BACKGROUND High‐grade astrocytomas (HGA) carry a dismal prognosis and compose nearly 20% of all childhood brain tumors. The role of high‐dose chemotherapy (HDCT) in the treatment of HGA remains unclear. METHODS In a nationwide study, The Children's Cancer Group (CCG) prospectively evaluated 102 children with HGA and postoperative residual disease for efficacy and toxicity of four courses of HDCT before radiotherapy (RT). Patients were randomly assigned to one of three couplets of drugs: carboplatin/etoposide (Regimen A), ifosfamide/etoposide (Regimen B), or cyclophosphamide/etoposide (Regimen C). After HDCT, all patients were to receive local RT followed by lomustine and vincristine. Twenty‐six patients were excluded after central neuroradiographic review (n = 8) or pathology review (n = 18). RESULTS Of 76 evaluable patients (median age, 11.95 yrs; range, 3–20 yrs), 30 patients relapsed during HDCT, and 11 others did not complete HDCT because of toxicity. Nonhematologic serious toxicities were common (29%), and 21% of patients did not receive RT. Objective response rates were not associated with amount of residual disease and did not statistically differ between regimens: 27% (Regimen A), 8% (Regimen B), and 29% (Regimen C). Overall survival (OS) was 24% ± 5% at 5 years and did not differ between groups. Median time to an event was longest for Regimen A (283 days compared with 83 and 91 days for Regimens B and C, respectively). The five‐year, event‐free survival (EFS) rate for all patients was 8% ± 3% and 14% ± 7% for Regimen A (P = 0.07). CONCLUSIONS OS and EFS were not affected by histologic grade. Patients who responded to HDCT had a nominally higher survival rate (P = 0.03 for trend). The authors conclude that these commonly used HDCT regimens provide no additional clinical benefit to conventional treatment in HGA, regardless of the amount of measurable residual tumor. Cancer 2005. © 2005 American Cancer Society.
A 1-year-old infant with von Recklinghausen's neurofibromatosis was seen because of increasing clitoral enlargement over a 7-month period. The mother, who also had neurofibromatosis, was treated 2 years beforehand for an acoustic neuroma. The child was treated by radical clitorectomy. Pathologic examination revealed malignant schwannoma, a tumor not previously described in this site. Vincristine, dactinomycin, and cyclophosphamide chemotherapy was given for 2 years to prevent local recurrence and metastatic spread. The child remains tumor free 2.5 years after diagnosis.
Hemorrhagic vesicles in a newborn present a challenging differential diagnosis including both infectious and neoplastic disorders. Patients should be evaluated in an efficient manner to arrive at the correct diagnosis as quickly as possible. We present here an interesting case that outlines the methodical workup that ultimately revealed the diagnosis of congenital Langerhans cell histiocytosis. After a diagnosis of Langerhans cell histiocytosis is made, it is important to evaluate the patient thoroughly for systemic involvement. Historically, the diagnosis of congenital self-healing Langerhans cell histiocytosis was used to delineate a benign self-limited disorder limited to the skin with spontaneous resolution during the first several months of life; this disorder may also be referred to as "self-regressive Langerhans cell histiocytosis." However, some newborns with initial skin-only Langerhans cell histiocytosis progress to have multisystem disease after spontaneous resolution has occurred. For this reason, the nomenclature is changing. We suggest using the term "skin-only Langerhans cell histiocytosis." Periodic long-term follow-up is recommended to monitor for relapse or progression to systemic disease.
pathways: 2WW 37, inpatients 15, angiograms 6, PE service 5, respiratory OPD 17, other MDT 4, OPD 11, GP 3. Only 4/107 patients (3.7%) had high suspicion for lung cancer at outset, -2 confirmed at surgery, 1 received radiotherapy (age 91yrs), 1 declined treatment. No further pathology was detected from surveillance. So far, a total of 246 CTs have been performed with 72 awaited (table 1). Fifteen patients had PET-CT (all low SUV). Fourteen underwent bronchoscopy (normal). Two had CT biopsy (benign), 2 declined biopsy, 2 were smaller at biopsy. One benign lesion was resected (patient choice). Only 28 patients have been discharged from surveillance; 10/28 resolved on 3month CT, 3/28 resolved on 6month CT, 15/28 stable on 12month CT. Fleischner guidance was accurately followed in 67%, most deviance due to delayed timing of 6month CT. Twenty-nine (27%) were discussed without documented nodule size. Conclusion Nodule surveillance has put a significant burden on local Thoracic-Oncology services. No unexpected pathology was encountered during this surveillance period. Until clear clinical and/or radiological identifying factors for high risk patients are understood and rationalised, nodule surveillance will have to continue. There are cost implications not only for Radiology and Respiratory services, but also to patients' emotional and physical well-being. This highlights the continued need for clear surveillance protocols supported by service development. A retrospective study to analyse the outcomes and costs of follow-up of incidental nodules (solitary and multiple) referred to our Department from 2010-2011. Method Consecutive nodule cases were identified by reviewing CT reports of 619 patients discussed at our Lung Cancer MDT from 2010-2011. Only clinically incidental nodules were included. Information was gathered using PACS and hospital records. In our department incidental nodules are seen once in clinic and then largely managed 'remotely' via correspondence. All nodules are managed to Fleischner guidelines.Costs for investigations/procedures/appointments were calculated using local 2012-13 reference costs. Manpower costs for MDTs and correspondence were calculated using a 'bottom-up' costing approach. Results 62 patients were referred with a new incidental nodule (s). Mean age was 66(34-92) with a 1:1 male:female ratio. 56% (35/62) had PS 0-1 and 56%(35/62) were current/ex-smokers. 66%(41/62) had a SPN. Mean size of largest nodule was 9mm. 11%(7/62) were diagnosed with malignancy, 6%(4/62) of pulmonary origin. The 3 non-pulmonary malignancies were renal, breast and metastatic squamous cell. New clinically important diagnoses were made in a further 11%(7/62) including TB/amyloid/ILD, whilst 78%(48/62) were benign.In the malignancy group, 71% (5/7) were current/ex-smokers, 86% (6/7) had a SPN with mean size 7.7mm and there was a higher likelihood of nodules enlarging on follow-up CTs (40% versus 2% at 2nd CT). 75%(3/4) of patients with lung malignancy underwent curative treatment. In the benign group (48), the me...
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