Nasal Embryonal Rhabdomyosarcoma in the Pediatric Population: Literature Review and Report of Midline Presentation.

BACKGROUND: Congenital midline nasal masses are rare anomalies and are typically benign nasal dermoid sinus cysts (NDSCs). Rhabdomyosarcomas (RMSs) are even less common, and only a fraction affect sites like the external nose, nasal cavity, nasopharynx, and paranasal sinuses. We review the clinical presentation and treatment of nasal, nasopharyngeal, and paranasal RMSs and report the first documented midline presentation. METHODS: We queried PubMed for articles with titles containing the terms rhabdomyosarcoma or sarcoma botryoides and nose, nasal, paranasal, sinonasal, nasopharynx, or nasopharyngeal. We then searched the references of each included article using the same parameters and continued this process iteratively until no new articles were found. RESULTS: The paranasal sinuses were the most commonly affected site, followed by the nasopharynx, nasal cavity, and external nose. Two patients presented with involvement of the external nose, but each presented with involvement of the right ala rather than a midline mass. The rates of intracranial extension and/or skull base involvement were comparable to those of NDSCs. The alveolar subtype was most common, followed by the embryonal subtype. CONCLUSIONS: Most midline nasal masses are benign; however, we report the first documented presentation of an RMS as a midline nasal mass. Accordingly, RMS should be included in the differential diagnosis of midline nasal masses in the pediatric population. Surgery for midline nasal masses is sometimes delayed due to the risks of interfering with developing structures and early anesthesia. However, early surgical treatment should be considered given this new differential and its predilection for early metastasis.

Vaccination in pediatric cancer survivors: Vaccination rates, immune status, and knowledge regarding compliance.

BACKGROUND: Vaccination recommendations for childhood cancer survivors are ambiguous. Limited data exist on vaccination rates and patient/caregiver knowledge of vaccination postchemotherapy. PROCEDURE: A single-institution study of childhood cancer survivors treated from 1996 to 2018. Study included a retrospective chart review assessing patient's vaccination status, survey of patient's/caregiver's knowledge/beliefs regarding vaccination postchemotherapy, and assessment of immunoglobulin titers. RESULTS: A total of 120 patient charts were included. Vaccination records were available for 82% (98/120) of patients, 57% (56/98) were up to date with vaccinations before chemotherapy, and 83% (81/98) received vaccinations after chemotherapy. Children who resumed vaccination postchemotherapy were younger at cancer diagnosis compared to those who did not resume vaccination (2 vs 4 years, P < .02). Median time since chemotherapy was higher in vaccinated versus unvaccinated patients (107 vs 60 months, P < .02). Immunoglobulin titers were assessed in 27 patients, and 74% (20/27) were not immune to one or more infections tested. Lack of immunity to pneumococcal strains was the most common. There was no difference in median age at diagnosis or time since chemotherapy completion in immune versus nonimmune patients. In 33 surveyed patients/caregivers, 33% (11/33) were not advised about resuming vaccinations postchemotherapy. Over one-third (12/33) of respondents were concerned about vaccination safety after chemotherapy, although 88% (29/33) agreed they would vaccinate if recommended by their pediatrician/pediatric oncologist. CONCLUSIONS: Most childhood cancer survivors resume vaccinations postchemotherapy. Considerable variability exists in vaccination timing after chemotherapy. Pediatric oncologists play a central role in educating patients/pediatricians about vaccination recommendations postchemotherapy.

Adolescent and Young Adult Oral Maxillofacial Tumors: A Single-Institution Case Series and Literature Review.

Adolescent and young adult (AYA) oral maxillofacial tumors are rare and account for ∼12% of all AYA cancers. Due to the low incidence of these malignancies, diagnostic considerations, therapeutic approaches, and factors affecting prognosis have been difficult to characterize. Given the anatomic structures located within the head and neck, patients are at risk for treatment-related morbidity that may adversely impact their quality of life. We present a single-institution case series of AYA patients with oral maxillofacial tumors treated at the University of Illinois at Chicago. A multidisciplinary treatment approach, including collaboration with the Oral Maxillofacial Surgery, Dentistry, and the Ear, Nose, and Throat teams along with the utilization of Children's Oncology Group treatment protocols, can serve as a model to address the challenges in the management of these complex cases.

A Tri-Institutional Approach to Address Disparities in Children's Oncology Group Clinical Trial Accrual for Adolescents and Young Adults and Underrepresented Minorities.

