cytogenetic analysis of colonies from this patient showed BFU-E to be 45,XY,-7 or 47,XY,+8 and CFU-GM to be 45,XY,-7 or 47,XY,+8 or 46,XY. In patient 2, most BFU-E were 45,XY,-7, although a few were 46,XY. These data indicate that malignant change in this disease involves hemopoietic stem cells capable of erythroid and in at least some cases, myeloid differentiation.

Acute Disease↗
PubMed1986

Bone marrow in children with acute lymphocytic leukemia: MR relaxation times.

Magnetic resonance imaging of the lumbar spine was performed in 17 children with acute lymphocytic leukemia (ALL): eight with newly diagnosed ALL, four with ALL in relapse, and five with ALL in remission. Eleven age-matched children were also imaged as controls. The T1 and T2 relaxation times of the bone marrow in the lumbar spine were calculated for all the children. The T1 relaxation times of the bone marrow were as follows (mean +/- standard deviation): newly diagnosed ALL, 968 msec +/- 68; ALL in relapse, 765 msec +/- 19; ALL in remission, 404 msec +/- 135; and age-matched controls, 441 msec +/- 82. T1 relaxation time was statistically significant in differentiating children with newly diagnosed ALL from normal children and from children with ALL in remission. In addition, T1 may be useful in differentiating children with ALL in relapse from children with ALL in remission and from healthy children. T2 was not significantly different among the four groups.

Adolescent↗
PubMedDate not supplied

Efficacy and safety of [131I]metaiodobenzylguanidine therapy for patients with refractory neuroblastoma.

Metaiodobenzylguanidine (MIBG) is a guanethidine derivative that is selectively concentrated in sympathetic nervous tissue. MIBG labeled with 123I or 131I has proven to be a specific and sensitive tool for detection of primary and metastatic pheochromocytoma and neuroblastoma. Eleven patients, with refractory stage IV neuroblastoma were treated with a total of 23 courses of 131I-MIBG, 100-400 mCi/m2/course. Total activity administered per course ranged from 90-550 mCi; maximum cumulative radioactivity per patient was 1356 mCi. The 131I-MIBG was given as a 2 hour infusion. Total body dose was calculated from whole body activity measurements, ranging from 73-250 cGy. The main toxicity was thrombocytopenia, with platelet nadirs to less than 25,000/microL in 5/23 courses (5 patients), all occurring in patients with greater than 25% replacement by tumor in the bone marrow. Neutropenia to a nadir of less than 500/microL was seen in only 2 patients, both with greater than 50% bone marrow replacement after 2 and 4 courses of 131I-MIBG, respectively. Tumor doses were calculated in patients with an evaluable measurable lesion, and ranged from 312-6329 cGy per course. Two of the eleven patients had partial responses, with one long-term survivor with stage IV neuroblastoma with no evidence of active disease now 4 years off treatment. Two other patients survive with stable disease after 3 treatments, at 3+ and 5+ months. Seven patients died with progressive disease. This study shows that treatment with 131I-MIBG is safe and can be effective in refractory neuroblastoma, particularly in patients who do not have extensive bone and bone marrow involvement.

3-Iodobenzylguanidine↗