Survival of high-risk pediatric neuroblastoma patients in a developing country.

Little information is available about survival of high-risk pediatric neuroblastoma patients in developing countries. We aimed to assess survival among high-risk pediatric neuroblastoma patients in La Plata, Argentina. Individuals eligible for our cohort were aged <20 yr when diagnosed with high-risk neuroblastoma and received cancer-directed therapy including stem cell transplantation at Hospital de Niños Sor Maria Ludovica between February 1999 and February 2015. We estimated overall survival probabilities using an extended Kaplan-Meier approach. Our study population comprised 39 high-risk neuroblastoma patients, of whom 39% were aged >4 yr at diagnosis, 54% were male, and 62% had adrenal neuroblastoma. We observed 18 deaths, and the median survival time of our study population was 1.7 yr. The five-yr overall survival probability was 24% (95% CL: 10%, 41%). In contrast, five-yr survival of high-risk neuroblastoma patients ranges between 23% and 76% in developed countries. Survival among high-risk neuroblastoma patients is generally poor regardless of geographic location, but our results illustrate dramatically worse survival for patients in a developing country. We speculate that the observed survival differences could be attenuated or eliminated with improvements in treatment and supportive care, but addressing these issues will require creative solutions because of resource limitations.

Diffusion-weighted MR imaging vs. multi-detector row CT: Direct comparison of capability for assessment of management needs for anterior mediastinal solitary tumors.

PURPOSE: To evaluate and compare the capability of diffusion-weighted MR imaging (DWI) and CT for assessment of management needs for anterior mediastinal solitary tumors. MATERIALS AND METHODS: Thirty-five patients with pathologically confirmed anterior mediastinal tumors were enrolled. The tumors were divided into two groups according to need for management: tumors not needing further intervention or treatment (group A; thymoma type A, AB and B1) and tumors needing further intervention and treatment (group B; other thymoma types and malignancies). The apparent diffusion coefficient (ADC) of each tumor was measured, and probabilities of malignancy and need for further intervention and treatment were visually assessed on CT. The differences in ADCs between group A and B and between malignancies and thymomas in group B were evaluated with the Mann-Whitney's U-test. Feasible threshold values for differentiation of group B from group A and distinguishing malignancies from thymomas assessed as group B were determined by the ROC-based positive test, and McNemar's test was used for comparing diagnostic capabilities of DWI with those of CT. RESULTS: ADCs for the two groups were significantly different (p<0.001). Application of the threshold value for differentiation of group B from A showed no significant difference (p>0.05). Application of the feasible threshold value for distinguishing malignant from thymomas assessed as group B showed that specificity (76.9%) and accuracy (85.2%) of DWI were significantly better than those of visual score (p<0.05). CONCLUSION: DWI has useful potential for the assessment of management needs for anterior mediastinum solitary tumors as well as CT.

Follow-up of patients with thoracic malignancies.

The optimal approach to the post-treatment management of patients with thoracic malignancies is a controversial topic. This is primarily because of the lack of widely accepted practice guidelines. Several guidelines have been promulgated for the follow-up of thoracic malignancies, but none have been tested in randomized controlled trials. The problem is an especially interesting one because little is known about how outcomes vary when the follow-up strategy is altered.

Residual mediastinal mass after treatment of Hodgkin's disease: a decision analysis.

A residual mediastinal mass after completion of initial treatment for Hodgkin's disease is a frequent clinical problem. Investigators have suggested three possible approaches to this important problem: 1) observation, 2) additional diagnostic tests with subsequent action based upon test results, or 3) immediate treatment for high-risk patients. The method of decision analysis was applied to determine the optimal management for residual mediastinal abnormalities following treatment of Hodgkin's disease with combined modalities of MOPP chemotherapy and radiation therapy. The three parameters of the importance for making the best decision were: 1) the probability that the mass is truly active disease, 2) the salvage success rate using MOPP or ABVD treatment and 3) the specificity of the gallium scan. The analysis favored the gallium imaging strategy as an initial management choice when the probability was greater than 3% that the residual mass represented active disease and the specificity of gallium imaging was greater than 56%. This strategy proved to be the most cost effective, as well. Additional chemotherapy was favored only when there was a greater than 99% probability that the mass represented active disease. A nomogram has been constructed combining all three parameters of importance for graphically determining the best decision.

Assessment of response to cancer therapy using fluorine-18-fluorodeoxyglucose and positron emission tomography.

In order to evaluate the usefulness of 18F-FDG PET in the assessment of therapeutic effects, FDG-PET studies were performed both before and after therapy in 26 patients with miscellaneous malignant tumors. The change in FDG uptake by therapy was compared with the change in tumor size and prognosis. All 26 lesions had a high FDG uptake before therapy. Five of seven lesions which had a relatively low FDG uptake before therapy showed no change or increase in tumor size by therapy. The decreased FDG uptake after therapy was more prominent in the partial response group than in the no change group. FDG uptake before therapy in the non-relapse group was higher than that in the relapse group. However, a decreased FDG uptake did not necessarily indicate a good prognosis. One patient with no change in tumor size and a decreased FDG uptake had no recurrence. This suggests that FDG-PET has a complementary role in the assessment of therapeutic effects.

