An overview of disparities in childhood cancer: Report on the Inaugural Symposium on Childhood Cancer Health Disparities,Houston, Texas, 2016

The Inaugural Symposium on Childhood Cancer Health Disparities was held in Houston, Texas, on November 2, 2016. The symposium was attended by 109 scientists and clinicians from diverse disciplinary backgrounds with interests in pediatric cancer disparities and focused on reviewing our current knowledge of disparities in cancer risk and outcomes for select childhood cancers. Following a full day of topical sessions, everyone participated in a brainstorming session to develop a working strategy for the continued expansion of research in this area. This meeting was designed to serve as a springboard for examination of childhood cancer disparities from a more unified and systematic approach and to enhance awareness of this area of need.     

Telomere length in leukocytes correlates with bone mineral density and is shorter in women with osteoporosis

Summary Telomere length decreases with age and is associated with osteoblast senescence. In 2,150 unselected women, leukocyte telomere length was significantly correlated with bone mineral density. Clinical osteoporosis was associated with shorter telomeres, suggesting that telomere length can be used as a marker of bone aging. Introduction The length of telomeres in proliferative cells diminishes with age. Telomere shortening and telomerase activity have been linked to in vitro osteoblast senescence and to increased secretion of pro-inflammatory cytokines. We explored whether bone mineral density correlates with telomere length in leukocytes. Materials and methods The relationship between leukocyte telomere length, bone mineral density (BMD) and osteoporosis (as defined by the World Health Organization) was examined in a cohort of 2,150 women from a population-based twin cohort aged 18–79. Results After adjusting for age, body mass index, menopausal status, smoking, hormone replacement therapy status, telomere length was positively correlated with BMD of the spine (p < 0.005), forearm (p < 0.013), but not the femoral neck (p < 0.06). Longer telomeres were associated with reduced the risk of clinical OP at two or more sites (odds ratio = 0.594 95% CI 0.42–0.84 p < 0.003) and in women over the age of 50, clinical osteoporosis was associated with 117 bp shorter telomere length (p < 0.02) equivalent to 5.2 years of telomeric aging. Conclusions Shortened leukocyte telomere length is independently associated with a decrease in BMD and the presence of osteoporosis in women. Our data provide evidence that leukocyte telomere length could be a marker of biological aging of bone.     

Anatomic mapping and evaluation of the esophagus in relation to the cervical vertebral body

The relationship of the esophagus to the cervical vertebral body (CVB), disc space and longus colli (LC) muscles, to our knowledge, has not been previously studied. The purpose of this study was to quantify the relationship of the esophagus to the CVB, disc space and LC. 30 patients were selected for a retrospective review of computed tomography (CT) scans. Measurements between the esophagus and the C5, C6, and C7 vertebral bodies as well as the C5/6 and C6/7 disc spaces were performed in the midline, 3 mm right and left of midline, and at the edge of the LC on both sides. The closest distance of the esophagus to the CVB and disc space occurs at the midline (range 1.02–1.31 mm at each level). The furthest distance occurred at the edge of the right LC (range 2.67–3.30 mm at each level). The mean distance from the edge of the right LC to the midline was significantly greater (P < 0.01) than mean distance from the edge of the left LC to the midline. No statistical significant differences were observed when comparing measurements at the individual vertebral bodies and disc spaces. The results of the study demonstrate that the esophagus lies in closest proximity to the CVB and disc space in the midline. A larger potential space exists between the esophagus and the CVB and disc space at the edge of the LC. These results may provide insight into a potential cause of post-operative dysphagia. Furthermore, it may help guide the future design of cervical plates to better utilize the potential space between the esophagus and the CVB and disc space at the edge of the LC.     

Esthesioneuroblastoma: the impact of treatment modality

BACKGROUND: We evaluated the impact of treatment modality on esthesioneuroblastoma. METHODS: Between 1976 and 1996, 25 patients with esthesioneuroblastoma were treated at Mallinckrodt Institute of Radiology. There were 11 male and 14 female patients; their ages ranged from 16 to 73 years (median, 57 years). The tumors were Kadish stage A in 3, Stage B in 13, C in 8, and modified D in 1 (cervical nodal metastasis). Seventeen patients were treated with surgery and radiation therapy, six were treated with irradiation alone, and two were treated with surgery only. Eight patients received neoadjuvant chemotherapy. Median follow-up was 8 years (range, 2-24 years). RESULTS: The 5-year actuarial overall survival, disease-free survival, and local tumor control rates were 66.3%, 56.3%, and 73.0%, respectively. Kadish stage was not a significant prognosticator for local control or disease-free survival. Five-year local control rates were 87.4% for the combination of surgery and radiation therapy and 51.2% for irradiation alone. Two patients with Kadish stage A and B disease underwent surgical resection alone; both failed locally. In contrast, 33.3% of patients (three of nine) with Kadish stage A or B disease who received adjuvant radiation therapy had a local recurrence develop. With adjuvant radiation therapy, the surgical margin status did not influence local tumor control. Among the eight patients who received neoadjuvant chemotherapy, six patients showed no response, one had partial response, and one showed a complete response. CONCLUSIONS: Surgical resection plus adjuvant radiation therapy yielded the best treatment outcome. More effective chemotherapy agents with a reproducible effectiveness are needed for patients with locally advanced esthesioneuroblastoma.     

