Renal Primitive Neuroectodermal Tumor With Inferior Vena Cava Thrombus: Case Series and Literature Review of a Rare but Challenging Entity |
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| Institution: | 1. Department of Urology, University Hospital Frankfurt, Frankfurt am Main, Germany;2. Cancer Prognostics and Health Outcomes Unit, Division of Urology, University of Montréal Health Center, Montréal, Québec, Canada;3. Martini-Klinik Prostate Cancer Center, University Hospital Hamburg-Eppendorf, Hamburg, Germany;4. Department of Urology, University of Naples Federico II, Naples, Italy;5. Department of Urology and Division of Experimental Oncology, URI, Urological Research Institute, San Raffaele Scientific Institute, Milan, Italy;6. Department of Urology, Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria;7. Institute for Urology and Reproductive Health, I.M. Sechenov First Moscow State Medical University, Moscow, Russia;8. Department of Urology, University of Jordan, Amman, Jordan;9. Department of Urologic Sciences, Vancouver Prostate Centre, University of British Columbia, Vancouver, British Colombia, Canada;10. Department of Urology, McGill University Health Centre, Montréal, Québec, Canada;1. Oncology Division, University College London Hospitals NHS Foundation Trust, London, UK;2. National Cancer Research Institute Teenage and Young Adult and Germ Cell Research Group, London, UK;3. Department of Medical Oncology, St Bartholomew''s Hospital, London, UK;4. It''s in the Bag Charity, Bristol Haematology and Oncology Centre, Bristol, UK;5. Department of Pathology, University of Cambridge, Cambridge, UK;6. Leeds Institute of Molecular Medicine, University of Leeds, Leeds, UK;7. Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK;1. Sarah Cannon Research Institute, London, United Kingdom;2. Guy''s Hospital, London, United Kingdom;3. Sarah Cannon Research Institute/Tennessee Oncology, Nashville, TN;4. Institute of Cancer Sciences, Manchester, United Kingdom;5. Memorial Sloan-Kettering Cancer Center, New York, New York;6. Merck & Co., Inc., Kenilworth, NJ;7. Sarah Cannon Research Institute and Cancer Institute, University College, London, United Kingdom;8. Alfred Health Melbourne, Monash University, Melbourne, Australia;9. Novartis Pharma AG, Basel, Switzerland;10. Novartis Oncology, East Hanover, NJ;11. Beth Israel Deaconess Medical Center, Boston, MA;1. Department of Medical Oncology, Istituto Oncologico della Svizzera Italiana (IOSI), Bellinzona, Switzerland;2. Department of Medical Oncology, IRCCS San Raffaele Hospital, Milan, Italy;3. Department of Radiology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy;4. Department of Nuclear Medicine, PET Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy;5. Department of Pathology, IRCCS San Raffaele Hospital, Milan, Italy;6. Department of Urology, IRCCS San Raffaele Hospital, Milan, Italy;7. Vita-Salute San Raffaele University, Milan, Italy;8. Foundation Medicine Inc., Cambridge, MA, United States;9. Upstate Medical University, Syracuse, NY, United States |
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| Abstract: | ObjectiveTo investigate the clinicopathological characteristics, treatments, and prognosis of patients with renal primitive neuroectodermal ectodermal tumors (rPNETs) with inferior vena cava (IVC) tumor thrombus.Patients and MethodsWe retrospectively reviewed 6 patients with rPNETs and IVC tumor thrombus between January 2005 and December 2019, and identified 39 published cases through a literature review. The clinicopathological characteristics, treatments, and survival data were analyzed.ResultsThe median patient age patients was 26 years, and the male to female ratio was approximately 1:1. The average tumor diameter was 12.5 cm. Seventeen patients (37.8%) showed metastasis at diagnosis. Forty-three cases (95.6%) were managed with surgical resection, and 35 (77.8%) received adjuvant chemotherapy after surgery. Follow-up data were available for 41 patients (median follow-up, 10 months; range, 4.5-13.0). The median overall survival (OS) and median progression-free survival (PFS) were both 30.0 months. Patients who received adjuvant chemotherapy had better PFS than those who underwent surgery only (30.0 months 95% confidence interval CI], 4.3-55.7] vs 5.0 months 95% CI, 1.0-9.0]; P = .036). In terms of OS, however, the difference between the 2 groups was not significant (30.0 months 95% CI, 8.4-52.6] vs 7.0 months 95% CI, 4.5-9.5]; P = .244).ConclusionsrPNET with IVCTT is an extremely rare entity that mostly occurs in young adults. Although multidisciplinary treatment is used, the prognosis of this disease remains unclear. RN with IVC tumor thrombectomy is a challenging procedure requiring vascular management techniques and experience. Adjuvant chemotherapy contributes to improved PFS, but not OS. Thus, early diagnosis and treatment play a key role in improving prognosis. |
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