Significance of commonly used prognostic factors differs for children with T cell acute lymphocytic leukemia (ALL), as compared to those with B-precursor ALL. A Pediatric Oncology Group (POG) studyT cell acute lymphocytic leukemia (T-ALL) and B-precursor ALL differ significantly in the clinical characteristics of the patients at presentation and in laboratory-defined characteristics of the leukemic cells. We assessed for pediatric patients with T-ALL the relative importance of prognostic factors previously demonstrated to predict outcome in B-precursor ALL. Presenting clinical and laboratory features were correlated with outcome for 441 children 12 months to 21 years of age with previously untreated T-ALL, registered on the Pediatric Oncology Group (POG) T3 protocol between 1986 and 1992. These T-ALL prognostic factor analyses were then compared to similar analyses for 1993 patients with B-precursor ALL enrolled during the same time period on the POG ALinC 14 protocol. Quantitative interaction between phenotype and each prognostic factor was studied to determine the relative importance of the prognostic factor for each of the two major immunophenotypes. We also analyzed the importance of maturational stage as a T-ALL prognostic factor, using a modified Ludwig definition of maturational stage. We conclude that several of the clinical and laboratory prognostic factors, which are used reliably for Bprecursor ALL, are much less predictive in T-ALL (ie age, WBC, consensus risk group, hyperdiploidy, presence of translocations and CALLA expression). There was no significant difference between the phenotypes in the prognostic importance of race or gender. Our data demonstrate a significant difference in outcome among the three maturational stages of Tcell ALL, with the intermediate group faring best. Using traditional risk group criteria to stratify patients with T-ALL for therapy may not be appropriate.
Head and neck sarcomas are a rare and heterogeneous group of tumors that pose management challenges. We report our experience with these tumors. Forty consecutive patients treated for 44 head and neck sarcomas between 1997 and 2014 were culled from our prospectively maintained head and neck database. Five patients were excluded. The adult cohort consisted 29 (83%) patients of a mean age of 57.7 years, with 33 sarcomas. The most common diagnoses were undifferentiated pleomorphic sarcoma (27%) and chondroblastic osteosarcoma (21%). Clear surgical margins were achieved in 24/33 (73%) lesions. Twenty-two patients received radiotherapy and/or chemotherapy. Fourteen patients developed local (n = 6), regional (n = 1) and distant (n = 7) recurrence. The overall 5-year survival was 66% with a mean survival interval of 66.5 months. Recurrent sarcoma, close (<1 mm) or involved surgical margins and advanced age were associated with statistically significantly reduced survival. The pediatric cohort consisted 6 (17%) patients, with a mean age of 9 years. Five patients had primary embryonal rhabdomyosarcomas and one had chondroblastic osteosarcoma. Clear surgical margins were achieved in five (83%) patients. All patients received adjuvant radiotherapy and/or chemotherapy. Mean survival interval was 102 months. Three patients developed local (n = 1) or distant (n = 2) recurrence. Twenty-three free and 8 pedicled flaps were performed in 25 patients. Eleven out of thirty-nine (28%) lesions in 11 patients developed a complication. In conclusion, head and neck sarcomas are best managed by a multidisciplinary team at a tertiary head and neck referral center and resection with clear margins is vital for disease control.
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