Imaging of brain tumors.

Neuroimaging plays a crucial role in diagnosis of brain tumors and in the decision-making process for therapy. Functional imaging techniques can reflect cellular density (diffusion imaging), capillary density (perfusion techniques), and tissue biochemistry (magnetic resonance [MR] spectroscopy). In addition, cortical activation imaging (functional MR imaging) can identify various loci of eloquent cerebral cortical function. Combining these new tools can increase diagnostic specificity and confidence. Familiarity with conventional and advanced imaging findings facilitates accurate diagnosis, differentiation from other processes, and optimal patient treatment. This article is a practical synopsis of pathologic, clinical, and imaging spectra of most common brain tumors.

Do long term survivors of ewing family of tumors experience low bone mineral density and increased fracture risk?

BACKGROUND: Multimodal treatment regimens for Ewing's sarcoma have led to survival rates approaching 70% of patients with no metastases at diagnosis. However, these treatments have long-term side effects. Low bone mineral density (BMD) and risk of fractures can occur owing in part to chemotherapy and limited mobility from local control of the primary tumor. QUESTIONS/PURPOSES: We performed this study to answer the following questions: (1) Do long-term survivors of the Ewing family of tumors sustain low BMD? (2) Which factors are associated with BMD in these patients? (3) Do they experience fractures? (4) Are BMD and fractures associated with each other? METHODS: We queried our institutional registry to identify all known survivors of Ewing tumors who were treated before 2005. Of 100 such patients, 67 (67%) responded to a postal survey to participate in this study, and an additional 11 (11%) patients were excluded according to prespecified criteria. In the remaining 56 long-term survivors (27 females, 29 males; mean±SD age at followup, 32±10 years; mean followup, 15±7 years), BMD was measured by dual-energy x-ray absorptiometry and history of fractures was assessed using a questionnaire. Associations were tested using univariate and multivariate models by stepwise variable selection procedure, including Bonferroni correction. RESULTS: Thirty-one of 56 (56%) patients had a pathologic BMD. Seven (13%) had osteoporosis and 24 (43%) had osteopenia. Factors related to low BMD after Bonferroni correction were the length of time between surgery and followup and the BMI at followup. Twenty-one patients reported 29 fractures. With the numbers available, BMD levels were not associated with fractures. CONCLUSIONS: We could not confirm some potentially important predictors for fractures to be associated with clinical events of interest. However, the data are valuable as hypothesis-generating pilot data for future, multicenter prospective studies. If BMD changes cannot explain the propensity of fractures, there may be other bone characteristics like microarchitectural changes of bone to more accurately explain the effect. LEVEL OF EVIDENCE: Level IV, prognostic study. See the Instructions for Authors for a complete description of levels of evidence.

Adult primitive neuroectodermal tumors: the prognostic value of supratentorial location.

Primitive neuroectodermal tumors (PNETs) are tumors which primarily consist of undifferentiated round neuroepithelial cells. Central nervous system PNETs can be divided into two genetically distinct groups: infratentorial PNET (iPNET)/medulloblastoma and supratentorial PNET (sPNET). Currently, the comparative outcome of adult patients with sPNETs and iPNETs is unknown. In this study we have utilized the Surveillance, Epidemiology, and End Results database to perform a comparative analysis of 103 cases of adult sPNET and 669 adult medulloblastoma cases. Additionally we have analyzed various factors to identify their prognostic significance and characterize the optimal treatment for these tumors. Patients with sPNETs were seen to have a significantly worse survival than those diagnosed with medulloblastomas (16 vs. 155 months, p < 0.0001). Elderly patients (15 vs. 114 months, p < 0.0001) and those over the age of 40 (68 vs. 147 months, p < 0.0001) experienced significantly worse survival than younger patients. In contrast, radiotherapy (143 vs. 26 months, p < 0.0001), surgical resection (116 vs. 22 months, p = 0.0010) and the extent of resection (EOR) (173 vs. 81 months, p = 0.0005) resulted in significantly improved patient survival. Multivariate analysis revealed age greater than 40 years (HR: 1.57; 95 % CI: 1.17-2.11; p = 0.0028) and sPNET pathology (HR: 3.41; 95 % CI: 2.47-4.72; p < 0.0001) to be poor prognostic factors for survival while radiotherapy (HR: 0.52; 95 % CI: 0.38-0.71; p < 0.0001) and the EOR (HR: 0.73; 95 % CI: 0.55-0.96; p = 0.023) were associated with significantly improved survival. The treatment of sPNETs should therefore include maximal surgical resection when feasible followed by radiotherapy as these treatments have been demonstrated to confer a survival benefit. Additional studies are needed to identify effective chemotherapeutics and specific treatment regimens for adults with sPNETs.

Fetal growth and the risk of childhood CNS tumors and lymphomas in Western Australia.

The etiology of childhood cancers is largely unknown, although the early age at diagnosis has led to particular interest in in utero and perinatal factors. Birth weight is the most frequently studied perinatal factor in relation to risk of childhood cancers, and results have been inconsistent. We investigated whether the risk of CNS tumors and lymphomas in children was associated with three measures of the appropriateness of intra-uterine growth: proportion of optimal birth weight (POBW), birth length (POBL) and weight for length (POWFL). A cohort of 576,633 infants born in Western Australia in 1980-2004 were followed from birth to diagnosis of a CNS tumor (n = 183) or lymphoma (n = 84) before age 15, death, or December 31, 2005, and analyzed with Cox regression. Overall, there was little evidence of any association between fetal growth and risk of CNS tumors, although risk of ependymoma/choroid plexus tumors was positively associated with POBL and negatively associated with POWFL. The risk of Hodgkin and Burkitt lymphoma increased with increasing fetal growth among boys only, whereas the increased risk observed with non-Hodgkin lymphoma was only in girls. These associations between fetal growth and disease risk were also observed among children not classified as high birth weight, suggesting that accelerated growth is more important than birth weight per se. Results were similar when cases were compared with their unaffected siblings, suggesting that the increased growth associated with cancer risk was not general to the family. The associations we observed are consistent with causal pathways involving fetal growth factors.