Thymoma: a clinico pathological study of 21 cases and assessment of prognostic features.

This is a retrospective and comprehensive study of 21 cases of thymoma treated during a period of 30 years (1954-1984). The tumors were staged into 3 categories: stage 1 for encapsulated completely resectable tumor, stage 2 for nonresectable intrathoracic tumor and stage 3 for tumor with extrathoracic spread. According to their lymphocytic content tumors were separated into 3 groups: 1) predominantly epithelioid (PE); 2) mixed cellular (MC) and predominantly lymphocytic (PL). Incidence of recurrence and survival were correlated with various treatment modalities. The tumor occurred in all age groups with highest incidence in the fourth decade. Six cases were asymptomatic. Myasthenia gravis was present only in one case. The most important prognostic factor was the stage of the tumor. Five-year survival was 69% for stage 2 and 0% for stage 3. All 12 patients who died with evidence of residual disease had PE tumors. Lymphocytic participation might be indicative of a residual functional competence and appears to confer a more favourable prognosis. This is a tumor of uncertain malignant potential which should be excised or debulked, and staged. Post-operative radiotherapy appears to prevent recurrence and improve the prognosis in stage 2. No therapeutic benefits were seen in the stage 3 cases. The value of chemotherapy is uncertain.

Three-dimensional volumetric assessment of response to treatment: stage I and II diffuse large cell lymphoma of the mediastinum.

From 1981 to 1986, 12 patients with Stage I and II diffuse large cell lymphoma of the mediastinum were treated with 4 or more cycles of multiagent chemotherapy and for nine patients this was followed by mediastinal irradiation. The response to treatment was assessed by three-dimensional volumetric analysis utilizing thoracic CT scans. The initial mean tumor volume of the five patients relapsing was 540 ml in contrast to an initial mean tumor volume of 360 ml for the seven patients remaining in remission. Of the eight patients in whom mediastinal lymphoma volumes could be assessed 1-2 months after chemotherapy prior to mediastinal irradiation, the three patients who have relapsed had volumes of 292, 92, and 50 ml (mean volume 145 ml) in contrast to five patients who have remained in remission with residual volume abnormalities of 4-87 ml (mean volume 32 ml). Four patients in prolonged remission with CT scans taken one year after treatment have been noted to have mediastinal tumor volumes of 0-28 ml with a mean value of 10 ml. This volumetric technique to assess the extent of mediastinal large cell lymphoma from thoracic CT scans appears to be a useful method to quantitate the amount of disease at presentation as well as objectively monitor response to treatment.

Stage IA to IIB mediastinal Hodgkin's disease: three-dimensional volumetric assessment of response to treatment.

From 1979 to 1986, the response to treatment of 53 patients with stage IA to IIB mediastinal Hodgkin's disease was evaluated by three-dimensional volumetric analysis using thoracic computed tomographic (CT) scans. The mean initial volume of mediastinal disease in 34 patients treated with mantle and para-aortic irradiation was 166 mL, whereas for 19 patients treated with two to six cycles of multiagent chemotherapy and mantle and para-aortic irradiation the mean initial volume was 446 mL. Preliminary data suggested that patients with mediastinal volumes of less than 200 mL had a lower mediastinal relapse rate (13%) than patients with volumes greater than 200 mL (32%). For 12 patients receiving six cycles of nitrogen mustard, vincristine, procarbazine, and prednisone (MOPP), those with a greater than 85% reduction in volume 1 to 2 months after chemotherapy had a lower incidence of mediastinal relapse (zero of six, 0%) compared with patients having 85% or less reduction in volume (four of six, 67%). The primary value of this technique is that it provides a sensitive assessment of response to treatment and may aid in monitoring the effectiveness of a given treatment.

New assessment of the prognostic significance of histopathology in Hodgkin's disease for laparotomy-negative stage I and stage II patients.

This paper describes preliminary radiotherapy results in 90 patients with Stage I and II Hodgkin's disease who were evaluated by laparotomy, including splenectomy, and liver and bone marrow biopsies. As a result of selection by laparotomy, the estimated five-year survival rate for these patients was 96%. No statistically significant differences were detected in the disease-free survival for patients with mixed cellularity, nodular sclerosis, and lymphocytic predominance disease. Since only one patient with lymphocytic depletion was in this series, no statement can be made regarding this rare histopathology. Patterns of new disease differed for Stage I and II patients. The major difference was that patients with nodular sclerosing Stage II presentations involving the mediastinum were at considerable risk of developing subsequent disease in the pulmonary parenchyma or the pleura. This finding, together with the demonstration that a histologic diagnosis of mixed cellularity did not carry an inferior prognosis, indicates the need for reassessment of the appropriateness of applying treatment programs based on results of lymphangiographically staged patients to Stage I and II patients evaluated by laparotomy.