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Retrospective Analysis of Retroperitoneal-Abdominal-Pelvic Ganglioneuromas: An International Study by the Transatlantic Australasian Retroperitoneal Sarcoma Working Group (TARPSWG)
S. Noh, C. Nessim, EZ. Keung, CL. Roland, D. Strauss, G. Sivarajah, M. Fiore, D. Biasoni, SPB. Cioffi, W. Mehtsun, FCM. Cananzi, F. Sicoli, V. Quagliuolo, J. Chen, C. Luo, RA. Gladdy, C. Swallow, W. Johnston, SJ. Ford, C. Evenden, F. Tirotta, M....
Jazyk angličtina Země Spojené státy americké
Typ dokumentu časopisecké články, Research Support, N.I.H., Extramural, práce podpořená grantem
Grantová podpora
P30 CA008748
NCI NIH HHS - United States
P30 CA023100
NCI NIH HHS - United States
R01 CA226803
NCI NIH HHS - United States
- MeSH
- dospělí MeSH
- ganglioneurom * chirurgie MeSH
- lidé MeSH
- nádory měkkých tkání * MeSH
- neuroblastom * MeSH
- progrese nemoci MeSH
- retroperitoneální nádory * chirurgie MeSH
- retrospektivní studie MeSH
- sarkom * chirurgie patologie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Research Support, N.I.H., Extramural MeSH
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 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 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
Harvard Medical School Boston MA
Sarcoma Melanoma and Rare Tumors Surgery Unit IRCCS Humanitas Research Hospital Rozzano Milan Italy
Citace poskytuje Crossref.org
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- $a Noh, Sangkyu $u Division of Surgical Oncology, Department of Surgery, Moores Cancer Center, University of California San Diego, UC San Diego Health Sciences, La Jolla, CA $u College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA
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