Medulloblastoma is the most common form of brain malignancy of childhood. The mainstay of epidemiological data regarding childhood medulloblastoma is derived from case series, hence population-based studies are warranted to improve the accuracy of survival estimates. To utilize a big-data approach to update survival estimates in a contemporary cohort of children with medulloblastoma. We performed a population-based retrospective observational cohort study utilizing the Surveillance, Epidemiology, and End Results Program database that captures all children, less than 20 years of age, between 1973 and 2012 in 18 geographical regions representing 28% of the US population. We included all participants with a presumed or histologically diagnosis of medulloblastoma. The main outcome of interest is survivors at 1, 5 and 10 years following diagnosis. A cohort of 1735 children with a median (interquartile range) age at diagnosis of 7 (4-11) years, with a diagnosis of medulloblastoma were identified. The incidence and prevalence of pediatric medulloblastoma has remained stable over the past 4 decades. There is a critical time point at 1990 when the overall survival has drastically improved. In the contemporary cohort (1990 onwards), the percentage of participants alive was 86, 70 and 63% at 1, 5 and 10 years, respectively. Multivariate Cox-Regression model demonstrated Radiation (HR 0.37; 95% CI 0.30-0.46, p < 0.001) and Surgery (HR 0.42; 95% CI 0.30-0.58, p < 0.001) independently predict survival. The probability of mortality from a neurological cause is <5% in patients who are alive 8 years following diagnosis. The SEER cohort analysis demonstrates significant improvements in pediatric medulloblastoma survival. In contrast to previous reports, the majority of patients survive in the modern era, and those alive 8 years following initial diagnosis are likely a long-term survivor. The importance of minimizing treatment-related toxicity is increasingly apparent given the likelihood of long-term survival.
The reported success of ETV/CPC for infantile hydrocephalus is higher in sub-Saharan Africa than developed nations. Large long-term prospective multi-center observational studies addressing patient-important outcomes are required to further evaluate the efficacy and safety of this re-emerging procedure.
Background In the pediatric population, bony defects of the extremities pose a significant challenge for which free vascularized fibular grafts (FVFGs) represent a valuable reconstructive option. The purpose of this study was to explore surgical outcomes, complications, and long-term functionality of FVFG for this patient group. Methods Using MEDLINE and PubMed databases, studies were identified of pediatric extremity reconstruction using FVFG which reported functional outcomes and/or complications. The operative logs at a tertiary pediatric center were then reviewed for cases of FVFG between January 2000 and 2017. Demographic characteristics, surgical indications, operative details, graft survival, bony union, complications, and functionality of the reconstruction were recorded. Results A total of 366 studies were identified with 23 ultimately meeting inclusion criteria in the systematic review. In the institutional series, 29 patients were included with mean age of 9.7 years (1–17 years). Indications for reconstruction included malignant bone tumor (n = 11), osteomyelitis (n = 9), congenital pseudoarthrosis (n = 6), and osteofibrous dysplasia (n = 3). Major postoperative complications included graft nonunion (24.1%), fracture (17.2%), and hardware failure (17.2%). Thirteen patients (44.8%) experienced delayed complications, while two (6.8%) experienced an immediate complication. Complications occurred in the donor site in 2 cases and the recipient site in all 13 cases. Long-term graft survival was achieved in 27 patients (93.1%), and 23 (79.3%) had full functional recovery, with an average Musculoskeletal Tumor Society score of 90% (60–100%). Mean follow-up was 5.17 years (2–12 years). Conclusion This review and institutional series demonstrate the versatility of FVFG to facilitate limb reconstruction in large defects or serve as a salvage option in complex cases. While immediate and donor-site complications are uncommon, delayed recipient-site complications are frequent, and patients and families should be counseled regarding this expectation. Though technically challenging, excellent long-term graft viability and functional recovery can be achieved in the large majority of patients.
Primary CNS extranodal marginal zone B-cell lymphoma (MZBL) is a rare low-grade non-Hodgkin lymphoma characterized predominantly by small B cells, plasma cells, monocytoid cells, and scattered large immunoblasts. As a slow-growing tumor that remains localized, primary CNS MZBL carries an excellent clinical prognosis. Here, we report two cases of primary CNS MZBL successfully treated using external beam radiation therapy along with a literature review.
