A Natural History Study of Children and Adults With Olfactory Neuroblastoma

Summary

Participation Requirements

Description

Background: Olfactory neuroblastoma (ONB), also known as esthesioneuroblastoma, is a malignant tumor of the nasal cavity believed to arise from the olfactory epithelium. ONB is rare, with a reported incidence of 0.4 per million (approximately 300 cases per year in the United States). ONB most freque...

Background: Olfactory neuroblastoma (ONB), also known as esthesioneuroblastoma, is a malignant tumor of the nasal cavity believed to arise from the olfactory epithelium. ONB is rare, with a reported incidence of 0.4 per million (approximately 300 cases per year in the United States). ONB most frequently arises in adults aged 20-60 years. Pediatric ONB is particularly rare, with only small case series or multi-institutional studies reported. However, a Surveillance Epidemiology and End Results (SEER) study of 47 pediatric patients with intranasal malignancy reported ONB as the most frequent pediatric intranasal malignancy (28%). Due to the location of ONB in the sinonasal tract and anterior skull base, the presenting symptoms are often non-specific, including nasal obstruction and epistaxis, thus patients often present at advanced stages. Furthermore, ONB has a propensity for local invasion and relatively high rates of distant spread most commonly to the neck, lungs, and bones. Clinical management is adults is generally surgical with adjuvant radiation. The natural history of pediatric and adult ONB is incompletely understood, and treatment options for recurrent or metastatic ONB are limited. The planned natural history study as part of the NCI POB Rare Tumor Patient Engagement Network (RTPEN) will allow for comprehensive evaluation and recommendations to these patients while longitudinally collecting clinical, epidemiologic, and biological data. Objective: To characterize the natural history of ONB including clinical presentation, family history, patterns of disease progression, response to current treatment methods, disease recurrence, and overall survival Eligibility: Patients of age >= 3 years old with histologically documented ONB. Subjects must be enrolled on NCI protocol 19-C-0016. Subjects must also enroll on NIDCD Protocol 18-DC-0051. Design: This protocol is a subprotocol to protocol 19-C-0016: "Natural History and Biospecimen Acquisition Study for Children and Adults with Rare Solid Tumors". After enrollment on the master protocol and having evaluations detailed, patient will be enrolled on this subprotocol specific for olfactory neuroblastoma. Participants will undergo a comprehensive study entry evaluation including acquisition of imaging of tumor sites. Medical histories will be documented, and patients followed throughout the course of their disease, with particular attention to patterns of disease recurrence and progression, response to therapies, duration of responses, and patient reported outcomes. Specimens will be obtained longitudinally, when feasible, and tumor growth rates will also be assessed throughout the course of the disease, when feasible. Blood and tumor samples may be obtained at study entry and while on study.

Tracking Information