OBJECTIVE: The Transatlantic Australasian Retroperitoneal Sarcoma Working Group conducted a retrospective study on the disease course and clinical management of ganglioneuromas. BACKGROUND: Ganglioneuromas are rare tumors derived from neural crest cells. Data on these tumors remain limited to case reports and single-institution case series. METHODS: Patients of all ages with pathologically confirmed primary retroperitoneal, intra-abdominal, and pelvic ganglioneuromas between January 1, 2000, and January 1, 2020, were included. We examined demographic, clinicopathologic, and radiologic characteristics, as well as clinical management. RESULTS: Overall, 328 patients from 29 institutions were included. The median age at diagnosis was 37 years with 59.1% of patients being female. Symptomatic presentation comprised 40.9% of cases, and tumors were often located in the extra-adrenal retroperitoneum (67.1%). At baseline, the median maximum tumor diameter was 7.2 cm. One hundred sixteen (35.4%) patients underwent active surveillance, whereas 212 (64.6%) patients underwent resection with 74.5% of operative cases achieving an R0/R1 resection. Serial tumor evaluations showed that malignant transformation to neuroblastoma was rare (0.9%, N=3). Tumors undergoing surveillance had a median follow-up of 1.9 years, with 92.2% of ganglioneuromas stable in size. With a median follow-up of 3.0 years for resected tumors, 84.4% of patients were disease free after resections, whereas recurrences were observed in 4 (1.9%) patients. CONCLUSIONS: Most ganglioneuromas have indolent disease courses and rarely transform to neuroblastoma. Thus, active surveillance may be appropriate for benign and asymptomatic tumors particularly when the risks of surgery outweigh the benefits. For symptomatic or growing tumors, resection may be curative.

Center for Sarcoma and Bone Oncology Dana Farber Cancer Institute Boston MA

College of Osteopathic Medicine of the Pacific Western University of Health Sciences Pomona CA

Department of Biomedical Sciences Humanitas University Pieve Emanuele Milan Italy

Department of Cancer Surgery Peter MacCallum Cancer Centre Melbourne Victoria Australia

Department of Comprehensive Cancer Care Masaryk Memorial Cancer Institute Brno Czech Republic

Department of Medical Oncology Bern University Hospital University of Bern Bern Switzerland

Department of Medical Oncology Campus Bio Medico University of Rome Rome Italy

Department of Paediatric Oncology University Hospitals Leuven Belgium

Department of Retroperitoneal Tumor Surgery Peking University International Hospital Beijing China

Department of Soft Tissue Bone Sarcoma and Melanoma Maria Sklodowska Curie National Research Institute of Oncology Warsaw Poland

Department of Surgery Brigham and Women's Hospital Boston MA

Department of Surgery Fondazione IRCCS Istituto Nazionale dei Tumori Milan Italy

Department of Surgery The Ottawa Hospital Ottawa Hospital Research Institute Ottawa ON Canada

Department of Surgery University of Washington Seattle WA

Department of Surgical Oncology Fox Chase Cancer Center Philadelphia PA

Department of Surgical Oncology Institut Curie PSL University Paris France

Department of Surgical Oncology Institute of Oncology Ljubljana Zaloška Ljubljana Slovenia

Department of Surgical Oncology Mount Sinai Hospital and Princess Margaret Cancer Centre Toronto ON Canada

Department of Surgical Oncology Royal Marsden Hospital London UK

Department of Surgical Oncology The University of Texas MD Anderson Cancer Center Houston TX

Department of Surgical Oncology University Hospitals Gasthuisberg Leuven Belgium

Division of Medical Oncology Sarcoma Unit Candiolo Cancer Institute FPO IRCCS Candiolo Italy

Division of Melanoma Sarcoma and Rare Tumors IRCCS European Institute of Oncology Milan Italy

Division of Musculoskeletal Oncology National Cancer Center Hospital Tokyo Japan

Division of Surgical Oncology Department of Surgery Mannheim University Medical Centre University of Heidelberg Mannheim Germany

Division of Surgical Oncology Department of Surgery Moores Cancer Center University of California San Diego UC San Diego Health Sciences La Jolla CA

Division of Surgical Oncology Department of Surgery Winship Cancer Institute Emory University Atlanta GA

Harvard Medical School Boston MA

Midlands Abdominal and Retroperitoneal Sarcoma Unit University Hospitals Birmingham NHS Foundation Trust Birmingham UK

N N Blokhin National Medical Research Center of Oncology of the Ministry of Health of Russia Moscow Russia

Sarcoma and Melanoma Unit Angel H Roffo Institute of Oncology University of Buenos Aires Buenos Aires Argentina

Sarcoma and Melanoma Unit General Surgery Department Buenos Aires British Hospital Buenos Aires Argentina

Sarcoma Melanoma and Rare Tumors Surgery Unit IRCCS Humanitas Research Hospital Rozzano Milan Italy

Surgical Oncology Unit Department of Surgery The Sackler Faculty of Medicine Tel Aviv Sourasky Medical Center Tel Aviv University Tel Aviv Israel

Unidad de Mama y Pared Abdominal Servicio de Cirugía General y Digestiva Hospital de la Santa Creu i Sant Pau Universidad Autónoma de Barcelona Barcelona España

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