Neuroectodermal and neuroendocrine tumors principally seen in children.

Neuroectodermal tumors comprise a large proportion of childhood neoplasms. Neuroblastic tumors, including neuroblastoma, ganglioneuroblastoma, and ganglioneuroma, are the most frequent extracranial solid cancers of childhood, occurring primarily in infants and toddlers. Primitive neuroectodermal tumors, including Ewing sarcoma and peripheral neuroepitheliomas, occur most frequently in older children and adolescents, and as pediatric sarcomas are second in frequency only to rhabdomyosarcomas. Rarer neuroectodermal tumors include desmoplastic small cell tumors, esthesioneuroblastomas, and melanotic neuroectodermal tumors, the first two entities occurring as rather site-specific lesions in the abdomen and nose, respectively. Diagnosis can be difficult due to the undifferentiated nature of many of these cancers, but ancillary studies, including electron microscopy, immunohistochemistry, cytogenetics, and molecular genetics, enhance their recognition. The molecular nature of childhood neuroectodermal tumors is as diverse as their histology, ranging from the fusion genes characterizing the Ewing sarcoma family of tumors to the proto-oncogene amplification seen in aggressive neuroblastomas.

Gastrointestinal neuroendocrine/neuroectodermal tumors.

This article reviews the cause and clinical and pathologic features of gastrointestinal carcinoid tumors and small cell carcinomas. Their pathogenesis and molecular features are reviewed. Tumor arrays within a given site, as in the stomach, are compared with one another to highlight their histologic features and differing biologies. General treatment guidelines are also provided.

Epidemiology of childhood brain tumors in Japan.

Using the vital statistics issued annually by the Japanese Government from 1960 to 1994, the mortality of brain tumors during childhood (0-14 years of age) in Japan was estimated. Since there are few nationwide registries of childhood cancers with a sufficiently high registration rate, the incidence of brain tumors was calculated using the Registry of Childhood Malignancies in Hokkaido Prefecture from 1969 to 1996. Though the mortality due to malignant diseases as a whole during childhood has been decreasing, and though there should be progress in therapeutic methods, the mortality due to brain tumors in children has been increasing. The incidence of brain tumors in Hokkaido Prefecture has been increasing as well. There was no tendency for the incidence of medulloblastoma to decrease. The incidence of childhood brain tumors has been increasing in Japan, though the cause is unknown. The period of the present study corresponded to a period of high economic growth. Thus, a study on environmental factors would be interesting.

Second primary cancers in patients with cutaneous malignant melanoma: a population-based study in Sweden.

To quantify the risk of second primary cancers among patients with cutaneous malignant melanoma, we studied 20,354 patients in the Swedish Cancer Register during 1958-88. A second primary cancer was reported in 1605 patients, compared with an expected number of 1109.5 [standardised incidence ratio (SIR) = 1.45, 95% confidence interval (CI) = 1.38-1.52]. The highest risk was found among patients younger than 60 years. The greatest risk was seen during the first year after diagnosis (SIR = 1.91, CI = 1.69-2.14), but even after long-term follow-up--15 years or more--the risk was still significantly elevated (SIR = 1.56, CI = 1.35-1.79). The strongest association was found for a second primary malignant melanoma (men, SIR = 10.0, CI = 8.26-12.00; women, SIR = 8.66, CI = 7.22-10.30) and non-melanoma skin cancer (men, SIR = 3.58, CI = 2.85-4.44; women, SIR = 2.41, CI = 1.71-3.29). The risk of second cancers associated with tissues of neuroectodermal origin was increased, especially tumours of the nervous system (men, SIR = 1.73, CI = 1.10-2.60; women, SIR = 2.03, CI = 1.45-2.78). The SIR of second cancers involving the immune system was also increased. An excess risk of endometrial cancer was seen (SIR = 1.41, CI = 1.03-1.88), but no significant associations existed for cancers of the breast, ovary, testis or other endocrine glands. Among tumours of the digestive tract, only colon cancer in men had a significantly increased SIR (1.33, CI = 1.00-1.74).

[Analysis of 110 cases of sacrococcygeal lesions in children].

110 cases of sacrococcygeal lesions seen in the Guangzhou Children's hospital were reviewed. 42 cases were anomalies, 66 cases were tumors and 2 were inflammatory granulomas. As compared to data in western literatures, the morbidity of anomalies in this series was lower than that of tumors, whereas in western reports the morbidity of anomalies was much higher than that of tumors. Another interesting point was that the morbidity of meningocele (MC) in this series was much higher than that of myelomeningocele (MMC) of spina bifida (7:1). According to a study in Australia, the MC:MMC was 1:5. We suggest that endodermal sinus tumor should be considered as an independent entity of there was no other tissue component (organlike component) found in tumor section. We also suggest that if neuroblastoma and/or other immature neuroectodermal component is observed in a teratoma, it should be classified as malignant rather than benign.