Ganglioneuroma: To Operate or Not to Operate

Abstract

There were a limited number of general minor complications in this series that did not include cases of regrowth or malignant transformation. However, these unfavorable events were occasionally reported in the literature. Since diagnosis of GN cannot be ascertained before removal of the mass, this should remain the aim of the treatment, although limiting the chances of complications to a minimum even if incomplete resection is the price to pay. Nonoperative attitudes should not be recommended in all cases, but they are certainly justified in some.

Characteristics and management of ganglioneuroma and ganglioneuroblastoma‐intermixed in children and adolescents

“…Several recent case series of patients with GN have advocated consideration of nonradical surgeries and/or observation approaches rather than complete surgical resection . To our knowledge, this report is the first to describe the natural history and growth over time for unresected GN and GNB‐I tumors.…”

“…Primary GN and GNB‐I present at older age (median 5–7 years) than other more aggressive subtypes of pNTs and are usually located in the thorax and abdomen . Patients may be symptomatic, most commonly with pain or respiratory distress, but frequently GN masses are diagnosed incidentally in evaluations for unrelated complaints . Both GN and GNB‐I have been shown to have favorable histologic features, including large amounts of Schwannian‐rich stroma, few immature neuroblasts, and low mitotic‐karyorrhexis index (MKI) .…”

“…The outcomes for GN and GNB‐I are excellent. Studies report 96–100% overall survival (OS), however, the morbidity from surgical resections is significant due to proximity to the spine, vessels, and organs. Complications, reported in 15–30% of cases, include hemorrhage, Horner's syndrome, intestinal obstruction, and death Often complete resection with negative margins is not possible, but tumor progression is rare in cases with residual disease …”

“…Since GN has favorable clinical and biological features, but significant postoperative complications, there is controversy as to whether these tumors must always be resected or can be safely observed without surgery, and whether there could be a risk for malignant transformation if not excised. Retrospective studies reporting surgical morbidities have suggested that due to the benign behavior of GN and GNB‐I, observation should be considered; however, there is no published evidence to support this approach, and specifically whether outcomes may be inferior.…”

Ganglioneuroma with Disseminated Bone Lesions

“…Malignant transformation, however, has been sporadically described and warrants proactive management. Although surgical resection is considered the preferred treatment, surgery carries a high risk of long‐term or fatal surgical complications . Therefore, a nonoperative approach and observation is justified in selected cases.…”