Cancer of retromolar trigone: long-term radiation therapy outcome

BACKGROUND: Cancer of the retromolar trigone is an uncommon head and neck cancer. In this retrospective study, we identified the prognostic factors and evaluated the therapeutic outcomes of patients treated with preoperative radiation therapy (RT), postoperative RT, and RT alone. METHODS: Between 1971 and 1994, 65 patients with histologically proven epidermoid carcinoma of the retromolar trigone were treated at the Mallinckrodt Institute of Radiology; 10 patients received preoperative RT (30-55.2 Gy), 39 received postoperative RT (46-66.6 Gy), and 15 were treated with RT alone (63-74 Gy). Surgery included 44 composite resections and 7 wide excisions. The minimum follow-up was 5 years. RESULTS: The 5-year disease-free survival rates were 90% with preoperative RT, 63% with postoperative RT, and 31% with RT alone. The 5-year disease-free survival rates were 76% for patients with T1 disease, 50% for T2, 72% for T3, and 54% for T4. The 5-year disease-free survival rates were 69% for patients with NO disease, 56% for N1, and 26% for N2. The locoregional recurrence rates were 10% (1 of 10) for preoperative RT, 23% (9 of 39) for postoperative RT, and 44% (7 of 16) for RT alone. On multivariate analysis, the significant factors for disease-free survival were treatment modality (p =.002) and N stage (p =.012); for locoregional control it was treatment modality (p =.046); and for distant metastasis it was N stage (p =.002). The incidence of bone necrosis, soft tissue necrosis, and severe trismus was 12% with postoperative RT, 11% with RT alone, and none with preoperative RT. CONCLUSIONS: Combination surgery with postoperative or preoperative RT offers better locoregional control and disease-free survival than RT alone for epidermoid carcinoma of the retromolar trigone. Lymph node status significantly influences the disease-free survival and distant metastasis rates.     

HbA(1c) levels are genetically determined even in type 1 diabetes: evidence from healthy and diabetic twins.

HbA(1c), a measure of blood glucose regulation, reflects glucose levels in the preceding months. In diabetes, HbA(1c) levels predict the risk of microvascular complications. The aim of this study was to determine whether genetic factors could influence HbA(1c) levels in normal subjects and type 1 diabetic patients. We performed a classical twin study of HbA(1c) in healthy nondiabetic female twins and 42 monozygotic (MZ) and 47 dizygotic (DZ) pairs. Interclass correlations (r) were higher in MZ (r = 0.77) compared with DZ (r = 0.53) twin pairs, suggesting a substantial genetic effect; this was confirmed by quantitative genetic model fitting. Additive genetic effects (heritability) explained 62% (95% CI 47-75) of population variance in HbA(1c); the remainder was attributable to the influence of unique environment (23% [15-36]) and age (14% [5-28]). Multivariate modeling showed that genetic factors also have a substantial influence on fasting glucose levels (51%). However, HbA(1c) heritability could not be explained by genes in common with fasting glucose. In the patients with type 1 diabetes, HbA(1c) levels were correlated in 33 MZ twins concordant for diabetes (r = 0.68; P < 0.001) but also in 45 MZ twins discordant for the disease (r = 0.52; P < 0.001). These significant correlations for HbA(1c) in both concordant and discordant pairs indicate a diabetes-independent familial effect. Thus, HbA(1c) levels are largely genetically determined and independent of the genes influencing fasting glucose. Even in type 1 diabetes, familial (i.e., diabetes-independent) factors influence protein glycation, implying that familial factors may explain, in part, the risk for microvascular complications, as indicated by high HbA(1c) levels.     

Titanium elastic nailing in femoral diaphyseal fractures of children in 6-16 years of age

Background:

Management of femoral diaphyseal fractures in the age group of 6-16 years is controversial. There has been a resurgence worldwide for operative fixation.

Materials and Methods:

Twenty-two children (18 boys, 4 girls) aged 6-16 years with recent (> 3 days) femoral diaphyseal fractures (20 closed, 2 open) were stabilized with Titanium Elastic Nail (TEN). These fractures were in proximal third (n=3), middle third (n=15) and in the distal third (n=4) 17 patients underwent surgery within seven days of their injury. The results were evaluated using Flynn''s scoring criteria. Statistical analysis was done using Fischer''s exact test.

Results:

All 22 patients were available for evaluation after a mean of 26 months (14-36 months) of followup. Radiological union in all cases were achieved in a mean time of 8.7 weeks. Full weight bearing was possible in a mean time of 8.8 weeks. Mean duration of hospital stay was 9.8 days. The results were excellent in 13 patients (59.0%), successful in six (27.2%) and poor in three patients (13.6%). All patients had early return to school.

Conclusion:

Intramedullary fixation titanium elastic nailing is an effective treatment of diaphyseal fractures of the femur in properly selected patients of the 6-16 years age group.