Purpose: Enrollment in Children's Oncology Group (COG) clinical trials has led to significant improvements in survival; however, disparities in survival persist, particularly among ethnic minorities, adolescents and young adults (AYAs), and the underinsured, partly due to inadequate access to cooperative group cancer clinical trials. In 2008, two COG sites University of Illinois at Chicago (UIC) and Rush University Medical Center, and a nonmember institution, John H Stroger Hospital, created a unified COG program utilizing one lead Institutional Review Board and research team. This study assesses the impact that the tri-institutional COG program had on clinical trial accrual for minority, AYA, and uninsured patients. Methods: Analysis and comparison of COG enrollment data from 2002 to 2008 (pre-merger) and 2008 to 2017 (post-merger) by age, ethnicity, insurance type, clinical trial type, oncologic diagnosis, and specialty of the enrolling physician were completed. Results: Following the merger, the total studies open to enrollment increased by 100%, enrollments increased by 446%, and, for each diagnoses, increased by more than 200%. Enrollment of ethnic minorities rose by 533%, most significantly for Hispanic patients by 925%. AYA enrollments increased by 822%. There was a 28-fold increase in enrollment of uninsured patients. Significantly more providers from various oncology specialties were engaged in enrolling patients and a consistent increase in the percentile standing of the program occurred after the merger. Conclusions: Creation of a tri-institutional COG research program was associated with significant increases in clinical trial enrollments, especially for underrepresented minorities, AYAs, and uninsured patients. The UIC/Rush/Stroger COG Program provides a novel and exemplary approach to address cancer health disparities for these vulnerable populations.

Primary Central Nervous System Malignant Melanoma in Children: A Case Series and Review of the Literature.

We describe 2 cases of rapidly progressive primary central nervous system malignant melanoma, and summarize 18 previously reported cases of this extremely rare tumor in children. Both patients presented with focal neurologic symptoms, with no evidence of skin or other organ system involvement. One patient was treated with temozolomide and etoposide, whereas the other was treated with multiple surgical resections, radiation therapy, and a trial of ipilimumab. New molecularly targeted and immune-based therapies used in metastatic melanoma in adults are potential new treatment options, but their efficacy and safety in pediatric patients needs to be established.

Serologic response and clinical efficacy of influenza vaccination in children and young adults on chemotherapy for cancer.

BACKGROUND: Influenza is a health risk to children receiving chemotherapy for cancer. An absolute lymphocyte count (ALC) >1,000 cells/mm(3) has been associated with the ability to produce an immune response to influenza vaccine during chemotherapy. However, clinical efficacy of influenza vaccination during chemotherapy remains unclear. PROCEDURE: We conducted a prospective cohort study in children receiving chemotherapy for cancer during two consecutive influenza seasons. Assessments of immune cells and serologic response were measured immediately before and after receiving influenza vaccine. Patients were monitored for influenza or influenza-like illness (ILI). RESULTS: Two hundred fifty-nine patients were studied over 2 years. The seroresponse rate was 62% (98/157). The median ALC at vaccination was higher in seroresponders than nonresponders, 854 cells/mm(3) versus 602 cells/mm(3) , respectively (P < 0.036). Univariate analysis showed that patients with an ALC <1,000 cells/mm(3) at the time of vaccination were twice as likely to be sero-nonresponders (P < 0.02, OR = 2.4, 95% CI: 1.1-5.0). Twelve percent (31/259) of patients developed influenza, of whom all had fever at presentation, 26% (8/31) required hospitalization, and 81% (25/31) had chemotherapy delays. No deaths were associated with influenza infection. The proportion of patients with influenza was not different between seroresponders and nonresponders. CONCLUSIONS: Influenza infection following immunization remains a source of morbidity in children undergoing chemotherapy. Lymphopenia at vaccination predicted sero-nonresponse. Seroresponse was not associated with a decreased frequency of influenza infection or ILI when compared to sero-nonresponders, suggesting clinical effectiveness of vaccination is likely multifactorial. Further investigation into the efficacy of the influenza vaccine is needed to refine immunization recommendations.

Chemotherapy in Children with Head and Neck Cancers: Perspectives and Review of Current Therapies.

Cancers of the head and neck in children represent a heterogeneous group of malignancies requiring a variety of treatment modalities. In many instances of childhood head and neck cancers, chemotherapy will be required for treatment, often in conjunction with surgery and/or radiation therapy. Chemotherapy in children with head and neck cancers poses unique challenges in terms of immediate as well as long-term toxicities. This article focuses on the common chemotherapeutic agents, with a particular focus on early and late effects, used in the treatment of children with head and neck cancers.

Secondary malignancies in pediatric cancer survivors: perspectives and review of the literature.

With continuing improvements in the successful treatment of pediatric malignancies, long term survivors of pediatric cancers and their providers are faced with new oncologic issues regarding long-term morbidities. As pediatric cancer survivors have matured into adulthood, the development of secondary malignancies has become a significant issue for these patients. Whether a consequence of treatment for the patient's original cancer, such as chemotherapy, ionizing radiation, or hematopoietic stem cell transplantation, secondary malignancies now present patients and providers with new challenges regarding treatment, surveillance and counseling. We review the major risk factors for secondary malignancies in pediatric cancer survivors, with particular emphasis on important molecular and cytogenetic risk factors, both inherited and acquired. We conclude with a discussion of recommendations for surveillance and counseling of these patients.