“Ganglioneuroblastoma” in Adults: Potentially Aggressive Tumors
S Chopra, J Stewart, P Zage, J Ater, JM Meis. UT MD Anderson Cancer Center, Houston, TX
Background: Neuroblastic tumors (neuroblastoma and ganglioneuroblastoma) in adults are extremely rare (0.3 cases per million per year according to SEER data). They reportedly have a worse prognosis than childhood cases regardless of subclassification. Despite distinct biologic differences with their pediatric counterparts, including lack of N-myc amplification, pediatric protocols are usually used to treat these patients. Ganglioneuromas are believed to arise from ganglioneuroblastomas that have matured. Identification of neuroblastic elements in predominantly ganglioneuromatous lesions (usually microscopic) depends upon meticulous sampling as well as uniform application of classification schemes.
Design: Cases diagnosed as ganglioneuroblastomas in adults (>18 years of age) were retrieved from 1985 – 2009 at a single institution, histologically reviewed and reclassified using INPC (International Neuroblastoma Pathology Committee) criteria. Seven cases were identified. Clinical information was tabulated as well.
Results: Patient ages ranged from 20 to 33 years (median 31 years) at presentation. All patients were female. Follow-up ranged from 0 to 6 years (median 5 months). Presenting symptoms included pelvic discomfort, pain and syncopal attacks; an asymptomatic mass was detected on pelvic exam in one patient. On histological review, 4 cases were confirmed to be ganglioneuroblastoma and 3 were reclassified as neuroblastoma. Ganglioneuroblastomas were further subclassified as nodular (1), differentiating (1) and intermixed types (2). There were metastases in 3 patients, including liver and spine (one patient) and lymph nodes (2 patients). These 3 patients expired: 1 patient 3 years after diagnosis and 2 patients after 6 years. All 4 patients with ganglioneuroblastoma and one with neuroblastoma were surgically treated. Two patients received chemotherapy. Treatment is unknown in one patient.
Conclusions: 1) Current classification schemes for neuroblastoma are inconsistently applied to adult tumors. 2) INPC criteria are probably not entirely applicable to adult neuroblastic tumors. 3) Macroscopic identification of nodular neuroblastic foci is subjective. Neuroblastic foci could be overlooked with inadequate sampling. 4) Additional clinical and pathologic review of adult neuroblastomas and ganglioneuromas could contribute to identification of risk factors in adult neuroblastic tumors and development of future treatment protocols tailored to adults.
Category: Bone & Soft Tissue
Tuesday, March 23, 2010 2:30 PM
Platform Session: Section E, Tuesday Afternoon