Purpose Patients who receive radiation therapy (RT) for prostate cancer are routinely positioned through radiographic means. We set out to establish a data-driven process that defines bladder volume required to meet V40/65 constraints using daily bladder ultrasound (US) and comparative cone beam CT (CBCT) before placing a patient on the treatment table. Methods and Materials This was a single institution retrospective study of 20 patients (390 CBCT scans) who received postprostatectomy RT. Each patient received a daily US before treatment. CBCT alignment was performed 3 times a week. The bladder and rectum were contoured on each CBCT and a session dose was recorded. A mixed-effect model was used to estimate trajectory slopes of radiation exposure with organs-at-risk volume increase. Slope differences by V40/65 for prostate fossa (PF) and pelvic lymph nodes (PF/pLN) were tested using a 3-way-interaction term with Bonferroni correction. Results For the 20 patients, 10 received treatment to PF and 10 received RT to the PF/pLN. Predefined bladder constraints were V65 < 50%, V40 < 70%, and rectal constraints were V65 < 35%, V40 < 55%. The CBCT bladder volume (76-578 cm 3 ) was greater than the pretreatment bladder US (87-466 cm 3 ) due to volume filling between measurements (r = 0.8 ± 0.05). Mixed model detected a statistically significant 3-way interaction ( P < .01) for bladder volume and V40/65. Both PF and PF/pLN patients showed improvement in V40/65 with an increase in bladder volume. For PF patients, bladder constraints were met when the US volume was >108 cm 3 and for PF/pLN patients when the US bladder volume was >200 cm 3 . Rectal filling showed no association with CBCT volume. Conclusions Daily US of the bladder before postprostatectomy RT allows for dosimetric predictions before daily treatment. This should translate into fewer CBCT for the patient and improved machine throughput. This technique is easy to institute and ensures organs-at-risk volumetric constraints are met based on daily US measurements.
Objectives: There is evidence that detection and treatment of oligometastases ( ≤ 5 lesions) may improve survival in breast cancer patients. However, there are no current national guidelines for screening of early, asymptomatic metastases. This study examined the patterns and timing of recurrence with respect to survival in node-positive breast cancer (NPBC) patients at higher risk for developing metastases.Methods: A single-institution retrospective review of NPBC patients treated with trimodality therapy was performed to collect patient and disease characteristics, recurrence location, method of detection, and survival outcome. Univariate and multivariate analyses were done to identify factors associated with recurrence.Results: Ninety-four NPBC patients treated at a safety-net hospital between 2008 and 2019 were identified. Twenty-one developed recurrence and were divided into oligometastatic (OM) (n = 10) or diffusely metastatic (DM) (n = 11) subgroups. Median recurrence-free survival in OM and DM was 18 and 36 months, respectively. Median overall survival (OS) for OM was not reached. Median OS for DM was 57 months. Four patients with OM progressed to diffuse disease in a median period of 17 months; median survival thereafter was 57 months. All patients with recurrence had distant metastases on initial detection, with the most common site being bone (14). Recurrence was most frequently detected by computed tomography (CT) (13), with the majority of disease located within the thorax region.Conclusions: All NPBC patients had distant metastasis at time of recurrence. Patients with OM had shorter interval to recurrence yet longer OS compared with DM. This study highlights improved surveillance imaging for timely detection of OM breast cancer that may yet be amenable to aggressive local salvage therapy to prevent progression to diffuse disease.
A lack of trained providers is an important contributor to the unmet burden of surgical disease treatment in low- and middle-income countries. The World Health Organization’s Commission on the International Recruitment of Health Personnel lays out guiding principles for addressing this workforce crisis. However, for surgical subspecialties such as plastic surgery, in-country training opportunities remain limited and there is a clear need for effective strategies to retain providers and develop sustainable solutions. We report the design and early implementation of a traveling fellowship in plastic surgery for providers at Jimma University Specialized Hospital in Jimma, Ethiopia. This fellowship is supported by Operation Smile and its network of international surgical volunteers. Since its inception, the program has trained 2 general surgeons with a commitment to helping train a total of 6 surgeons to establish a self-sustaining service. Key innovations include multiple international sites to facilitate broad subspecialty training, commitment of participants to return to Jimma upon completion of the program to establish a local training service, and coordination with national governing bodies to ensure program recognition and support. Ongoing challenges include physical resource limitations and coordination with a wide array of stakeholders. Nongovernmental organizations also have a role to play in supporting the Ministries of Health in scaling up human resources for improved health within their countries. Operation Smile’s traveling fellowship demonstrates a feasible method of addressing the health workforce crisis by providing specialized training and facilitating the development of surgical teaching programs capable of sustainably serving local communities.
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